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Sample records for acute neurological diseases

  1. Acute neurologic disease in Porcine rubulavirus experimentally infected piglets.

    Herrera, Jenifer; Gómez-Núñez, Luis; Lara-Romero, Rocío; Diosdado, Fernando; Martínez-Lara, Atalo; Jasso, Miguel; Ramírez-Mendoza, Humberto; Pérez-Torres, Armando; Rivera-Benítez, José Francisco

    2017-02-15

    The objective of this study was to evaluate the clinical disease, humoral response and viral distribution of recent Porcine rubulavirus (PorPV) isolates in experimentally infected pigs. Four, 6-piglet (5-days old) groups were employed (G1-84, G2-93, G3-147, and G4-T). Three viral strains were used for the experimental infection: the reference strain LPMV-1984 (Michoacán 1984) and two other strains isolated in 2013, one in Queretaro (Qro/93/2013) and the other in Michoacán (Mich/147/2013). Each strain was genetically characterized by amplification and sequencing of the gene encoding hemagglutinin-neuroamidase (HN). The inoculation was performed through the oronasal and ocular routes, at a dose of 1×10(6)TCID50/ml. Subsequently, the signs were evaluated daily and necropsies were performed on 3 different days post infection (dpi). We recorded all micro- and macroscopic lesions. Organs from the nervous, lymphatic, and respiratory system were analyzed by quantifying the viral RNA load and the presence of the infectious virus. The presence of the viral antigen in organs was evidenced through immunohistochemistry. Seroconversion was evaluated through the use of a hemagglutination inhibition test. In the characterization of gene HN, only three substitutions were identified in strain Mich/147/2013, two in strain LPMV/1984 (fourth passage) and one in strain Qro/93/2013, with respect to reference strain LPMV-84, these changes had not been identified as virulence factors in previously reported strains. Neurological alterations associated with the infection were found in all three experimental groups starting from 3dpi. Groups G1-84 and G3-147 presented the most exacerbated nervous signs. Group G2-93 only presented milder signs including slight motor incoordination, and an increased rectal temperature starting from day 5 post infection (PI). The main histopathological findings were the presence of a mononuclear inflammatory infiltrate (lymphocytic/monocytic) surrounding the

  2. Metabolic assessment and enteral tube feeding usage in children with acute neurological diseases

    Heitor Pons Leite

    Full Text Available OBJECTIVE: To report on acquired experience of metabolic support for children with acute neurological diseases, emphasizing enteral tube feeding usage and metabolic assessment, and also to recommend policies aimed towards improving its implementation. DESIGN: Retrospective analysis. SETTING: Pediatric Intensive Care Unit of Hospital do Servidor Público Estadual de São Paulo. SUBJECTS: 44 patients consecutively admitted to the Pediatric ICU over a period of 3 years who were given nutrition and metabolic support for at least 72 hours. Head trauma, CNS infections and craniotomy post-operative period following tumor exeresis were the main diagnoses. MEASUREMENTS: Records of protein-energy intake, nutrient supply route, nitrogen balance and length of therapy. RESULTS: From a total of 527 days of therapy, single parenteral nutrition was utilized for 34.3% and single enteral tube feeding for 79.1% of that period. 61.4% of the children were fed exclusively via enteral tube feeding, 9.1% via parenteral and 39.5 % by both routes. The enteral tube feeding was introduced upon admission and transpyloric placement was successful in 90% of the cases. Feeding was started 48 hours after ICU admission. The caloric goal was achieved on the 7th day after admission, and thereafter parenteral nutrition was interrupted. The maximum energy supply was 104.2 ± 23.15 kcal/kg. The median length of therapy was 11 days (range 4-38. None of the patients on tube feeding developed GI tract bleeding, pneumonia or bronchoaspiration episodes and, of the 4 patients who were given exclusive TPN, 2 developed peptic ulcer. The initial urinary urea nitrogen was 7.11 g/m2 and at discharge 6.44 g/m2. The protein supply increased from 1.49 g/kg to 3.65 g/kg (p< 0.01. The nitrogen balance increased from -7.05 to 2.2 g (p< 0.01. CONCLUSIONS: Children with acute neurological diseases are hypercatabolic and have high urinary nitrogen losses. The initial negative nitrogen balance can be

  3. [Depression and neurological diseases].

    Piber, D; Hinkelmann, K; Gold, S M; Heesen, C; Spitzer, C; Endres, M; Otte, C

    2012-11-01

    In many neurological diseases a depressive syndrome is a characteristic sign of the primary disease or is an important comorbidity. Post-stroke depression, for example, is a common and relevant complication following ischemic brain infarction. Approximately 4 out of every 10 stroke patients develop depressive disorders in the course of the disease which have a disadvantageous effect on the course and the prognosis. On the other hand depression is also a risk factor for certain neurological diseases as was recently demonstrated in a meta-analysis of prospective cohort studies which revealed a much higher stroke risk for depressive patients. Furthermore, depression plays an important role in other neurological diseases with respect to the course and quality of life, such as Parkinson's disease, multiple sclerosis and epilepsy. This article gives a review of the most important epidemiological, pathophysiological and therapeutic aspects of depressive disorders as a comorbidity of neurological diseases and as a risk factor for neurological diseases.

  4. Potential Therapeutic Effects of Neurotrophins for Acute and Chronic Neurological Diseases

    Junying Cai

    2014-01-01

    Full Text Available The neurotrophins (NTs nerve growth factor (NGF, brain-derived neurotrophic factor (BDNF, NT-3, and NT-4/5 are proteins that regulate cell proliferation, differentiation, and survival in both the developing and mature central nervous system (CNS by binding to two receptor classes, Trk receptors and p75 NTR. Motivated by the broad growth- and survival-promoting effects of these proteins, numerous studies have attempted to use exogenous NTs to prevent the death of cells that are associated with neurological disease or promote the regeneration of severed axons caused by mechanical injury. Indeed, such neurotrophic effects have been repeatedly demonstrated in animal models of stroke, nerve injury, and neurodegenerative disease. However, limitations, including the short biological half-lives and poor blood-brain permeability of these proteins, prevent routine application from treating human disease. In this report, we reviewed evidence for the neuroprotective efficacy of NTs in animal models, highlighting outstanding technical challenges and discussing more recent attempts to harness the neuroprotective capacity of endogenous NTs using small molecule inducers and cell transplantation.

  5. Happiness and neurological diseases.

    Barak, Yoram; Achiron, Anat

    2009-04-01

    Happiness is an emotional state reflecting positive feelings and satisfaction with life, which, as an outcome in disease states or as an end point in clinical trials, is a neglected concept in most therapeutic areas. In neurological disease, happiness is important as it can be diminished either as a direct result of damage to neuronal tissue or as a reaction to a poor prognosis. The monitoring and maintenance of happiness and wellbeing have historically been considered to be peripheral to medicine. However, as happiness interacts with the patient's physical health, it is an important parameter to assess alongside all aspects of any given disease. Happiness provides a reliable overview of the patient's general status over and above standard parameters for quality of life, and is more wide-ranging than the narrow measures of disease activity or treatment efficacy that are the focus of most clinical trials. In many studies, happiness has been associated with health and success in most areas of life, including performance at work, sporting achievement and social functioning. For approximately a decade, previously studied aspects of psychology have been grouped under the label of positive psychology (PoP). Principles of this discipline are now being used to guide some treatments in neurological and psychiatric diseases. PoP aims to define patient wellbeing in scientific terms and to increase understanding of happiness, meaning in life, resilience and character strengths, as well as to determine how this knowledge can be applied clinically to promote health. Some evidence has emerged recently suggesting that improvements in patient status can result from interventions to improve the patient's level of happiness in diseases, including epilepsy, Huntington's disease, multiple sclerosis, Parkinson's disease and stroke. Several effective approaches to increase happiness employ activities to engage and stimulate patients who might otherwise be unoccupied and isolated. In

  6. Cytokine Therapies in Neurological Disease.

    Azodi, Shila; Jacobson, Steven

    2016-07-01

    Cytokines are a heterogeneous group of glycoproteins that coordinate physiological functions. Cytokine deregulation is observed in many neurological diseases. This article reviews current research focused on human clinical trials of cytokine and anticytokine therapies in the treatment of several neurological disease including stroke, neuromuscular diseases, neuroinfectious diseases, demyelinating diseases, and neurobehavioral diseases. This research suggests that cytokine therapy applications may play an important role in offering new strategies for disease modulation and treatment. Further, this research provides insights into the causal link between cytokine deregulation and neurological diseases.

  7. Neurology of acute organophosphate poisoning

    Singh Gagandeep

    2009-01-01

    Full Text Available Acute organophosphate (OP poisoning is one of the most common poisonings in emergency medicine and toxicological practice in some of the less-developed nations in South Asia. Traditionally, OP poisoning comes under the domain of emergency physicians, internists, intensivists, and toxicologists. However, some of the complications following OP poisoning are neurological and involve neurologists. The pathophysiological basis for the clinical manifestations of OP poisoning is inactivation of the enzyme, acetylcholinesterase at the peripheral nicotinic and muscarinic and central nervous system (CNS nerve terminals and junctions. Nicotinic manifestations occur in severe cases and late in the course; these comprise of fasciculations and neuromuscular paralysis. There is a good correlation between the electrophysiological abnormalities and the severity of the clinical manifestations. Neurophysiological abnormalities characteristic of nicotinic junctions (mainly neuromuscular junction dysfunction include: (1 single, supramaximal electrical-stimulus-induced repetitive response/s, (2 decrement-increment response to high frequency (30 Hz repetitive nerve stimulation (RNS, and (3 decremental response to high frequency (30 Hz RNS. Atropine ameliorates muscarinic manifestations. Therapeutic agents that can ameliorate nicotinic manifestations, mainly neuromuscular, are oximes. However, the evidence for this effect is inconclusive. This may be due to the fact that there are several factors that determine the therapeutic effect of oximes. These factors include: The OP compound responsible for poisoning, duration of poisoning, severity of poisoning, and route of exposure. There is also a need to study the effect of oximes on the neurophysiological abnormalities.

  8. Neurological findings of Lyme disease.

    Pachner, A. R.; Steere, A. C.

    1984-01-01

    Neurologic involvement of Lyme disease typically consists of meningitis, cranial neuropathy, and radiculoneuritis, alone or in combination, lasting for months. From 1976 to 1983, we studied 38 patients with Lyme meningitis. Headache and mild neck stiffness, which fluctuated in intensity, and lymphocytic pleocytosis were the common findings. Half of the patients also had facial palsies, which were unilateral in 12 and bilateral in seven. In addition, 12 patients had motor and/or sensory radiculoneuropathies; asymmetric weakness of extremities was the most common finding. Although incomplete presentations of neurologic involvement of Lyme disease may be confused with other entities, the typical constellation of neurologic symptoms represents a unique clinical picture. PMID:6516450

  9. [Nutritional and metabolic aspects of neurological diseases].

    Planas Vilà, Mercè

    2014-01-01

    The central nervous system regulates food intake, homoeostasis of glucose and electrolytes, and starts the sensations of hunger and satiety. Different nutritional factors are involved in the pathogenesis of several neurological diseases. Patients with acute neurological diseases (traumatic brain injury, cerebral vascular accident hemorrhagic or ischemic, spinal cord injuries, and cancer) and chronic neurological diseases (Alzheimer's Disease and other dementias, amyotrophic lateral sclerosis, Parkinson's Disease) increase the risk of malnutrition by multiple factors related to nutrient ingestion, abnormalities in the energy expenditure, changes in eating behavior, gastrointestinal changes, and by side effects of drugs administered. Patients with acute neurological diseases have in common the presence of hyper metabolism and hyper catabolism both associated to a period of prolonged fasting mainly for the frequent gastrointestinal complications, many times as a side effect of drugs administered. During the acute phase, spinal cord injuries presented a reduction in the energy expenditure but an increase in the nitrogen elimination. In order to correct the negative nitrogen balance increase intakes is performed with the result of a hyper alimentation that should be avoided due to the complications resulting. In patients with chronic neurological diseases and in the acute phase of cerebrovascular accident, dysphagia could be present which also affects intakes. Several chronic neurological diseases have also dementia, which lead to alterations in the eating behavior. The presence of malnutrition complicates the clinical evolution, increases muscular atrophy with higher incidence of respiratory failure and less capacity to disphagia recuperation, alters the immune response with higher rate of infections, increases the likelihood of fractures and of pressure ulcers, increases the incapacity degree and is an independent factor to increase mortality. The periodic nutritional

  10. [Sleep disorders in neurological diseases].

    Kotterba, S

    2015-06-01

    Sleep disorders can be diagnosed in approximately 15 % of the population and have been shown to increase with age. The relationship between sleep disorders and neurological disorders, however, is still insufficiently considered in the clinical practice. Sleep disorders can be an early symptom of the disease, such as the presence of rapid eye movement (REM) sleep behavior disorder (RBD) as an early indicator of neurodegeneration. Sleep disorders have also been shown to be a main symptom of various neurological syndromes, such as in restless legs syndrome (RLS), periodic limb movement disorder (PLMD) and narcolepsy. The international classification of sleep disorders 2nd edition (ICSD 2) describes the main diagnoses, insomnia, circadian rhythm sleep disorders, sleep-related breathing disorders and hypersomnia but all of these can also appear as symptoms in various neurological diseases. Parasomnias are largely considered a differential diagnosis to nocturnal epilepsy. In this review, the main sleep disorders are described with a particular focus on how they relate to neurological diseases; in particular, how they influence disease-related symptoms and how they affect the course of the disease.

  11. Somatic comorbidity in neurological disease.

    Nuyen, J.; Bos, G.A.M. van den; Groenewegen, P.P.; Schellevis, F.G.

    2004-01-01

    Background: Patients with comorbidity in general have a higher risk of dying, a poorer quality of life and greater use of health services. Relativel few studies have examined the occurrence of somatic comorbid conditions in neurological diseases. Aim: Therefore, the size of somatic comorbidity in fo

  12. Neurological complications in chronic kidney disease

    Ria Arnold

    2016-10-01

    Full Text Available Patients with chronic kidney disease (CKD are frequently afflicted with neurological complications. These complications can potentially affect both the central and peripheral nervous systems. Common neurological complications in CKD include stroke, cognitive dysfunction, encephalopathy, peripheral and autonomic neuropathies. These conditions have significant impact not only on patient morbidity but also on mortality risk through a variety of mechanisms. Understanding the pathophysiological mechanisms of these conditions can provide insights into effective management strategies for neurological complications. This review describes clinical management of neurological complications in CKD with reference to the contributing physiological and pathological derangements. Stroke, cognitive dysfunction and dementia share several pathological mechanisms that may contribute to vascular impairment and neurodegeneration. Cognitive dysfunction and dementia may be differentiated from encephalopathy which has similar contributing factors but presents in an acute and rapidly progressive manner and may be accompanied by tremor and asterixis. Recent evidence suggests that dietary potassium restriction may be a useful preventative measure for peripheral neuropathy. Management of painful neuropathic symptoms can be achieved by pharmacological means with careful dosing and side effect considerations for reduced renal function. Patients with autonomic neuropathy may respond to sildenafil for impotence. Neurological complications often become clinically apparent at end-stage disease, however early detection and management of these conditions in mild CKD may reduce their impact at later stages.

  13. Neurological manifestations in Fabry disease

    Joseph Bruno Bidin Brooks; Yara Dadalti Fragoso

    2016-01-01

    Fabry disease (FD) is a rare, progressive, multisystem and highly debilitating disease. FD is an X-linked lysosome storage disorder that results in α-galactosidase A deifciency. The subsequent accumulation of glycosphingolipids is more evident in vascular endothelium and smooth-muscle cells. The resulting effect of the deposition is generalized inlfammation and vasculopathy, which can also affect the central and peripheral nervous system. FD progresses with kidney dysfunction, angiokeratoma of the skin, cardiomyopathy, cerebrovascular events and neurological disorders. In the present review, the neurological manifestations of FD are summarized with emphasis on cerebral vasculopathy, cochlear nerve dysfunction, psychiatric and cognitive symptoms, autonomic dysfunction and peripheral neuropathy. Enzyme replacement therapy is also discussed in the light of its more prominent effects when administered early in life, which make it essential to diagnose FD as soon as possible.

  14. Neurological manifestations in Fabry's disease

    Møller, Anette Torvin; Jensen, Troels Staehelin

    2007-01-01

    Fabry's disease is an X-linked lysosomal storage disorder caused by a defect in the gene that encodes the lysosomal enzyme alpha-galactosidase A. Symptoms arise because of accumulation of globotriaosylceramide in multiple organs, resulting in severely reduced quality of life and premature death....... Neurological symptoms, such as burning sensations (occasionally accompanied by acroparesthesia) and stroke, are among the first to appear, and occur in both male and female patients. A delay in establishing the diagnosis of Fabry's disease can cause unnecessary problems, especially now that enzyme replacement...

  15. Neurological disorders and celiac disease.

    Casella, Giovanni; Bordo, Bianca M; Schalling, Renzo; Villanacci, Vincenzo; Salemme, Marianna; Di Bella, Camillo; Baldini, Vittorio; Bassotti, Gabrio

    2016-06-01

    Celiac disease (CD) determines neurologic manifestations in 10% of all CD patients. We describe the most common clinical manifestations as cerebellar ataxia, gluten encephalopathy, multiple sclerosis, peripheral neuropathies, sensorineural hearing loss, epilepsy, headache, depression, cognitive deficiencies and other less described clinical conditions. Our aim is to perform, as more as possible, a review about the most recent update on the topics in international literature. It is important to consider clinical neurological manifestations in celiac patients and to research these conditions also in the follow-up because they may start also one year after the start of gluten free diet (GFD) as peripheral neuropathy. The association with autism is analysed and possible new association with non-celiac gluten sensitivity (NCGS) are considered.

  16. [Neurological diseases and nutrition -- what can we do?].

    Tamási, Péter

    2014-12-21

    Neurological diseases and nutrition are in complex relationship. In the first part of this review the nutritional consequences of acute neurological diseases is presented, with special emphasis on traumatic injuries of the nervous system and stroke. Nutritional therapy of these patients is described in detail. In addition, chronic, degenerative neurological pathological conditions are also discussed, including nutritional consequences and possibilities of therapy. Some ethical and legal issues are also considered. The second part of this review article describes neurological consequences of nutritional problems, both deficits of macro- and micronutrients and toxic effects.

  17. Acute cerebellar ataxia: A neurological manifestation in malaria

    Peddametla Shravan Kumar

    2014-01-01

    Full Text Available Malaria is a vector-borne disease transmitted by the bite of an infected female anopheles mosquito presents with varied clinical manifestations. Neurological manifestations include headaches, confusion, convulsions, hemiplegia, ataxia, cerebral palsy, cortical blindness, and Guillain-Barre syndrome (GBS. We are presenting a case report of acute cerebellar ataxia in a 20-year-old male patient who presented with fever and positive for Plasmodium vivax and Plasmodium falciparum malaria antibodies.

  18. Neurologic Diseases in Special Care Patients.

    Robbins, Miriam R

    2016-07-01

    Neurologic diseases can have a major impact on functional capacity. Patients with neurologic disease require individualized management considerations depending on the extent of impairment and impact on functional capacity. This article reviews 4 of the more common and significant neurologic diseases (Alzheimer disease, cerebrovascular accident/stroke, multiple sclerosis, and Parkinson disease) that are likely to present to a dental office and provides suggestions on the dental management of patients with these conditions.

  19. The Spectrum of Neurological Manifestations Associated with Gaucher Disease

    Tamanna Roshan Lal

    2017-03-01

    Full Text Available Gaucher disease, the most common lysosomal storage disorder, is due to a deficiency in the enzyme glucocerebrosidase. This leads to the accumulation of its normal substrate, glucocerebroside, in tissue macrophages, affecting the hematological, visceral, bone and neurologic systems. Gaucher disease is classified into three broad phenotypes based upon the presence or absence of neurological involvement: type 1 (non-neuronopathic, type 2 (acute neuronopathic, and type 3 (subacute neuronopathic. Phenotypically, there is a wide spectrum of visceral and neurological manifestations. Enzyme replacement is effective in managing the visceral disease; however, treating the neurological manifestations has proved to be more challenging. This review discusses the various neurological manifestations encountered in Gaucher disease, and provides a brief overview regarding the treatment and ongoing research challenges.

  20. [Neurological disease and facial recognition].

    Kawamura, Mitsuru; Sugimoto, Azusa; Kobayakawa, Mutsutaka; Tsuruya, Natsuko

    2012-07-01

    To discuss the neurological basis of facial recognition, we present our case reports of impaired recognition and a review of previous literature. First, we present a case of infarction and discuss prosopagnosia, which has had a large impact on face recognition research. From a study of patient symptoms, we assume that prosopagnosia may be caused by unilateral right occipitotemporal lesion and right cerebral dominance of facial recognition. Further, circumscribed lesion and degenerative disease may also cause progressive prosopagnosia. Apperceptive prosopagnosia is observed in patients with posterior cortical atrophy (PCA), pathologically considered as Alzheimer's disease, and associative prosopagnosia in frontotemporal lobar degeneration (FTLD). Second, we discuss face recognition as part of communication. Patients with Parkinson disease show social cognitive impairments, such as difficulty in facial expression recognition and deficits in theory of mind as detected by the reading the mind in the eyes test. Pathological and functional imaging studies indicate that social cognitive impairment in Parkinson disease is possibly related to damages in the amygdalae and surrounding limbic system. The social cognitive deficits can be observed in the early stages of Parkinson disease, and even in the prodromal stage, for example, patients with rapid eye movement (REM) sleep behavior disorder (RBD) show impairment in facial expression recognition. Further, patients with myotonic dystrophy type 1 (DM 1), which is a multisystem disease that mainly affects the muscles, show social cognitive impairment similar to that of Parkinson disease. Our previous study showed that facial expression recognition impairment of DM 1 patients is associated with lesion in the amygdalae and insulae. Our study results indicate that behaviors and personality traits in DM 1 patients, which are revealed by social cognitive impairment, are attributable to dysfunction of the limbic system.

  1. [Neurological complications of inflammatory bowel diseases].

    Cieplik, N; Stangel, M; Bachmann, O

    2013-02-01

    Inflammatory bowel diseases, such as Crohn's disease, ulcerative colitis, autoantibody driven celiac disease and infectious Whipple's disease can all be associated with neurological symptoms. The neurological manifestation may occur even before the gastrointestinal symptoms or the enteropathic symptoms can even be absent as in celiac disease. These diseases can be caused by malresorption and lack of vitamins due to enteral inflammation as well as (auto-)immunological mechanisms and drug-associated side effects. Thus, inflammatory bowel diseases have to be considered in the differential diagnosis. In this review the most common neurological manifestations of these diseases will be described as well as the diagnostic approach.

  2. Orthotropic liver transplantation for intractable neurological manifestations of Wilson's disease.

    Sutariya, Vaibhav K; Tank, Anad H; Modi, Pranjal R

    2015-01-01

    Wilson's disease (WD) is an inherited autosomal recessive disorder characterized by copper accumulation and toxicity, affecting mainly the liver and brain. Orthotopic liver transplantation (OLT) is the definitive therapy for patients with WD. Acute fulminant hepatic failure and decompensated cirrhosis are well-established indications for OLT. Patients with severe neurologic impairment can also be benefited by OLT. Here, we present a patient who underwent OLT for isolated neurological WD.

  3. Astaxanthin as a Potential Neuroprotective Agent for Neurological Diseases.

    Wu, Haijian; Niu, Huanjiang; Shao, Anwen; Wu, Cheng; Dixon, Brandon J; Zhang, Jianmin; Yang, Shuxu; Wang, Yirong

    2015-09-11

    Neurological diseases, which consist of acute injuries and chronic neurodegeneration, are the leading causes of human death and disability. However, the pathophysiology of these diseases have not been fully elucidated, and effective treatments are still lacking. Astaxanthin, a member of the xanthophyll group, is a red-orange carotenoid with unique cell membrane actions and diverse biological activities. More importantly, there is evidence demonstrating that astaxanthin confers neuroprotective effects in experimental models of acute injuries, chronic neurodegenerative disorders, and neurological diseases. The beneficial effects of astaxanthin are linked to its oxidative, anti-inflammatory, and anti-apoptotic characteristics. In this review, we will focus on the neuroprotective properties of astaxanthin and explore the underlying mechanisms in the setting of neurological diseases.

  4. Astaxanthin as a Potential Neuroprotective Agent for Neurological Diseases

    Haijian Wu

    2015-09-01

    Full Text Available Neurological diseases, which consist of acute injuries and chronic neurodegeneration, are the leading causes of human death and disability. However, the pathophysiology of these diseases have not been fully elucidated, and effective treatments are still lacking. Astaxanthin, a member of the xanthophyll group, is a red-orange carotenoid with unique cell membrane actions and diverse biological activities. More importantly, there is evidence demonstrating that astaxanthin confers neuroprotective effects in experimental models of acute injuries, chronic neurodegenerative disorders, and neurological diseases. The beneficial effects of astaxanthin are linked to its oxidative, anti-inflammatory, and anti-apoptotic characteristics. In this review, we will focus on the neuroprotective properties of astaxanthin and explore the underlying mechanisms in the setting of neurological diseases.

  5. [Neurological diseases in the aged].

    Kameyama, M

    1990-12-01

    In this paper, I described clinical and basic problems on neurology of the aged patients. These studies have been done in various institutions with many co-workers. 1) A PET study revealed some age differences on CBF, CMRO2, or CMRgl. But these results are not so rigid in which much of individual variations should be considered in interpretation. Calendar age is not always compatible to biological age. 2) Saccular aneurysms in the brain artery were found in 7.3% of 1200 routine autopsy series of the aged subjects. Aneurysms with external diameter exceeding 6 mm had been fatally ruptured in 14 (78%) of 18 subjects. 3) Variations of the pyramidal crossing are found responsible for bizarre clinical manifestations. Non-crossing component was more prominent in the right pyramidal tract; consequently, right pyramidal tracts including ventral and lateral one seemed to have more extensive representation in the spinal cord level. 4) I123-IMP SPECT study showed a reduced uptake in the area 4 or area 4-6 of the ALS patients. 5) I introduced a new simplified Wartenberg's maneuver, which is useful for detection of subtle pyramidal dysfunctions. 6) Cases with central pontine myelinolysis and those of paraneoplastic syndrome were presented with an emphasis on their patho-chemical mechanisms. 7) Lewis-Sumner syndrome showing multifocal persistent conduction block is not rare in the aged, in which we have already had some useful therapeutic methods. 8) Dementia complicated with neurodegenerative disease was discussed on its clinical and chemical features of mental disturbances. In ALS-dementia, CSF-homovanilic acid reduced significantly than in the control and L-dopa was effective in some patients. 9) Vascular and Alzheimer-type dementias were presented and discussed on their pathogenetic mechanism according to our recent studies with review of literature.

  6. THE NEUROLOGICAL FACE OF CELIAC DISEASE

    Sedat IŞIKAY

    2015-09-01

    Full Text Available BackgroundSeveral neurological disorders have also been widely described in celiac disease patients.ObjectiveThe aim of this study was to determine the incidence of accompanying different neurologic manifestations in children with celiac disease at the time of diagnosis and to discuss these manifestations in the light of the recent literature.MethodsThis prospective cross sectional study included 297 children diagnosed with celiac disease. The medical records of all patients were reviewed.ResultsIn neurological evaluation, totally 40 (13. 5% of the 297 celiac patients had a neurological finding including headache, epilepsy, migraine, mental retardation, breath holding spells, ataxia, cerebral palsy, attention deficit hyperactivity disorder, Down syndrome and Turner syndrome in order of frequency. There was not any significant difference between the laboratory data of the patients with and without neurological manifestations. However; type 3a biopsy was statistically significantly more common among patients without neurological manifestations, while type 3b biopsy was statistically significantly more common among patients with neurological manifestations.ConclusionIt is important to keep in mind that in clinical course of celiac disease different neurological manifestations may be reported.

  7. Hyponatremia in neurological diseases in ICU

    Lath Rahul

    2005-01-01

    Full Text Available Hyponatremia is the commonest electrolyte disturbance encountered in the neurological and neurosurgical intensive care units. It can present with signs and symptoms mimicking a neurological disease and can worsen the existing neurological deficits. Hyponatremia in neurological disorders is usually of the hypo-osmolar type caused either due to the Syndrome of Inappropriate Secretion of Anti Diuretic Hormone (SIADH or Cerebral Salt Wasting Syndrome (CSWS. It is important to distinguish between these two disorders, as the treatment of the two differ to a large extent. In SIADH, the fluid intake is restricted, whereas in CSWS the treatment involves fluid and salt replacement.

  8. Imaging of acute neurological conditions in pregnancy and the puerperium

    Dineen, R. [Department of Neuroradiology, Queen' s Medical Centre, Nottingham (United Kingdom)]. E-mail: robert.dineen@nhs.net; Banks, A. [Department of Anaesthesia, Queen' s Medical Centre, Nottingham (United Kingdom); Lenthall, R. [Department of Neuroradiology, Queen' s Medical Centre, Nottingham (United Kingdom)

    2005-11-01

    Eclampsia is one of the most common acute neurological events occurring during pregnancy. However, there are many other conditions that can present during pregnancy and the puerperium and that may either mimic eclampsia or produce other acute neurological manifestations. Frequently the symptoms and signs are non-specific, and it can be difficult to differentiate between these conditions on clinical grounds alone. Neuroradiological studies can provide valuable diagnostic information, and interventional radiological procedures may play a part in the subsequent management of these conditions. This review focuses on the imaging of acute neurological conditions which may be associated with, or present during, pregnancy and the puerperium.

  9. Neurologic Manifestations of Childhood Rheumatic Diseases

    Reza SHIARI

    2012-10-01

    Full Text Available Children with rheumatic disorders may have a wide variety of clinical features ranging from fever or a simple arthritis to complex multisystem autoimmune diseases. Information about the prevalence of neurological manifestations in children with rheumatologic disorders is limited. This review describes the neurologic complications of childhood Rheumatic disease either solely or combined with symptoms of other organs involvement, as a primary manifestation or as a part of other symptoms, additionally.

  10. Neurological Disorders in Adult Celiac Disease

    Hugh J Freeman

    2008-01-01

    Full Text Available Celiac disease may initially present as a neurological disorder. Alternatively, celiac disease may be complicated by neurological changes. With impaired nutrient absorption, different deficiency syndromes may occur and these may be manifested clinically with neurological changes. However, in patients with deficiency syndromes, extensive involvement of the small intestine with celiac disease is often evident. There are a number of reports of celiac disease associated with neuropathy, ataxia, dementia and seizure disorder. In these reports, there is no clear relationship with nutrient deficiency and a precise mechanism for the neurological changes has not been defined. A small number of patients have been reported to have responded to vitamin E administration, but most do not. In some, gluten antibodies have also been described, especially in those with ataxia, but a consistent response to a gluten-free diet has not been defined. Screening for celiac disease should be considered in patients with unexplained neurological disorders, including ataxia and dementia. Further studies are needed, however, to determine if a gluten-free diet will lead to improvement in the associated neurological disorder.

  11. Stem-cell therapy for neurologic diseases

    Shilpa Sharma

    2015-01-01

    Full Text Available With the advent of research on stem cell therapy for various diseases, an important need was felt in the field of neurological diseases. While congenital lesion may not be amenable to stem cell therapy completely, there is a scope of partial improvement in the lesions and halt in further progression. Neuro degenerative lesions like Parkinson′s disease, multiple sclerosis and amyotrophic lateral sclerosis have shown improvement with stem cell therapy. This article reviews the available literature and summarizes the current evidence in the various neurologic diseases amenable to stem cell therapy, the plausible mechanism of action, ethical concerns with insights into the future of stem cell therapy.

  12. Neurologic Manifestations of Childhood Rheumatic Diseases

    Reza SHIARI

    2013-01-01

    . Halimiasl A, Hosseini AH, Shiari R, Ghadamli P,Mojtahedzadeh S. Concomitant Coronary Artery Aneurysm and Myocarditis as a Rare Manifestation of Kawasaki Disease: A Case Report. J Compr Ped.2012;3(1:34-6. 11. Ichiyama T, Nishikawa M, Hayashi T, Koga M, Tashiro N, Furukawa S. Cerebral hypoperfusion during acute Kawasaki disease. Stroke. 1998 Jul;29(7:1320-1. 12. Muneuchi J, Kusuhara K, Kanaya Y, Ohno T, Furuno K,Kira R, Mihara F, Hara T. Magnetic resonance studies of brain lesions in patients with Kawasaki disease. Brain Dev. 2006 Jan;28(1:30-3. 13. Scolding NJ, Wilson H, Hohlfeld R, Polman C, Leite I,Gilhus N. The recognition, diagnosis and management of cerebral vasculitis: a European survey. Eur J Neurol. 2002Jul;9(4:343-7. 14. Guillevin L, Durand-Gasselin B, Cevallos R, Gayraud M, Lhote F, Callard P et al. Microscopic polyangiitis:clinical and laboratory findings in eighty-five patients.Arthritis Rheum. 1999 Mar;42(3:421-30. 15. Rahman A, Isenberg DA. Systemic lupus erythematosus.N Engl J Med. 2008 Feb;358(9:929-39. 16. Farivar S, Shiari R, Nejad Hosseinian M, Eshtad S. New genetic finding in systemic lupus erythematosus. Genet inthe 3rd Millen 2008;6(2:1333-8. 17. Sibbitt WL Jr, Brandt JR, Johnson CR, Maldonado ME,Patel SR, Ford CC et al. The incidence and prevalence of neuropsychiatric syndromes in pediatric onset systemic lupus erythematosus. J Rheumatol. 2002 Jul;29(7:1536-42. 18. Harel L, Sandborg C, Lee T, von Scheven E.Neuropsychiatric manifestations in pediatric systemic lupus erythematosus and association with antiphospholipid antibodies. J Rheumatol. 2006 Sep;33(9:1873-7. 19. Hawro T, Bogucki A, Sysa-Jedrzejowska A, BogaczewiczJ, Wozniacka A. [Neurological disorders in systemic lupus erythematosus patients]. Pol Merkur Lekarski. 2009 Jan;26(151:43-8. 20. Greenberg BM. The neurologic manifestations of systemic lupus erythematosus. Neurologist. 2009May;15(3:115-21.21. Sofat N, Malik O, Higgens CS. Neurological involvementin patients with rheumatic disease. QJM

  13. Astrocytes : a central element in neurological diseases

    Pekny, Milos; Pekna, Marcela; Messing, Albee; Steinhäuser, Christian; Lee, Jin Moo; Parpura, Vladimir; Hol, Elly M.; Sofroniew, Michael V.; Verkhratsky, Alexei

    2016-01-01

    The neurone-centred view of the past disregarded or downplayed the role of astroglia as a primary component in the pathogenesis of neurological diseases. As this concept is changing, so is also the perceived role of astrocytes in the healthy and diseased brain and spinal cord. We have started to unr

  14. Need for palliative care for neurological diseases.

    Provinciali, Leandro; Carlini, Giulia; Tarquini, Daniela; Defanti, Carlo Alberto; Veronese, Simone; Pucci, Eugenio

    2016-10-01

    The new concept of palliative care supports the idea of palliation as an early approach to patients affected by disabling and life-limiting disease which focuses on the patient's quality of life along the entire course of disease. This model moves beyond the traditional concept of palliation as an approach restricted to the final stage of disease and widens the fields of intervention. There is a growing awareness of the importance of palliative care not only in oncological diseases but also in many other branches of medicine, and it appears particularly evident in the approach to many of the most frequent neurological diseases that are chronic, incurable and autonomy-impairing illnesses. The definition and implementation of palliative goals and procedures in neurology must take into account the specific features of these conditions in terms of the complexity and variability of symptoms, clinical course, disability and prognosis. The realization of an effective palliative approach to neurological diseases requires specific skills and expertise to adapt the concept of palliation to the peculiarities of these diseases; this approach should be realized through the cooperation of different services and the action of a multidisciplinary team in which the neurologist should play a central role to identify and face the patient's needs. In this view, it is paramount for the neurologist to be trained in these issues to promote the integration of palliative care in the care of neurological patients.

  15. The Neurologic Manifestations of Mitochondrial Disease

    Parikh, Sumit

    2010-01-01

    The nervous system contains some of the body's most metabolically demanding cells that are highly dependent on ATP produced via mitochondrial oxidative phosphorylation. Thus, the neurological system is consistently involved in patients with mitochondrial disease. Symptoms differ depending on the part of the nervous system affected. Although almost…

  16. Astrocytes: a central element in neurological diseases.

    Pekny, Milos; Pekna, Marcela; Messing, Albee; Steinhäuser, Christian; Lee, Jin-Moo; Parpura, Vladimir; Hol, Elly M; Sofroniew, Michael V; Verkhratsky, Alexei

    2016-03-01

    The neurone-centred view of the past disregarded or downplayed the role of astroglia as a primary component in the pathogenesis of neurological diseases. As this concept is changing, so is also the perceived role of astrocytes in the healthy and diseased brain and spinal cord. We have started to unravel the different signalling mechanisms that trigger specific molecular, morphological and functional changes in reactive astrocytes that are critical for repairing tissue and maintaining function in CNS pathologies, such as neurotrauma, stroke, or neurodegenerative diseases. An increasing body of evidence shows that the effects of astrogliosis on the neural tissue and its functions are not uniform or stereotypic, but vary in a context-specific manner from astrogliosis being an adaptive beneficial response under some circumstances to a maladaptive and deleterious process in another context. There is a growing support for the concept of astrocytopathies in which the disruption of normal astrocyte functions, astrodegeneration or dysfunctional/maladaptive astrogliosis are the primary cause or the main factor in neurological dysfunction and disease. This review describes the multiple roles of astrocytes in the healthy CNS, discusses the diversity of astroglial responses in neurological disorders and argues that targeting astrocytes may represent an effective therapeutic strategy for Alexander disease, neurotrauma, stroke, epilepsy and Alzheimer's disease as well as other neurodegenerative diseases.

  17. [Neurological complications of inflammatory bowel disease].

    Bermejo, Pedro Emilio; Burgos, Aurora

    2008-05-10

    Although ulcerative colitis and Crohn's disease have traditionally been considered to be inflammatory diseases limited to the gastrointestinal tract, it has been shown that both pathologies are frequently accompanied by various extraintestinal disorders. There is an increasing evidence that they may also manifest in the nervous system, including the peripheral and the central parts. Although some of these neurological complications have been known for a long time, such as cerebrovascular disease, vasculitis and autoinmune processes including neuropathies and cerebral demyelination, others have been recently described. With the exception of some of this complications such as the thromboembolism, evidence for a casual relationship relies merely on single case reports or case series. In this article, we try to review the existing evidence on neurological manifestations of both variants of inflammatory bowel disease.

  18. Neurological disorders and inflammatory bowel diseases.

    Casella, Giovanni; Tontini, Gian Eugenio; Bassotti, Gabrio; Pastorelli, Luca; Villanacci, Vincenzo; Spina, Luisa; Baldini, Vittorio; Vecchi, Maurizio

    2014-07-21

    Extraintestinal manifestations occur in about one-third of patients living with inflammatory bowel disease (IBD) and may precede the onset of gastrointestinal symptoms by many years. Neurologic disorders associated with IBD are not frequent, being reported in 3% of patients, but they often represent an important cause of morbidity and a relevant diagnostic issue. In addition, the increasing use of immunosuppressant and biological therapies for IBD may also play a pivotal role in the development of neurological disorders of different type and pathogenesis. Hence, we provide a complete and profound review of the main features of neurological complications associated with IBD, with particular reference to those related to drugs and with a specific focus on their clinical presentation and possible pathophysiological mechanisms.

  19. Wilson's disease and other neurological copper disorders.

    Bandmann, Oliver; Weiss, Karl Heinz; Kaler, Stephen G

    2015-01-01

    The copper metabolism disorder Wilson's disease was first defined in 1912. Wilson's disease can present with hepatic and neurological deficits, including dystonia and parkinsonism. Early-onset presentations in infancy and late-onset manifestations in adults older than 70 years of age are now well recognised. Direct genetic testing for ATP7B mutations are increasingly available to confirm the clinical diagnosis of Wilson's disease, and results from biochemical and genetic prevalence studies suggest that Wilson's disease might be much more common than previously estimated. Early diagnosis of Wilson's disease is crucial to ensure that patients can be started on adequate treatment, but uncertainty remains about the best possible choice of medication. Furthermore, Wilson's disease needs to be differentiated from other conditions that also present clinically with hepatolenticular degeneration or share biochemical abnormalities with Wilson's disease, such as reduced serum ceruloplasmin concentrations. Disordered copper metabolism is also associated with other neurological conditions, including a subtype of axonal neuropathy due to ATP7A mutations and the late-onset neurodegenerative disorders Alzheimer's disease and Parkinson's disease.

  20. Neurological Manifestations In Inflammatory Bowel Disease

    youssef HNACH

    2015-06-01

    Full Text Available IntroductionThe purpose of this retrospective study was to report neurological manifestations noted in patients who were monitored for inflammatory bowel disease, in order to document the pathophysiological, clinical, progressive, and therapeutic characteristics of this entity.Material and methodsWe conducted a retrospective study on patients monitored -in the gastroenterology service in Ibn Sina Hospital in Rabat, Morocco- for inflammatory bowel disease from 1992 till 2013 and who developed neurological manifestations during its course. Patients with iatrogenic complications were excluded, as well as patients with cerebrovascular risk factors.ResultsThere were 6 patients, 4 of whom have developed peripheral manifestations. Electromyography enabled the diagnosis to be made and the outcome was favorable with disappearance of clinical manifestations and normalization of the electromyography.The other 2 patients, monitored for Crohn’s disease, developed ischemic stroke. Cerebral computed tomography angiography provided positive and topographic diagnosis. Two patients were admitted to specialized facilities.ConclusionNeurological manifestations in inflammatory bowel disease are rarely reported.  Peripheral neuropathies and stroke remain the most common manifestations. The mechanisms of these manifestations are not clearly defined yet. Currently, we hypothesize the interaction of immune mediators.

  1. Neurologic complications of valvular heart disease.

    Cruz-Flores, Salvador

    2014-01-01

    Valvular heart disease (VHD) is frequently associated with neurologic complications; cerebral embolism is the most common of these since thrombus formation results from the abnormalities in the valvular surfaces or from the anatomic and physiologic changes associated with valve dysfunction, such as atrial or ventricular enlargement, intracardiac thrombi, and cardiac dysrhythmias. Prosthetic heart valves, particularly mechanical valves, are very thrombogenic, which explains the high risk of thromboembolism and the need for anticoagulation for the prevention of embolism. Infective endocarditis is a disease process with protean manifestations that include not only cerebral embolism but also intracranial hemorrhage, mycotic aneurysms, and systemic manifestations such as fever and encephalopathy. Other neurologic complications include nonbacterial thrombotic endocarditis, a process associated with systemic diseases such as cancer and systemic lupus erythematosus. For many of these conditions, anticoagulation is the mainstay of treatment to prevent cerebral embolism, therefore it is the potential complications of anticoagulation that can explain other neurologic complications in patients with VHD. The prevention and management of these complications requires an understanding of their natural history in order to balance the risks posed by valvular disease itself against the risks and benefits associated with treatment.

  2. Neuroelectrophysiological studies on neurological autoimmune diseases

    Yin-hong LIU

    2014-09-01

    Full Text Available The neuroelectrophysiological manifestations of four clinical typical neurological autoimmune diseases including multiple sclerosis (MS, Guillain-Barré syndrome (GBS, myasthenia gravis (MG, and polymyositis and dermatomyositis were reviewed in this paper. The diagnostic value of evoked potentials for multiple sclerosis, nerve conduction studies (NCS for Guillain-Barré syndrome, repetitive nerve stimulation (RNS and single-fiber electromyography (SFEMG for myasthenia gravis, and needle electromyography for polymyositis and dermatomyositis were respectively discussed. This review will help to have comprehensive understanding on electrophysiological examinations and their clinical significance in the diagnosis of neurological autoimmune diseases. doi: 10.3969/j.issn.1672-6731.2014.09.004

  3. Approaching neurological diseases to reduce mobility limitations in older persons.

    Lauretani, Fulvio; Ceda, Gian Paolo; Pelliccioni, Pio; Ruffini, Livia; Nardelli, Anna; Cherubini, Antonio; Maggio, Marcello

    2014-01-01

    The rapidly increasing elderly population poses a major challenge for future health-care systems. Neurological diseases in older persons are particularly common and coexist with other clinical conditions. This is not surprising given that, for example, even patients with Alzheimer Disease (AD) could have relevant extrapyramidal signs at the moment of the diagnosis with motor signs having more negative prognostic value. Longitudinal studies conducted on Parkinson Disease (PD) showed that, after 20 years, dementia is not only present in almost all survivors but is also the main factor influencing nursing home admission. Recently, it has been reported the importance of Comprehensive Geriatric Assessment (CGA: comprehensive evaluation of cognition, depressive symptoms, mobility and functional assessment) as a tool reducing morbidity in frail older patients admitted to any acute hospital unit. The CGA should be considered as a technological device, for physicians who take care of older persons affected by overlapping neurological diseases. CGA is an extraordinary and cost effective instrument even in patients with advanced neurological diseases where allows to collect valuable information for an effective plan of management.

  4. Epigenetic mechanisms in neurological and neurodegenerative diseases.

    Jorge eLandgrave-Gómez

    2015-02-01

    Full Text Available The role of epigenetic mechanisms in the function and homeostasis of the central nervous system (CNS and its regulation in diseases is one of the most interesting processes of contemporary neuroscience. In the last decade, a growing body of literature suggests that long-term changes in gene transcription associated with CNS´s regulation and neurological disorders are mediated via modulation of chromatin structure.Epigenetics, introduced for the first time by Waddington in the early 1940s, has been traditionally referred to a variety of mechanisms that allow heritable changes in gene expression even in the absence of DNA mutation. However, new definitions acknowledge that many of these mechanisms used to perpetuate epigenetic traits in dividing cells are used by neurons to control a variety of functions dependent on gene expression. Indeed, in the recent years these mechanisms have shown their importance in the maintenance of a healthy CNS. Moreover, environmental inputs that have shown effects in CNS diseases, such as nutrition, that can modulate the concentration of a variety of metabolites such as acetyl-coenzyme A (acetyl-coA, nicotinamide adenine dinucleotide (NAD+ and beta hydroxybutyrate (β-HB, regulates some of these epigenetic modifications, linking in a precise way environment with gene expression.This manuscript will portray what is currently understood about the role of epigenetic mechanisms in the function and homeostasis of the CNS and their participation in a variety of neurological disorders. We will discuss how the machinery that controls these modifications plays an important role in processes involved in neurological disorders such as neurogenesis and cell growth. Moreover, we will discuss how environmental inputs modulate these modifications producing metabolic and physiological alterations that could exert beneficial effects on neurological diseases. Finally, we will highlight possible future directions in the field of

  5. Trends in Mitochondrial Therapeutics for Neurological Disease.

    Leitão-Rocha, Ana; Guedes-Dias, Pedro; Pinho, Brígida R; Oliveira, Jorge M A

    2015-01-01

    Neuronal homeostasis is critically dependent on healthy mitochondria. Mutations in mitochondrial DNA (mtDNA), in nuclear-encoded mitochondrial components, and age-dependent mitochondrial damage, have all been connected with neurological disorders. These include not only typical mitochondrial syndromes with neurological features such as encephalomyopathy, myoclonic epilepsy, neuropathy and ataxia; but also secondary mitochondrial involvement in neurodegenerative disorders such as Alzheimer's, Parkinson's and Huntington's disease. Unravelling the molecular aetiology of mitochondrial dysfunction opens new therapeutic prospects for diseases thus far lacking effective treatments. In this review we address recent advances on preventive strategies, such as pronuclear, spindle-chromosome complex, or polar body genome transfer to replace mtDNA and avoid disease transmission to newborns; we also address experimental mitochondrial therapeutics aiming to benefit symptomatic patients and prevent disease manifestation in those at risk. Specifically, we focus on: (1) gene therapy to reduce mutant mtDNA, such as anti-replicative therapies and mitochondriatargeted nucleases allowing favourable heteroplasmic shifts; (2) allotopic expression of recoded wild-type mitochondrial genes, including targeted tRNAs and xenotopic expression of cognate genes to compensate for pathogenic mutations; (3) mitochondria targeted-peptides and lipophilic cations for in vivo delivery of antioxidants or other putative therapeutics; and (4) modulation of mitochondrial dynamics at the level of biogenesis, fission, fusion, movement and mitophagy. Further advances in therapeutic development are hindered by scarce in vivo models for mitochondrial disease, with the bulk of available data coming from cellular models. Nevertheless, wherever available, we also address data from in vivo experiments and clinical trials, focusing on neurological disease models.

  6. Study on subsequent neurologic complications in children with acute leukemia

    Kobayashi, Naoaki; Shimazaki, Haruyo; Hoshi, Yasutaka; Akatsuka, Jun-ichi (Jikei Univ., Tokyo (Japan). School of Medicine)

    1989-06-01

    Twenty-seven children with acute leukemia were studied in order to detect the subsequent neurologic complications due to chemotherapy and radiation therapy. Twenty-four patients with ALL received central nervous system prophylaxis including cranial irradiation. The methods of evaluation consisted of electroencephalogram (EEG), computed tomography of the head (CT scan), soft neurological sign, intelligence quotient (IQ) and Bender Gestalt test. The patients with relapse showed severe abnormalities in various kinds of examinations. Younger children at diagnosis were associated with a higher abnormality rate of soft neurological signs and Bender Gestalt test. Factors which were found to be closely associated with a lower IQ score included younger children at diagnosis and longer duration of remission time. These results indicate the need for caution for the dosage of cranial irradiation for younger patients in CNS prophylaxis, and improvement of a lower IQ score in long-term survivors requires further investigation as to the appropriate intellectual environment for their development after remission. (author).

  7. Efficacy of Intravenous Immunoglobulin in Neurological Diseases.

    Lünemann, Jan D; Quast, Isaak; Dalakas, Marinos C

    2016-01-01

    Owing to its anti-inflammatory efficacy in various autoimmune disease conditions, intravenous immunoglobulin (IVIG)-pooled IgG obtained from the plasma of several thousands individuals-has been used for nearly three decades and is proving to be efficient in a growing number of neurological diseases. IVIG therapy has been firmly established for the treatment of Guillain-Barré syndrome, chronic inflammatory demyelinating polyneuropathy, and multifocal motor neuropathy, either as first-line therapy or adjunctive treatment. IVIG is also recommended as rescue therapy in patients with worsening myasthenia gravis and is beneficial as a second-line therapy for dermatomyositis and stiff-person syndrome. Subcutaneous rather than intravenous administration of IgG is gaining momentum because of its effectiveness in patients with primary immunodeficiency and the ease with which it can be administered independently from hospital-based infusions. The demand for IVIG therapy is growing, resulting in rising costs and supply shortages. Strategies to replace IVIG with recombinant products have been developed based on proposed mechanisms that confer the anti-inflammatory activity of IVIG, but their efficacy has not been tested in clinical trials. This review covers new developments in the immunobiology and clinical applications of IVIG in neurological diseases.

  8. Statins in acute neurologic disease:which one, which dose, when to start, and when not to stop

    Bong-Su Kang; Gene Sung; May Kim-Tenser; Nerses Sanossian

    2016-01-01

    Statins could have physiologic properties that may beneift patients that have been diagnosed with various acute neurological diseases. This review aims tosummarize the literature pertaining to stain use in acute neurological disease such as subarachnoid hemorrhage, intracerebral hemorrhage (ICH), cerebral ischemia (CI), traumatic brain injury, status epilepticus and meningitis. The authors reviewed published abstracts and manuscripts pertaining to experimental and clinical trials relevant to statins in acute neurological disease. Although acute statin therapy in the setting of subarachnoid hemorrhage might reduce delayed cerebral ischemia and mortality, it should not be considered standard care at this time. Acute statins therapy has not demonstrated anybeneift yet folowing an ICH or CI. Acute statin withdrawal may worsen outcome in acute CI. Observational and case-control studies suggest that pretreatment with statin at time of onset may be associated with better outcomes. Even though preclinical studies have shown statins to have beneifcial effects, there has been no clinical evidence. In conclusion, current published studies have not shown that acute statin therapy has any beneifcal effects in acute neurologic diseases and therefore further large randomized clinical trials are needed.

  9. Dysfunctional HCN ion channels in neurological diseases.

    DiFrancesco, Jacopo C; DiFrancesco, Dario

    2015-01-01

    Hyperpolarization-activated cyclic nucleotide-gated (HCN) channels are expressed as four different isoforms (HCN1-4) in the heart and in the central and peripheral nervous systems. HCN channels are activated by membrane hyperpolarization at voltages close to resting membrane potentials and carry the hyperpolarization-activated current, dubbed If (funny current) in heart and Ih in neurons. HCN channels contribute in several ways to neuronal activity and are responsible for many important cellular functions, including cellular excitability, generation, and modulation of rhythmic activity, dendritic integration, transmission of synaptic potentials, and plasticity phenomena. Because of their role, defective HCN channels are natural candidates in the search for potential causes of neurological disorders in humans. Several data, including growing evidence that some forms of epilepsy are associated with HCN mutations, support the notion of an involvement of dysfunctional HCN channels in different experimental models of the disease. Additionally, some anti-epileptic drugs are known to modify the activity of the Ih current. HCN channels are widely expressed in the peripheral nervous system and recent evidence has highlighted the importance of the HCN2 isoform in the transmission of pain. HCN channels are also present in the midbrain system, where they finely regulate the activity of dopaminergic neurons, and a potential role of these channels in the pathogenesis of Parkinson's disease has recently emerged. The function of HCN channels is regulated by specific accessory proteins, which control the correct expression and modulation of the neuronal Ih current. Alteration of these proteins can severely interfere with the physiological channel function, potentially predisposing to pathological conditions. In this review we address the present knowledge of the association between HCN dysfunctions and neurological diseases, including clinical, genetic, and physiopathological

  10. Dysfunctional HCN ion channels in neurological diseases

    Jacopo C. DiFrancesco

    2015-03-01

    Full Text Available Hyperpolarization-activated cyclic nucleotide-gated (HCN channels are expressed as four different isoforms (HCN1-4 in the heart and in the central and peripheral nervous systems. HCN channels are activated by membrane hyperpolarization at voltages close to resting membrane potentials and carry the hyperpolarization-activated current, dubbed If (funny current in heart and Ih in neurons. HCN channels contribute in several ways to neuronal activity and are responsible for many important cellular functions, including cellular excitability, generation and modulation of rhythmic activity, dendritic integration, transmission of synaptic potentials and plasticity phenomena. Because of their role, defective HCN channels are natural candidates in the search for potential causes of neurological disorders in humans. Several data, including growing evidence that some forms of epilepsy are associated with HCN mutations, support the notion of an involvement of dysfunctional HCN channels in different experimental models of the disease. Additionally, some anti-epileptic drugs are known to modify the activity of the Ih current. HCN channels are widely expressed in the peripheral nervous system and recent evidence has highlighted the importance of the HCN2 isoform in the transmission of pain. HCN channels are also present in the midbrain system, where they finely regulate the activity of dopaminergic neurons, and a potential role of these channels in the pathogenesis of Parkinson’s disease has recently emerged. The function of HCN channels is regulated by specific accessory proteins, which control the correct expression and modulation of the neuronal Ih current. Alteration of these proteins can severely interfere with the physiological channel function, potentially predisposing to pathological conditions. In this review we address the present knowledge of the association between HCN dysfunctions and neurological diseases, including clinical, genetic and

  11. Coenzyme Q10 and Neurological Diseases

    Gabriele Siciliano

    2009-12-01

    Full Text Available Coenzyme Q10 (CoQ10, or ubiquinone is a small electron carrier of the mitochondrial respiratory chain with antioxidant properties. CoQ10 supplementation has been widely used for mitochondrial disorders. The rationale for using CoQ10 is very powerful when this compound is primary decreased because of defective synthesis. Primary CoQ10 deficiency is a treatable condition, so heightened “clinical awareness” about this diagnosis is essential. CoQ10 and its analogue, idebenone, have also been widely used in the treatment of other neurodegenerative disorders. These compounds could potentially play a therapeutic role in Parkinson’s disease, Huntington’s disease, amyotrophic lateral sclerosis, Friedreich’s ataxia, and other conditions which have been linked to mitochondrial dysfunction. This article reviews the physiological roles of CoQ10, as well as the rationale and the role in clinical practice of CoQ10 supplementation in different neurological diseases, from primary CoQ10 deficiency to neurodegenerative disorders.

  12. Magnetic resonance imaging in neurologic diseases

    Chang, Kee Hyun; Han, Man Chung; Wan, Chu Wan; Myung, Ho Jin; Choi, Kil Soo [Seoul National University College of Medicine, Seoul (Korea, Republic of); Ahn, Chang Beom; Oh, Chang Hyun; Cho, Zang Hee [Koear Advanced Institute of Science, Seoul (Korea, Republic of)

    1985-02-15

    Magnetic resonance (MR) imaging with 0.15 Tesla resistive magnet developed by Korea Advanced Institute of Science were performed in 27 patients with various neurologic diseases and compared with x-ray computed tomography (CT). The purpose of the paper is to evaluate the image quality, the diagnostic value and limitation, and the optimal pulse sequence of MR imagings with a resistive magnet. The MR images were obtained by using a variety of pulse sequence with spin echo technique including saturation recovery. T2-weighted spin echo, and/or inversion recovery with various pulse repetition (TR) and echo delay (TE) times. The MR imaging demonstrated the capability of detecting the lesions shown on CT in al cases and also detected an additional finding in one case (multiple sclerosis) which was not seen on CT. The MR imaging appeared to be more useful than CT in the evaluation of syringomyelia of spinal cord and white matter disease, while it failed to demonstrated small calcific lesion or inflammatory nodule (less than 1 cm) shown on CT and has shown somewhat poor contrast resolution in the case of meingloma. The spatial resolution of saturation recovery images was similar or superior to CT, whereas the contrast resolution of saturation recovery was inferior to CT. While the saturation recovery images have shown false negative findings in 5 patients (19%), the inversion recovery and T2-weighted spin echo have shown consistently positive findings. The inversive recovery and T2-weighted spin echo images demonstrated better contrast discrimination between normal and pathologic conditions than the saturation recovery images, but somewhat poorer spatial resolution. Authors suggest that the MR images of both the saturation recovery with 300/30 and T2-weighted spin echo with 1000/90 be used as a routine procedure and additional inversion recovery of 1300/300/30 sequence as a option if white matter disease is suspected.

  13. Risk of neurological diseases among survivors of electric shocks

    Grell, Kathrine; Meersohn, Andrea; Schüz, Joachim

    2012-01-01

    Several studies suggest a link between electric injuries and neurological diseases, where electric shocks may explain elevated risks for neuronal degeneration and, subsequently, neurological diseases. We conducted a retrospective cohort study on the risk of neurological diseases among people...... in Denmark who had survived an electric accident in 1968-2008. The cohort included 3,133 people and occurrences of neurological diseases were determined by linkage to the nationwide population-based Danish National Register of Patients. The numbers of cases observed at first hospital contact in the cohort...... were compared with the respective rates of first hospital contacts for neurological diseases in the general population. We observed significantly increased risks for peripheral nerve diseases (standardized hospitalization ratio (SHR), 1.66; 95% confidence interval (CI), 1.22-2.22), for migraine (SHR, 1...

  14. Neurological Effects of Acute Carbon Monoxide Poisoning in Children

    Coskun YARAR

    2009-11-01

    Full Text Available Carbon monoxide poisoning (COP is one of the most common causes of mortality and morbidity due to poisoning in all over the world. Although the incidence of COP has not been known exactly in the childhood, almost one-third of CO exposures occurred in children. The data regarding COP in children are inconclusive. Children may be more vulnerable to CO exposure than adults as a result of their high respiration and metabolic rates, high oxygen metabolism, and immature central nervous system. Recent researches proposed new theories about neurological effects of CO toxicity. The clinical presentations associated acute COP may be various and nonspecific. Unrecognized CO exposure may lead to significant morbidity and mortality. CO exposed children often become symptomatic earlier, and recover more rapidly, than similarly CO exposed adults. Mild clinical signs and symptoms associated with COP are headache, dizziness, weakness, lethargy, and myalgia; however, severe signs and symptoms such as blurred vision, syncope, convulsion, coma, cardiopulmonary arrest and death can also accompany with COP. Neurologic manifestations can include altered mental status at different degrees, neck stiffness, tremor, ataxia, and positive Babinski's sign. Delayed neurologic sequels (DNS of COP might be seen in children like adults. DNS symptoms and signs in children include memory problems, mental retardation, mutism, fecal and urinary incontinence, motor deficits, facial palsy, psychosis, chronic headache, seizures, and epilepsy. After CO exposure children must be cared to detect and treat DNS. Although hyperbaric oxygen therapy (HBOT is reported to prevent development of DNS, its indications, application duration and procedures are controversial in both of the children and adults. Although their predictive values are limited, exposing to CO more than eight hours and suffering from CO-induced coma, cardiac arrest, lactic acidosis, high COHb levels, and pathologic findings

  15. Acute flaccid paraplegia: neurological approach, diagnostic workup, and therapeutic options

    Gentian Vyshka

    2015-03-01

    Full Text Available Acute flaccid paraplegia is a clinical occurrence with extreme importance, due to the dramatic presentation, the severity of the underlying disorder, and the generally poor prognosis that follows such a condition. Among etiological factors, the traumatic events are of particular interest, with the clinical treating dealing with a severely ill patient, following fall from height, motor vehicle collisions, and direct shocks applied over the vertebral column. The non-traumatic list is more numerous; however the severity of the acute paraplegia is not necessarily of a lesser degree. Viral infections, autoimmune disorders, and ischemic events involving feeding spinal arteries have been imputed. However, chemical and medications injected during procedures or accidentally intrathecal administration can produce acute flaccid paraplegia. A careful neurological assessment and complete electrophysiological and imaging studies must follow. In spite of the poor prognosis, different therapeutic options have been proposed and applied. Neurosurgical and orthopedic interventions are often necessary when trauma is present, with high dose glucocorticoids treatment preceding the intervention, in a hope to decrease edema-related compression over the spinal cord. Immunoglobulins and plasmapheresis are logical and helpful options when a polyradiculoneuritis produces such a clinical picture. The role of decompression, as neurosurgical exclusivity, has been considered as well.

  16. Neurological diseases of ruminant livestock in Australia. I: general neurological examination, necropsy procedures and neurological manifestations of systemic disease, trauma and neoplasia.

    Finnie, J W; Windsor, P A; Kessell, A E

    2011-07-01

    Disease surveillance is an integral part of most veterinary practices in Australia. The aim of this series of invited reviews is to facilitate the differential and ultimately definitive diagnosis of some of the previously known, as well as the novel and emerging, neurological disorders of ruminant livestock, which is of particular importance in the surveillance for transmissible spongiform encephalopathies. General principles of a systematic neurological examination, necropsy procedures and the neurological manifestations of systemic disease, trauma and neoplasia are described here.

  17. Molecular Imaging in Traditional Chinese Medicine Therapy for Neurological Diseases

    Zefeng Wang; Haitong Wan; Jinhui Li; Hong Zhang(Department of Physics and Center for Quantum Spacetime (CQUeST), Sogang University, 35 Baekbeom-ro, Mapo-gu, Seoul, 121-742 Korea); Mei Tian

    2013-01-01

    With the speeding tendency of aging society, human neurological disorders have posed an ever increasing threat to public health care. Human neurological diseases include ischemic brain injury, Alzheimer’s disease, Parkinson’s disease, and spinal cord injury, which are induced by impairment or specific degeneration of different types of neurons in central nervous system. Currently, there are no more effective treatments against these diseases. Traditional Chinese medicine (TCM) is focused on, ...

  18. Childhood central nervous system leukemia: historical perspectives, current therapy, and acute neurological sequelae

    Laningham, Fred H. [St. Jude Children' s Research Hospital, Division of Diagnostic Imaging, Department of Radiological Sciences, Memphis, TN (United States); University of Tennessee Health Sciences Center, Memphis, TN (United States); Kun, Larry E. [St. Jude Children' s Research Hospital, Division of Radiation Oncology, Department of Radiological Sciences, Memphis, TN (United States); University of Tennessee Health Sciences Center, Memphis, TN (United States); Reddick, Wilburn E.; Ogg, Robert J. [St. Jude Children' s Research Hospital, Division of Translational Imaging Research, Department of Radiological Sciences, Memphis, TN (United States); Morris, E.B. [St. Jude Children' s Research Hospital, Department of Oncology, Memphis, TN (United States); Pui, Ching-Hon [St. Jude Children' s Research Hospital, Department of Oncology, Memphis, TN (United States); University of Tennessee Health Sciences Center, Memphis, TN (United States)

    2007-11-15

    During the past three decades, improvements in the treatment of childhood leukemia have resulted in high cure rates, particularly for acute lymphoblastic leukemia (ALL). Unfortunately, successful therapy has come with a price, as significant morbidity can result from neurological affects which harm the brain and spinal cord. The expectation and hope is that chemotherapy, as a primary means of CNS therapy, will result in acceptable disease control with less CNS morbidity than has been observed with combinations of chemotherapy and radiotherapy over the past several decades. In this review we discuss the poignant, historical aspects of CNS leukemia therapy, outline current methods of systemic and CNS leukemia therapy, and present imaging findings we have encountered in childhood leukemia patients with a variety of acute neurological conditions. A major objective of our research is to understand the neuroimaging correlates of acute and chronic effects of cancer and therapy. Specific features related to CNS leukemia and associated short-term toxicities, both disease- and therapy-related, are emphasized in this review with the specific neuroimaging findings. Specific CNS findings are similarly important when treating acute myelogenous leukemia (AML), and details of leukemic involvement and toxicities are also presented in this entity. Despite contemporary treatment approaches which favor the use of chemotherapy (including intrathecal therapy) over radiotherapy in the treatment of CNS leukemia, children still occasionally experience morbid neurotoxicity. Standard neuroimaging is sufficient to identify a variety of neurotoxic sequelae in children, and often suggest specific etiologies. Specific neuroimaging findings frequently indicate a need to alter antileukemia therapy. It is important to appreciate that intrathecal and high doses of systemic chemotherapy are not innocuous and are associated with acute, specific, recognizable, and often serious neurological

  19. Outcome of carotid endarterectomy for acute neurological deficit.

    Mussa, Firas F; Aaronson, Nicole; Lamparello, Patrick J; Maldonado, Thomas S; Cayne, Neal S; Adelman, Mark A; Riles, Thomas S; Rockman, Caron B

    2009-01-01

    We reviewed our experience with urgent carotid intervention in the setting of acute neurological deficits. Between June 1992 and August 2008, a total of 3145 carotid endarterectomies (CEA) were performed. Twenty-seven patients (<1.0%) were categorized as urgent. The mean age was 74.1 years (range 56-93 years) with 16 (60%) men, and 11 (40%) women, Symptoms included extremity weakness or paralysis (n=13), amaurosis fugax (n=6), speech difficulty (n=2), and syncope, (n=3). Three patients exhibited a combination of these symptoms. Three open thrombectomy were performed. Regional anesthesia was used in 13 patients (52%). Seventeen patients (67%), required shunt placement. At 30-days, 2 patient (7%) suffered a stroke, and 1 (4%) died. Urgent CEA can be performed safely. A stroke rate of 7% is acceptable in those who may otherwise suffer a dismal outcome without intervention.

  20. Remote Physical Activity Monitoring in Neurological Disease: A Systematic Review.

    Valerie A J Block

    Full Text Available To perform a systematic review of studies using remote physical activity monitoring in neurological diseases, highlighting advances and determining gaps.Studies were systematically identified in PubMed/MEDLINE, CINAHL and SCOPUS from January 2004 to December 2014 that monitored physical activity for ≥24 hours in adults with neurological diseases. Studies that measured only involuntary motor activity (tremor, seizures, energy expenditure or sleep were excluded. Feasibility, findings, and protocols were examined.137 studies met inclusion criteria in multiple sclerosis (MS (61 studies; stroke (41; Parkinson's Disease (PD (20; dementia (11; traumatic brain injury (2 and ataxia (1. Physical activity levels measured by remote monitoring are consistently low in people with MS, stroke and dementia, and patterns of physical activity are altered in PD. In MS, decreased ambulatory activity assessed via remote monitoring is associated with greater disability and lower quality of life. In stroke, remote measures of upper limb function and ambulation are associated with functional recovery following rehabilitation and goal-directed interventions. In PD, remote monitoring may help to predict falls. In dementia, remote physical activity measures correlate with disease severity and can detect wandering.These studies show that remote physical activity monitoring is feasible in neurological diseases, including in people with moderate to severe neurological disability. Remote monitoring can be a psychometrically sound and responsive way to assess physical activity in neurological disease. Further research is needed to ensure these tools provide meaningful information in the context of specific neurological disorders and patterns of neurological disability.

  1. Molecular imaging in traditional Chinese medicine therapy for neurological diseases.

    Wang, Zefeng; Wan, Haitong; Li, Jinhui; Zhang, Hong; Tian, Mei

    2013-01-01

    With the speeding tendency of aging society, human neurological disorders have posed an ever increasing threat to public health care. Human neurological diseases include ischemic brain injury, Alzheimer's disease, Parkinson's disease, and spinal cord injury, which are induced by impairment or specific degeneration of different types of neurons in central nervous system. Currently, there are no more effective treatments against these diseases. Traditional Chinese medicine (TCM) is focused on, which can provide new strategies for the therapy in neurological disorders. TCM, including Chinese herb medicine, acupuncture, and other nonmedication therapies, has its unique therapies in treating neurological diseases. In order to improve the treatment of these disorders by optimizing strategies using TCM and evaluate the therapeutic effects, we have summarized molecular imaging, a new promising technology, to assess noninvasively disease specific in cellular and molecular levels of living models in vivo, that was applied in TCM therapy for neurological diseases. In this review, we mainly focus on applying diverse molecular imaging methodologies in different TCM therapies and monitoring neurological disease, and unveiling the mysteries of TCM.

  2. Yellow fever vaccine-associated neurological disease, a suspicious case.

    Beirão, Pedro; Pereira, Patrícia; Nunes, Andreia; Antunes, Pedro

    2017-03-02

    A 70-year-old man with known cardiovascular risk factors, presented with acute onset expression aphasia, agraphia, dyscalculia, right-left disorientation and finger agnosia, without fever or meningeal signs. Stroke was thought to be the cause, but cerebrovascular disease investigation was negative. Interviewing the family revealed he had undergone yellow fever vaccination 18 days before. Lumbar puncture revealed mild protein elevation. Cultural examinations, Coxiella burnetti, and neurotropic virus serologies were negative. Regarding the yellow fever virus, IgG was identified in serum and cerebrospinal fluid (CSF), with negative IgM and virus PCR in CSF. EEG showed an encephalopathic pattern. The patient improved gradually and a week after discharge was his usual self. Only criteria for suspect neurotropic disease were met, but it's possible the time spent between symptom onset and lumbar puncture prevented a definite diagnosis of yellow fever vaccine-associated neurological disease. This gap would have been smaller if the vaccination history had been collected earlier.

  3. Suicide and patients with neurologic diseases. Methodologic problems

    Stenager, E N; Stenager, Egon

    1992-01-01

    OBJECTIVE: The suicide risk in patients with many neurologic diseases has been reported to be greater than that in the general population. Studies on the subject are, however, often encumbered with methodologic problems. We appraised these problems and, based on an evaluation, reappraised knowledge...... of the suicide risk in patients with specific neurologic diseases. DATA SOURCE: Using the computerized database MEDLINE, we identified all published reports with the key words suicide, attempted suicide, and neurologic diseases. STUDY SELECTION: We assessed and reviewed studies concerning the most common...... neurologic diseases for methodologic problems in the study design. DATA EXTRACTION: The following methodologic problems emerged during our review: (1) choice of study type, ie, autopsy study or follow-up study; (2) choice of study population; (3) choice of control groups; (4) epidemiologic...

  4. KEGG DISEASE / Acute encephalitis [KEGG DISEASE

    Full Text Available DISEASE: H01417 Entry H01417Disease Name Acute encephalitis Description Acute encep...ns Infections caused by dsDNA viruses H01417Acute encephalitis Human diseases in ICD-10 classification [BR:b...of the central nervous system G04Encephalitis, myelitis and encephalomyelitis H01417Acute encephalitis Patho...elines for management. Journal Eur J Neurol 12:331-43 (2005) KEGG DISEASE / Acute encephalitis ...

  5. Interferon-gamma in progression to chronic demyelination and neurological deficit following acute EAE

    Renno, T; Taupin, V; Bourbonnière, L;

    1998-01-01

    The cytokine interferon-gamma (IFNgamma) is implicated in the induction of acute CNS inflammation, but it is less clear what role if any IFNgamma plays in progression to chronic demyelination and neurological deficit. To address this issue, we have expressed IFNgamma in myelinating oligodendrocytes....... In contrast to control mice, which remit from EAE with resolution of glial reactivity and leukocytic infiltration, transgenics showed chronic neurological deficits. While activated microglia/macrophages persisted in demyelinating lesions for over 100 days, CD4(+) T lymphocytes were no longer present in CNS....... IFNgamma therefore may play a role in chronic demyelination and long-term disability following the induction of demyelinating disease. Because IFNgamma may have neural as well as immune-infiltrating origins, these findings generate a new perspective on its role in the CNS....

  6. PET molecular imaging in stem cell therapy for neurological diseases

    Wang, Jiachuan; Zhang, Hong [Second Affiliated Hospital of Zhejiang University School of Medicine, Department of Nuclear Medicine, Hangzhou, Zhejiang (China); Zhejiang University, Medical PET Center, Hangzhou (China); Institute of Nuclear Medicine and Molecular Imaging of Zhejiang University, Hangzhou (China); Key Laboratory of Medical Molecular Imaging of Zhejiang Province, Hangzhou (China); Tian, Mei [University of Texas, M.D. Anderson Cancer Center, Department of Experimental Diagnostic Imaging, Houston, TX (United States)

    2011-10-15

    Human neurological diseases such as Alzheimer's disease, Parkinson's disease, Huntington's disease, spinal cord injury and multiple sclerosis are caused by loss of different types of neurons and glial cells in the brain and spinal cord. At present, there are no effective therapies against these disorders. Discovery of the therapeutic potential of stem cells offers new strategies for the treatment of neurological diseases. Direct assessment of stem cells' survival, interaction with the host and impact on neuronal functions after transplantation requires advanced in vivo imaging techniques. Positron emission tomography (PET) is a potential molecular imaging modality to evaluate the viability and function of transplanted tissue or stem cells in the nervous system. This review focuses on PET molecular imaging in stem cell therapy for neurological diseases. (orig.)

  7. Patient-Specific Pluripotent Stem Cells in Neurological Diseases

    Serpen Durnaoglu

    2011-01-01

    Full Text Available Many human neurological diseases are not currently curable and result in devastating neurologic sequelae. The increasing availability of induced pluripotent stem cells (iPSCs derived from adult human somatic cells provides new prospects for cellreplacement strategies and disease-related basic research in a broad spectrum of human neurologic diseases. Patient-specific iPSC-based modeling of neurogenetic and neurodegenerative diseases is an emerging efficient tool for in vitro modeling to understand disease and to screen for genes and drugs that modify the disease process. With the exponential increase in iPSC research in recent years, human iPSCs have been successfully derived with different technologies and from various cell types. Although there remain a great deal to learn about patient-specific iPSC safety, the reprogramming mechanisms, better ways to direct a specific reprogramming, ideal cell source for cellular grafts, and the mechanisms by which transplanted stem cells lead to an enhanced functional recovery and structural reorganization, the discovery of the therapeutic potential of iPSCs offers new opportunities for the treatment of incurable neurologic diseases. However, iPSC-based therapeutic strategies need to be thoroughly evaluated in preclinical animal models of neurological diseases before they can be applied in a clinical setting.

  8. Recent advances in metabolomics in neurological disease, and future perspectives.

    Zhang, Ai-hua; Sun, Hui; Wang, Xi-jun

    2013-10-01

    Discovery of clinically relevant biomarkers for diseases has revealed metabolomics has potential advantages that classical diagnostic approaches do not. The great asset of metabolomics is that it enables assessment of global metabolic profiles of biofluids and discovery of biomarkers distinguishing disease status, with the possibility of enhancing clinical diagnostics. Most current clinical chemistry tests rely on old technology, and are neither sensitive nor specific for a particular disease. Clinical diagnosis of major neurological disorders, for example Alzheimer's disease and Parkinson's disease, on the basis of current clinical criteria is unsatisfactory. Emerging metabolomics is a powerful technique for discovering novel biomarkers and biochemical pathways to improve diagnosis, and for determination of prognosis and therapy. Identifying multiple novel biomarkers for neurological diseases has been greatly enhanced with recent advances in metabolomics that are more accurate than routine clinical practice. Cerebrospinal fluid (CSF), which is known to be a rich source of small-molecule biomarkers for neurological and neurodegenerative diseases, and is in close contact with diseased areas in neurological disorders, could potentially be used for disease diagnosis. Metabolomics will drive CSF analysis, facilitate and improve the development of disease treatment, and result in great benefits to public health in the long-term. This review covers different aspects of CSF metabolomics and discusses their significance in the postgenomic era, emphasizing the potential importance of endogenous small-molecule metabolites in this emerging field.

  9. KEGG DISEASE / Acute alcohol sensitivity [KEGG DISEASE

    Full Text Available DISEASE: H01071 Entry H01071Disease Name Acute alcohol sensitivity Description Alde...bolism Congenital disorders of carbohydrate metabolism H01071Acute alcohol sensit...eases. Journal Cardiovasc Res 88:51-7 (2010) KEGG DISEASE / Acute alcohol sensitivity ...

  10. Human pluripotent stem cells: applications and challenges in neurological diseases

    Youssef eHIBAOUI

    2012-07-01

    Full Text Available The ability to generate human pluripotent stem cells (hPSCs holds great promise for the understanding and the treatment of human neurological diseases in modern medicine. The hPSCs are considered for their in vitro use as research tools to provide relevant cellular model for human diseases, drug discovery and toxicity assays and for their in vivo use in regenerative medicine applications. In this review, we highlight recent progress, promises and challenges of hPSC applications in human neurological disease modelling and therapies.

  11. Prediction and prognostication of neurological deterioration in patients with acute ICH

    Ovesen, Christian; Christensen, Anders Fogh; Havsteen, Inger;

    2015-01-01

    OBJECTIVE: Patients with intracerebral haemorrhage (ICH) are at high risk of neurological deterioration (ND). We aimed at establishing predictors of early ND (END) as well as late ND (LND) and at exploring the impact of neurological stability during the first week on long-term prognosis. DESIGN: We...... conducted this study as a retrospective cohort study. ND was evaluated based on the consciousness and severity of neurological symptoms. ND during the first 24 h after admission was defined as early ND and from 24 h to 7 days as LND. Patients were followed up until February 2015. PARTICIPANTS: We included...... 300 patients with acute ICH (≤4.5 h from symptom onset) who were admitted to our institution from March 2009 to January 2015. SETTING: Section of Acute Neurology, Department of Neurology, Bispebjerg Hospital is a specialised referral centre receiving patients with acute stroke from the entire capital...

  12. Awareness Status of Chronic Disabling Neurological Diseases among Elderly Veterans

    Ji-Ping Tan; Lin-Qi Zhu; Jun Zhang; Shi-Min Zhang; Xiao-Yang Lan; Bo Cui; Yu-Cheng Deng; Ying-Hao Li; Guang-Hua Ye; Lu-Ning Wang

    2015-01-01

    Background: The awareness, treatment and prevention of chronic diseases are generally poor among the elderly population of China, whereas the prevention and control of chronic diseases in elderly veteran communities have been ongoing for more than 30 years. Therefore, investigating the awareness status of chronic disabling neurological diseases (CDND) and common chronic diseases (CCD) among elderly veterans may provide references for related programs among the elderly in the general populatio...

  13. [Analysis of clinical pathway in changing and disabling neurological diseases].

    Cordesse, V; Jametal, T; Guy, C; Lefebvre, S; Roussel, M; Ruggeri, J; Schimmel, P; Holstein, J; Meininger, V

    2013-01-01

    Neurological diseases are characterized by the complexity of care and by a constant and changing disability. More and more frequently, their impact on the clinical pathway remains unknown. Seven postgraduate rehabilitation students (Master coordination du handicap, université Pierre-et-Marie-Curie, Paris) reconstructed the clinical pathway of 123 patients with various neurological diseases: multiple sclerosis, Alzheimer disease, amyotrophic lateral sclerosis, spinal trauma, Parkinson disease and brain tumors. There was a significant correlation between disease duration and the number of specialists involved in care, the number of prescribed drugs and the number of short-term hospitalizations; there was no correlation with age. This result suggests that with time an increasing number of complications related to the initial neurological disease developed. Hospitalization in rehabilitation units was highly correlated with the degree of disability and also with the help received by the patients during the course of their disease. This result suggests that these hospitalizations were a direct consequence of burn out among relatives. General practitioners (GP) were highly involved only during the initial part of the pathway, and their involvement rapidly declined thereafter, suggesting a probable relation with the specificities and the complexity of care for neurological diseases which induces a progressive transfer of responsibilities from the GP to the hospital. Social care was always incomplete and occurred too late during the course of the disease. The feeling by the patients that their care pathway was chaotic was highly correlated with the quality of the information given to the patient at the time of the announcement of their disease. This study confirms that cares for neurological diseases is highly specific and that expert centers and coordination networks are in a key position to ensure an efficient care pathway.

  14. Parkinson's disease between internal medicine and neurology.

    Csoti, Ilona; Jost, Wolfgang H; Reichmann, Heinz

    2016-01-01

    General medical problems and complications have a major impact on the quality of life in all stages of Parkinson's disease. To introduce an effective treatment, a comprehensive analysis of the various clinical symptoms must be undertaken. One must distinguish between (1) diseases which arise independently of Parkinson's disease, and (2) diseases which are a direct or indirect consequence of Parkinson's disease. Medical comorbidity may induce additional limitations to physical strength and coping strategies, and may thus restrict the efficacy of the physical therapy which is essential for treating hypokinetic-rigid symptoms. In selecting the appropriate medication for the treatment of any additional medical symptoms, which may arise, its limitations, contraindications and interactions with dopaminergic substances have to be taken into consideration. General medical symptoms and organ manifestations may also arise as a direct consequence of the autonomic dysfunction associated with Parkinson's disease. As the disease progresses, additional non-parkinsonian symptoms can be of concern. Furthermore, the side effects of Parkinson medications may necessitate the involvement of other medical specialists. In this review, we will discuss the various general medical aspects of Parkinson's disease.

  15. The role of cannabinoids and leptin in neurological diseases.

    Agar, E

    2015-12-01

    Cannabinoids exert a neuroprotective influence on some neurological diseases, including Alzheimer's, Parkinson's, Huntington's, multiple sclerosis and epilepsy. Synthetic cannabinoid receptor agonists/antagonists or compounds can provide symptom relief or control the progression of neurological diseases. However, the molecular mechanism and the effectiveness of these agents in controlling the progression of most of these diseases remain unclear. Cannabinoids may exert effects via a number of mechanisms and interactions with neurotransmitters, neurotropic factors and neuropeptides. Leptin is a peptide hormone involved in the regulation of food intake and energy balance via its actions on specific hypothalamic nuclei. Leptin receptors are widely expressed throughout the brain, especially in the hippocampus, basal ganglia, cortex and cerebellum. Leptin has also shown neuroprotective properties in a number of neurological disorders, such as Parkinson's and Alzheimer's. Therefore, cannabinoid and leptin hold therapeutic potential for neurological diseases. Further elucidation of the molecular mechanisms underlying the effects on these agents may lead to the development of new therapeutic strategies for the treatment of neurological disorders.

  16. Lyme disease: neurology, neurobiology, and behavior.

    Halperin, John J

    2014-05-01

    The Lyme disease controversy can be largely linked to the misconception that neurobehavioral effects of illness constitute evidence of nervous system infection. Appropriate differentiation between neuroborreliosis (nervous system Borrelia burgdorferi infection) and Lyme encephalopathy (altered nervous system function in individuals with systemic but not nervous system infection)-or encephalopathies of other etiologies-would lessen the controversy considerably, as the attribution of nonspecific symptoms to supposed ongoing central nervous system infection is a major factor perpetuating the debate. Epidemiologic considerations suggest that the entities referred to as "posttreatment Lyme disease" and "chronic Lyme disease" may not actually exist but rather reflect anchoring bias, linking common, nonspecific symptoms to an antecedent medical event. On the other hand, there are data suggesting possible mechanisms by which posttreatment Lyme disease could occur.

  17. Dynamic diseases in neurology and psychiatry

    Milton, John; Black, Deborah

    1995-03-01

    Thirty-two (32) periodic diseases of the nervous system are identified in which symptoms and/or signs recur. In 10/32, the recurrence of a symptom complex is one of the defining features of the illness, whereas in 22/32 oscillatory signs occur in the setting of an ongoing nervous system disorder. We discuss the possibility that these disorders may be dynamic diseases.

  18. Epigenetics and triplet repeat neurological diseases

    Sathiji eNageshwaran

    2015-12-01

    Full Text Available The term ‘junk DNA’ has been reconsidered following the delineation of the functional significance of repetitive DNA regions. Typically associated with centromeres and telomeres, DNA repeats are found in nearly all organisms throughout their genomes. Repetitive regions are frequently heterchromatinised resulting in silencing of intrinsic and nearby genes. However, this is not a uniform rule, with several genes known to require such an environment to permit transcription. Repetitive regions frequently exist as dinucleotide, trinucleotide and tetranucleotide repeats. The association between repetitive regions and disease was emphasised following the discovery of abnormal trinucleotide repeats underlying spinal and bulbar muscular atrophy (Kennedy’s disease and fragile X syndrome of mental retardation (FRAXA in 1991. In this review we provide a brief overview of epigenetic mechanisms and then focus on several diseases caused by DNA triplet-repeat expansions, which exhibit diverse epigenetic effects. It is clear that the emerging field of epigenetics is already generating novel potential therapeutic avenues for this group of largely incurable diseases.

  19. Obsessive–Compulsive Symptoms in Neurologic Disease: A Review

    M. S. George

    1992-01-01

    Full Text Available Obsessive–compulsive disorder (OCD is an increasingly recognized disorder with a prevalence of 2–3% (Robins et al., 1984. Once thought to be psychodynamic in origin, OCD is now generally recognized as having a neurobiological cause. Although the exact pathophysiology of OCD in its pure form remains unknown, there are numerous reports of obsessive–compulsive symptoms arising in the setting of known neurological disease. In this paper, we review the reported cases of obsessive–compulsive symptoms associated with neurologic diseases and outline the known facts about the underlying neurobiology of OCD. Finally, we synthesize these findings into a proposed theory of the pathophysiology of OCD, in both its pure form and when it accompanies other neurological illness.

  20. Which neurological diseases are most likely to be associated with "symptoms unexplained by organic disease".

    Stone, J; Carson, A; Duncan, R; Roberts, R; Coleman, R; Warlow, C; Murray, G; Pelosi, A; Cavanagh, J; Matthews, K; Goldbeck, R; Sharpe, M

    2012-01-01

    Many patients with a diagnosis of neurological disease, such as multiple sclerosis, have symptoms or disability that is considered to be in excess of what would be expected from that disease. We aimed to describe the overall and relative frequency of symptoms 'unexplained by organic disease' in patients attending general neurology clinics with a range of neurological disease diagnoses. Newly referred outpatients attending neurology clinics in all the NHS neurological centres in Scotland, UK were recruited over a period of 15 months. The assessing neurologists recorded their initial neurological diagnoses and also the degree to which they considered the patient's symptoms to be explained by organic disease. Patients completed self report scales for both physical and psychological symptoms. The frequency of symptoms unexplained by organic disease was determined for each category of neurological disease diagnoses. 3,781 patients participated (91% of those eligible). 2,467 patients had a diagnosis of a neurological disease (excluding headache disorders). 293 patients (12%) of these patients were rated as having symptoms only "somewhat" or "not at all" explained by that disease. These patients self-reported more physical and more psychological symptoms than those with more explained symptoms. No category of neurological disease was more likely than the others to be associated with such symptoms although patients with epilepsy had fewer. A substantial proportion of new outpatients with diagnoses of neurological disease also have symptoms regarded by the assessing neurologist as being unexplained by that disease; no single neurological disease category was more likely than others to be associated with this phenomenon.

  1. [Neurology].

    Sokolov, Arseny A; Rossetti, Andrea O; Michel, Patrik; Benninger, David; Nater, Bernard; Wider, Christian; Hirt, Lorenz; Kuntzer, Thierry; Démonet, Jean-François; Du Pasquier, Renaud A; Vingerhoets, François

    2016-01-13

    In 2015, cerebral stimulation becomes increasingly established in the treatment of pharmacoresistant epilepsy. Efficacy of endovascular treatment has been demonstrated for acute ischemic stroke. Deep brain stimulation at low frequency improves dysphagia and freezing of gait in Parkinson patients. Bimagrumab seems to increase muscular volume and force in patients with inclusion body myositis. In cluster-type headache, a transcutaneous vagal nerve stimulator is efficient in stopping acute attacks and also reducing their frequency. Initial steps have been undertaken towards modulating memory by stimulation of the proximal fornix. Teriflunomide is the first oral immunomodulatory drug for which efficacy has been shown in preventing conversion from clinical isolated syndrome to multiple sclerosis.

  2. Ethical clinical translation of stem cell interventions for neurologic disease

    Cote, David J; Bredenoord, Annelien L; Smith, Timothy R

    2017-01-01

    The application of stem cell transplants in clinical practice has increased in frequency in recent years. Many of the stem cell transplants in neurologic diseases, including stroke, Parkinson disease, spinal cord injury, and demyelinating diseases, are unproven-they have not been tested...... in prospective, controlled clinical trials and have not become accepted therapies. Stem cell transplant procedures currently being carried out have therapeutic aims, but are frequently experimental and unregulated, and could potentially put patients at risk. In some cases, patients undergoing such operations...... are not included in a clinical trial, and do not provide genuinely informed consent. For these reasons and others, some current stem cell interventions for neurologic diseases are ethically dubious and could jeopardize progress in the field. We provide discussion points for the evaluation of new stem cell...

  3. Olfaction in Neurologic and Neurodegenerative Diseases: A Literature Review

    Godoy, Maria Dantas Costa Lima

    2015-01-01

    Full Text Available Introduction Loss of smell is involved in various neurologic and neurodegenerative diseases, such as Parkinson disease and Alzheimer disease. However, the olfactory test is usually neglected by physicians at large. Objective The aim of this study was to review the current literature about the relationship between olfactory dysfunction and neurologic and neurodegenerative diseases. Data Synthesis Twenty-seven studies were selected for analysis, and the olfactory system, olfaction, and the association between the olfactory dysfunction and dementias were reviewed. Furthermore, is described an up to date in olfaction. Conclusion Otolaryngologist should remember the importance of olfaction evaluation in daily practice. Furthermore, neurologists and physicians in general should include olfactory tests in the screening of those at higher risk of dementia.

  4. New progress in brain aging and its related neurological diseases

    Ming-wei ZHU

    2014-03-01

    Full Text Available Brain aging-related neurological diseases including Alzheimer's disease (AD, Parkinson's disease (PD and cerebral amyloid angiopathy (CAA have become one of the major diseases endangering the health of old people in China. Although the mechanism of brain aging and pathogenesis of its related neurodegenerative diseases remain unclear, protein pathological studies such as tau, α-synuclein (α-Syn, TDP-43 and amyloid-β protein (Aβ based on brain tissue bank and case registration database are opening the door to solve the mystery in the brain aging process and unlock pathogenesis of aging-related neurodegenerative diseases. Research on functional neuroimaging including 11C-PIB PET and 18F-FDDNP PET in Alzheimer's disease and 18F-FDG PET in Parkinson's disease, and biomarkers such as total-tau, phosphorylated-tau, and the 42 amino acid fragment of β-amyloid in cerebrospinal fluid (CSF in the preclinical stages of Alzheimer's disease now become hot topics in the field of elderly dementia and movement disorders. Clinicopathological correlation research of Alzheimer's disease, Parkinson's disease and cerebral amyloid angiopathy is also one of focuses in the geriatric neurological diseases. doi: 10.3969/j.issn.1672-6731.2014.03.004

  5. The neurologic examination in patients with probable Alzheimer's disease.

    Huff, F J; Boller, F; Lucchelli, F; Querriera, R; Beyer, J; Belle, S

    1987-09-01

    Abnormal findings on a standardized neurologic examination were compared between patients with a clinical diagnosis of probable Alzheimer's disease (AD) and healthy control subjects. Aside from mental status findings, the most useful examination findings for differentiating AD from control subjects were the presence of release signs, olfactory deficit, impaired stereognosis or graphesthesia, gait disorder, tremor, and abnormalities on cerebellar testing. These abnormalities probably reflect the different areas of the central nervous system that are affected pathologically in AD. In the clinical diagnosis of AD, particular attention should be given to these aspects of the neurologic examination.

  6. Cerebrospinal fluid biomarkers in neurological diseases in children.

    Shahim, Pashtun; Månsson, Jan-Eric; Darin, Niklas; Zetterberg, Henrik; Mattsson, Niklas

    2013-01-01

    Analysis of cerebrospinal fluid (CSF) biomarkers is an integral part of neurology. Basic CSF biomarkers, such as CSF/serum albumin ratio and CSF cell counts, have been used to diagnose inflammatory and infectious CNS disorders in adults and children for decades. During recent years, however, numerous biomarkers for neuronal and astroglial injury, as well as disease-specific protein inclusions, have been developed for neurodegenerative disorders in adults. The overall aim of this paper is to give an updated overview of some of these biomarkers with special focus on their possible relevance to neurological disorders in children and adolescents.

  7. Lineage 2 west nile virus as cause of fatal neurologic disease in horses, South Africa.

    Venter, Marietjie; Human, Stacey; Zaayman, Dewald; Gerdes, Gertruida H; Williams, June; Steyl, Johan; Leman, Patricia A; Paweska, Janusz Tadeusz; Setzkorn, Hildegard; Rous, Gavin; Murray, Sue; Parker, Rissa; Donnellan, Cynthia; Swanepoel, Robert

    2009-06-01

    Serologic evidence suggests that West Nile virus (WNV) is widely distributed in horses in southern Africa. However, because few neurologic cases have been reported, endemic lineage 2 strains were postulated to be nonpathogenic in horses. Recent evidence suggests that highly neuroinvasive lineage 2 strains exist in humans and mice. To determine whether neurologic cases are being missed in South Africa, we tested 80 serum or brain specimens from horses with unexplained fever (n = 48) and/or neurologic signs (n = 32) for WNV. From March 2007 through June 2008, using reverse transcription-PCR (RT-PCR) and immunoglobulin (Ig) M ELISA, we found WNV RNA or IgM in 7/32 horses with acute neurologic disease; 5 horses died or were euthanized. In 5/7 horses, no other pathogen was detected. DNA sequencing for all 5 RT-PCR-positive cases showed the virus belonged to lineage 2. WNV lineage 2 may cause neurologic disease in horses in South Africa.

  8. Epigenetic mechanisms of gene expression regulation in neurological diseases.

    Gos, Monika

    2013-01-01

    Neurological diseases are a heterogenous group of disorders that are related to alterations in nervous system function. The genetic background of neurological diseases is heterogenous and may include chromosomal aberrations, specific gene mutations and epigenetic defects. This review is aimed at presenting of selected diseases that are associated with different epigenetic alterations. The imprinting defects on chromosome 15 are the cause of Prader-Willi and Angelman syndromes that both are characterized by intellectual disability, developmental delay and specific behavioral phenotype. Besides the imprinting defect, these diseases can also be caused by deletion of chromosome 15 or uniparental disomy. Aberrant epigenetic regulation is also specific for Fragile X syndrome that is caused by expansion of CGG repeats in the FMR1 gene that leads to global methylation of the promoter region and repression of FMR1 transcription. A number of neurological diseases, mainly associated with intellectual impairment, may be caused by mutations in genes encoding proteins involved in epigenetic regulation. The number of such diseases is rapidly growing thanks to the implementation of genomic sequencing for the identification of their molecular causes. One of the best known diseases linked to defects in epigenetic modifiers is Rett syndrome caused by a mutation in the MECP2 gene or its variant - Rett-like syndrome caused by a mutation in CDKL5 or FOXG1 genes. As the epigenetic signature is potentially reversible, much attention is focused on possible therapies with drugs that influence DNA or histone modifications. This is especially important in the case of neurological disorders in which epigenetic changes are observed as the effect of the disease.

  9. Biomarker discovery in neurological diseases: a metabolomic approach

    Afaf El-Ansary

    2009-12-01

    Full Text Available Afaf El-Ansary, Nouf Al-Afaleg, Yousra Al-YafaeeBiochemistry Department, Science College, King Saud University, Riyadh, Saudi ArabiaAbstract: Biomarkers are pharmacological and physiological measurements or specific biochemicals in the body that have a particular molecular feature that makes them useful for measuring the progress of disease or the effects of treatment. Due to the complexity of neurological disorders, it is very difficult to have perfect markers. Brain diseases require plenty of markers to reflect the metabolic impairment of different brain cells. The recent introduction of the metabolomic approach helps the study of neurological diseases based on profiling a multitude of biochemical components related to brain metabolism. This review is a trial to elucidate the possibility to use this approach to identify plasma metabolic markers related to neurological disorders. Previous trials using different metabolomic analyses including nuclear magnetic resonance spectroscopy, gas chromatography combined with mass spectrometry, liquid chromatography combined with mass spectrometry, and capillary electrophoresis will be traced.Keywords: metabolic biomarkers, neurological disorders. metabolome, nuclear magnetic resonance, mass spectrometry, chromatography

  10. Dysprosody nonassociated with neurological diseases--a case report.

    Pinto, José Antonio; Corso, Renato José; Guilherme, Ana Cláudia Rocha; Pinho, Sílvia Rebelo; Nóbrega, Monica de Oliveira

    2004-03-01

    Dysprosody also known as pseudo-foreign dialect, is the rarest neurological speech disorder. It is characterized by alterations in intensity, in the timing of utterance segments, and in rhythm, cadency, and intonation of words. The terms refers to changes as to duration, fundamental frequency, and intensity of tonic and atonic syllables of the sentences spoken, which deprive an individual's particular speech of its characteristics. The cause of this disease is usually associated with neurological pathologies such as brain vascular accidents, cranioencephalic traumatisms, and brain tumors. The authors report a case of dysprosody attended to at the Núcleo de Otorrinolaringologia e Cirurgia de Cabeça e Pescoço de São Paulo (NOSP). It is about a female patient with bilateral III degree Reinke's edema and normal neurological examinations that started presenting characteristics of the German dialect following a larynx microsurgery.

  11. Insomnia in central neurologic diseases--occurrence and management

    Mayer, Geert; Jennum, Poul; Riemann, Dieter

    2011-01-01

    The objective of this review is to highlight the impact of insomnia in central neurological disorders by providing information on its prevalence and give recommendations for diagnosis and treatment. Insomnia in neurological disorders is a frequent, but underestimated symptom. Its occurrence may...... of insomnia is primarily based on medical history and validated questionnaires. Actigraphy is a helpful diagnostic tool for assessing the circadian sleep-wake rhythm. For differential diagnosis and to measure the duration of sleep full polysomnography may be recommended. Prior to initiating treatment...... the cause of insomnia must be clearly identified. First line treatment aims at the underlying neurologic disease. The few high quality treatment studies show that short term treatment with hypnotics may be recommended in most disorders after having ruled out high risk for adverse effects. Sedating...

  12. Liver transplantation for hepatic and neurological Wilson's disease.

    Geissler, I; Heinemann, K; Rohm, S; Hauss, J; Lamesch, P

    2003-06-01

    Wilson's disease (WD) is an autosomal-recessive inherited disorder of copper metabolism characterized by excessive deposition of copper throughout the body. If medical treatment fails in cases of fulminant hepatic failure and progressive hepatic dysfunction due to advanced cirrhosis, liver transplantation (OLTx) has been demonstrated to be a valuable treatment option. Between December 1993 and December 2002, 225 OLTxs in 198 patients were performed in our institution. In this consecutive series six patients (three females and three males) were liver grafted for WD. The follow-up ranged from 3 to 7 years. All patients are alive with well-functioning grafts at present. The ceruloplasmin levels increased after transplantation and remained normal. The Kayser-Fleischer ring disappeared in all patients, and urinary copper excretion normalized. The neurological manifestations in the two patients with severe neurological symptoms showed after 2 to 5 years a downward tendency; in one the ataxic movements disappeared completely. The psychiatric disorder in one patient disappeared as well the mild neurological symptoms in the patient with CHILD A cirrhosis. These two patients are fully recovered and returned to work. OLTx should be considered as a treatment option in patients with severe progressive neurological deficits even in cases with stable liver function since liver grafting definitely cures the underlying biochemical defect. In such cases an early decision for liver transplantation is justified because neurological deficits may become irreversible.

  13. Comorbid diseases at patients with HIV-induced neurological disorders

    Sholomova E.l.

    2016-09-01

    Full Text Available Objective: to estimate the structure and frequency of detection of secondary diseases in patients with neurological manifestations of HIV infection. Materials and methods. The study involved 304 patients infected with HIV. Results. The defeat of the nervous system in HIV infection occur encephalopathy, cerebral vascular lesions, meningitis, subacute encephalitis, secondary CNS lesions. The number of CD4-lymphocytes in HIV-infected patients with neurological disorders was significantly lower. Most of them have comorbid diseases. The most commonly diagnosed hepatitis С and B, herpes, cytomegalovirus infection, chlamydia, Candida, toxoplasmosis and tuberculosis, mixed infection. Hepatitis В and С and herpes are the most widely represented in patients with HIV-induced encephalopathy and cerebrovascular form of HIV. The presence of cytomegalovirus infection is correlated with the development of subacute encephalitis. Conclusion. Manifestations of nervous system pathology in HIV polymorphic and correlated with the presence of secondary comorbid pathology. Such conditions are due to underlying disease immunological parameters.

  14. Epigenetic mechanisms in neurological diseases: genes, syndromes, and therapies.

    Urdinguio, Rocio G; Sanchez-Mut, Jose V; Esteller, Manel

    2009-11-01

    Epigenetic mechanisms such as DNA methylation and modifications to histone proteins regulate high-order DNA structure and gene expression. Aberrant epigenetic mechanisms are involved in the development of many diseases, including cancer. The neurological disorder most intensely studied with regard to epigenetic changes is Rett syndrome; patients with Rett syndrome have neurodevelopmental defects associated with mutations in MeCP2, which encodes the methyl CpG binding protein 2, that binds to methylated DNA. Other mental retardation disorders are also linked to the disruption of genes involved in epigenetic mechanisms; such disorders include alpha thalassaemia/mental retardation X-linked syndrome, Rubinstein-Taybi syndrome, and Coffin-Lowry syndrome. Moreover, aberrant DNA methylation and histone modification profiles of discrete DNA sequences, and those at a genome-wide level, have just begun to be described for neurodegenerative disorders such as Alzheimer's disease, Parkinson's disease, and Huntington's disease, and in other neurological disorders such as multiple sclerosis, epilepsy, and amyotrophic lateral sclerosis. In this Review, we describe epigenetic changes present in neurological diseases and discuss the therapeutic potential of epigenetic drugs, such as histone deacetylase inhibitors.

  15. Ultrasound treatment of neurological diseases--current and emerging applications.

    Leinenga, Gerhard; Langton, Christian; Nisbet, Rebecca; Götz, Jürgen

    2016-03-01

    Like cardiovascular disease and cancer, neurological disorders present an increasing challenge for an ageing population. Whereas nonpharmacological procedures are routine for eliminating cancer tissue or opening a blocked artery, the focus in neurological disease remains on pharmacological interventions. Setbacks in clinical trials and the obstacle of access to the brain for drug delivery and surgery have highlighted the potential for therapeutic use of ultrasound in neurological diseases, and the technology has proved useful for inducing focused lesions, clearing protein aggregates, facilitating drug uptake, and modulating neuronal function. In this Review, we discuss milestones in the development of therapeutic ultrasound, from the first steps in the 1950s to recent improvements in technology. We provide an overview of the principles of diagnostic and therapeutic ultrasound, for surgery and transient opening of the blood-brain barrier, and its application in clinical trials of stroke, Parkinson disease and chronic pain. We discuss the promising outcomes of safety and feasibility studies in preclinical models, including rodents, pigs and macaques, and efficacy studies in models of Alzheimer disease. We also consider the challenges faced on the road to clinical translation.

  16. Neurologic and psychiatric manifestations of celiac disease and gluten sensitivity.

    Jackson, Jessica R; Eaton, William W; Cascella, Nicola G; Fasano, Alessio; Kelly, Deanna L

    2012-03-01

    Celiac Disease (CD) is an immune-mediated disease dependent on gluten (a protein present in wheat, rye or barley) that occurs in about 1% of the population and is generally characterized by gastrointestinal complaints. More recently the understanding and knowledge of gluten sensitivity (GS), has emerged as an illness distinct from celiac disease with an estimated prevalence 6 times that of CD. Gluten sensitive people do not have villous atrophy or antibodies that are present in celiac disease, but rather they can test positive for antibodies to gliadin. Both CD and GS may present with a variety of neurologic and psychiatric co-morbidities, however, extraintestinal symptoms may be the prime presentation in those with GS. However, gluten sensitivity remains undertreated and underrecognized as a contributing factor to psychiatric and neurologic manifestations. This review focuses on neurologic and psychiatric manifestations implicated with gluten sensitivity, reviews the emergence of gluten sensitivity distinct from celiac disease, and summarizes the potential mechanisms related to this immune reaction.

  17. Cyclodextrins, blood-brain barrier, and treatment of neurological diseases.

    Vecsernyés, Miklós; Fenyvesi, Ferenc; Bácskay, Ildikó; Deli, Mária A; Szente, Lajos; Fenyvesi, Éva

    2014-11-01

    Biological barriers are the main defense systems of the homeostasis of the organism and protected organs. The blood-brain barrier (BBB), formed by the endothelial cells of brain capillaries, not only provides nutrients and protection to the central nervous system but also restricts the entry of drugs, emphasizing its importance in the treatment of neurological diseases. Cyclodextrins are increasingly used in human pharmacotherapy. Due to their favorable profile to form hydrophilic inclusion complexes with poorly soluble active pharmaceutical ingredients, they are present as excipients in many marketed drugs. Application of cyclodextrins is widespread in formulations for oral, parenteral, nasal, pulmonary, and skin delivery of drugs. Experimental and clinical data suggest that cyclodextrins can be used not only as excipients for centrally acting marketed drugs like antiepileptics, but also as active pharmaceutical ingredients to treat neurological diseases. Hydroxypropyl-β-cyclodextrin received orphan drug designation for the treatment of Niemann-Pick type C disease. In addition to this rare lysosomal storage disease with neurological symptoms, experimental research revealed the potential therapeutic use of cyclodextrins and cyclodextrin nanoparticles in neurodegenerative diseases, stroke, neuroinfections and brain tumors. In this context, the biological effects of cyclodextrins, their interaction with plasma membranes and extraction of different lipids are highly relevant at the level of the BBB.

  18. Blood levels of glial fibrillary acidic protein (GFAP in patients with neurological diseases.

    Christoph A Mayer

    Full Text Available BACKGROUND AND PURPOSE: The brain-specific astroglial protein GFAP is a blood biomarker candidate indicative of intracerebral hemorrhage in patients with symptoms suspicious of acute stroke. Comparably little, however, is known about GFAP release in other neurological disorders. In order to identify potential "specificity gaps" of a future GFAP test used to diagnose intracerebral hemorrhage, we measured GFAP in the blood of a large and rather unselected collective of patients with neurological diseases. METHODS: Within a one-year period, we randomly selected in-patients of our university hospital for study inclusion. Patients with ischemic stroke, transient ischemic attack and intracerebral hemorrhage were excluded. Primary endpoint was the ICD-10 coded diagnosis reached at discharge. During hospital stay, blood was collected, and GFAP plasma levels were determined using an advanced prototype immunoassay at Roche Diagnostics. RESULTS: A total of 331 patients were included, covering a broad spectrum of neurological diseases. GFAP levels were low in the vast majority of patients, with 98.5% of cases lying below the cut-off that was previously defined for the differentiation of intracerebral hemorrhage and ischemic stroke. No diagnosis or group of diagnoses was identified that showed consistently increased GFAP values. No association with age and sex was found. CONCLUSION: Most acute and chronic neurological diseases, including typical stroke mimics, are not associated with detectable GFAP levels in the bloodstream. Our findings underline the hypothesis that rapid astroglial destruction as in acute intracerebral hemorrhage is mandatory for GFAP increase. A future GFAP blood test applied to identify patients with intracerebral hemorrhage is likely to have a high specificity.

  19. Central Venous Line and Acute Neurological Deficit: A Case Series

    SeyedHossein Ahmadi

    2015-10-01

    Full Text Available Central venous catheter (CVC insertion is a practical way to assess patients hemodynamic specially in cardiovascular surgery but this relatively simple junior level procedure is not risk free and its common reported complications include; pneumothorax, hydrothorax, hemothorax, local hematoma, cardiac tamponade, vascular injury, thrombosis, embolism, and catheter disruption. Here in this article we are going to present 6 patients with very unusual presentation of CVC complication which was neurological deficit presented by agitation , unconsciousness, disorientation to time and place and  hemiparesis. All patients undergone neurologic consult and brain computed tomography. Final diagnosis was brain ischemic damage and finally we kept them on conservative management; fortunately we did not have any permanent damage.

  20. [The rational immunotherapy with intravenous immunoglobulin in neurologic diseases].

    Korsak, Jolanta

    2011-06-01

    Intravenous immunoglobulins (IVIG) have been used mainly as a supplement therapy in hypogammglobulinemia patients. IVIG have been proved effective in treating TTP and recently they have been used in treating many autoimmune and inflammatory diseases. In neurologic diseases IVIG have immunomodulating features dependent on IgG Fc-fragment. Which diseases benefit from the use of IVIG was the find from controlled clinical trials in accordance with Evidence Based Medicine. IVIG have been proved effective in Guillaine-Barre syndrom, chronic inflammatory demyelinating polyradiculoneuropathy, miastenia, dermathomyositis and sclerosis multiple. The recommendations include the frequency of IVIG administering its dosage and duration of treatment.

  1. Node of Ranvier Disruption as a Cause of Neurological Diseases

    Keiichiro Susuki

    2013-07-01

    Full Text Available Dysfunction and/or disruption of nodes of Ranvier are now recognized as key contributors to the pathophysiology of various neurological diseases. One reason is that the excitable nodal axolemma contains a high density of Nav (voltage-gated Na+ channels that are required for the rapid and efficient saltatory conduction of action potentials. Nodal physiology is disturbed by altered function, localization, and expression of voltage-gated ion channels clustered at nodes and juxtaparanodes, and by disrupted axon–glial interactions at paranodes. This paper reviews recent discoveries in molecular/cellular neuroscience, genetics, immunology, and neurology that highlight the critical roles of nodes of Ranvier in health and disease.

  2. Ionotropic GABA and Glutamate Receptor Mutations and Human Neurologic Diseases.

    Yuan, Hongjie; Low, Chian-Ming; Moody, Olivia A; Jenkins, Andrew; Traynelis, Stephen F

    2015-07-01

    The advent of whole exome/genome sequencing and the technology-driven reduction in the cost of next-generation sequencing as well as the introduction of diagnostic-targeted sequencing chips have resulted in an unprecedented volume of data directly linking patient genomic variability to disorders of the brain. This information has the potential to transform our understanding of neurologic disorders by improving diagnoses, illuminating the molecular heterogeneity underlying diseases, and identifying new targets for therapeutic treatment. There is a strong history of mutations in GABA receptor genes being involved in neurologic diseases, particularly the epilepsies. In addition, a substantial number of variants and mutations have been found in GABA receptor genes in patients with autism, schizophrenia, and addiction, suggesting potential links between the GABA receptors and these conditions. A new and unexpected outcome from sequencing efforts has been the surprising number of mutations found in glutamate receptor subunits, with the GRIN2A gene encoding the GluN2A N-methyl-d-aspartate receptor subunit being most often affected. These mutations are associated with multiple neurologic conditions, for which seizure disorders comprise the largest group. The GluN2A subunit appears to be a locus for epilepsy, which holds important therapeutic implications. Virtually all α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor mutations, most of which occur within GRIA3, are from patients with intellectual disabilities, suggesting a link to this condition. Similarly, the most common phenotype for kainate receptor variants is intellectual disability. Herein, we summarize the current understanding of disease-associated mutations in ionotropic GABA and glutamate receptor families, and discuss implications regarding the identification of human mutations and treatment of neurologic diseases.

  3. [Features of neurologic semiotics at chronic obstructive pulmonary disease].

    Litvinenko, I V; Baranov, V L; Kolcheva, Iu A

    2011-01-01

    Chronic obstructive pulmonary disease (COPD) is actual pathology, when it forms the mixed hypoxemia. In the conditions of a chronic hypoxemia structures of organism with high level of metabolic processes, namely brain tissues, suffer. Character of defeat of the central nervous system at that pathology is insufficiently studied. In this article we studied and analysed the presence of such changes as depression, anxiety, cognitive impairment and features of neurologic semiotics at COPD in 50 patients.

  4. Cerebellar transcranial direct current stimulation in neurological disease

    Ferrucci, Roberta; Bocci, Tommaso; Cortese, Francesca; Ruggiero, Fabiana; Priori, Alberto

    2016-01-01

    Several studies have highlighted the therapeutic potential of transcranial direct current stimulation (tDCS) in patients with neurological diseases, including dementia, epilepsy, post-stroke dysfunctions, movement disorders, and other pathological conditions. Because of this technique’s ability to modify cerebellar excitability without significant side effects, cerebellar tDCS is a new, interesting, and powerful tool to induce plastic modifications in the cerebellum. In this report, we review...

  5. [Neurological manifestations of Behçet's disease].

    Noel, N; Drier, A; Wechsler, B; Piette, J-C; De Paz, R; Dormont, D; Cacoub, P; Saadoun, D

    2014-02-01

    Neurological manifestations of Behçet's disease (BD) occur in 5.3 to more than 50% of patients. They are divided into two major forms: "parenchymal" lesions, which include mainly meningoencephalitis as opposed to "extra-parenchymal" lesions (i.e. cerebral venous thrombosis and arterial aneurysms). Myelitis or peripheral neuropathy is exceptional. The neuro-Behçet syndrome (NBS) should be considered in the setting of neurological manifestations, particularly headache and pyramidal signs, in a young man diagnosed with BD. However, its recognition may be difficult when neurological manifestations are the presenting features of BD (one third of cases), and requires a thorough knowledge of clinical manifestations and morphological lesions. Thus, parenchymal NB lesions classically exhibit inflammatory characteristics on MRI and are located at the meso-diencephalic junction and in the brainstem, rarely with a supratentorial extension. Meningitis is not systematically associated, and may be absent in about 30% of cases. The pathogenesis of these lesions is incompletely understood, but inflammatory infiltrates include mainly neutrophils and activated T cells (mainly Th17). Differential diagnoses include infectious diseases (herpes, listeria, tuberculosis), and inflammatory diseases (i.e. multiple sclerosis and sarcoidosis). A prompt recognition of NBS should lead to initiate adequate therapies in order to limit the risk of sequelae, relapses or death.

  6. Spasmodic dysphonia: description of the disease and associated neurologic disorders

    Coelho, Marina Serrato

    2010-06-01

    Full Text Available Introduction: Spasmodic dysphonia (SD is a problem that affects speech and vocalization, one of the most devastating disorders of oral communication. It is characterized by vocal quality tensaestrangulada, harshly and / or interspersed with abrupt vocal attack and a great tension in the vocal tract. The etiology of spasmodic dysphonia is unclear. Some authors point to psychogenic causes, neurological or even unknown. Objective: To assess the prevalence of muscular dystonias and other neurological symptoms in patients with ED. Method: A retrospective study of 10 cases with diagnosis of ED for symptoms and neurological disorders associated. Results: There was a significant predominance of the disease in females (9:1. The average age of onset of symptoms was 32 years, ranging between 14 and 60 years. The mean disease duration was 10 years. Among the patients, 87.5% had a diagnosis of disorders of movement made by a neurologist, including orofacial dystonias (50%, essential tremor (50% and spastic paraparesis (12%. Conclusion: The presence of movement disorders followed almost all cases of spasmodic dysphonia. More studies are needed to clarify the pathophysiological basis of disease.

  7. Cell therapy: the final frontier for treatment of neurological diseases.

    Dutta, Susmita; Singh, Gurbind; Sreejith, Sailaja; Mamidi, Murali Krishna; Husin, Juani Mazmin; Datta, Indrani; Pal, Rajarshi; Das, Anjan Kumar

    2013-01-01

    Neurodegenerative diseases are devastating because they cause increasing loss of cognitive and physical functions and affect an estimated 1 billion individuals worldwide. Unfortunately, no drugs are currently available to halt their progression, except a few that are largely inadequate. This mandates the search of new treatments for these progressively degenerative diseases. Neural stem cells (NSCs) have been successfully isolated, propagated, and characterized from the adult brains of mammals, including humans. The confirmation that neurogenesis occurs in the adult brain via NSCs opens up fresh avenues for treating neurological problems. The proof-of-concept studies demonstrating the neural differentiation capacity of stem cells both in vitro and in vivo have raised widespread enthusiasm toward cell-based interventions. It is anticipated that cell-based neurogenic drugs may reverse or compensate for deficits associated with neurological diseases. The increasing interest of the private sector in using human stem cells in therapeutics is evidenced by launching of several collaborative clinical research activities between Pharma giants and research institutions or small start-up companies. In this review, we discuss the major developments that have taken place in this field to position stem cells as a prospective candidate drug for the treatment of neurological disorders.

  8. Neurological manifestations, diagnosis, and treatment of celiac disease: A comprehensive review

    Shahriar Nikpour

    2012-01-01

    Celiac disease or gluten sensitivity may initially present as one or more neurological signs and/or symptoms. On the other hand, it may be associated with or complicated by neurological manifestations. Neurological presentations are rare in children but as many as 36% of adult patients present with neurological changes. With severe malnutrition after progression of celiac disease, different vitamin deficiencies may develop. Such problems can in turn overlap with previous neurological abnormal...

  9. Understanding neurological disease mechanisms in the era of epigenetics.

    Qureshi, Irfan A; Mehler, Mark F

    2013-06-01

    The burgeoning field of epigenetics is making a significant impact on our understanding of brain evolution, development, and function. In fact, it is now clear that epigenetic mechanisms promote seminal neurobiological processes, ranging from neural stem cell maintenance and differentiation to learning and memory. At the molecular level, epigenetic mechanisms regulate the structure and activity of the genome in response to intracellular and environmental cues, including the deployment of cell type-specific gene networks and those underlying synaptic plasticity. Pharmacological and genetic manipulation of epigenetic factors can, in turn, induce remarkable changes in neural cell identity and cognitive and behavioral phenotypes. Not surprisingly, it is also becoming apparent that epigenetics is intimately involved in neurological disease pathogenesis. Herein, we highlight emerging paradigms for linking epigenetic machinery and processes with neurological disease states, including how (1) mutations in genes encoding epigenetic factors cause disease, (2) genetic variation in genes encoding epigenetic factors modify disease risk, (3) abnormalities in epigenetic factor expression, localization, or function are involved in disease pathophysiology, (4) epigenetic mechanisms regulate disease-associated genomic loci, gene products, and cellular pathways, and (5) differential epigenetic profiles are present in patient-derived central and peripheral tissues.

  10. Could a neurological disease be a part of Mozart's pathography?

    Ivkić, Goran; Erdeljić, Viktorija

    2011-01-01

    As expected, since we recently celebrated the 250th anniversary of birth of Wolfgang Amadeus Mozart, there has been again a renewal of interest in his short but intensive life, as well as in the true reason of his untimely dead. Mozart lived and died in time when the medical knowledge was based mostly on subjective observations, without the established basics of standardized medical terminology and methodology. This leaves a great space for hypothesizing about his health problems, as well as about the cause of his death. The medical academic community attributed to Mozart approximately 150 different medical diagnoses. There is much speculation on the possible causes of Mozart's death: uremia, infection, rheumatic fever, trichinellosis, etc. Recently some authors have raised the question about a possible concomitant neurological disease. According to available records, Mozart has shown some elements of cyclotimic disorder, epilepsy and Gilles de la Tourette syndrome. Furthermore, the finding of a temporal fracture on (allegedly) Mozart's skull, gives a way to speculations about the possibility of a chronic subdural hematoma and its compressive effect on the temporal lobe. Despite numerous theories on Mozart's pathography that also include a concomitant neurological disorder, the medical and history records about Mozart's health status indicate that he probably had suffered from an infective illness, followed most likely by the reactivation of rheumatic fever, which was followed by strong immunologic reaction in the last days of his life. Taking all the above into consideration, it is reasonably to conclude that Mozart's neurological disturbances were caused by the intensity of the infective disease, and not primarily by a neurological disease.

  11. Cerebrospinal fluid biomarkers of neurodegeneration in chronic neurological diseases.

    Tumani, Hayrettin; Teunissen, Charlotte; Süssmuth, Sigurd; Otto, Markus; Ludolph, Albert C; Brettschneider, Johannes

    2008-07-01

    Chronic neurological diseases (CND) like amyotrophic lateral sclerosis (ALS), dementia or multiple sclerosis (MS) share a chronic progressive course of disease that frequently leads to the common pathological pathway of neurodegeneration, including neuroaxonal damage, apoptosis and gliosis. There is an ongoing search for biomarkers that could support early diagnosis of CND and help to identify responders to interventions in therapeutic treatment trials. Cerebrospinal fluid (CSF) is a promising source of biomarkers in CND, since the CSF compartment is in close anatomical contact with the brain interstitial fluid, where biochemical changes related to CND are reflected. We review recent advances in CSF biomarkers research in CND and thereby focus on markers associated with neurodegeneration.

  12. Neurological Manifestations, Diagnosis, and Treatment of Celiac Disease: A Comprehensive Review

    Shahriar Nikpour

    2012-01-01

    Full Text Available Celiac disease or gluten sensitivity may initially present asone or more neurological signs and/or symptoms. On the other hand, it may be associated with or complicated by neurological manifestations. Neurological presentations are rare in children but as many as 36% of adult patients present with neurological changes. With severe malnutrition after progression of celiac disease, different vitamin deficiencies may develop. Such problems can in turn overlap with previous neurological abnormalities including ataxia,epilepsy, neuropathy, dementia, and cognitive disorders. Inthis study, we aimed to review the neurological aspects of celiac disease. Early diagnosis and treatment could prevent related disability in patients with celiac disease.

  13. Insomnia in central neurologic diseases--occurrence and management

    Mayer, Geert; Jennum, Poul; Riemann, Dieter

    2011-01-01

    The objective of this review is to highlight the impact of insomnia in central neurological disorders by providing information on its prevalence and give recommendations for diagnosis and treatment. Insomnia in neurological disorders is a frequent, but underestimated symptom. Its occurrence may...... be a direct consequence of the disease itself or may be secondary to pain, depression, other sleep disorders or the effects of medications. Insomnia can have a significant impact on the patient's cognitive and physical function and may be associated with psychological distress and depression. Diagnosis...... of insomnia is primarily based on medical history and validated questionnaires. Actigraphy is a helpful diagnostic tool for assessing the circadian sleep-wake rhythm. For differential diagnosis and to measure the duration of sleep full polysomnography may be recommended. Prior to initiating treatment...

  14. An overview of neurological and neuromuscular signs in mitochondrial diseases.

    Chaussenot, A; Paquis-Flucklinger, V

    2014-05-01

    Mitochondrial disorders have a broad clinical spectrum and are genetically heterogeneous, involving two genomes. These disorders may be develop at any age, with isolated or multiple system involvement, and any pattern of inheritance. Neurological involvement is the most frequent, and concerns muscular, peripheral and central nervous system. Among these diverse signs, some are suggestive of mitochondrial disease, such as progressive external ophthalmoplegia, exercise intolerance, psychomotor regression, stroke-like episodes, refractory epilepsy and Epilepsia Partialis Continua. Others are less specific and mitochondrial hypothesis may be evocated because of either association of different neuromuscular signs or a multisystemic involvement. This review describes the wealth of this neurological and neuromuscular symptomatology through different syndromes reported in the literature, according to preponderant signs and to modes of inheritance, as key elements to guide genetics testing.

  15. Complications of Immunosuppressive/Immunomodulatory Therapy in Neurological Diseases

    Nath, Avindra

    2016-01-01

    Opinion statement The first critical step in the appropriate treatment of neurological infectious disease accompanying immunosuppressive states or immunomodulatory medication is to properly identify the offending organism. Broadly immunosuppressive conditions will predispose to both common and uncommon infectious diseases. There are substantial differences between neurological infectious disorders complicating disturbances of the innate immunity (neutrophils, monocytes and macrophages) and those due to abnormal adaptive immunity (humoral and cellular immunity). Similarly, there are differences in the types of infections with impaired humoral immunity compared to disturbed cellular immunity and between T- and B-cell disorders. HIV/AIDS has been a model of acquired immunosuppression and the nature of opportunistic infections with which it has been associated has been well characterized and generally correlates well with the degree of CD4 lymphopenia. Increasingly, immunotherapies target specific components of the immune system, such as an adhesion molecule or its ligand or surface receptors on a special class of cells. These targeted perturbations of the immune system increase the risk of particular infectious diseases. For instance, natalizumab, an α4β1 integrin inhibitor that is highly effective in multiple sclerosis, increases the risk of progressive multifocal leukoencephalopathy for reasons that still remain unclear. It is likely that other therapies that result in a disruption of a specific component of the immune system will be associated with other unique opportunistic infections. The risk of multiple simultaneous neurological infections in the immunosuppressed host must always be considered, particularly with a failure to respond to a therapeutic regimen. With respect to appropriate and effective therapy, diagnostic accuracy assumes primacy, but occasionally broad spectrum therapy is necessitated. For a number of opportunistic infectious disorders

  16. Uncommon acute neurologic presentation of canine distemper in 4 adult dogs.

    Galán, Alba; Gamito, Araceli; Carletti, Beatrice E; Guisado, Alicia; de las Mulas, Juana Martín; Pérez, José; Martín, Eva M

    2014-04-01

    Four uncommon cases of canine distemper (CD) were diagnosed in vaccinated adult dogs. All dogs had acute onset of neurologic signs, including seizures, abnormal mentation, ataxia, and proprioceptive deficits. Polymerase chain reaction for CD virus was positive on cerebrospinal fluid in 2 cases. Due to rapid deterioration the dogs were euthanized and CD was confirmed by postmortem examination.

  17. Emerging Links between Homeostatic Synaptic Plasticity and Neurological Disease

    Dion eDickman

    2013-11-01

    Full Text Available Homeostatic signaling systems are ubiquitous forms of biological regulation, having been studied for hundreds of years in the context of diverse physiological processes including body temperature and osmotic balance. However, only recently has this concept been brought to the study of excitatory and inhibitory electrical activity that the nervous system uses to establish and maintain stable communication. Synapses are a primary target of neuronal regulation with a variety of studies over the past 15 years demonstrating that these cellular junctions are under bidirectional homeostatic control. Recent work from an array of diverse systems and approaches has revealed exciting new links between homeostatic synaptic plasticity and a variety of seemingly disparate neurological and psychiatric diseases. These include autism spectrum disorders, intellectual disabilities, schizophrenia, and Fragile X Syndrome. Although the molecular mechanisms through which defective homeostatic signaling may lead to disease pathogenesis remain unclear, rapid progress is likely to be made in the coming years using a powerful combination of genetic, imaging, electrophysiological, and next generation sequencing approaches. Importantly, understanding homeostatic synaptic plasticity at a cellular and molecular level may lead to developments in new therapeutic innovations to treat these diseases. In this review we will examine recent studies that demonstrate homeostatic control of postsynaptic protein translation, retrograde signaling, and presynaptic function that may contribute to the etiology of complex neurological and psychiatric diseases.

  18. NMDARs in neurological diseases: a potential therapeutic target.

    Gonzalez, Janneth; Jurado-Coronel, Juan Camilo; Ávila, Marcos Fidel; Sabogal, Angélica; Capani, Francisco; Barreto, George E

    2015-05-01

    N-methyl-D-aspartate ionotropic glutamate receptor (NMDARs) is a ligand-gated ion channel that plays a critical role in excitatory neurotransmission, brain development, synaptic plasticity associated with memory formation, central sensitization during persistent pain, excitotoxicity and neurodegenerative diseases in the central nervous system (CNS). Within iGluRs, NMDA receptors have been the most actively investigated for their role in neurological diseases, especially neurodegenerative pathologies such as Alzheimer's and Parkinson's diseases. It has been demonstrated that excessive activation of NMDA receptors (NMDARs) plays a key role in mediating some aspects of synaptic dysfunction in several CNS disorders, so extensive research has been directed on the discovery of compounds that are able to reduce NMDARs activity. This review discusses the role of NMDARs on neurological pathologies and the possible therapeutic use of agents that target this receptor. Additionally, we delve into the role of NMDARs in Alzheimer's and Parkinson's diseases and the receptor antagonists that have been tested on in vivo models of these pathologies. Finally, we put into consideration the importance of antioxidants to counteract oxidative capacity of the signaling cascade in which NMDARs are involved.

  19. Emerging links between homeostatic synaptic plasticity and neurological disease.

    Wondolowski, Joyce; Dickman, Dion

    2013-11-21

    Homeostatic signaling systems are ubiquitous forms of biological regulation, having been studied for hundreds of years in the context of diverse physiological processes including body temperature and osmotic balance. However, only recently has this concept been brought to the study of excitatory and inhibitory electrical activity that the nervous system uses to establish and maintain stable communication. Synapses are a primary target of neuronal regulation with a variety of studies over the past 15 years demonstrating that these cellular junctions are under bidirectional homeostatic control. Recent work from an array of diverse systems and approaches has revealed exciting new links between homeostatic synaptic plasticity and a variety of seemingly disparate neurological and psychiatric diseases. These include autism spectrum disorders, intellectual disabilities, schizophrenia, and Fragile X Syndrome. Although the molecular mechanisms through which defective homeostatic signaling may lead to disease pathogenesis remain unclear, rapid progress is likely to be made in the coming years using a powerful combination of genetic, imaging, electrophysiological, and next generation sequencing approaches. Importantly, understanding homeostatic synaptic plasticity at a cellular and molecular level may lead to developments in new therapeutic innovations to treat these diseases. In this review we will examine recent studies that demonstrate homeostatic control of postsynaptic protein translation, retrograde signaling, and presynaptic function that may contribute to the etiology of complex neurological and psychiatric diseases.

  20. The saccadic and neurological deficits in type 3 Gaucher disease.

    William Benko

    Full Text Available Our objective was to characterize the saccadic eye movements in patients with type 3 Gaucher disease (chronic neuronopathic in relationship to neurological and neurophysiological abnormalities. For approximately 4 years, we prospectively followed a cohort of 15 patients with Gaucher type 3, ages 8-28 years, by measuring saccadic eye movements using the scleral search coil method. We found that patients with type 3 Gaucher disease had a significantly higher regression slope of duration vs amplitude and peak duration vs amplitude compared to healthy controls for both horizontal and vertical saccades. Saccadic latency was significantly increased for horizontal saccades only. Downward saccades were more affected than upward saccades. Saccade abnormalities increased over time in some patients reflecting the slowly progressive nature of the disease. Phase plane plots showed individually characteristic patterns of abnormal saccade trajectories. Oculo-manual dexterity scores on the Purdue Pegboard test were low in virtually all patients, even in those with normal cognitive function. Vertical saccade peak duration vs amplitude slope significantly correlated with IQ and with the performance on the Purdue Pegboard but not with the brainstem and somatosensory evoked potentials. We conclude that, in patients with Gaucher disease type 3, saccadic eye movements and oculo-manual dexterity are representative neurological functions for longitudinal studies and can probably be used as endpoints for therapeutic clinical trials.ClinicalTrials.gov NCT00001289.

  1. Scavenger receptor b2 as a receptor for hand, foot, and mouth disease and severe neurological diseases.

    Yamayoshi, Seiya; Fujii, Ken; Koike, Satoshi

    2012-01-01

    Enterovirus 71 (EV71) is one of the major causative agents of hand, foot, and mouth disease (HFMD). Infection with EV71 is occasionally associated with severe neurological diseases such as acute encephalitis, acute flaccid paralysis, and cardiopulmonary failure. Because cellular receptors for viruses play an important role in cell, tissue, and species tropism, it is important to identify and characterize the receptor molecule. Recently, cellular receptors and host factors that stimulate EV71 infection have been identified. Several lines of evidence suggest that scavenger receptor class B, member 2 (SCARB2) plays critical roles in efficient EV71 infection and the development of disease in humans. In this review, we will summarize the findings of recent studies on EV71 infection and on the roles of SCARB2.

  2. Shiga toxin Mediated Neurologic Changes in Murine Model of Disease.

    Suman Pradhan

    2016-09-01

    Full Text Available Seizures and neurologic involvement have been reported in patients infected with Shiga toxin (Stx producing E. coli, and hemolytic uremic syndrome (HUS with neurologic involvement is associated with more severe outcome. We investigated the extent of renal and neurologic damage in mice following injection of the highly potent form of Stx, Stx2a, and less potent Stx1. As observed in previous studies, Stx2a brought about moderate to acute tubular necrosis of proximal and distal tubules in the kidneys. Brain sections stained with hematoxylin and eosin (H&E appeared normal, although some red blood cell congestion was observed. Microglial cell responses to neural injury include up-regulation of surface-marker expression (e.g. Iba1 and stereotypical morphological changes. Mice injected with Stx2a showed increased Iba1 staining, mild morphological changes associated with microglial activation (thickening of processes, and increased microglial staining per unit area. Microglial changes were observed in the cortex, hippocampus, and amygdala regions, but not the nucleus. Magnetic resonance imaging (MRI of Stx2a-treated mice revealed no hyper-intensities in the brain, although magnetic resonance spectroscopy (MRS revealed significantly decreased levels of phosphocreatine in the thalamus. Less dramatic changes were observed following Stx1 challenge. Neither immortalized microvascular endothelial cells from the cerebral cortex of mice (bEnd.3 nor primary human brain microvascular endothelial cells were found to be susceptible to Stx1 or Stx2a. The lack of susceptibility to Stx for both cell types correlated with an absence of receptor expression. These studies indicate Stx causes subtle, but identifiable changes in the mouse brain.

  3. Shiga Toxin Mediated Neurologic Changes in Murine Model of Disease

    Pradhan, Suman; Pellino, Christine; MacMaster, Kayleigh; Coyle, Dennis; Weiss, Alison A.

    2016-01-01

    Seizures and neurologic involvement have been reported in patients infected with Shiga toxin (Stx) producing E. coli, and hemolytic uremic syndrome (HUS) with neurologic involvement is associated with more severe outcome. We investigated the extent of renal and neurologic damage in mice following injection of the highly potent form of Stx, Stx2a, and less potent Stx1. As observed in previous studies, Stx2a brought about moderate to acute tubular necrosis of proximal and distal tubules in the kidneys. Brain sections stained with hematoxylin and eosin (H&E) appeared normal, although some red blood cell congestion was observed. Microglial cell responses to neural injury include up-regulation of surface-marker expression (e.g., Iba1) and stereotypical morphological changes. Mice injected with Stx2a showed increased Iba1 staining, mild morphological changes associated with microglial activation (thickening of processes), and increased microglial staining per unit area. Microglial changes were observed in the cortex, hippocampus, and amygdala regions, but not the nucleus. Magnetic resonance imaging (MRI) of Stx2a-treated mice revealed no hyper-intensities in the brain, although magnetic resonance spectroscopy (MRS) revealed significantly decreased levels of phosphocreatine in the thalamus. Less dramatic changes were observed following Stx1 challenge. Neither immortalized microvascular endothelial cells from the cerebral cortex of mice (bEnd.3) nor primary human brain microvascular endothelial cells were found to be susceptible to Stx1 or Stx2a. The lack of susceptibility to Stx for both cell types correlated with an absence of receptor expression. These studies indicate Stx causes subtle, but identifiable changes in the mouse brain.

  4. Neurological disease in wild loggerhead sea turtles Caretta caretta.

    Jacobson, Elliott R; Homer, Bruce L; Stacy, Brian A; Greiner, Ellis C; Szabo, Nancy J; Chrisman, Cheryl L; Origgi, Francesco; Coberley, Sadie; Foley, Allen M; Landsberg, Jan H; Flewelling, Leanne; Ewing, Ruth Y; Moretti, Richie; Schaf, Susan; Rose, Corinne; Mader, Douglas R; Harman, Glenn R; Manire, Charles A; Mettee, Nancy S; Mizisin, Andrew P; Shelton, G Diane

    2006-06-12

    Beginning in October 2000, subadult loggerhead sea turtles Caretta caretta showing clinical signs of a neurological disorder were found in waters off south Florida, USA. Histopathology indicated generalized and neurologic spirorchiidiasis. In loggerhead sea turtles (LST) with neurospirorchiidiasis, adult trematodes were found in the meninges of the brain and spinal cord of 7 and 3 affected turtles respectively, and multiple encephalic intravascular or perivascular eggs were associated with granulomatous or mixed leukocytic inflammation, vasculitis, edema, axonal degeneration and occasional necrosis. Adult spirorchiids were dissected from meningeal vessels of 2 of 11 LST brains and 1 of 10 spinal cords and were identified as Neospirorchis sp. Affected LST were evaluated for brevetoxins, ciguatoxins, saxitoxins, domoic acid and palytoxin. While tissues from 7 of 20 LST tested positive for brevetoxins, the levels were not considered to be in a range causing acute toxicosis. No known natural (algal blooms) or anthropogenic (pollutant spills) stressors co-occurred with the turtle mortality. While heavy metal toxicosis and organophosphate toxicosis were also investigated as possible causes, there was no evidence for their involvement. We speculate that the clinical signs and pathologic changes seen in the affected LST resulted from combined heavy spirorchiid parasitism and possible chronic exposure to a novel toxin present in the diet of LST.

  5. MRI of acute cervical injury: correlation with neurologic deficit

    Hyun, Chang Dong; Kwon, Soon Tae; Lim, Seung Chul; Shin, Myung Jin; Han, Boo Kyung; Kim, Sang Joon; Park, Man Soo; Yoon, Hyun Ki; Suh, Dae Chul [Asan Medical Center University of Ulsan College of Medicine, Seoul (Korea, Republic of)

    1995-10-15

    To evaluate MRI findings of spinal cord according to mechanism in acute cervical spinal injury. 25 patients under went MRI within 1 month after acute cervical trauma. Axial T1Wl (TR/TE: 500/20), gradient-echo (TR/TE: 300/14), sagittal T1Wl (TR/TE: 500/20), proton (TR/TE: 2000. 20 msec), T2Wl (TR/TE: 2000/80) were performed. In 11 patients, post-enhancement T1Wl was done. Change of spinal cord signal intensity on MRI in addition to the presence of abnormal changes of vertebral body, intervertebral disc and paraspinal soft tissue were evaluated. 15 patients had flexion injury, seven had extension injury and three had injury of unknown mechanism. Twelve patients showed iso-signal intensity on T2Wl and high signal intensity on T2Wl. Three patients showed low signal intensity on T1Wl and high signal intensity on T2Wl. Spinal cord hemorrhage occured in 10 patients. We found cord swelling in nine patients and cord compression in 12 patients. In nine patients with cord swelling, extent of cord injury was more than one segment of vertebral body. Ligamentous injury, disc injury, soft tissue injury occurred in 16 (64%), 17 (68%), 15 (60%) patients respectively. Vertebral body fracture was found in 17 patients (68%). The levels of fracture were C6 (eight patients) and C5 (five patients). MRI is valuable in exaluetion of the spinal cord, intervertebral disc, and soft tissue lesions in acute cervical spinal injury. Prognosis is worse in flexion injury than in extension injury, and is well correlated with cord hemorrhage and lesion extent.

  6. Neurological findings in Alzheimer's disease and normal aging.

    Galasko, D; Kwo-on-Yuen, P F; Klauber, M R; Thal, L J

    1990-06-01

    To determine the potential value of abnormal neurological findings as markers of Alzheimer's disease (AD) and their relationship to the stage of AD, we compared standardized neurological examinations in 135 community-dwelling patients with AD and 91 nondemented elderly individuals. After correcting for differences in age and education between the two groups, we found that rigidity, stooped posture, graphesthesia, neglect of simultaneous tactile stimuli (face-hand test), and snout, grasp, and glabella reflexes were present significantly more often in patients with AD than in control subjects. These findings increased in prevalence in patients with AD according to the severity of dementia. However, in a multivariate logistic regression model only the grasp reflex, graphesthesia, and the face-hand test were statistically significantly associated with the degree of cognitive impairment. Although abnormal neurological findings occur regularly in AD, they are too infrequent early in the course of AD to serve as diagnostic markers. Prospective studies are needed to determine whether patients with the early onset of extrapyramidal or other findings form a distinct subgroup of AD.

  7. Enterovirus 71 infection-associated acute flaccid paralysis: a case series of long-term neurologic follow-up.

    Lee, Hsiu-Fen; Chi, Ching-Shiang

    2014-10-01

    The authors undertook long-term neurologic outcomes of 27 patients aged 0 to 15 years with enterovirus 71-related acute flaccid paralysis from June 1998 to July 2012. Motor function outcome was graded from class I (complete recovery) to class V (permanent paralytic limbs). Twelve of 20 patients (60%) who received intravenous immunoglobulin for treatment of acute flaccid paralysis had motor function outcomes in classes III to V. The median duration of follow-up was 6 months, during which time 7 of 13 patients (54%) with central nervous system infection, 3 of 6 patients (50%) with autonomic nervous system dysregulation, and 3 of 8 patients (37%) with heart failure showed motor function outcomes in classes III to V. These findings suggested that the usage of intravenous immunoglobulin and the severity of disease staging at disease onset might not be able to predict long-term motor function outcomes.

  8. Human gene therapy and imaging in neurological diseases

    Jacobs, Andreas H.; Winkler, Alexandra [Max Planck-Institute for Neurological Research, Center of Molecular Medicine (CMMC) and Department of Neurology, Cologne (Germany); MPI for Neurological Research, Laboratory for Gene Therapy and Molecular Imaging, Cologne (Germany); Castro, Maria G.; Lowenstein, Pedro [University of California Los Angeles (United States). Department of Medicine

    2005-12-01

    Molecular imaging aims to assess non-invasively disease-specific biological and molecular processes in animal models and humans in vivo. Apart from precise anatomical localisation and quantification, the most intriguing advantage of such imaging is the opportunity it provides to investigate the time course (dynamics) of disease-specific molecular events in the intact organism. Further, molecular imaging can be used to address basic scientific questions, e.g. transcriptional regulation, signal transduction or protein/protein interaction, and will be essential in developing treatment strategies based on gene therapy. Most importantly, molecular imaging is a key technology in translational research, helping to develop experimental protocols which may later be applied to human patients. Over the past 20 years, imaging based on positron emission tomography (PET) and magnetic resonance imaging (MRI) has been employed for the assessment and ''phenotyping'' of various neurological diseases, including cerebral ischaemia, neurodegeneration and brain gliomas. While in the past neuro-anatomical studies had to be performed post mortem, molecular imaging has ushered in the era of in vivo functional neuro-anatomy by allowing neuroscience to image structure, function, metabolism and molecular processes of the central nervous system in vivo in both health and disease. Recently, PET and MRI have been successfully utilised together in the non-invasive assessment of gene transfer and gene therapy in humans. To assess the efficiency of gene transfer, the same markers are being used in animals and humans, and have been applied for phenotyping human disease. Here, we review the imaging hallmarks of focal and disseminated neurological diseases, such as cerebral ischaemia, neurodegeneration and glioblastoma multiforme, as well as the attempts to translate gene therapy's experimental knowledge into clinical applications and the way in which this process is being

  9. Pictorial essay: Acute neurological complications in children with acute lymphoblastic leukemia

    Seema A Kembhavi

    2012-01-01

    Full Text Available Acute lymphoblastic leukemia (ALL is the commonest childhood malignancy with high cure rates due to recent advances in central nervous system (CNS prophylaxis. The disease per se, as well as the prophylactic therapy, predisposes the child to complications such as cerebrovascular events, infections, drug toxicities, etc. The purpose of this study is to highlight the pathophysiology and the imaging features (with appropriate examples of these complications and to propose a diagnostic algorithm based on MRI. Interpreting these scans in the light of clinical inputs very often helps the radiologist reach an appropriate diagnosis and help treatment and management.

  10. Therapeutic plasma exchange in patients with neurological diseases: multicenter retrospective analysis.

    Kaya, Emin; Keklik, Muzaffer; Sencan, Mehmet; Yilmaz, Mehmet; Keskin, Ali; Kiki, Ilhami; Erkurt, Mehmet Ali; Sivgin, Serdar; Korkmaz, Serdal; Okan, Vahap; Doğu, Mehmet Hilmi; Unal, Ali; Cetin, Mustafa; Altuntaş, Fevzi; Ilhan, Osman

    2013-06-01

    Therapeutic plasma exchange (TPE), is a procedure, changing pathologic substances in the plasma of patients with replacement fluid. TPE has an increasing list of indications in recent years such as neurological, connective tissue, hematological, nephrological, endocrinological and metabolic disorders. We report our multicenter data about therapeutic plasma exchange in patients with neurological diseases. Six University Hospitals' aphaeresis units medical records about neurologic diseases were reviewed retrospectively. Hundred and fifteen patients and 771 TPE sessions from six aphaeresis units' were included to this study. Of the 115 patients, 53 (46%) were men and 62 (54%) were women. The median age was 50 (range: 5-85) years. Of these patients 58.3% were Guillain-Barre syndrome (GBS), 17.4% were acute disseminated encephalomyelitis (ADEM), 10.4% were chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), 7% were multiple sclerosis, 6.1% were myasthenia gravis (MG) and 0.9% were Wilson disease (WD). The median number of TPE sessions per patient was 5 (range 1-72). Human albumin was used as a replacement fluid in 66% and fresh frozen plasma was used in 34% of cases. TPE was done through central venous catheters in 66%, and peripheral venous access in 34% of patients. Some complications were seen in patients (18.3%) during TPE sessions. These complications were, complications related to catheter placement procedure (8.7%), hypotension (3.5%), hypocalcaemia (3.5%) and allergic reactions (1.7%). The complication ratios were 2.7% in total 771 TPE procedures. TPE procedure was terminated in 6% of sessions depending on these complications. Overall responses to TPE were noted in 89.5% of patients. In conclusion; Therapeutic plasma exchange is an effective treatment option in several neurologic diseases.

  11. Torsade de pointes indicates early neurologic damage in acute ischemic stroke.

    Huang, Li-Yen; Lin, Wei-Shiang; Lin, Wen-Yu; Cheng, Cheng-Chung; Cheng, Shu-Meng; Tsai, Tsung-Neng

    2013-12-01

    Torsade de pointes (TdP) is a life-threatening polymorphic ventricular tachycardia that is related to QT prolongation. Although QT prolongation is commonly seen in acute stroke, TdP is rare. We report the case of a 78-year-old woman with ischemic stroke who presented with TdP as the initial manifestation of early neurologic deterioration. We hypothesized that an increase in intracranial pressure may result in neurohormonal activation, QT prolongation, and then myocardial damage, leading to TdP. We highlight that new onset of TdP in a patient with stroke may reflect neurologic deterioration, requiring further evaluation and specific intervention.

  12. Induced pluripotent stem cells and neurological disease models.

    Cai, Sa; Chan, Ying-Shing; Shum, Daisy Kwok-Yan

    2014-02-25

    The availability of human stem cells heralds a new era for in vitro cell-based modeling of neurodevelopmental and neurodegenerative diseases. Adding to the excitement is the discovery that somatic cells of patients can be reprogrammed to a pluripotent state from which neural lineage cells that carry the disease genotype can be derived. These in vitro cell-based models of neurological diseases hold promise for monitoring of disease initiation and progression, and for testing of new drug treatments on the patient-derived cells. In this review, we focus on the prospective applications of different stem cell types for disease modeling and drug screening. We also highlight how the availability of patient-specific induced pluripotent stem cells (iPS cells) offers a unique opportunity for studying and modeling human neurodevelopmental and neurodegenerative diseases in vitro and for testing small molecules or other potential therapies for these disorders. Finally, the limitations of this technology from the standpoint of reprogramming efficiency and therapeutic safety are discussed.

  13. Neurological Disease Rises from Ocean to Bring Model for Human Epilepsy to Life

    John S. Ramsdell

    2010-06-01

    Full Text Available Domoic acid of macroalgal origin was used for traditional and medicinal purposes in Japan and largely forgotten until its rediscovery in diatoms that poisoned 107 people after consumption of contaminated mussels. The more severely poisoned victims had seizures and/or amnesia and four died; however, one survivor unexpectedly developed temporal lobe epilepsy (TLE a year after the event. Nearly a decade later, several thousand sea lions have stranded on California beaches with neurological symptoms. Analysis of the animals stranded over an eight year period indicated five clusters of acute neurological poisoning; however, nearly a quarter have stranded individually outside these events with clinical signs of a chronic neurological syndrome similar to TLE. These poisonings are not limited to sea lions, which serve as readily observed sentinels for other marine animals that strand during domoic acid poisoning events, including several species of dolphin and whales. Acute domoic acid poisoning is five-times more prominent in adult female sea lions as a result of the proximity of their year-round breeding grounds to major domoic acid bloom events. The chronic neurological syndrome, on the other hand, is more prevalent in young animals, with many potentially poisoned in utero. The sea lion rookeries of the Channel Islands are at the crossroads of domoic acid producing harmful algal blooms and a huge industrial discharge site for dichlorodiphenyltrichloroethane (DDTs. Studies in experimental animals suggest that chronic poisoning observed in immature sea lions may result from a spatial and temporal coincidence of DDTs and domoic acid during early life stages. Emergence of an epilepsy syndrome from the ocean brings a human epilepsy model to life and provides unexpected insights into interaction with legacy contaminants and expression of disease at different life stages.

  14. Neurological images and the predictors for neurological sequelae of epidemic herpangina/hand-foot-mouth disease with encephalomyelitis.

    Tsai, Jeng-Dau; Kuo, Hung-Tsung; Chen, Shan-Ming; Lue, Ko-Huang; Sheu, Ji-Nan

    2014-04-01

    Since 1998 in Taiwan, enterovirus (EV) 71 epidemics have caused encephalomyelitis and placed a significant burden on parents and physicians. In this study, we present clinical manifestations, magnetic resonance (MR) imaging findings, and neurological sequelae on epidemic EV-infected patients with encephalomyelitis. Of the 46 patients, 14 patients presented with neurological sequelae; of them, 3 patients suffered from complications of mental regression. Predictors of unfavorable neurological sequelae were myoclonic jerks (> 4 times/night) and pleocytosis (167/μL) of the cerebrospinal fluid (CSF). Results from viral culture and MR imaging indicated that positive identification of EV71 infection was associated significantly with lesions on MR imaging. Our results show that hand-foot-mouth disease carries a higher risk of encephalomyelitis and that frequent myoclonic jerks and pleocytosis of the CSF are risk factors for subsequent neurological sequelae. Positive identification of EV71 might be useful as a predictor of lesions in MR imaging.

  15. Samuel Alexander Kinnier Wilson. Wilson's disease, Queen Square and neurology.

    Broussolle, E; Trocello, J-M; Woimant, F; Lachaux, A; Quinn, N

    2013-12-01

    This historical article describes the life and work of the British physician Samuel Alexander Kinnier Wilson (1878-1937), who was one of the world's greatest neurologists of the first half of the 20th century. Early in his career, Wilson spent one year in Paris in 1903 where he learned from Pierre-Marie at Bicêtre Hospital. He subsequently retained uninterrupted links with French neurology. He also visited in Leipzig the German anatomist Paul Flechsig. In 1904, Wilson returned to London, where he worked for the rest of his life at the National Hospital for the Paralysed and Epileptic (later the National Hospital for Nervous Diseases, and today the National Hospital for Neurology and Neurosurgery) in Queen Square, and also at Kings' College Hospital. He wrote on 'the old motor system and the new', on disorders of motility and muscle tone, on the epilepsies, on aphasia, apraxia, tics, and pathologic laughing and crying, and most importantly on Wilson's disease. The other objective of our paper is to commemorate the centenary of Wilson's most important work published in 1912 in Brain, and also in Revue Neurologique, on an illness newly recognized and characterized by him entitled "Progressive lenticular degeneration, a familial nervous disease associated with liver cirrhosis". He analyzed 12 clinical cases, four of whom he followed himself, but also four cases previously published by others and a further two that he considered in retrospect had the same disease as he was describing. The pathological profile combined necrotic damage in the lenticular nuclei of the brain and hepatic cirrhosis. This major original work is summarized and discussed in the present paper. Wilson not only delineated what was later called hepato-lenticular degeneration and Wilson's disease, but also introduced for the first time the terms extrapyramidal syndrome and extrapyramidal system, stressing the role of the basal ganglia in motility. The present historical work emphasizes the special

  16. Major neurological disease and occupational exposure to organic solvents.

    Seaton, A; Jellinek, E H; Kennedy, P

    1992-09-01

    Five patients are described who presented with major organic brain disease affecting one or more of pyramidal and extrapyramidal tracts, cerebellum, and higher cortical functions. All had a history of 10 years or more of regular occupational exposure to solvents in confined spaces, three in painting inside ships and the others in weapons maintenance and printing. All had been regularly exposed to high air vapour peaks as well as to skin contamination. Four showed some evidence of improvement after the exposure ceased. None was initially suspected of having a toxic encephalopathy by the consultant to whom he was referred. The spectrum of neurological disease presented by these men mirrors closely that described in solvent abusers. All were forced by illness to retire from their work, a circumstance which might have in the past have led to such conditions being missed in cross-sectional studies, which in general have not shown evidence of major disease. We suggest that when such disease occurs nowadays, its cause is usually not suspected. Further epidemiological study of the problem is necessary.

  17. Acute axonal polyneuropathy with predominant proximal involvement: an uncommon neurological complication of bariatric surgery

    2006-01-01

    Bariatric surgery is frequently indicated in the treatment of morbid obesity. Previously unreported complications have been associated to this surgery; among them, neurological complications have gained attention. We report the case of a 25-year-old man submitted to gastric surgery for treatment of morbid obesity who developed, two months after surgery, acute proximal weakness in lower limbs. The electroneuromyography revealed axonal peripheral polyneuropathy with predominant proximal involve...

  18. Non-coding RNAs in chromatin disease involving neurological defects

    Floriana eDella Ragione

    2014-02-01

    Full Text Available Novel classes of small and long non-coding RNAs (ncRNAs are increasingly becoming apparent, being engaged in diverse structural, functional and regulatory activities. They take part in target gene silencing, play roles in transcriptional, post-transcriptional and epigenetic processes, such as chromatin remodeling, nuclear reorganization with the formation of silent compartments and fine-tuning of gene recruitment into them. Among their functions, non-coding RNAs are thought to act either as guide or scaffold for epigenetic modifiers that write, erase and read the epigenetic signature over the genome. Studies on human disorders caused by defects in epigenetic modifiers and involving neurological phenotypes highlight the disruption of diverse classes of non-coding RNAs. Noteworthy, these molecules mediate a wide spectrum of neuronal functions, including brain development, and synaptic plasticity. These findings imply a significant contribution of ncRNAs in pathophysiology of the aforesaid diseases and provide new concepts for potential therapeutic applications.

  19. EFFECT OF ACUPUNCTURE ON NEUROLOGICAL DEFECTS AND DAILY LIFE ABILITY IN PATIENTS WITH ACUTE CEREBRAL HEMORRHAGE

    周爽; 方邦江; 孙国杰

    2003-01-01

    Objective: To observe the effect of acupuncture of Shuigou (GV 26), Neiguan (PC 6), Zusanli (ST36), etc. on neurological defects and daily life ability in patients with acute cerebral hemorrhage. Methods: Fifty-eight cases of acute cerebral hemorrhage patients were randomized into control group (n = 28) and treatment group (n =30). Patients of two groups were both treated with intravenous infusion of Mannitol and other expectant medicines. In addition, patients of treatment group were also treated with acupuncture therapy, once daily and continuously for one month. Before and after treatment, the scores of neurological defects and daily life ability (Barthel Index) were givenfor assessing the therapeutic effect. Results: Following treatment, both scores of neurological defects of two groups decreased significantly (P < 0.05), and the score of treatment group was strikingly lower than that of control group (P<0.05). After treatment, values of Barthel Index (BI) of two groups increased considerably in comparison with pretreatment (P< 0.01 ), and the value of BI of treatment group was bigger than that of control group (P< 0.05). Conclusion: Acupuncture can improve acute cerebral hemorrhage patients' nervous function and daily life ability.

  20. Neuroinflammation and neurological alterations in chronic liver diseases

    Carmina Montoliu

    2015-01-01

    Full Text Available Several million people with chronic liver diseases (cirrhosis, hepatitis show neurological alterations, named hepatic encephalopathy (HE with cognitive and motor alterations that impair quality of life and reduces life span. Inflammation acts synergistically with hyperammonemia to induce cognitive and motor alterations in patients with chronic liver disease and minimal hepatic encephalopathy (MHE. Previous studies in animal models have suggested that neuroinflammation is a major player in HE. This would also be the case in patients with liver cirrhosis or hepatitis C with HE. Rats with MHE show microglial activation and neuroinflammation that is associated with cognitive impairment and hypokinesia. The anti-inflammatory drug ibuprofen reduces microglial activation and neuroinflammation and restores cognitive and motor functions in rats with MHE. Chronic hyperammonemia per se induces neuroinflammation. Both peripheral inflammation and hyperammonemia would contribute to neuroinflammation in chronic liver failure. Therefore, neuroinflammation may be a key therapeutic target to improve the cognitive and motor alterations in MHE and overt HE. Identifying new targets to reduce neuroinflammation in MHE without inducing secondary effects would serve to develop new therapeutic tools to reverse the cognitive and motor alterations in patients with HE associated with chronic liver diseases.

  1. Dialectics and Implications of Natural Neurotropic Autoantibodies in Neurological Disease and Rehabilitation

    A. B. Poletaev

    2004-01-01

    Full Text Available The role of natural idiotypic (Id-Abs and anti-idiotypic (AId-Abs autoantibodies against neuro-antigens observed in different neurological disorders is not fully understood. In particular, limited experimental evidence has been provided concerning the qualitative and quantitative serological response after acute injuries of the central nervous system or during chronic mental diseases. In this study, we analyzed the specific Id-Abs and AId-Abs serological reactivities against 4 neuro-antigens in a large population of patients with ischemic stroke, schizophrenia, as well as healthy individuals. Patients with ischemic stroke were tested at different time points following the acute stroke episode and a correlation was attempted between autoantibodies response and different patterns of functional recovery. Results showed variable and detectable Id-Abs and AId-Abs in different proportions of all three populations of subjects. Among patients with different functional recovery after ischemic stroke, a difference in time-related trends of Id-Abs and AId-Abs was encountered. Our observations suggest that changes in the production of natural neurotropic Abs may engender a positive homeostatic, beside a possible pathogenic effect, in specific neurological disorders.

  2. Enfermedad neurologica por adenovirus Neurologic disease due to adenovirus infection

    Cristina L. Lema

    2005-06-01

    Full Text Available El objetivo de este trabajo fue determinar la prevalencia de adenovirus (ADV en las infecciones del sistema nervioso central (SNC. Se analizaron 108 muestras de líquido cefalorraquídeo (LCR provenientes de 79 casos de encefalitis, 7 meningitis y 22 de otras patologías neurológicas, recibidas en el período 2000-2002. Cuarenta y nueve (47.35% se obtuvieron de pacientes inmunocomprometidos. La presencia de ADV se investigó mediante reacción en cadena de la polimerasa en formato anidado (Nested-PCR. La identificación del genogrupo se realizó mediante análisis filogenético de la secuencia nucleotídica parcial de la región que codifica para la proteína del hexón. Se detectó la presencia de ADV en 6 de 108 (5.5% muestras de LCR analizadas. Todos los casos positivos pertenecieron a pacientes con encefalitis que fueron 79, (6/79, 7.6%. No se observó diferencia estadísticamente significativa entre los casos de infección por ADV en pacientes inmunocomprometidos e inmunocompetentes (p>0.05. Las cepas de ADV detectadas se agruparon en los genogrupos B1 y C. En conclusión, nuestros resultados describen el rol de los ADV en las infecciones neurológicas en Argentina. La información presentada contribuye al conocimiento de su epidemiología, en particular en casos de encefalitis.The aim of this study was to assess the prevalence of adenovirusm (ADV infections in neurological disorders. A total of 108 cerebrospinal fluid (CSF samples from 79 encephalitis cases, 7 meningitis and 22 other neurological diseases analysed in our laboratory between 2000 and 2002 were studied. Forty nine (47.4% belonged to immunocompromised patients. Viral genome was detected using nested polymerase chain reaction (Nested-PCR and ADV genotypes were identified using partial gene sequence analysis of hexon gene. Adenovirus were detected in 6 of 108 (5.5% CSF samples tested. All of these were from encephalitis cases, 6/79, representing 7.6% of them. No statistically

  3. Increased neurofilament light chain blood levels in neurodegenerative neurological diseases.

    Johanna Gaiottino

    Full Text Available Neuronal damage is the morphological substrate of persisting neurological disability. Neurofilaments (Nf are cytoskeletal proteins of neurons and their release into cerebrospinal fluid has shown encouraging results as a biomarker for neurodegeneration. This study aimed to validate the quantification of the Nf light chain (NfL in blood samples, as a biofluid source easily accessible for longitudinal studies.We developed and applied a highly sensitive electrochemiluminescence (ECL based immunoassay for quantification of NfL in blood and CSF.Patients with Alzheimer's disease (AD (30.8 pg/ml, n=20, Guillain-Barré-syndrome (GBS (79.4 pg/ml, n=19 or amyotrophic lateral sclerosis (ALS (95.4 pg/ml, n=46 had higher serum NfL values than a control group of neurological patients without evidence of structural CNS damage (control patients, CP (4.4 pg/ml, n=68, p<0.0001 for each comparison, p=0.002 for AD patients and healthy controls (HC (3.3 pg/ml, n=67, p<0.0001. Similar differences were seen in corresponding CSF samples. CSF and serum levels correlated in AD (r=0.48, p=0.033, GBS (r=0.79, p<0.0001 and ALS (r=0.70, p<0.0001, but not in CP (r=0.11, p=0.3739. The sensitivity and specificity of serum NfL for separating ALS from healthy controls was 91.3% and 91.0%.We developed and validated a novel ECL based sandwich immunoassay for the NfL protein in serum (NfL(Umea47:3; levels in ALS were more than 20-fold higher than in controls. Our data supports further longitudinal studies of serum NfL in neurodegenerative diseases as a potential biomarker of on-going disease progression, and as a potential surrogate to quantify effects of neuroprotective drugs in clinical trials.

  4. Cannabinoids: New Promising Agents in the Treatment of Neurological Diseases

    Sabrina Giacoppo

    2014-11-01

    Full Text Available Nowadays, Cannabis sativa is considered the most extensively used narcotic. Nevertheless, this fame obscures its traditional employ in native medicine of South Africa, South America, Turkey, Egypt and in many regions of Asia as a therapeutic drug. In fact, the use of compounds containing Cannabis and their introduction in clinical practice is still controversial and strongly limited by unavoidable psychotropic effects. So, overcoming these adverse effects represents the main open question on the utilization of cannabinoids as new drugs for treatment of several pathologies. To date, therapeutic use of cannabinoid extracts is prescribed in patients with glaucoma, in the control of chemotherapy-related vomiting and nausea, for appetite stimulation in patients with anorexia-cachexia syndrome by HIV, and for the treatment of multiple sclerosis symptoms. Recently, researcher efforts are aimed to employ the therapeutic potentials of Cannabis sativa in the modulation of cannabinoid receptor activity within the central nervous system, particularly for the treatment of neurodegenerative diseases, as well as psychiatric and non-psychiatric disorders. This review evaluates the most recent available data on cannabinoids utilization in experimental and clinical studies, and highlights their beneficial effects in the prevention of the main neurological diseases and for the clinical treatment of symptoms with them correlated.

  5. Awareness Status of Chronic Disabling Neurological Diseases among Elderly Veterans

    Ji-Ping Tan; Lin-Qi Zhu; Jun Zhang; Shi-Min Zhang; Xiao-Yang Lan; Bo Cui; Yu-Cheng Deng

    2015-01-01

    Background:The awareness,treatment and prevention of chronic diseases are generally poor among the elderly population of China,whereas the prevention and control of chronic diseases in elderly veteran communities have been ongoing for more than 30 years.Therefore,investigating the awareness status of chronic disabling neurological diseases (CDND) and common chronic diseases (CCD) among elderly veterans may provide references for related programs among the elderly in the general population.Methods:A cross-sectional survey was conducted among veterans ≥60 years old in veteran communities in Beijing.The awareness of preventive strategies against dementia,Alzheimer's disease (AD),Parkinson's disease (PD),sleep disorders,cerebrovascular disease (CVD) and CCD such as hypertension,and the approaches used to access this information,including media,word of mouth (verbal communication among the elderly) and health care professionals,were investigated via face-to-face interviews.Results:The awareness rates for CCD and CVD were approximately 100%,but that forAD was the lowest at <10%.The awareness rates for sleep disorders,PD and dementia,were 51.0-89.4%.Media was the most commonly selected mode of communication by which veterans acquired knowledge about CCD and CVD.Media was used by approximately 80% of veterans.Both health care professionals and word of mouth were used by approximately 50% of veterans.With respect to the source of information about CDND excluding AD,the rates of the use of health care professionals,word of mouth and media were 10.6-28.2%,56.5-76.5%,and approximately 50%,respectively.Conclusions:The awareness of CDND among elderly veterans was significantly lower than that of CCD.More information about CDND should be disseminated by health care professionals.Appropriate guidance will promote the rapid and extensive dissemination of information about the prevention of CDND by media and word-of-mouth peer education.

  6. (-)-Epigallocatechin-3-gallate (EGCG) modulates neurological function when intravenously infused in acute and, chronically injured spinal cord of adult rats.

    Renno, Waleed M; Al-Khaledi, Ghanim; Mousa, Alyaa; Karam, Shaima M; Abul, Habib; Asfar, Sami

    2014-02-01

    Spinal cord injury (SCI) causes severe and long lasting motor and sensory deficits, chronic pain, and autonomic dysreflexia. (-)-epigallocatechin-3-gallate (EGCG) has shown to produce neuroprotective effect in a broad range of neurodegenerative disease animal models. This study designed to test the efficacy of intravenous infusion of EGCG for 36 h, in acutely injured rats' spinal cord: within first 4 h post-injury and, in chronically SC injured rats: after one year of injury. Functional outcomes measured using standard BBB scale, The Louisville Swim Scale (LSS) and, pain behavior assessment tests. 72 Female adult rats subjected to moderate thoracic SCI using MASCIS Impactor, blindly randomized as the following: (I) Acute SCI + EGCG (II) Acute SCI + saline. (III) Chronic SCI + EGCG. (IV) Chronic SCI + saline and, sham SCI animals. EGCG i.v. treatment of acute and, chronic SCI animals resulted in significantly better recovery of motor and sensory functions, BBB and LSS (P spinal cord increased (P < 0.001). Percent areas of GAP-43 and GFAP immunohistochemistry showed significant (P < 0.05) increase. We conclude that the therapeutic window of opportunity for EGCG to depict neurological recovery in SCI animals, is viable up to one year post SCI when intravenously infused for 36 h.

  7. Neurological and cardiac complications in a cohort of children with end-stage renal disease.

    Albaramki, Jumana H; Al-Ammouri, Iyad A; Akl, Kamal F

    2016-05-01

    Adult patients with chronic kidney disease are at risk of major neurologic and cardiac complications. The purpose of this study is to review the neurological and cardiac complications in children with end-stage renal disease (ESRD). A retrospective review of medical records of children with ESRD at Jordan University Hospital was performed. All neurological and cardiac events were recorded and analyzed. Data of a total of 68 children with ESRD presenting between 2002 and 2013 were reviewed. Neurological complications occurred in 32.4%; seizures were the most common event. Uncontrolled hypertension was the leading cause of neurological events. Cardiac complications occurred in 39.7%, the most common being pericardial effusion. Mortality from neurological complications was 45%. Neurological and cardiac complications occurred in around a third of children with ESRD with a high mortality rate. More effective control of hypertension, anemia, and intensive and gentle dialysis are needed.

  8. Neurological and cardiac complications in a cohort of children with end-stage renal disease

    Jumana H Albaramki

    2016-01-01

    Full Text Available Adult patients with chronic kidney disease are at risk of major neurologic and cardiac complications. The purpose of this study is to review the neurological and cardiac complications in children with end-stage renal disease (ESRD. A retrospective review of medical records of children with ESRD at Jordan University Hospital was performed. All neurological and cardiac events were recorded and analyzed. Data of a total of 68 children with ESRD presenting between 2002 and 2013 were reviewed. Neurological complications occurred in 32.4%; seizures were the most common event. Uncontrolled hypertension was the leading cause of neurological events. Cardiac complications occurred in 39.7%, the most common being pericardial effusion. Mortality from neurological complications was 45%. Neurological and cardiac complications occurred in around a third of children with ESRD with a high mortality rate. More effective control of hypertension, anemia, and intensive and gentle dialysis are needed.

  9. When dialogue fails. Music therapy with elderly with neurological degenerative diseases

    Wigram, Anthony Lewis

    2004-01-01

    When dialogue fails. Music therapy with elderly with neurological degenerative diseases. In persons suffering from neurological degenerative diseases we often see the following symptoms: difficulties in remembering, concentrating, perceiving input, and controlling and timing movements. Normal every...... interaction with others means that psychosocial needs are not met, and this leads to secondary symptoms of the neurological degeneration. Secondary symptoms might be expressed as repetitive behaviour, catastrophic reactions and situationally inappropriate behaviour. In a music therapeutical setting...

  10. Inherited neuronal ion channelopathies: new windows on complex neurological diseases.

    Catterall, William A; Dib-Hajj, Sulayman; Meisler, Miriam H; Pietrobon, Daniela

    2008-11-12

    Studies of genetic forms of epilepsy, chronic pain, and migraine caused by mutations in ion channels have given crucial insights into molecular mechanisms, pathogenesis, and therapeutic approaches to complex neurological disorders. Gain-of-function missense mutations in the brain type-I sodium channel Na(V)1.1 are a primary cause of generalized epilepsy with febrile seizures plus. Loss-of-function mutations in Na(V)1.1 channels cause severe myoclonic epilepsy of infancy, an intractable childhood epilepsy. Studies of a mouse model show that this disease is caused by selective loss of sodium current and excitability of GABAergic inhibitory interneurons, which leads to hyperexcitability, epilepsy, and ataxia. Mutations in the peripheral sodium channel Na(V)1.7 cause familial pain syndromes. Gain-of-function mutations cause erythromelalgia and paroxysmal extreme pain disorder as a result of hyperexcitability of sensory neurons, whereas loss-of-function mutations cause congenital indifference to pain because of attenuation of action potential firing. These experiments have defined correlations between genotype and phenotype in chronic pain diseases and focused attention on Na(V)1.7 as a therapeutic target. Familial hemiplegic migraine is caused by mutations in the calcium channel, Ca(V)2.1, which conducts P/Q-type calcium currents that initiate neurotransmitter release. These mutations increase activation at negative membrane potentials and increase evoked neurotransmitter release at cortical glutamatergic synapses. Studies of a mouse genetic model show that these gain-of-function effects lead to cortical spreading depression, aura, and potentially migraine. Overall, these experiments indicate that imbalance in the activity of excitatory and inhibitory neurons is an important underlying cause of these diseases.

  11. Sindbis and Middelburg Old World Alphaviruses Associated with Neurologic Disease in Horses, South Africa

    van Niekerk, Stephanie; Human, Stacey; Williams, June; van Wilpe, Erna; Pretorius, Marthi; Swanepoel, Robert

    2015-01-01

    Old World alphaviruses were identified in 52 of 623 horses with febrile or neurologic disease in South Africa. Five of 8 Sindbis virus infections were mild; 2 of 3 fatal cases involved co-infections. Of 44 Middelburg virus infections, 28 caused neurologic disease; 12 were fatal. Middelburg virus likely has zoonotic potential. PMID:26583836

  12. Acute graft versus host disease

    Vogelsang Georgia B

    2007-09-01

    Full Text Available Abstract Acute graft-versus-host disease (GVHD occurs after allogeneic hematopoietic stem cell transplant and is a reaction of donor immune cells against host tissues. Activated donor T cells damage host epithelial cells after an inflammatory cascade that begins with the preparative regimen. About 35%–50% of hematopoietic stem cell transplant (HSCT recipients will develop acute GVHD. The exact risk is dependent on the stem cell source, age of the patient, conditioning, and GVHD prophylaxis used. Given the number of transplants performed, we can expect about 5500 patients/year to develop acute GVHD. Patients can have involvement of three organs: skin (rash/dermatitis, liver (hepatitis/jaundice, and gastrointestinal tract (abdominal pain/diarrhea. One or more organs may be involved. GVHD is a clinical diagnosis that may be supported with appropriate biopsies. The reason to pursue a tissue biopsy is to help differentiate from other diagnoses which may mimic GVHD, such as viral infection (hepatitis, colitis or drug reaction (causing skin rash. Acute GVHD is staged and graded (grade 0-IV by the number and extent of organ involvement. Patients with grade III/IV acute GVHD tend to have a poor outcome. Generally the patient is treated by optimizing their immunosuppression and adding methylprednisolone. About 50% of patients will have a solid response to methylprednisolone. If patients progress after 3 days or are not improved after 7 days, they will get salvage (second-line immunosuppressive therapy for which there is currently no standard-of-care. Well-organized clinical trials are imperative to better define second-line therapies for this disease. Additional management issues are attention to wound infections in skin GVHD and fluid/nutrition management in gastrointestinal GVHD. About 50% of patients with acute GVHD will eventually have manifestations of chronic GVHD.

  13. Urodynamic profile in acute transverse myelitis patients: Its correlation with neurological outcome

    Gupta, Anupam; Kumar, Sushruth Nagesh; Taly, Arun B.

    2017-01-01

    Objective: The objective of this study was to observe urodynamic profile of acute transverse myelitis (ATM) patients and its correlation with neurological outcome. Patients and Methods: This prospective study was conducted in the neurorehabilitation unit of a tertiary university research hospital from July 2012 to June 2014. Forty-three patients (19 men) with ATM with bladder dysfunction, admitted in the rehabilitation unit, were included in this study. Urodynamic study (UDS) was performed in all the patients. Their neurological status was assessed using ASIA impairment scale and functional status was assessed using spinal cord independence measure. Bladder management was based on UDS findings. Results: In total, 17 patients had tetraplegia and 26 had paraplegia. Thirty-six patients (83.7%) had complaints of increased frequency and urgency of urine with 26 patients reported at least one episode of urge incontinence. Seven patients reported obstructive urinary complaints in the form of straining to void with 13 patients reported both urgency and straining to void and 3 also had stress incontinence. Thirty-seven (86.1%) patients had neurogenic overactive detrusor with or without sphincter dyssynergia and five patients had acontractile detrusor on UDS. No definitive pattern was observed between neurological status and bladder characteristics. All patients showed significant neurological and functional recovery with inpatient rehabilitation (P < 0.05 and P < 0.001, respectively). Conclusions: The problem of neurogenic bladder dysfunction is integral to ATM. Bladder management in these patients should be based on UDS findings. Bladder characteristics have no definitive pattern consistent with the neurological status. PMID:28149080

  14. Neurological Diseases, Disorders and Injuries in Canada: Highlights of a National Study.

    Bray, Garth M; Huggett, Deanna L

    2016-01-01

    The National Population Health Study of Neurological Conditions, a partnership between Neurological Health Charities Canada and the Government of Canada, was the largest study of neurological diseases, disorders, and injuries ever conducted in Canada. Undertaken between 2009 and 2013, the expansive program of research addressed the epidemiology, impacts, health services, and risk factors of 18 neurological conditions and estimated the health outcomes and costs of these conditions in Canada through 2031. This review summarizes highlights from the component projects of the study as presented in the synthesis report, Mapping Connections: An Understanding of Neurological Conditions in Canada. The key findings included new prevalence and incidence estimates, documentation of the diverse and often debilitating effects of neurological conditions, and identification of the utilization, economic costs, and current limitations of related health services. The study findings will support health charities, governments, and other stakeholders to reduce the impact of neurological conditions in Canada.

  15. Diagnostic imaging of acute neurologic symptoms in children; Bildgebende Diagnostik neurologischer Akutsituationen im Kindesalter

    Unsinn, K.M.; Gassner, I. [Universitaetsklinik fuer Kinder- und Jugendheilkunde, Innsbruck (Austria). Kinderradiologie; Freund, M.C.; Schocke, M.; Cihak, C. [Universitaetsklinik fuer Radiodiagnostik, Innsbruck (Austria)

    2002-03-01

    The diagnostic imaging of children with acute, non-traumatic, neurologic symptoms enables a fast and non-invasive localization and diagnosis. A spectrum of typical disorders will be described dependent on the location of neurologic symptoms (central, spinal, or peripheral nervous system). Different non-invasive imaging modalities e.g. US with colour-coded doppler, CT, MRI are utilized dependent on age of the patient and neurologic symptoms. The purpose of this article is to describe the spectrum of diagnostic imaging for each of these common disorders. (orig.) [German] Die bildgebende Diagnostik akuter neurologischer Stoerungen nichttraumatischer Genese im Kindesalter ermoeglicht eine rasche, nichtinvasive Lokalisation und artdiagnostische Zuordnung. Typische Krankheitsbilder werden in Abhaengigkeit von der Lokalisation - zerebral, spinal, peripher-nerval - dargestellt. In Abhaengigkeit vom Patientenalter und der neurologischen Symptomatik kommen unterschiedliche nichtinvasive Untersuchungsmethoden zur Anwendung wie z.B. Ultraschall mit FKDS, CT, MRT. Ziel dieses Uebersichtsreferates ist die Darstellung des Stellenwertes der bildgebenden Diagnostik in der Abklaerung der haeufigsten akuten-neurologischen Krankheitsbilder im Kindesalter. (orig.)

  16. Cerebrospinal Fluid Proteome of Patients with Acute Lyme Disease

    Angel, Thomas E.; Jacobs, Jon M.; Smith, Robert P.; Pasternack, Mark S.; Elias, Susan; Gritsenko, Marina A.; Shukla, Anil K.; Gilmore, Edward C.; McCarthy, Carol; Camp, David G.; Smith, Richard D.

    2012-10-05

    Acute Lyme disease results from transmission of and infection by the bacterium Borrelia burgdorferi following a tick bite. During acute infection, bacteria can disseminate to the central nervous system (CNS) leading to the development of Lyme meningitis. Here we have analyzed pooled cerebrospinal fluid (CSF) allowing for a deep view into the proteome for a cohort of patients with early-disseminated Lyme disease and CSF inflammation leading to the identification of proteins that reflect host responses, which are distinct for subjects with acute Lyme disease. Additionally, we analyzed individual patient samples and quantified changes in protein abundance employing label-free quantitative mass spectrometry based methods. The measured changes in protein abundances reflect the impact of acute Lyme disease on the CNS as presented in CSF. We have identified 89 proteins that differ significantly in abundance in patients with acute Lyme disease. A number of the differentially abundant proteins have been found to be localized to brain synapse and thus constitute important leads for better understanding of the neurological consequence of disseminated Lyme disease.

  17. Correlation between serum neuron specific enolase and functional neurological outcome in patients of acute ischemic stroke

    Sana Zaheer

    2013-01-01

    Full Text Available Context: The use of biomarkers to predict stroke prognosis is gaining particular attention nowadays. Neuron specific enolase (NSE, which is a dimeric isoenzyme of the glycolytic enzyme enolase and is found mainly in the neurons is one such biomarker. Aims: This study was carried out on patients of acute ischemic stroke with the aims to determine the correlation between NSE levels on the day of admission with infarct volume, stroke severity, and functional neurological outcome on day 30. Materials and Methods: Seventy five patients of acute ischemic stroke admitted in the Department of Medicine were included in the study. Levels of NSE were determined on day 1 using the human NSE ELISA kit (Alpha Diagnostic International Texas 78244, USA. Volume of infarct was measured by computed tomography (CT scan using the preinstalled software Syngo (version A40A of Siemen′s medical solutions (Forchheim, Germany. Stroke severity at admission was assessed using Glasgow coma scale (GCS and functional neurological outcome was assessed using modified Rankin scale (mRS on day 30. Statistical Analysis Used: Statistical analysis was performed using the SPSS software for windows version 15.0 (SPSS. Results: A positive correlation was found between concentration of NSE on day 1 and infarct volume determined by CT scan (r = 0.955, P < 0.001. A strong negative correlation was found between GCS at presentation and concentration of NSE on day 1 (r = −0.806, P < 0.001. There was a positive correlation between NSE levels at day 1 and functional neurological outcome assessed by mRS at day 30 (r = 0.744, P < 0.001. Conclusions: Serum levels of NSE in first few days of ischemic stroke can serve as a useful marker to predict stroke severity and early functional outcome. However, larger studies with serial estimation of NSE are needed to establish these observations more firmly.

  18. Adult Hip Flexion Contracture due to Neurological Disease: A New Treatment Protocol—Surgical Treatment of Neurological Hip Flexion Contracture

    Alberto Nicodemo

    2014-01-01

    Full Text Available Congenital, traumatic, or extrinsic causes can lead people to paraplegia; some of these are potentially; reversible and others are not. Paraplegia can couse hip flexion contracture and, consequently, pressure sores, scoliosis, and hyperlordosis; lumbar and groin pain are strictly correlated. Scientific literature contains many studies about children hip flexion related to neurological diseases, mainly caused by cerebral palsy; only few papers focus on this complication in adults. In this study we report our experience on surgical treatment of adult hip flexion contracture due to neurological diseases; we have tried to outline an algorithm to choose the best treatment avoiding useless or too aggressive therapies. We present 5 cases of adult hips flexion due to neurological conditions treated following our algorithm. At 1-year-follow-up all patients had a good clinical outcome in terms of hip range of motion, pain and recovery of walking if possible. In conclusion we think that this algorithm could be a good guideline to treat these complex cases even if we need to treat more patients to confirm this theory. We believe also that postoperation physiotherapy it is useful in hip motility preservation, improvement of muscular function, and walking ability recovery when possible.

  19. A case-control study of the prevalence of neurological diseases in inflammatory bowel disease (IBD).

    Gondim, Francisco de Assis Aquino; Oliveira, Gisele Ramos de; Teles, Benedito Cadorno V; Souza, Marcellus H L P; Braga, Lucia L B C; Messias, Erick L

    2015-02-01

    Neurological diseases are common in inflammatory bowel disease (IBD) patients, but their exact prevalence is unknown. Method We prospectively evaluated the presence of neurological disorders in 121 patients with IBD [51 with Crohn's disease (CD) and 70 with ulcerative colitis (UC)] and 50 controls (gastritis and dyspepsia) over 3 years. Results Our standard neurological evaluation (that included electrodiagnostic testing) revealed that CD patients were 7.4 times more likely to develop large-fiber neuropathy than controls (p = 0.045), 7.1 times more likely to develop any type of neuromuscular condition (p = 0.001) and 5.1 times more likely to develop autonomic complaints (p = 0.027). UC patients were 5 times more likely to develop large-fiber neuropathy (p = 0.027) and 3.1 times more likely to develop any type of neuromuscular condition (p = 0.015). Conclusion In summary, this is the first study to prospectively establish that both CD and UC patients are more prone to neuromuscular diseases than patients with gastritis and dyspepsia.

  20. A Case-Control study of the prevalence of neurological diseases in inflammatory bowel disease (IBD

    Francisco de Assis Aquino Gondim

    2015-02-01

    Full Text Available Neurological diseases are common in inflammatory bowel disease (IBD patients, but their exact prevalence is unknown. Method We prospectively evaluated the presence of neurological disorders in 121 patients with IBD [51 with Crohn's disease (CD and 70 with ulcerative colitis (UC] and 50 controls (gastritis and dyspepsia over 3 years. Results Our standard neurological evaluation (that included electrodiagnostic testing revealed that CD patients were 7.4 times more likely to develop large-fiber neuropathy than controls (p = 0.045, 7.1 times more likely to develop any type of neuromuscular condition (p = 0.001 and 5.1 times more likely to develop autonomic complaints (p = 0.027. UC patients were 5 times more likely to develop large-fiber neuropathy (p = 0.027 and 3.1 times more likely to develop any type of neuromuscular condition (p = 0.015. Conclusion In summary, this is the first study to prospectively establish that both CD and UC patients are more prone to neuromuscular diseases than patients with gastritis and dyspepsia.

  1. 应用简化急性生理评分Ⅱ预测危重神经疾病患者死亡概率%Predicting possibility of mortality in critically ill patients with neurological diseases by using Simplified Acute Physiology Score Ⅱ

    马联胜; 宿英英; 李霞; 刘甜甜; 陈卫碧

    2010-01-01

    Objective To evaluate feasibility and reliability of using Simplified Acute Physiology Score Ⅱ (SAPS Ⅱ)in predicting mortality in critically ill patients with neurological diseases.Methods All 653 patients hospitalized in neurological intensive care unit (N-ICU) from Jan 2005 to Dec 2007 were retrospectively studied.SAPSⅡ scores were scaled upon admission at 24,48 and 72 h,and possibility of hospital mortality (PHM) was calculated based on SAPS Ⅱ score.Neurological diseases diagnosis made initially at time of hospitalization was classified into five categories:intracranial hemorrhage,cerebral infarction,neurologic infection,neuromuscular diseases and other neurologic diseases.At each of four time points,the SAPSⅡscores were compared between the survival group and death group,and the relationship of SAPS Ⅱ score and prognosis were analyzed.The calibration of the SAPS Ⅱ were accessed with the Hosmer-Lemeshow goodness-of-fit chi-squared statistic and the discrimination with area under the receiver operating characteristic curve (AUROC).Multivariate logistic regression was used to analyze the relationship between disease category and prognosis.Results SAPS Ⅱ scores in the death group (46.53±12.22,47.28±13.84,48.58±14.18 and 49.06±14.61)at each time point were significantly higher than those in the survival group (34.70±11.78,30.28±12.24,29.79±12.36 and 29.69±12.96;t=11.12,14.02,14.43 and 13.49 at 0,24,48 and 72 h,respectively,P<0.01).Furthermore,univariate logistic regression analyses demonstrated that SAPS Ⅱ score was correlated with prognosis (OR=1.080,1.100,1.109,1.100 at 0,24,48 and 72 h,respectively,P<0.01).The scores at 48 and 72 h were more accurate in predicting mortality.SAPS Ⅱ had good calibration at each time points (x2=5.305,7.557,6.369 and 8.540,P>0.05),however,the consistency of expected mortality with observed mortality was satisfactory only at 48 and 72 h(82.6%,83.4%),so was the discrimination ( AUROC=0

  2. Posterior reversible encephalopathy syndrome (PRES, an acute neurological syndrome due to reversible multifactorial brain edema: a case report

    Camilla Cicognani

    2013-04-01

    Full Text Available Background: The essential features of Posterior Reversible Encephalopathy Syndrome (PRES are headache, mental changes, seizures, visual symptoms and often arterial hypertension. Brain RMN typically shows cortico-sottocortical parieto-occipital edema, with a bilateral and symmetric distribution. PRES develops in clinical conditions as hypertensive encephalopathy, preeclampsia/ eclampsia, autoimmune diseases, after transplantation, infections and as an adverse effect of immunosuppressive drugs or chemotherapy. It usually completely reverses with treatment, although permanent sequelae are possible in case of delayed or missed diagnosis. Case report: We describe the case of a transsexual (M!F and tetraplegic patient, admitted for neck and low back pain. She suddenly developed headache, confusion, seizures and severe hypertension with normal blood tests. RMN showed multiple cortico-sottocortical areas of vasogenic and citotoxic edema in temporo-occipital, parietal, frontal, and cerebellar regions. Soon after the beginning of the antihypertensive therapy, clinical recovery was observed, as well as the disappearance of edema at RMN. Discussion and conclusions: Although PRES is usually associated with definite pathological conditions, it is not always the case, as was for the patient here described, who had no predisposing factors in her past clinical history, and presented hypertension only in the acute phase of the syndrome. Since, moreover, PRES usually presents with acute non specific features and it can be misdiagnosed with other serious diseases, the clinician will be helped by the knowledge of this syndrome to promptly start diagnostic workup and treatments, and avoid permanent neurological deficits.

  3. [Case presentation with critical analysis of Maurice Ravel's neurological disease].

    Baeck, E

    2001-01-01

    In the last 4 years of his life, the famous French composer Maurice Ravel (1875-1937) was afflicted with a progressive neurological illness that destroyed his artistic realization but preserved his musical sensibility and judgment. He died after craniotomy. An attempt has been made to compare conflicting testimonies and to correct erroneous accounts of the events in order to refine the differential diagnosis of Ravel's presenile decline. Moreover, in the last decades new neurological entities have been described which prompted an update on the exact nature of his illness. The composer probably suffered from corticobasal degeneration. However, in the absence of a post-mortem examination, the diagnosis must remain speculative.

  4. Is Further Examination Necessary in Patients with Behcets Disease Without Any Neurological Signs or Symptoms?

    Halit YAsAR

    2015-09-01

    Conclusion: Visually evoked potential examination may be used as a conductive method to detect the subclinical neurological pathologies in Behcets disease. The possible silent neurological involvement should be evaluated with further neuro-screening methods. [Dis Mol Med 2015; 3(3.000: 29-34

  5. Predictors of major neurological improvement after intravenous thrombolysis in acute ischemic stroke: A hospital-based study from south India

    Boddu Demudu

    2010-01-01

    Full Text Available Background : Despite the increasing use of recombinant tissue plasminogen activator (rt-PA in acute ischemic stroke, uncertainty persists about the short- and long-term outcome of the thrombolysed patients. Objective : To identify predictors of major neurological improvement at 24 h after intravenous rt-PA administration in patients of acute ischemic stroke and their relationship with outcome at 12 months. Materials and Methods : We analyzed the data of the patients with acute ischemic stroke treated as per the National Institute of Neurological Disorders and Stroke (NINDS criteria with intravenous rt-PA between January 2000 and June 2009 at a tertiary care center in south India. Major neurological improvement was defined by an 8-point improvement in National Institute of Health Stroke Scale (NIHSS score or an NIHSS score of 0 or 1 at 24 h. Good outcome was defined as a 12-month modified Rankin Scale (mRS of 0 to 1. Results : Of the 72 patients with acute ischemic stroke treated with intravenous rt-PA, 23 (32% patients had major neurological improvement at 24 h. Age <60 years (OR 1.9, 95% CI 1.7 to3.2, admission glucose levels <8 mmol/L (OR 3.87, 95% CI 1.9 to 9.2 and mild to moderate baseline stroke severity (NIHSS median score 10+ 6 were associated with major neurological improvement after adjusting for co variables. Major neurological improvement at 24 h was an independent predictor of good outcome (mRS=1 at 12 months (OR 13.9, 95% CI 6.84 to 40.2. Conclusions : Age <60 years, glucose levels <8 mmol/L and mild to moderate stroke severity (NIHSS median score 10±6 was associated with major neurological improvement after intravenous rt-PA. Major neurological improvement at 24 h after the administration of intravenous thrombolysis independently predicted good outcome at 12 months.

  6. Aggressive and acute periodontal diseases.

    Albandar, Jasim M

    2014-06-01

    Inflammatory periodontal diseases are highly prevalent, although most of these diseases develop and progress slowly, often unnoticed by the affected individual. However, a subgroup of these diseases include aggressive and acute forms that have a relatively low prevalence but show a rapid-course, high rate of progression leading to severe destruction of the periodontal tissues, or cause systemic symptoms that often require urgent attention from healthcare providers. Aggressive periodontitis is an early-onset, destructive disease that shows a high rate of periodontal progression and distinctive clinical features. A contemporary case definition of this disease is presented. Population studies show that the disease is more prevalent in certain geographic regions and ethnic groups. Aggressive periodontitis is an infectious disease, and recent data show that in affected subjects the subgingival microbiota is composed of a mixed microbial infection, with a wide heterogeneity in the types and proportions of microorganisms recovered. Furthermore, there are significant differences in the microbiota of the disease among different geographic regions and ethnicities. There is also evidence that the Aggregatibacter actinomycetemycomitans-JP2 clone may play an important role in the development of the disease in certain populations. The host response plays an important role in the susceptibility to aggressive periodontitis, where the immune response may be complex and involve multiple mechanisms. Also, genetic factors seem to play an important role in the pathogenesis of this disease, but the mechanisms of increased susceptibility are complex and not yet fully understood. The available data suggest that aggressive periodontitis is caused by mutations either in a few major genes or in multiple small-effect genes, and there is also evidence of gene-gene and gene-environment interaction effects. Diagnostic methods for this disease, based on a specific microbiologic, immunologic or

  7. Managing dysphagia. Special problems in patients with neurologic disease.

    Horner, J; Massey, E W

    1991-04-01

    Swallowing is a brief but intricate process. When this process is interrupted, as in patients with neurologic disorders, problems such as aspiration and risk of malnutrition can occur. The authors of this article discuss an individualized approach to evaluation and management of neurogenic oropharyngeal dysphagia. Three cases illustrate the diversity of causes, signs and symptoms, and clinical course.

  8. Cerebrolysin effects on neurological outcomes and cerebral blood flow in acute ischemic stroke

    Amiri-Nikpour MR

    2014-12-01

    Full Text Available Mohammad Reza Amiri-Nikpour,1 Surena Nazarbaghi,1 Babak Ahmadi-Salmasi,1 Tayebeh Mokari,2 Urya Tahamtan,2 Yousef Rezaei3 1Department of Neurology, Imam Khomeini Hospital, 2School of Medicine, 3Seyyed-al-Shohada Heart Center, Urmia University of Medical Sciences, Urmia, Iran Background: Cerebrolysin, a brain-derived neuropeptide, has been shown to improve the neurological outcomes of stroke, but no study has demonstrated its effect on cerebral blood flow. This study aimed to determine the cerebrolysin impact on the neurological outcomes and cerebral blood flow. Methods: In a randomized, double-blinded, placebo-controlled trial, 46 patients who had acute focal ischemic stroke were randomly assigned into two groups to receive intravenously either 30 mL of cerebrolysin diluted in normal saline daily for 10 days (n=23 or normal saline alone (n=23 adjunct to 100 mg of aspirin daily. All patients were examined using the National Institutes of Health Stroke Scale and transcranial Doppler to measure the mean flow velocity and pulsatility index (PI of their cerebral arteries at baseline as well as on days 30, 60, and 90. Results: The patients’ mean age was 60±9.7 years, and 51.2% of patients were male. The National Institutes of Health Stroke Scale was significantly lower in the cerebrolysin group compared with the placebo group on day 60 (median 10, interquartile range 9–11, P=0.008 and day 90 (median 11, interquartile range 10–13.5, P=0.001. The median of PI in the right middle cerebral artery was significantly lower in the cerebrolysin group compared with the placebo group on days 30, 60, and 90 (P<0.05. One patient in the cerebrolysin group and two patients in the placebo group died before day 30 (4.3% versus 8.7%. Conclusion: Cerebrolysin can be useful to improve the neurological outcomes and the PI of middle cerebral artery in patients with acute focal ischemic stroke. Keywords: ischemic stroke, cerebrolysin, neuroprotection, NIHSS, mean

  9. The protective and therapeutic function of small heat shock proteins in neurological diseases.

    Brownell, Sara E; Becker, Rachel A; Steinman, Lawrence

    2012-01-01

    Historically, small heat shock proteins (sHSPs) have been extensively studied in the context of being intracellular molecular chaperones. However, recent studies looking at the role of sHSPs in neurological diseases have demonstrated a near universal upregulation of certain sHSPs in damaged and diseased brains. Initially, it was thought that sHSPs are pathological in these disease states because they are found in the areas of damage. However, transgenic overexpression and exogenous administration of sHSPs in various experimental disease paradigms have shown just the contrary - that sHSPs are protective, not pathological. This review examines sHSPs in neurological diseases and highlights the potential for using these neuroprotective sHSPs as novel therapeutics. It first addresses the endogenous expression of sHSPs in a variety of neurological disorders. Although many studies have examined the expression of sHSPs in neurological diseases, there are no review articles summarizing these data. Furthermore, it focuses on recent studies that have investigated the therapeutic potential of sHSPs for neurological diseases. Finally, it will explain what we think is the function of endogenous sHSPs in neurological diseases.

  10. Acute flaccid paraplegia:neurological approach, diagnostic workup, and therapeutic options

    Gentian Vyshka; Altin Kuqo; Serla Grabova; Eris Ranxha; Liro Buda; Jera Kruja

    2015-01-01

    Acute flaccid paraplegia is a clinical occurrence with extreme importance, due to the dramatic presentation, the severity of the underlying disorder, and the generally poor prognosis that follows such a condition. Among etiological factors, the traumatic events are of particular interest, with the clinical treating dealing with a severely ill patient, following fall from height, motor vehicle collisions, and direct shocks applied over the vertebral column. The non-traumatic list is more numerous;however the severity of the acute paraplegia is not necessarily of a lesser degree. Viral infections, autoimmune disorders, and ischemic events involving feeding spinal arteries have been imputed. However, chemical and medications injected during procedures or accidentally intrathecal administration can produce acute flaccid paraplegia. A careful neurological assessment and complete electrophysiological and imaging studies must follow. In spite of the poor prognosis, different therapeutic options have been proposed and applied. Neurosurgical and orthopedic interventions are often necessary when trauma is present, with high dose glucocorticoids treatment preceding the intervention, in a hope to decrease edema-related compression over the spinal cord. Immunoglobulins and plasmapheresis are logical and helpful options when a polyradiculoneuritis produces such a clinical picture. The role of decompression, as neurosurgical exclusivity, has been considered as well.

  11. Exposure to lipophilic chemicals as a cause of neurological impairments, neurodevelopmental disorders and neurodegenerative diseases

    Zeliger, Harold I.

    2013-01-01

    Many studies have associated environmental exposure to chemicals with neurological impairments (NIs) including neuropathies, cognitive, motor and sensory impairments; neurodevelopmental disorders (NDDs) including autism and attention deficit hyperactivity disorder (ADHD); neurodegenerative diseases (NDGs) including Alzheimer′s disease, Parkinson's disease and amyotrophic lateral sclerosis (ALS). The environmental chemicals shown to induce all these diseases include persistent organic pollutan...

  12. Minds on replay: musical hallucinations and their relationship to neurological disease.

    Golden, Erin C; Josephs, Keith A

    2015-12-01

    The phenomenon of musical hallucinations, in which individuals perceive music in the absence of an external auditory stimulus, has been described sparingly in the literature through small case reports and series. Musical hallucinations have been linked to multiple associated conditions, including psychiatric and neurologic disease, brain lesions, drug effect, and hearing impairment. This study aimed to review the demographics of subjects with musical hallucinations and to determine the prevalence of neurological disorders, particularly neurodegenerative disease. Through the Mayo medical record, 393 subjects with musical hallucinations were identified and divided into five categories based on comorbid conditions that have been associated with musical hallucinations: neurological, psychiatric, structural, drug effect and not otherwise classifiable. Variables, including hearing impairment and the presence of visual and other auditory hallucinations, were evaluated independently in all five groups. The mean age at onset of the hallucinations was 56 years, ranging from 18 to 98 years, and 65.4% of the subjects were female. Neurological disease and focal brain lesions were found in 25% and 9% of the total subjects, respectively. Sixty-five subjects were identified with a neurodegenerative disorder, with the Lewy body disorders being the most common. Visual hallucinations were more common in the group with neurological disease compared to the psychiatric, structural, and not otherwise classifiable groups (P neurological disease and brain lesions represent a substantial proportion, and that Lewy body disorders are the most commonly associated neurodegenerative diseases. A future prospective study would be helpful to further delineate an association between musical hallucinations and neurodegenerative disease.

  13. KEGG DISEASE / Acute myeloid leukemia (AML) [KEGG DISEASE

    Full Text Available DISEASE: H00003 Entry H00003Disease Name Acute myeloid leukemia (AML) Description Acute.... Category Cancer Brite Human diseases [BR:br08402] Cancers Cancers of haematopoietic and lymphoid tissues H00003Acute...atopoietic and related tissue C92Myeloid leukaemia H00003Acute myeloid leukemia (AML) Cancer-accociated carb...ohydrates [br08441.html] H00003 Pathway hsa05221Acute myeloid leukemiahsa05202Transcriptional misregulation ... or t(16; 16)(p13, q22), (CBF-beta/MYH11) ICD-O: 9866/3, Tumor type: Acute promyelocytic leukaemia (AML with

  14. Cerebrospinal fluid proteome of patients with acute Lyme disease.

    Angel, Thomas E; Jacobs, Jon M; Smith, Robert P; Pasternack, Mark S; Elias, Susan; Gritsenko, Marina A; Shukla, Anil; Gilmore, Edward C; McCarthy, Carol; Camp, David G; Smith, Richard D; Warren, H Shaw

    2012-10-05

    During acute Lyme disease, bacteria can disseminate to the central nervous system (CNS), leading to the development of meningitis and other neurologic symptoms. Here we have analyzed pooled cerebrospinal fluid (CSF) allowing a deep view into the proteome for patients diagnosed with early disseminated Lyme disease and CSF inflammation. Additionally, we analyzed individual patient samples and quantified differences in protein abundance employing label-free quantitative mass spectrometry-based methods. We identified 108 proteins that differ significantly in abundance in patients with acute Lyme disease from controls. Comparison between infected patients and control subjects revealed differences in proteins in the CSF associated with cell death localized to brain synapses and others that likely originate from brain parenchyma.

  15. Hippotherapy acute impact on heart rate variability non-linear dynamics in neurological disorders.

    Cabiddu, Ramona; Borghi-Silva, Audrey; Trimer, Renata; Trimer, Vitor; Ricci, Paula Angélica; Italiano Monteiro, Clara; Camargo Magalhães Maniglia, Marcela; Silva Pereira, Ana Maria; Rodrigues das Chagas, Gustavo; Carvalho, Eliane Maria

    2016-05-15

    Neurological disorders are associated with autonomic dysfunction. Hippotherapy (HT) is a therapy treatment strategy that utilizes a horse in an interdisciplinary approach for the physical and mental rehabilitation of people with physical, mental and/or psychological disabilities. However, no studies have been carried out which evaluated the effects of HT on the autonomic control in these patients. Therefore, the objective of the present study was to investigate the effects of a single HT session on cardiovascular autonomic control by time domain and non-linear analysis of heart rate variability (HRV). The HRV signal was recorded continuously in twelve children affected by neurological disorders during a HT session, consisting in a 10-minute sitting position rest (P1), a 15-minute preparatory phase sitting on the horse (P2), a 15-minute HT session (P3) and a final 10-minute sitting position recovery (P4). Time domain and non-linear HRV indices, including Sample Entropy (SampEn), Lempel-Ziv Complexity (LZC) and Detrended Fluctuation Analysis (DFA), were calculated for each treatment phase. We observed that SampEn increased during P3 (SampEn=0.56±0.10) with respect to P1 (SampEn=0.40±0.14, p<0.05), while DFA decreased during P3 (DFA=1.10±0.10) with respect to P1 (DFA=1.26±0.14, p<0.05). A significant SDRR increase (p<0.05) was observed during the recovery period P4 (SDRR=50±30ms) with respect to the HT session period P3 (SDRR=30±10ms). Our results suggest that HT might benefit children with disabilities attributable to neurological disorders by eliciting an acute autonomic response during the therapy and during the recovery period.

  16. Acute axonal polyneuropathy with predominant proximal involvement: an uncommon neurological complication of bariatric surgery

    Machado Flavia Costa Nunes

    2006-01-01

    Full Text Available Bariatric surgery is frequently indicated in the treatment of morbid obesity. Previously unreported complications have been associated to this surgery; among them, neurological complications have gained attention. We report the case of a 25-year-old man submitted to gastric surgery for treatment of morbid obesity who developed, two months after surgery, acute proximal weakness in lower limbs. The electroneuromyography revealed axonal peripheral polyneuropathy with predominant proximal involvement. After treatment with immunoglobulin and vitamin supplementation, rapid clinical and neurophysiologic recovery was observed. We describe the clinical and electroneuromyographic features of this case, stressing the difficulty of initial diagnosis, particularly in the differential diagnosis with Guillain-Barré syndrome. We discuss the importance of nutritional follow-up and the eventual indication of routine vitamin supplementation in these patients.

  17. Acute axonal polyneuropathy with predominant proximal involvement: an uncommon neurological complication of bariatric surgery.

    Machado, Flavia Costa Nunes; Valério, Berenice Cataldo Oliveira; Morgulis, Roberto Naun Franco; Nunes, Karlo Faria; Mazzali-Verst, Sílvia

    2006-09-01

    Bariatric surgery is frequently indicated in the treatment of morbid obesity. Previously unreported complications have been associated to this surgery; among them, neurological complications have gained attention. We report the case of a 25-year-old man submitted to gastric surgery for treatment of morbid obesity who developed, two months after surgery, acute proximal weakness in lower limbs. The electroneuromyography revealed axonal peripheral polyneuropathy with predominant proximal involvement. After treatment with immunoglobulin and vitamin supplementation, rapid clinical and neurophysiologic recovery was observed. We describe the clinical and electroneuromyographic features of this case, stressing the difficulty of initial diagnosis, particularly in the differential diagnosis with Guillain-Barré syndrome. We discuss the importance of nutritional follow-up and the eventual indication of routine vitamin supplementation in these patients.

  18. Urgent carotid endarterectomy in patients with acute neurological ischemic events within six hours after symptoms onset.

    Gajin, P; Radak, Dj; Tanaskovic, S; Babic, S; Nenezic, D

    2014-06-01

    To analyze the outcome of urgent carotid endarterectomy (CEA) performed within less than six hours in patients with crescendo transient ischemic attack (TIA) and stroke in progression. From January 1998 to December 2008, 58 urgent CEAs were done for acute neurological ischemic events--46 patients with crescendo TIA and 12 patients with stroke in progression. Brain computed tomography (CT) was done prior and after the surgery. Disability level was assessed prior to and after urgent CEA using modified Rankin scale. Median follow-up was 42.1 ± 16.6 months. In the early postoperative period stroke rate was 0% for the patients in crescendo TIA group while in patients with stroke in progression group 3 patients (25%) had positive postoperative brain CT, yet neurological status significantly improved. Mid-term stroke rate was 2.2% in crescendo TIA group and 8.3% in stroke in progression group. In the early postoperative period there were no lethal outcomes, mid-term mortality was 8.3% in stroke in progression while in crescendo TIA group lethal outcomes were not observed. In conclusion, based on our results urgent CEA is a safe and effective treatment option for patients with crescendo TIA and stroke in progression with acceptable rate of postoperative complications.

  19. Systems biological approach on neurological disorders: a novel molecular connectivity to aging and psychiatric diseases

    Santosh Winkins

    2011-01-01

    Full Text Available Abstract Background Systems biological approach of molecular connectivity map has reached to a great interest to understand the gene functional similarities between the diseases. In this study, we developed a computational framework to build molecular connectivity maps by integrating mutated and differentially expressed genes of neurological and psychiatric diseases to determine its relationship with aging. Results The systematic large-scale analyses of 124 human diseases create three classes of molecular connectivity maps. First, molecular interaction of disease protein network generates 3632 proteins with 6172 interactions, which determines the common genes/proteins between diseases. Second, Disease-disease network includes 4845 positively scored disease-disease relationships. The comparison of these disease-disease pairs with Medical Subject Headings (MeSH classification tree suggests 25% of the disease-disease pairs were in same disease area. The remaining can be a novel disease-disease relationship based on gene/protein similarity. Inclusion of aging genes set showed 79 neurological and 20 psychiatric diseases have the strong association with aging. Third and lastly, a curated disease biomarker network was created by relating the proteins/genes in specific disease contexts, such analysis showed 73 markers for 24 diseases. Further, the overall quality of the results was achieved by a series of statistical methods, to avoid insignificant data in biological networks. Conclusions This study improves the understanding of the complex interactions that occur between neurological and psychiatric diseases with aging, which lead to determine the diagnostic markers. Also, the disease-disease association results could be helpful to determine the symptom relationships between neurological and psychiatric diseases. Together, our study presents many research opportunities in post-genomic biomarkers development.

  20. Liver cirrhosis in patients newly diagnosed with neurological phenotype of Wilson's disease.

    Przybyłkowski, Adam; Gromadzka, Grażyna; Chabik, Grzegorz; Wierzchowska, Agata; Litwin, Tomasz; Członkowska, Anna

    2014-01-01

    Wilson's disease (WD) can manifest itself in different clinical forms, the neurological and hepatic ones being the most common. It is suggested that neurological signs and psychiatric symptoms develop secondary to liver involvement. The aim of this study was to characterize the liver disease in patients newly diagnosed with the neurological form of WD. Treatment-naive patients diagnosed with WD were classified into three phenotypic groups: hepatic, neurological and pre-symptomatic. Liver involvement was ascertained through surrogate markers: abdominal ultrasound and laboratory parameters. In addition, study participants were screened for esophageal varices. Of 53 consecutively diagnosed WD patients, 23 individuals (43.4%) had a predominantly neurological presentation. In this group, cirrhosis was diagnosed in 11 (47.8%) subjects. Esophageal varices were present in all of them. In every patient with neurological WD, there was at least one sign of hepatic disease on ultrasound examination, indicating universal presence of liver involvement. The prevalence of surrogate signs of cirrhosis was similar in patients with the neurological and in those with the hepatic phenotype.

  1. Focus on acute diarrhoeal disease

    Fabio Baldi; Maria Antonia Bianco; Gerardo Nardone; Alberto Pilotto; Emanuela Zamparo

    2009-01-01

    Diarrhoea is an alteration of normal bowel movement characterized by an increase in the water content,volume, or frequency of stools. Diarrhoea needs to be classified according to the trends over time (acute or chronic) and to the characteristics of the stools (watery, fatty, inflammatory). Secretory diarrhoeas,mostly acute and of viral aetiology in more than 70% of cases, are by far the most important subtype of diarrhoeas in terms of frequency, incidence and mortality (over 2.5 million deaths/year in developing countries). Natural and synthetic opiates such as morphine, codeine, and loperamide which react with endogenous opiates (enkephalins, beta-endorphins,dynorphins) mainly act on intestinal motility and slow down transit. An antidiarrhoeal drug developed in recent years, racecadotril, acts as an enkephalinase inhibitor.Clinical studies have shown that it is just as effective as loperamide in resolving acute diarrhoea but with greater reduction in pain and abdominal distension.Some studies have explored the prevalence of diarrhoea in old age. An epidemiological study carried out in Italy by 133 General Practitioners on 5515 elderly outpatients reported a prevalence of diarrhoea, defined according to the Rome criteria, of 9.1%. Infectious diseases (19%) and drug use (16%) were the most commoncauses of diarrhoea in old age. Regardless of the cause,the treatment of elderly patients with diarrhoea must include rehydration and nutritional support. Every year,more than 50 million tourists travel from industrialized countries to places where hygiene levels are poor. At least 75% of those travelling for short periods mention health problems, and in particular traveller's diarrhoea.

  2. Regulations in the United States for cell transplantation clinical trials in neurological diseases

    He Zhu; Yuanqing Tan; Qi Gu; Weifang Han; Zhongwen Li; Jason S Meyer; Baoyang Hu

    2015-01-01

    Objective: This study aimed to use a systematic approach to evaluate the current utilization, safety, and effectiveness of cell therapies for neurological diseases in human. And review the present regulations, considering United States (US) as a representative country, for cell transplantation in neurological disease and discuss the challenges facing the field of neurology in the coming decades. Methods:A detailed search was performed in systematic literature reviews of cellular‐based therapies in neurological diseases, using PubMed, web of science, and clinical trials. Regulations of cell therapy products used for clinical trials were searched from the Food and Drug Administration (FDA) and the National Institutes of Health (NIH). Results: Seven most common types of cell therapies for neurological diseases have been reported to be relatively safe with varying degrees of neurological recovery. And a series of regulations in US for cellular therapy was summarized including preclinical evaluations, sourcing material, stem cell manufacturing and characterization, cell therapy product, and clinical trials. Conclusions:Stem cell‐based therapy holds great promise for a cure of such diseases and will value a growing population of patients. However, regulatory permitting activity of the US in the sphere of stem cells, technologies of regenerative medicine and substitutive cell therapy are selective, theoretical and does not fit the existing norm and rules. Compiled well‐defined regulations to guide the application of stem cell products for clinical trials should be formulated.

  3. Acute neurological signs as the predominant clinical manifestation in four dogs with Angiostrongylus vasorum infections in Denmark

    Pors Susanne E

    2011-06-01

    Full Text Available Abstract Four dogs with acute neurological signs caused by haemorrhages in the central nervous system were diagnosed with Angiostrongylus vasorum infection as the underlying aetiology. Two dogs presented with brain lesions, one dog with spinal cord lesions and one with lesions in both the brain and spinal cord. Only one dog presented with concurrent signs of classical pulmonary angiostrongylosis (respiratory distress, cough, and only two dogs displayed overt clinical signs of haemorrhages. Results of coagulation assays were inconsistent. Neurological signs reflected the site of pathology and included seizures, various cranial nerve deficits, vestibular signs, proprioceptive deficits, ataxia and paraplegia. One dog died and three were euthanised due to lack of improvement despite medical treatment. This emphasises canine angiostrongylosis as a potential cause of fatal lesions of the central nervous system and the importance of including A. vasorum as a differential diagnosis in young dogs with acute neurological signs in Denmark.

  4. Acute intermittent porphyria with syndrome of inappropriate antidiuretic hormone secretion (SIADH and neurological crisis, successfully treated with haemodialysis

    P. S. Singh

    2014-04-01

    Full Text Available We report a 35 years old male, a case of Acute Intermittent Porphyria (AIP with Syndrome of Inappropriate Antidiuretic Hormone secretion (SIADH and neurological crisis for its rarity. Since specific parenteral medication (hemin was not available, patient was empirically treated with haemodialysis with satisfactory outcome. [Int J Res Med Sci 2014; 2(2.000: 795-797

  5. Is acute recurrent pancreatitis a chronic disease?

    Mariani, Alberto; Testoni, Pier Alberto

    2008-01-01

    Whether acute recurrent pancreatitis is a chronic disease is still debated and a consensus is not still reached as demonstrated by differences in the classification of acute recurrent pancreatitis. There is major evidence for considering alcoholic pancreatitis as a chronic disease ab initio while chronic pancreatitis lesions detectable in biliary acute recurrent pancreatitis (ARP) seem a casual association. Cystic fibrosis transmembrane conductance regulator (CFTR) gene mutation, hereditary a...

  6. Red cell distribution width and neurological scoring systems in acute stroke patients

    Kara H

    2015-03-01

    Full Text Available Hasan Kara,1 Selim Degirmenci,1 Aysegul Bayir,1 Ahmet Ak,1 Murat Akinci,1 Ali Dogru,1 Fikret Akyurek,2 Seyit Ali Kayis3 1Department of Emergency Medicine, Faculty of Medicine, Selcuk University, Konya, Turkey; 2Department of Biochemistry, Faculty of Medicine, Selcuk University, Konya, Turkey; 3Department of Biostatistics, Faculty of Medicine, Karabuk University, Karabuk, Turkey Objectives: The purpose of the present study was to evaluate the association between the red blood cell distribution width (RDW and the Glasgow Coma Scale (GCS, Canadian Neurological Scale (CNS, and National Institutes of Health Stroke Scale (NIHSS scores in patients who had acute ischemic stroke. Methods: This prospective observational cohort study included 88 patients who have had acute ischemic stroke and a control group of 40 patients who were evaluated in the Emergency Department for disorders other than acute ischemic stroke. All subjects had RDW determined, and stroke patients had scoring with the GCS, CNS, and NIHSS scores. The GCS, CNS, and NIHSS scores of the patients were rated as mild, moderate, or severe and compared with RDW. Results: Stroke patients had significantly higher median RDW than control subjects. The median RDW values were significantly elevated in patients who had more severe rather than milder strokes rated with all three scoring systems (GCS, CNS, and NIHSS. The median RDW values were significantly elevated for patients who had moderate rather than mild strokes rated by GCS and CNS and for patients who had severe rather than mild strokes rated by NIHSS. The area under the receiver operating characteristic curve was 0.760 (95% confidence interval, 0.676–0.844. Separation of stroke patients and control groups was optimal with RDW 14% (sensitivity, 71.6%; specificity, 67.5%; accuracy, 70.3%. Conclusion: In stroke patients who have symptoms <24 hours, the RDW may be useful in predicting the severity and functional outcomes of the stroke

  7. Helicobacter pylori and neurological diseases: Married by the laws of inflammation.

    Alvarez-Arellano, Lourdes; Maldonado-Bernal, Carmen

    2014-11-15

    The purpose of this paper is to review current information about the role of inflammation caused by Helicobacter pylori (H. pylori) infection in neurological diseases such as Parkinson's disease, Alzheimer's disease, Guillain-Barré syndrome, multiple sclerosis, and other inflammatory diseases including ischemic stroke. Infection with H. pylori usually persists throughout life, resulting in a chronic inflammatory response with local secretion of numerous inflammatory mediators including chemokines [interleukin (IL)-8, macrophage chemotactic protein (MCP)-1, growth-regulated oncogene (GRO)-α] and cytokines [IL-1β, tumor necrosis factor (TNF)-α, IL-6, IL-12, interferon (IFN)-γ], which can pass into the circulation and have a systemic effect. The persistence of detectable systemic and local concentrations of inflammatory mediators is likely to alter the outcome of neurological diseases. These proinflammatory factors can induce brain inflammation and the death of neurons and could eventually be associated to Parkinson's disease and also may be involved in the development of Alzheimer's disease. However, most neurological diseases are the result of a combination of multiple factors, but the systemic inflammatory response is a common component and determinant in the onset, evolution, and outcome of diseases. However, more studies are needed to allow understanding of the effects and mechanisms by which the inflammatory response generated by H. pylori infection affects neurological diseases.

  8. Neurologic and Psychiatric Manifestations of Celiac Disease and Gluten Sensitivity

    Jackson, Jessica R.; Eaton, William W; Cascella, Nicola G.; Fasano, Alessio; Kelly, Deanna L.

    2012-01-01

    Celiac Disease (CD) is an immune-mediated disease dependent on gluten (a protein present in wheat, rye or barley) that occurs in about 1% of the population and is generally characterized by gastrointestinal complaints. More recently the understanding and knowledge of gluten sensitivity (GS), has emerged as an illness distinct from celiac disease with an estimated prevalence 6 times that of CD. Gluten sensitive people do not have villous atrophy or antibodies that are present in celiac disease...

  9. Iatrogenic neurology.

    Sposato, Luciano A; Fustinoni, Osvaldo

    2014-01-01

    Iatrogenic disease is one of the most frequent causes of hospital admissions and constitutes a growing public health problem. The most common type of iatrogenic neurologic disease is pharmacologic, and the central and peripheral nervous systems are particularly vulnerable. Despite this, iatrogenic disease is generally overlooked as a differential diagnosis among neurologic patients. The clinical picture of pharmacologically mediated iatrogenic neurologic disease can range from mild to fatal. Common and uncommon forms of drug toxicity are comprehensively addressed in this chapter. While the majority of neurologic adverse effects are listed and referenced in the tables, the most relevant issues are further discussed in the text.

  10. Reaction time of patients with Parkinson's disease, with reference to asymmetry of neurological signs.

    Yokochi, F. (Fusako); Nakamura, R; Narabayashi, H

    1985-01-01

    Electromyographic reaction times of the left and the right finger extensor muscles in extension movement of the wrist were examined in 42 patients with Parkinson's disease, and 20 normal subjects. Compared to the normal subjects and the patients with neurological signs confined to the right side, the patients with neurological signs on the left side or on both sides showed slowing of reaction times regardless of the side of responding hand. The patients with asymmetry of bilateral neurologica...

  11. Disease modeling and drug screening for neurological diseases using human induced pluripotent stem cells.

    Xu, Xiao-hong; Zhong, Zhong

    2013-06-01

    With the general decline of pharmaceutical research productivity, there are concerns that many components of the drug discovery process need to be redesigned and optimized. For example, the human immortalized cell lines or animal primary cells commonly used in traditional drug screening may not faithfully recapitulate the pathological mechanisms of human diseases, leading to biases in assays, targets, or compounds that do not effectively address disease mechanisms. Recent advances in stem cell research, especially in the development of induced pluripotent stem cell (iPSC) technology, provide a new paradigm for drug screening by permitting the use of human cells with the same genetic makeup as the patients without the typical quantity constraints associated with patient primary cells. In this article, we will review the progress made to date on cellular disease models using human stem cells, with a focus on patient-specific iPSCs for neurological diseases. We will discuss the key challenges and the factors that associated with the success of using stem cell models for drug discovery through examples from monogenic diseases, diseases with various known genetic components, and complex diseases caused by a combination of genetic, environmental and other factors.

  12. Disease modeling and drug screening for neurological diseases using human induced pluripotent stem cells

    Xiao-hong XU; Zhong ZHONG

    2013-01-01

    With the general decline of pharmaceutical research productivity,there are concerns that many components of the drug discovery process need to be redesigned and optimized.For example,the human immortalized cell lines or animal primary cells commonly used in traditional drug screening may not faithfully recapitulate the pathological mechanisms of human diseases,leading to biases in assays,targets,or compounds that do not effectively address disease mechanisms.Recent advances in stem cell research,especially in the development of induced pluripotent stem cell (iPSC) technology,provide a new paradigm for drug screening by permitting the use of human cells with the same genetic makeup as the patients without the typical quantity constraints associated with patient primary cells.In this article,we will review the progress made to date on cellular disease models using human stem cells,with a focus on patient-specific iPSCs for neurological diseases.We will discuss the key challenges and the factors that associated with the success of using stem cell models for drug discovery through examples from monogenic diseases,diseases with various known genetic components,and complex diseases caused by a combination of genetic,environmental and other factors.

  13. The pharmacology of neurotrophic treatment with Cerebrolysin: brain protection and repair to counteract pathologies of acute and chronic neurological disorders.

    Masliah, E; Díez-Tejedor, E

    2012-04-01

    Neurotrophic factors are considered as part of the therapeutic strategy for neurological disorders like dementia, stroke and traumatic brain injury. Cerebrolysin is a neuropeptide preparation which mimics the action of endogenous neurotrophic factors on brain protection and repair. In dementia models, Cerebrolysin decreases β-amyloid deposition and microtubule-associated protein tau phosphorylation by regulating glycogen synthase kinase-3β and cyclin-dependent kinase 5 activity, increases synaptic density and restores neuronal cytoarchitecture. These effects protect integrity of the neuronal circuits and thus result in improved cognitive and behavioral performance. Furthermore, Cerebrolysin enhances neurogenesis in the dentate gyrus, the basis for neuronal replacement therapy in neurodegenerative diseases. Experimental studies in stroke animal models have shown that Cerebrolysin stabilizes the structural integrity of cells by inhibition of calpain and reduces the number of apoptotic cells after ischemic lesion. Cerebrolysin induces restorative processes, decreases infarct volume and edema formation and promotes functional recovery. Stroke-induced neurogenesis in the subventricular zone was also promoted by Cerebrolysin, thus supporting the brain's self-repair after stroke. Both, traumatic brain and spinal cord injury conditions stimulate the expression of natural neurotrophic factors to promote repair and regeneration processes -axonal regeneration, neuronal plasticity and neurogenesis- that is considered to be crucial for the future recovery. Neuroprotective effects of Cerebrolysin on experimentally induced traumatic spinal cord injury have shown that Cerebrolysin prevents apoptosis of lesioned motoneurons and promotes functional recovery. This section summarizes the most relevant data on the pharmacology of Cerebrolysin obtained from in vitro assays (biochemical and cell cultures) and in vivo animal models of acute and chronic neurological disorders.

  14. The interplay between microRNAs and histone deacetylases in neurological diseases.

    Bourassa, Megan W; Ratan, Rajiv R

    2014-11-01

    Neurological conditions, such as Alzheimer's disease and stroke, represent a prevalent group of devastating illnesses with few treatments. Each of these diseases or conditions is in part characterized by the dysregulation of many genes, including those that code for microRNAs (miRNAs) and histone deacetylases (HDACs). Recently, a complex relationship has been uncovered linking miRNAs and HDACs and their ability to regulate one another. This provides a new avenue for potential therapeutics as the ability to reinstate a careful balance between miRNA and HDACs has lead to improved outcomes in a number of in vitro and in vivo models of neurological conditions. In this review, we will discuss recent findings on the interplay between miRNAs and HDACs and its implications for pathogenesis and treatment of neurological conditions, including amyotrophic lateral sclerosis, Alzheimer's disease, Huntington's disease and stroke.

  15. Effect of adjuvant argatroban therapy on neurological function, endothelial injury and inflammation state in patient with acute cerebral infarction

    Nan Che

    2016-01-01

    Objective:To analyze the effect of adjuvant argatroban therapy on neurological function, endothelial injury and inflammation state in patient with acute cerebral infarction.Methods:A total of 118 patients with acute cerebral infarction were divided into observation group and control group according to the random number table, control group received conventional treatment, observation group received argatroban + conventional treatment, and then differences in TCD cerebral blood flow, serum neurological function, endothelial injury and inflammatory marker levels were compared between two groups after treatment.Results:TCD MCA and ACA values of observation group after treatment were higher than those of control group (P<0.05); serum neurological function indexes copeptin, NT-proBNP, PAO and S-100B levels of observation group after treatment were lower than those of control group, endothelial injury index ET-1 level was lower than that of control group, NO and CGRP levels were higher than those of control group, and inflammatory markers hs-CRP, TNF-α, IL-6, MMP-9 and Lp-PLA2 levels were lower than those of control group (P<0.05).Conclusions:Adjuvant argatroban therapy can optimize the overall condition in patients with acute cerebral infarction, and plays a positive role in improving the neurological function, reducing endothelial injury and inflammation state, etc.

  16. Four-Stage Audit Demonstrating Increased Uptake of HIV Testing in Acute Neurology Admissions Using Staged Practical Interventions.

    Dilraj Singh Sokhi

    Full Text Available UK National Guidelines (UKNG advise HIV testing in clinically indicated neurological presentations. We audited the impact of our practical strategies to increase uptake of HIV testing at a regional acute neurology admissions unit.We audited HIV testing in 4 periods over 2 years: before we designed a UKNG-based "HIV testing in Neurology" protocol ("pre-protocol"; after dissemination of the protocol alone ("post-protocol"; post-protocol dissemination combined with both a tailored departmental admissions clerking proforma to prompt for HIV testing & consenting, and regular focussed tutorials to doctors on HIV testing in neurological patients ("post-proforma"; and finally one year after the post-proforma period ("+1 year". We also looked at the total number of HIV tests sent from the unit during the two-year period. We assessed significance using Fisher's exact test.47.8% of all acute neurology non-stroke admissions were eligible for HIV testing during all the audit periods. Testing rates were as follows: pre-protocol 21.9%; post-protocol 36.6%; post-proforma 83.3%; and at +1 year 65.4% (p<0.05 for both post-protocol and +1 year when compared to pre-protocol. Documentation of consent for HIV testing improved from 25% to 67.6% with the HIV-tailored clerking proforma. The total number of HIV tests requested from the unit doubled in the post-proforma period compared to pre-protocol (p<0.05.the combination of an HIV testing protocol, a tailored departmental clerking proforma and regular focussed teaching to doctors on indications for HIV testing led to a sustained increase in HIV testing uptake in our regional acute neurology admissions unit.

  17. Epidemiology of neurological diseases in elderly people: what did we learn from the Rotterdam Study?

    Hofman, A.; de Jong, P.T.V.M.; Duijn, C.M. van; Breteler, M.M.

    2006-01-01

    The Rotterdam Study is a prospective cohort study that has been ongoing since 1990 in the city of Rotterdam, the Netherlands, among 7983 people aged 55 years or older. One part of the study targets neurological diseases, others deal with cardiovascular, ophthalmological, and endocrine diseases. The

  18. Brain molecular aging, promotion of neurological disease and modulation by sirtuin 5 longevity gene polymorphism.

    Glorioso, Christin; Oh, Sunghee; Douillard, Gaelle Guilloux; Sibille, Etienne

    2011-02-01

    Mechanisms determining characteristic age-of-onset for neurological diseases are largely unknown. Normal brain aging associates with robust and progressive transcriptome changes ("molecular aging"), but the intersection with disease pathways is mostly uncharacterized. Here, using cross-cohort microarray analysis of four human brain areas, we show that neurological disease pathways largely overlap with molecular aging and that subjects carrying a newly-characterized low-expressing polymorphism in a putative longevity gene (Sirtuin5; SIRT5(prom2)) have older brain molecular ages. Specifically, molecular aging was remarkably conserved across cohorts and brain areas, and included numerous developmental and transcription-regulator genes. Neurological disease-associated genes were highly overrepresented within age-related genes and changed almost unanimously in pro-disease directions, together suggesting an underlying genetic "program" of aging that progressively promotes disease. To begin testing this putative pathway, we developed and used an age-biosignature to assess five candidate longevity gene polymorphisms' association with molecular aging rates. Most robustly, aging was accelerated in cingulate, but not amygdala, of subjects carrying a SIRT5 promoter polymorphism (+9 years, p=0.004), in concordance with cingulate-specific decreased SIRT5 expression. This effect was driven by a set of core transcripts (+24 years, p=0.0004), many of which were mitochondrial, including Parkinson's disease genes, PINK-1 and DJ-1/PARK7, hence suggesting that SIRT5(prom2) may represent a risk factor for mitochondrial dysfunction-related diseases, including Parkinson's, through accelerated molecular aging of disease-related genes. Based on these results we speculate that a "common mechanism" may underlie age-of-onset across several neurological diseases. Confirming this pathway and its regulation by common genetic variants would provide new strategies for predicting, delaying, and

  19. Blood-brain interfaces and bilirubin-induced neurological diseases.

    Ghersi-Egea, J F; Gazzin, S; Strazielle, N

    2009-01-01

    The endothelium of the brain microvessels and the choroid plexus epithelium form highly specialized cellular barriers referred to as blood-brain interfaces through which molecular exchanges take place between the blood and the neuropil or the cerebrospinal fluid, respectively. Within the brain, the ependyma and the pia-glia limitans modulate exchanges between the neuropil and the cerebrospinal fluid. All these interfaces are key elements of neuroprotection and fulfill trophic functions; both properties are critical to harmonious brain development and maturation. By analogy to hepatic bilirubin detoxification pathways, we review the transport and metabolic mechanisms which in all these interfaces may participate in the regulation of bilirubin cerebral bioavailability in physiologic conditions, both in adult and in developing brain. We specifically address the role of ABC and OATP transporters, glutathione-S-transferases, and the potential involvement of glucuronoconjugation and oxidative metabolic pathways. Regulatory mechanisms are explored which are involved in the induction of these pathways and represent potential pharmacological targets to prevent bilirubin accumulation into the brain. We then review the possible alteration of the neuroprotective and trophic barrier functions in the course of bilirubin-induced neurological dysfunctions resulting from hyperbilirubinemia. Finally, we highlight the role of the blood-brain and blood-CSF barriers in regulating the brain biodisposition of candidate drugs for the treatment or prevention of bilirubin-induced brain injury.

  20. Acute exacerbations of fibrotic interstitial lung disease.

    Churg, Andrew; Wright, Joanne L; Tazelaar, Henry D

    2011-03-01

    An acute exacerbation is the development of acute lung injury, usually resulting in acute respiratory distress syndrome, in a patient with a pre-existing fibrosing interstitial pneumonia. By definition, acute exacerbations are not caused by infection, heart failure, aspiration or drug reaction. Most patients with acute exacerbations have underlying usual interstitial pneumonia, either idiopathic or in association with a connective tissue disease, but the same process has been reported in patients with fibrotic non-specific interstitial pneumonia, fibrotic hypersensitivity pneumonitis, desquamative interstitial pneumonia and asbestosis. Occasionally an acute exacerbation is the initial manifestation of underlying interstitial lung disease. On biopsy, acute exacerbations appear as diffuse alveolar damage or bronchiolitis obliterans organizing pneumonia (BOOP) superimposed upon the fibrosing interstitial pneumonia. Biopsies may be extremely confusing, because the acute injury pattern can completely obscure the underlying disease; a useful clue is that diffuse alveolar damage and organizing pneumonia should not be associated with old dense fibrosis and peripheral honeycomb change. Consultation with radiology can also be extremely helpful, because the fibrosing disease may be evident on old or concurrent computed tomography scans. The aetiology of acute exacerbations is unknown, and the prognosis is poor; however, some patients survive with high-dose steroid therapy.

  1. Approach to Neurometabolic Diseases from a Pediatric Neurological Point of View

    Parvaneh KARIMZADEH

    2015-01-01

    Full Text Available How to Cite This Article: Karimzadeh P. Approach to Neurometabolic Diseases from a Pediatric Neurological Point of View. Iran J Child Neurol. 2015 Winter;9(1: 1-16. AbstractObjectiveNeurometabolic disorders are an important group of diseases that mostly arepresented in newborns and infants.Neurological manifestations are the prominent signs and symptoms in this groupof diseases. Seizures are a common sign and are often refractory to antiepileptic drugs in untreated neurometabolic patients.The onset of symptoms for neurometabolic disorders appears after an interval ofnormal or near normal growth and development.Additionally, affected childrenmay fare well until a catabolic crisis occurs.Patients with neurometabolic disorders during metabolic decompensation havesevere clinical presentation, which include poor feeding, vomiting, lethargy,seizures, and loss of consciousness.This symptom is often fatal but severe neurological insult and regression inneurodevelopmental milestones can result as a prominent sign in patients whosurvived.Acute symptoms should be immediately treated regardless of the cause.A number of patients with neurometabolic disorders respond favorably and, insome instances, dramatically respond to treatment.Early detection and early intervention is invaluable in some patients to preventcatabolism and normal or near normal neurodevelopmental milestones.This paper discusses neurometabolic disorders, approaches to this group ofdiseases (from the view of a pediatric neurologist, clinical and neurologicalmanifestations, neuroimaging and electroencephalography findings, earlydetection, and early treatment. ReferencesFiliano JJ. Neurometabolic Disease in the Newborn. ClinPerinatol 33, 2006, 411-479.Van Karnebeek CDM, Stockler S. Treatable inborn errors of metabolism causing intellectual disability: A systematic literature review. Molecular Genetics and Metabolism, 105, 2012, 369-381.Abdel-Salam GMH, Abdel-Kader AA, Effat L, Gouda A

  2. Acute Ischemic Stroke and Acute on Chronic Kidney Disease

    Raja Ahsan Aftab

    2016-06-01

    Full Text Available Ischemic stroke is due to either local thrombus formation or emboli that occlude a cerebral artery, together with chronic kidney disease represent major mortality and morbidity. Here wer present a case of 53 years old Malay man, admitted to a hospital in Malaysia complaining of sudden onset of weakness on right sided upper and lower limb associated with slurred speech. Patient was also suffering from uncontrolled hypertension, hyperlipidemia, chronic kidney disease stage 4, and diabetes mellitus(un controlled. He was diagnosed with acute ischemic stroke with cranial nerve 7 palsy (with right hemiparesis, acute on chronic kidney disease precipitated by dehydration and ACE inhibitor, and hyperkalemia. Patients with ischemic disease and chronic kidney disaese require constant monitering and carefull selected pharmacotherapy. Patient was placed under observation and was prescribed multiple pharamacotherpay to stabalise detoriating condition. Keywords: ischemic disease; chronic kidney disease; uncontrolled hypertension. | PubMed

  3. Is acute recurrent pancreatitis a chronic disease?

    Alberto Mariani; Pier Alberto Testoni

    2008-01-01

    Whether acute recurrent pancreaUtis is a chronic disease is still debated and a consensus is not still reached as demonstrated by differences in the classification of acute recurrent pancreatitis.There is major evidence for considering alcoholic pancreatitis as a chronic disease ab initio while chronic pancreatitis lesions detectable in biliary acute recurrent pancreatitis (ARP) seem a casual association.Cystic fibrosis transmembrane con ductance regulator (CFTR) gene mutation,hereditary and obstructive pancreatitis seem an acute disease that progress to chronic pancreatitis,likely as a consequence of the activation and proliferation of pancreatic stellate cells that produce and activate collagen and therefore fibrosis.From the diagnostic point of view,in patients with acute recurrent pancreatitis Endoscopic ultrasound (EUS) seems the more reliable technique for an accurate evaluation and follow-up of some ductal and parenchymal abnormalities suspected for early chronic pancreatitis.

  4. Disregard of neurological impairments associated with neglected tropical diseases in Africa.

    Quansah, Emmanuel; Sarpong, Esther; Karikari, Thomas K

    2016-06-01

    Neglected tropical diseases (NTDs) affect people in the bottom billion poorest in the world. These diseases are concentrated in rural areas, conflict zones and urban slums in Africa and other tropical areas. While the World Health Organization recognizes seventeen priority NTDs, the list of conditions present in Africa and elsewhere that are eligible to be classified as NTDs is much longer. Although NTDs are generally marginalized, their associated neurological burden has been almost completely disregarded. However, reports indicate that trichuriasis, schistosomiasis and hookworm infection, among others, cause impairments in memory and cognition, negatively affecting school attendance rates and educational performance particularly among children, as well as agricultural productivity among adults. Consequently, the neurological impairments have substantial influence on education and economic productivity, thus aggravating and perpetuating poverty in affected societies. However, inadequate research, policy and public health attention has been paid to the neurological burdens associated with NTDs. In order to appropriately address these burdens, we recommend the development of policy interventions that focus on the following areas: (i) the introduction of training programs to develop the capacity of scientists and clinicians in research, diagnostic and treatment approaches (ii) the establishment of competitive research grant schemes to fund cutting-edge research into these neurological impairments, and (iii) the development of public health interventions to improve community awareness of the NTD-associated neurological problems, possibly enhancing disease prevention and expediting treatment.

  5. Fahr's disease: a rare neurological presentation in a tropical setting

    Otu, Akaninyene Asuquo; Anikwe, Jude Chinedu; Cocker, Derek

    2015-01-01

    Key Clinical Message While rare, Fahr's disease should be considered as a differential diagnosis for seizures, movement disorders, or cognitive impairment in tropical settings. Classically, bilateral calcification of the basal ganglia is seen on CT. Endemic infections, metabolic, and toxic causes should be excluded. Treatment using Levodopa is often beneficial. PMID:26509011

  6. When dialogue fails. Music therapy with elderly with neurological degenerative diseases

    Ridder, Hanne Mette Ochsner

    2004-01-01

    and the ability to understand ‘what is going on’. “You need to have just the right level of activation to perform optimally” (LeDoux 1998, p. 289). Stimulating and sedating effects of music or songs are obtained by musical parameters, such as tempo, rhythm, timbre, volume, pitch, phrasing, dynamic, and timing......When dialogue fails. Music therapy with elderly with neurological degenerative diseases. In persons suffering from neurological degenerative diseases we often see the following symptoms: difficulties in remembering, concentrating, perceiving input, and controlling and timing movements. Normal every...... interaction with others means that psychosocial needs are not met, and this leads to secondary symptoms of the neurological degeneration. Secondary symptoms might be expressed as repetitive behaviour, catastrophic reactions and situationally inappropriate behaviour. In a music therapeutical setting...

  7. Thrombolysis in Acute Cerebrovascular Disease

    刘泽霖

    2003-01-01

    @@ Large-scale trials have shown that thrombolytic therapy reduces mortality and preserves left ventricular function in patients with acute myocardial infarction (AMI). That's a rationale for the use of thrombolytic agents in the management of ischemic stroke.

  8. Translational neurophysiology in sheep: measuring sleep and neurological dysfunction in CLN5 Batten disease affected sheep.

    Perentos, Nicholas; Martins, Amadeu Q; Watson, Thomas C; Bartsch, Ullrich; Mitchell, Nadia L; Palmer, David N; Jones, Matthew W; Morton, A Jennifer

    2015-04-01

    Creating valid mouse models of slowly progressing human neurological diseases is challenging, not least because the short lifespan of rodents confounds realistic modelling of disease time course. With their large brains and long lives, sheep offer significant advantages for translational studies of human disease. Here we used normal and CLN5 Batten disease affected sheep to demonstrate the use of the species for studying neurological function in a model of human disease. We show that electroencephalography can be used in sheep, and that longitudinal recordings spanning many months are possible. This is the first time such an electroencephalography study has been performed in sheep. We characterized sleep in sheep, quantifying characteristic vigilance states and neurophysiological hallmarks such as sleep spindles. Mild sleep abnormalities and abnormal epileptiform waveforms were found in the electroencephalographies of Batten disease affected sheep. These abnormalities resemble the epileptiform activity seen in children with Batten disease and demonstrate the translational relevance of both the technique and the model. Given that both spontaneous and engineered sheep models of human neurodegenerative diseases already exist, sheep constitute a powerful species in which longitudinal in vivo studies can be conducted. This will advance our understanding of normal brain function and improve our capacity for translational research into neurological disorders.

  9. Stem cell transplantation in neurological diseases: improving effectiveness in animal models.

    Raffaella eAdami

    2014-05-01

    Full Text Available Neurological diseases afflict a growing proportion of the human population. There are two reasons for this: first, the average age of the population (especially in the industrialised world is increasing, and second, the diagnostic tools to detect these pathologies are now more sophisticated and can be used on a higher percentage of the population. In many cases, neurological disease has a pharmacological treatment which, as in the case of Alzheimer’s disease, Parkinson’s disease, Epilepsy, and Multiple Sclerosis can reduce the symptoms and slow down the course of the disease but cannot reverse its effects or heal the patient.In the last two decades the transplantation approach, by means of stem cells of different origin, has been suggested for the treatment of neurological diseases. The choice of slightly different animal models and the differences in methods of stem cell preparation make it difficult to compare the results of transplantation experiments. Moreover, the translation of these results into clinical trials with human subjects is difficult and has so far met with little success.This review seeks to discuss the reasons for these difficulties by considering the differences between human and animal cells (including isolation, handling and transplantation and between the human disease model and the animal disease model.

  10. Helicobacter pylori and neurological diseases: Married by the laws of inflammation

    Lourdes; álvarez-Arellano; Carmen; Maldonado-Bernal

    2014-01-01

    The purpose of this paper is to review current infor-mation about the role of inflammation caused by He-licobacter pylori(H. pylori) infection in neurological diseases such as Parkinson’s disease, Alzheimer’s dis-ease, Guillain-Barré syndrome, multiple sclerosis, and other inflammatory diseases including ischemic stroke. Infection with H. pylori usually persists throughout life, resulting in a chronic inflammatory response with local secretion of numerous inflammatory mediators includ-ing chemokines [interleukin(IL)-8, macrophage che-motactic protein, growth-regulated oncogene(GRO)-α, chemokine(C-X-C motif) ligand 1] and cytokines [IL-1β, tumor necrosis factor-α, IL-6, IL-12, interferon-g], which can pass into the circulation and have a systemic effect. The persistence of detectable systemic and lo-cal concentrations of inflammatory mediators is likely to alter the outcome of neurological diseases. These proinflammatory factors can induce brain inflammation and the death of neurons and could eventually be asso-ciated to Parkinson’s disease and also may be involved in the development of Alzheimer’s disease. However,most neurological diseases are the result of a combina-tion of multiple factors, but the systemic inflammatory response is a common component and determinant in the onset, evolution, and outcome of diseases. How-ever, more studies are needed to allow understanding of the effects and mechanisms by which the inflamma-tory response generated by H. pylori infection affects neurological diseases.

  11. EXAMINER executive function battery and neurologic morbidity in pediatric sickle cell disease.

    Schatz, Jeffrey; Stancil, Melita; Katz, Tal; Sanchez, Carmen E

    2014-01-01

    Sickle cell disease (SCD) is blood disorder with a high risk for cerebral vascular morbidities that impact neurocognitive functioning. Specific cognitive abilities are known to be more sensitive to neurologic effects of SCD than IQ scores, yet there is little consensus about which measures to use to assess neurocognitive functioning. We evaluated the ability of the Executive Abilities: Methods and Instruments for Neurobehavioral Evaluation and Research (EXAMINER) Battery to detect neurologic effects in SCD. Thirty-two youth with SCD and sixty demographically-matched comparison youth completed the EXAMINER Battery and selected tests from the Woodcock-Johnson Tests of Cognitive Ability, 3rd edition (WJ-III). Neurologic severity was examined via clinical history for morbidities and midsagittal corpus callosum (CC) area. Results indicated cognitive performance decreased with increasing neurologic morbidity across all cognitive measures; two of four EXAMINER factors were related to CC area. The association with clinical history and midsagittal CC area appeared at least as large for the Examiner Battery scores as for the WJ-III measures. The Examiner Battery showed sensitivity to neurologic history and white matter effects in SCD; this new measure compares favorably to established measures of disease-related neurocognitive effects, but would benefit from further development.

  12. Glyphosate, pathways to modern diseases III: Manganese, neurological diseases, and associated pathologies

    Anthony Samsel

    2015-01-01

    Full Text Available Manganese (Mn is an often overlooked but important nutrient, required in small amounts for multiple essential functions in the body. A recent study on cows fed genetically modified Roundup ® -Ready feed revealed a severe depletion of serum Mn. Glyphosate, the active ingredient in Roundup ® , has also been shown to severely deplete Mn levels in plants. Here, we investigate the impact of Mn on physiology, and its association with gut dysbiosis as well as neuropathologies such as autism, Alzheimer′s disease (AD, depression, anxiety syndrome, Parkinson′s disease (PD, and prion diseases. Glutamate overexpression in the brain in association with autism, AD, and other neurological diseases can be explained by Mn deficiency. Mn superoxide dismutase protects mitochondria from oxidative damage, and mitochondrial dysfunction is a key feature of autism and Alzheimer′s. Chondroitin sulfate synthesis depends on Mn, and its deficiency leads to osteoporosis and osteomalacia. Lactobacillus, depleted in autism, depend critically on Mn for antioxidant protection. Lactobacillus probiotics can treat anxiety, which is a comorbidity of autism and chronic fatigue syndrome. Reduced gut Lactobacillus leads to overgrowth of the pathogen, Salmonella, which is resistant to glyphosate toxicity, and Mn plays a role here as well. Sperm motility depends on Mn, and this may partially explain increased rates of infertility and birth defects. We further reason that, under conditions of adequate Mn in the diet, glyphosate, through its disruption of bile acid homeostasis, ironically promotes toxic accumulation of Mn in the brainstem, leading to conditions such as PD and prion diseases.

  13. Recent onset neck pain with associated neurological deficit--Pott's disease remains an important differential diagnosis.

    Bourke, M G

    2010-11-05

    The incidence of spinal tuberculosis is increasing in developed nations. In Ireland, half of all cases seen in the most recent decade for which figures are available were diagnosed in 2005-2007, the three most recent years for which there is complete data. We discuss a patient who presented with neurological complications due to destructive spinal tuberculous disease affecting the sixth cervical vertebra.

  14. Dolphins, dogs, and robot seals for the treatment of neurological disease.

    Burton, Adrian

    2013-09-01

    A growing body of evidence suggests that animal-assisted therapies and activities involving all kinds of real and even robotic animals can have beneficial eff ects in people with neurological disease or mental illness. But what is the quality of that evidence and do these interventions really provide any health benefits? Adrian Burton investigates.

  15. Creation of an open-access, mutation-defined fibroblast resource for neurological disease research.

    Selina Wray

    Full Text Available Our understanding of the molecular mechanisms of many neurological disorders has been greatly enhanced by the discovery of mutations in genes linked to familial forms of these diseases. These have facilitated the generation of cell and animal models that can be used to understand the underlying molecular pathology. Recently, there has been a surge of interest in the use of patient-derived cells, due to the development of induced pluripotent stem cells and their subsequent differentiation into neurons and glia. Access to patient cell lines carrying the relevant mutations is a limiting factor for many centres wishing to pursue this research. We have therefore generated an open-access collection of fibroblast lines from patients carrying mutations linked to neurological disease. These cell lines have been deposited in the National Institute for Neurological Disorders and Stroke (NINDS Repository at the Coriell Institute for Medical Research and can be requested by any research group for use in in vitro disease modelling. There are currently 71 mutation-defined cell lines available for request from a wide range of neurological disorders and this collection will be continually expanded. This represents a significant resource that will advance the use of patient cells as disease models by the scientific community.

  16. [A spectrum of neurological diseases with anti-VGKC antibody].

    Arimura, Kimiyoshi; Watanabe, Osamu; Nagado, Tatsui

    2007-11-01

    Anti-VGKC antibody causing peripheral nerve hyperexcitability is already an established clinical entity. Recently, many patients with non-herpetic limbic encephalitis (NHLE) with anti-VGKC antibody have been reported. The characteristic clinical features are low serum Na+ concentration and good response to immunotherapy. Anti-VGK antibody positive NHLE is relatively frequent among immune-mediated NHLE. It is important to know that this disease is responsive to immunotherapy. Furthermore, anti-VGKC antibody is also positive in some intractable epilepsies. These findings suggest that anti-VGKC is correlated with hyperexcitability in both the peripheral and central nervous system and that the spectrum of anti-VGKC antibody syndrome is now expanding.

  17. Molecular imaging in neurological diseases; Molekulare Bildgebung bei neurologischen Erkrankungen

    Reimold, M.; Fougere, C. la [Universitaetsklinikum Tuebingen, Abteilung Nuklearmedizin und Klinische Molekulare Bildgebung, Department Radiologie, Tuebingen (Germany)

    2016-07-15

    In neurodegeneration and in neuro-oncology, the standard imaging procedure, magnetic resonance imaging (MRI), shows limited sensitivity and specificity. Molecular imaging with specific positron-emission tomography (PET) and single-photon emission computed tomography (SPECT) tracers allows various molecular targets and metabolic processes to be assessed and is thus a valuable adjunct to MRI. Two important examples are referred to here: amino acid transport for neuro-oncological issues, and the recently approved PET tracers for detecting amyloid depositions during the preclinical stage of Alzheimer's disease. This review discusses the clinical relevance and indications for the following nuclear medicine imaging procedures: amyloid PET, {sup 18}F-fluorodeoxyglucose (FDG)-PET, and dopamine transporter (DaT)-SPECT for the diagnosis of dementia and the differential diagnosis of Parkinson's disease, in addition to amino acid PET for the diagnosis of brain tumors and somatostatin receptor imaging in meningioma. (orig.) [German] Die Magnetresonanztomographie (MRT) weist als Standardverfahren bei neurodegenerativen und neuroonkologischen Fragestellungen eine eingeschraenkte Sensitivitaet und Spezifitaet auf. Die nuklearmedizinische molekulare Bildgebung mit spezifischen Positronenemissionstomographie(PET)- und single-photon-emission-computed-tomography(SPECT)-Tracern ermoeglicht die Darstellung verschiedener molekularer Targets bzw. Stoffwechselprozesse und stellt damit eine wichtige Ergaenzung zur MRT dar. Hier sei exemplarisch auf die Darstellung des Aminosaeuretransports im Rahmen neuroonkologischer Fragestellungen verwiesen, sowie auf die bereits im praeklinischen Stadium der Alzheimer-Demenz nachweisbaren Amyloidablagerungen mit hierfuer seit Kurzem zugelassenen PET-Tracern. Dieser Uebersichtsbeitrag bespricht die klinische Bedeutung bzw. die Indikationen der folgenden nuklearmedizinischen Untersuchungsverfahren: der Amyloid-PET, der {sup 18}F

  18. Predicting targets of compounds against neurological diseases using cheminformatic methodology.

    Nikolic, Katarina; Mavridis, Lazaros; Bautista-Aguilera, Oscar M; Marco-Contelles, José; Stark, Holger; do Carmo Carreiras, Maria; Rossi, Ilaria; Massarelli, Paola; Agbaba, Danica; Ramsay, Rona R; Mitchell, John B O

    2015-02-01

    Recently developed multi-targeted ligands are novel drug candidates able to interact with monoamine oxidase A and B; acetylcholinesterase and butyrylcholinesterase; or with histamine N-methyltransferase and histamine H3-receptor (H3R). These proteins are drug targets in the treatment of depression, Alzheimer's disease, obsessive disorders, and Parkinson's disease. A probabilistic method, the Parzen-Rosenblatt window approach, was used to build a "predictor" model using data collected from the ChEMBL database. The model can be used to predict both the primary pharmaceutical target and off-targets of a compound based on its structure. Molecular structures were represented based on the circular fingerprint methodology. The same approach was used to build a "predictor" model from the DrugBank dataset to determine the main pharmacological groups of the compound. The study of off-target interactions is now recognised as crucial to the understanding of both drug action and toxicology. Primary pharmaceutical targets and off-targets for the novel multi-target ligands were examined by use of the developed cheminformatic method. Several multi-target ligands were selected for further study, as compounds with possible additional beneficial pharmacological activities. The cheminformatic targets identifications were in agreement with four 3D-QSAR (H3R/D1R/D2R/5-HT2aR) models and by in vitro assays for serotonin 5-HT1a and 5-HT2a receptor binding of the most promising ligand (71/MBA-VEG8).

  19. Liver transplantation in neurological Wilson's Disease: is there indication? A case report.

    Mocchegiani, F; Gemini, S; Vincenzi, P; Montalti, R; Vecchi, A; Nicolini, D; Federici, A; Coletta, M; Pansini, M; Lanari, J; Svegliati Baroni, G; Risaliti, A; Vivarelli, M

    2014-09-01

    Wilson's disease (WD) is an autosomal recessive disorder characterized by copper overload. In this disease, inadequate hepatic excretion leads to copper accumulation in the liver, brain, kidney, and cornea. Severe neurological symptoms can develop in patients with WD, often in the absence of relevant liver damage: it is unclear whether liver transplantation (LT) could reverse neurological symptoms, and at present LT is not recommended in this setting. We report a case of regression of neurological symptoms in a patient affected by WD with prevalent neurological involvement. A 19-year-old man with disabling neuropsychiatric symptoms from WD that included frontal ataxia, akinesia, dystonia, tremors, and behavioral disorders in the presence of preserved liver function (Model for End-Stage Liver Disease score=7; Child-Turcotte-Pugh score=A5) underwent LT in November 2009. At the time of LT, encephalic magnetic resonance imaging (MRI) indicated diffuse neurodegenerative alterations involving subtentorial and supratentorial structures; bilateral Kayser-Fleischer ring was present. Four years after LT, laboratory tests show normalized copper metabolism and excellent liver function test results. Encephalic MRI shows a substantial improvement of already-known signal alterations at nuclei thalamus and putamen, mesencephalon, and pons. Kayser-Fleischer ring disappeared from the right eye, but a little remnant is still visible in the left eye. At neurological examination, all of the previous symptoms and signs are no longer present and behavioral disorders are no longer present; psychosocial functions are completely restored. The present case provides some evidence that LT may be a valid therapeutic option for WD patients with marked neurological impairment, particularly in those no longer responsive to chelation therapy.

  20. (123)I-Meta-iodobenzylguanidine Sympathetic Imaging: Standardization and Application to Neurological Diseases.

    Nakajima, Kenichi; Yamada, Masahito

    2016-09-01

    (123)I-meta-iodobenzylguanidine (MIBG) has become widely applied in Japan since its introduction to clinical cardiology and neurology practice in the 1990s. Neurological studies found decreased cardiac uptake of (123)I-MIBG in Lewy-body diseases including Parkinson's disease and dementia with Lewy bodies. Thus, cardiac MIBG uptake is now considered a biomarker of Lewy body diseases. Although scintigraphic images of (123)I-MIBG can be visually interpreted, an average count ratio of heart-to-mediastinum (H/M) has commonly served as a semi-quantitative marker of sympathetic activity. Since H/M ratios significantly vary according to acquisition and processing conditions, quality control should be appropriate, and quantitation should be standardized. The threshold H/M ratio for differentiating Lewy-body disease is 2.0-2.1, and was based on standardized H/M ratios to comparable values of medium-energy collimators. Parkinson's disease can be separated from various types of parkinsonian syndromes using cardiac (123)I-MIBG, whereas activity is decreased on images of Lewy-body diseases using both (123)I-ioflupane for the striatum and (123)I-MIBG. Despite being a simple index, the H/M ratio of (123)I-MIBG uptake is reproducible and can serve as an effective tool to support a diagnosis of Lewy-body diseases in neurological practice.

  1. Thymosin β4 as a restorative/regenerative therapy for neurological injury and neurodegenerative diseases.

    Chopp, Michael; Zhang, Zheng Gang

    2015-01-01

    Thymosin β4 (Tβ4) promotes CNS and peripheral nervous system (PNS) plasticity and neurovascular remodeling leading to neurological recovery in a range of neurological diseases. Treatment of neural injury and neurodegenerative disease 24 h or more post-injury and disease onset with Tβ4 enhances angiogenesis, neurogenesis, neurite and axonal outgrowth, and oligodendrogenesis, and thereby, significantly improves functional and behavioral outcomes. We propose that oligodendrogenesis is a common link by which Tβ4 promotes recovery after neural injury and neurodegenerative disease. The ability to target many diverse restorative processes via multiple molecular pathways that drive oligodendrogenesis and neurovascular remodeling may be mediated by the ability of Tβ4 to alter cellular expression of microRNAs (miRNAs). However, further investigations on the essential role of miRNAs in regulating protein expression and the remarkable exosomal intercellular communication network via exosomes will likely provide insight into mechanisms of action and means to amplify the therapeutic effects of Tβ4.

  2. Measles, mumps, rubella, and human parvovirus B19 infections and neurologic disease.

    Bale, James F

    2014-01-01

    While the systemic disorders associated with measles, mumps, and rubella viruses and human parvovirus B19 tend to be mild, each virus can produce potentially life-threatening neurologic disease in human hosts, especially when these viruses infect young children. Two of the viruses, rubella and parvovirus B19, can be vertically transmitted to fetuses during maternal infection and cause congenital infection. Neurologic complications are common after intrauterine infection with the rubella virus, a condition known as the congenital rubella syndrome. Two, measles and rubella viruses, can induce "slow viral" infections, serious, disorders that can occur several years after the initial exposure to the virus and typically have fatal outcomes.

  3. Neurological complications in late-stage hospitalized patients with HIV disease

    Rakendra Singh

    2011-01-01

    Full Text Available Background and Objective: The nervous system is the most frequent and serious targets of human immunodeficiency virus (HIV infection. In spite of a wide prevalence of neurological manifestations in HIV there are not many studies to look into it, especially from this part of the world. We investigated various neurological manifestations of HIV and their association with CD4 and CD8 counts at the time of presentation. Materials and Methods: All HIV-infected patients who presented to 750 bedded teaching hospital in North India were subjected to thorough neurological and neuropsychological evaluation. Wherever indicated, neuroimaging, cerebrospinal fluid study, electromyography, and nerve-conduction studies were performed to confirm the diagnosis. CD4 and CD8 counts were calculated. Results: A total of 416 HIV-positive patients were seen. Of them 269 were males. A total of 312 neurological events were identified in 268 patients having evidence of neurological involvement. HIV-associated dementia (HAD was the most common cause of morbidity (33.65%, followed by CNS infections (21.63%. Most common CNS infection was tuberculosis (65.56%. CD4 counts in CNS infections and HAD were 64.8/ml and 83.52/ml, respectively. Most of the patients in our study had low scores on MMSE (22.32. Conclusions: Even in the absence of overt neurological disease, subclinical involvement in the form of subtle cognitive and motor decline is found to occur with greater frequency. Most of these patients have lower CD4 and CD8 counts, thus substantiating the proposition that neuroAIDS is a late manifestation. Significant correlation exists between CD4 counts and type of neurological manifestation. We concluded that neuropsychological assessment should be mandatory for all HIV-positive patients.

  4. MicroRNAs: Key Regulators in the Central Nervous System and Their Implication in Neurological Diseases

    Dan-Dan Cao

    2016-05-01

    Full Text Available MicroRNAs (miRNAs are a class of small, well-conserved noncoding RNAs that regulate gene expression post-transcriptionally. They have been demonstrated to regulate a lot of biological pathways and cellular functions. Many miRNAs are dynamically regulated during central nervous system (CNS development and are spatially expressed in adult brain indicating their essential roles in neural development and function. In addition, accumulating evidence strongly suggests that dysfunction of miRNAs contributes to neurological diseases. These observations, together with their gene regulation property, implicated miRNAs to be the key regulators in the complex genetic network of the CNS. In this review, we first focus on the ways through which miRNAs exert the regulatory function and how miRNAs are regulated in the CNS. We then summarize recent findings that highlight the versatile roles of miRNAs in normal CNS physiology and their association with several types of neurological diseases. Subsequently we discuss the limitations of miRNAs research based on current studies as well as the potential therapeutic applications and challenges of miRNAs in neurological disorders. We endeavor to provide an updated description of the regulatory roles of miRNAs in normal CNS functions and pathogenesis of neurological diseases.

  5. MicroRNAs: Key Regulators in the Central Nervous System and Their Implication in Neurological Diseases.

    Cao, Dan-Dan; Li, Lu; Chan, Wai-Yee

    2016-05-28

    MicroRNAs (miRNAs) are a class of small, well-conserved noncoding RNAs that regulate gene expression post-transcriptionally. They have been demonstrated to regulate a lot of biological pathways and cellular functions. Many miRNAs are dynamically regulated during central nervous system (CNS) development and are spatially expressed in adult brain indicating their essential roles in neural development and function. In addition, accumulating evidence strongly suggests that dysfunction of miRNAs contributes to neurological diseases. These observations, together with their gene regulation property, implicated miRNAs to be the key regulators in the complex genetic network of the CNS. In this review, we first focus on the ways through which miRNAs exert the regulatory function and how miRNAs are regulated in the CNS. We then summarize recent findings that highlight the versatile roles of miRNAs in normal CNS physiology and their association with several types of neurological diseases. Subsequently we discuss the limitations of miRNAs research based on current studies as well as the potential therapeutic applications and challenges of miRNAs in neurological disorders. We endeavor to provide an updated description of the regulatory roles of miRNAs in normal CNS functions and pathogenesis of neurological diseases.

  6. Diarrheal Diseases - Acute and Chronic

    ... acute, watery diarrhea are caused by viruses (viral gastroenteritis). The most common ones in children are rotavirus and in adults are norovirus (this is sometimes called “cruise ship diarrhea” due to well publicized epidemics). Bacteria are a common cause of traveler’s diarrhea. Causes – ...

  7. Frontiers in therapeutic development of allopregnanolone for Alzheimer's disease and other neurological disorders

    Ronald W. Irwin

    2014-07-01

    Full Text Available Allopregnanolone (Allo, a neurosteroid, has emerged as a promising promoter of endogenous regeneration in brain. In a mouse model of Alzheimer’s disease, Allo induced neurogenesis, oligodendrogenesis, white matter generation and cholesterol homeostasis while simultaneously reducing β-amyloid and neuroinflammatory burden. Allo activates signaling pathways and gene expression required for regeneration of neural stem cells and their differentiation into neurons. In parallel, Allo activates systems to sustain cholesterol homeostasis and reduce β-amyloid generation. To advance Allo into studies for chronic human neurological conditions, we examined translational and clinical parameters: dose, regimen, route, formulation, outcome measures, and safety regulations. A treatment regimen of once per week at sub-sedative doses of Allo was optimal for regeneration and reduction in Alzheimer’s pathology. This regimen had a high safety profile following chronic exposure in aged normal and Alzheimer’s mice. Formulation of Allo for multiple routes of administration has been developed for both preclinical and clinical testing. Preclinical evidence for therapeutic efficacy of Allo spans multiple neurological diseases including Alzheimer’s, Parkinson’s, multiple sclerosis, Niemann-Pick, diabetic neuropathy, status epilepticus, and traumatic brain injury. To successfully translate Allo as a therapeutic for multiple neurological disorders, it will be necessary to tailor dose and regimen to the targeted therapeutic mechanisms and disease etiology. Treatment paradigms conducted in accelerated disease models in young animals have a low probability of successful translation to chronic diseases in adult and aged humans. Gender, genetic risks, stage and burden of disease are critical determinants of efficacy. This review focuses on recent advances in development of Allo for Alzheimer’s disease that have the potential to accelerate therapeutic translation for

  8. Neurological recovery at age 92 after acute trauma and operative spinal decompression

    Hazem Eltahawy, MD, PhD, FRCS, FACS

    2016-09-01

    Full Text Available People aged > 80 years are among the fastest growing segments of most Western societies. With improved lifestyles and medical care, complex surgical interventions will be increasingly offered to elderly patients. Questions will arise about the value of performing major surgery in patients near their postulated end of life. Here, we describe a near-full neurological recovery from a profound neurological deficit that occurred as a result of a spinal fracture after a fall. To our knowledge, this is the first report of neurological recovery at such an advanced age.

  9. Proton MRS in Behcet's disease with and without neurological findings

    Baysal, T.; Sarac, K.; Dusak, A. [Department of Radiology, Inonu University School of Medicine, 44069, Malatya (Turkey); Ozisik, H.I.; Ozcan, C. [Department of Neurology, Inonu University School of Medicine, 44069, Malatya (Turkey); Karlidag, R. [Department of Psychiatry, Inonu University School of Medicine, 44069, Malatya (Turkey); Baysal, O. [Department of Physical Therapy and Rehabilitation, Inonu University School of Medicine, 44069, Malayta (Turkey); Hazneci, E. [Department of Dermatology, Inonu University School of Medicine, 44069, Malatya (Turkey)

    2003-12-01

    Our aim was to investigate whether neurological impairment in Behcet's disease (BD) can be assessed by means of proton MRS and whether it can assist in prognosis. We used single-voxel MRS to measure metabolites in regions of normal-appearing pons, basal ganglia and periventricular white matter (PWM) in 32 patients with chronic BD patients with and without neurological deficits and 29 control subjects. Patients had significantly higher N-acetylaspartate (NAA)/creatine (Cr) and choline (Cho)/Cr ratios in the basal ganglia than the controls. The Cho/Cr ratio in the PWM was also significantly higher in the patients. MRS enabled clear discrimination of patients and controls and also revealed spectral differences between non-neuro-Behcet's disease and neuro-Behcet's disease in the basal ganglia. MRS can be used to assess brain involvement in BD even if structural changes are absent. (orig.)

  10. tDCS-enhanced motor and cognitive function in neurological diseases.

    Flöel, Agnes

    2014-01-15

    Transcranial direct current stimulation (tDCS) is a non-invasive brain stimulation tool that is now being widely used in neuroscientific and clinical research in humans. While initial studies focused on modulation of cortical excitability, the technique quickly progressed to studies on motor and cognitive functions in healthy humans and in patients with neurological diseases. In the present review we will first provide the reader with a brief background on the basic principles of tDCS. In the main part, we will outline recent studies with tDCS that aimed at enhancing behavioral outcome or disease-specific symptoms in patients suffering from mild cognitive impairment, Alzheimer's disease, movement disorders, and epilepsy, or persistent deficits after stroke. The review will close with a summary statement on the present use of tDCS in the treatment of neurological disorders, and an outlook to further developments in this realm. tDCS may be an ideal tool to be administered in parallel to intensive cognitive or motor training in neurological disease, but efficacy for the areas of activities and participation still needs to be established in controlled randomized trials. Its use in reducing disease-specific symptoms like dystonia or epileptic seizures is still unclear.

  11. Transgenic Monkey Model of the Polyglutamine Diseases Recapitulating Progressive Neurological Symptoms

    Ishibashi, Hidetoshi; Minakawa, Eiko N.; Motohashi, Hideyuki H.; Takayama, Osamu; Popiel, H. Akiko; Puentes, Sandra; Owari, Kensuke; Nakatani, Terumi; Nogami, Naotake; Yamamoto, Kazuhiro; Yonekawa, Takahiro; Tanaka, Yoko; Fujita, Naoko; Suzuki, Hikaru; Aizawa, Shu; Nagano, Seiichi; Yamada, Daisuke; Wada, Keiji; Kohsaka, Shinichi

    2017-01-01

    Abstract Age-associated neurodegenerative diseases, such as Alzheimer’s disease, Parkinson’s disease, and the polyglutamine (polyQ) diseases, are becoming prevalent as a consequence of elongation of the human lifespan. Although various rodent models have been developed to study and overcome these diseases, they have limitations in their translational research utility owing to differences from humans in brain structure and function and in drug metabolism. Here, we generated a transgenic marmoset model of the polyQ diseases, showing progressive neurological symptoms including motor impairment. Seven transgenic marmosets were produced by lentiviral introduction of the human ataxin 3 gene with 120 CAG repeats encoding an expanded polyQ stretch. Although all offspring showed no neurological symptoms at birth, three marmosets with higher transgene expression developed neurological symptoms of varying degrees at 3–4 months after birth, followed by gradual decreases in body weight gain, spontaneous activity, and grip strength, indicating time-dependent disease progression. Pathological examinations revealed neurodegeneration and intranuclear polyQ protein inclusions accompanied by gliosis, which recapitulate the neuropathological features of polyQ disease patients. Consistent with neuronal loss in the cerebellum, brain MRI analyses in one living symptomatic marmoset detected enlargement of the fourth ventricle, which suggests cerebellar atrophy. Notably, successful germline transgene transmission was confirmed in the second-generation offspring derived from the symptomatic transgenic marmoset gamete. Because the accumulation of abnormal proteins is a shared pathomechanism among various neurodegenerative diseases, we suggest that this new marmoset model will contribute toward elucidating the pathomechanisms of and developing clinically applicable therapies for neurodegenerative diseases. PMID:28374014

  12. Cerebrolysin effects on neurological outcomes and cerebral blood flow in acute ischemic stroke

    Amiri-Nikpour MR; Nazarbaghi S; Ahmadi-Salmasi B; Mokari. T.; Tahamtan U; Rezaei Y

    2014-01-01

    Mohammad Reza Amiri-Nikpour,1 Surena Nazarbaghi,1 Babak Ahmadi-Salmasi,1 Tayebeh Mokari,2 Urya Tahamtan,2 Yousef Rezaei3 1Department of Neurology, Imam Khomeini Hospital, 2School of Medicine, 3Seyyed-al-Shohada Heart Center, Urmia University of Medical Sciences, Urmia, Iran Background: Cerebrolysin, a brain-derived neuropeptide, has been shown to improve the neurological outcomes of stroke, but no study has demonstrated its effect on cerebral blood flow. This study aimed to determine the ce...

  13. Depressive symptoms in Parkinson’s disease and in non-neurological medical illnesses

    Assogna, Francesca; Fagioli, Sabrina; Cravello, Luca; Meco, Giuseppe; Pierantozzi, Mariangela; Stefani, Alessandro; Imperiale, Francesca; Caltagirone, Carlo; Pontieri, Francesco E; Spalletta, Gianfranco

    2013-01-01

    Background Patients with neurological and non-neurological medical illnesses very often complain of depressive symptoms that are associated with cognitive and functional impairments. We compared the profile of depressive symptoms in Parkinson’s disease (PD) patients with that of control subjects (CS) suffering from non-neurological medical illnesses. Methods One-hundred PD patients and 100 CS were submitted to a structured clinical interview for identification of major depressive disorder (MDD) and minor depressive disorder (MIND), according to the Diagnostic and Statistical Manual of Mental Disorders, 4th edition, text revision (DSM-IV-TR), criteria. The Hamilton Depression Rating Scale (HDRS) and the Beck Depression Inventory (BDI) were also administered to measure depression severity. Results When considering the whole groups, there were no differences in depressive symptom frequency between PD and CS apart from worthlessness/guilt, and changes in appetite reduced rates in PD. Further, total scores and psychic and somatic subscores of HDRS and BDI did not differ between PD and CS. After we separated PD and CS in those with MDD, MIND, and no depression (NODEP), comparing total scores and psychic/somatic subscores of HDRS and BDI, we found increased total depression severity in NODEP PD and reduced severity of the psychic symptoms of depression in MDD PD, with no differences in MIND. However, the severity of individual symptom frequency of depression was not different between PD and CS in MDD, MIND, and NODEP groups. Conclusion Although MDD and MIND phenomenology in PD may be very similar to that of CS with non-neurological medical illnesses, neurological symptoms of PD may worsen (or confound) depression severity in patients with no formal/structured DSM-IV-TR, diagnosis of depressive mood disorders. Thus, a thorough assessment of depression in PD should take into consideration the different impacts of neurological manifestations on MDD, MIND, and NODEP. PMID

  14. EFhd2, a Protein Linked to Alzheimer's Disease and Other Neurological Disorders.

    Vega, Irving E

    2016-01-01

    EFhd2 is a conserved calcium binding protein linked to different neurological disorders and types of cancer. Although, EFhd2 is more abundant in neurons, it is also found in other cell types. The physiological function of this novel protein is still unclear, but it has been shown in vitro to play a role in calcium signaling, apoptosis, actin cytoskeleton, and regulation of synapse formation. Recently, EFhd2 was shown to promote cell motility by modulating the activity of Rac1, Cdc42, and RhoA. Although, EFhd2's role in promoting cell invasion and metastasis is of great interest in cancer biology, this review focusses on the evidence that links EFhd2 to Alzheimer's disease (AD) and other neurological disorders. Altered expression of EFhd2 has been documented in AD, Parkinson's disease, Huntington's disease, Amyotrophic Lateral Sclerosis, and schizophrenia, indicating that Efhd2 gene expression is regulated in response to neuropathological processes. However, the specific role that EFhd2 plays in the pathophysiology of neurological disorders is still poorly understood. Recent studies demonstrated that EFhd2 has structural characteristics similar to amyloid proteins found in neurological disorders. Moreover, EFhd2 co-aggregates and interacts with known neuropathological proteins, such as tau, C9orf72, and Lrrk2. These results suggest that EFhd2 may play an important role in the pathophysiology of neurodegenerative diseases. Therefore, the understanding of EFhd2's role in health and disease could lead to decipher molecular mechanisms that become activated in response to neuronal stress and degeneration.

  15. Evaluation of the role of SNCA variants in survival without neurological disease.

    Michael G Heckman

    Full Text Available BACKGROUND: A variety of definitions of successful aging have been proposed, many of which relate to longevity, freedom from disease and disability, or preservation of high physical and cognitive function. Many behavioral, biomedical, and psychological factors have been linked with these various measures of successful aging, however genetic predictors are less understood. Parkinson's disease (PD is an age-related neurodegenerative disorder, and variants in the α-synuclein gene (SNCA affect susceptibility to PD. This exploratory study examined whether SNCA variants may also promote successful aging as defined by survival without neurological disease. METHODS: We utilized 769 controls without neurological disease (Mean age: 79 years, Range: 33-99 years and examined the frequency of 20 different SNCA variants across age groups using logistic regression models. We also included 426 PD cases to assess the effect of these variants on PD risk. RESULTS: There was a significant decline in the proportion of carriers of the minor allele of rs10014396 as age increased (P = 0.021, from 30% in controls younger than 60 to 14% in controls 90 years of age or older. Findings were similar for rs3775439, where the proportion of carriers of the minor allele declined from 32% in controls less than 60 years old to 19% in those 90 or older (P = 0.025. A number of SNCA variants, not including rs10014396 or rs3775439, were significantly associated with susceptibility to PD. CONCLUSIONS: In addition to its documented roles in PD and α-synucleinopathies, our results suggest that SNCA has a role in survival free of neurological disease. Acknowledging that our findings would not have withstood correction for multiple testing, validation in an independent series of aged neurologically normal controls is needed.

  16. Depressive symptoms in Parkinson’s disease and in non-neurological medical illnesses

    Assogna F

    2013-03-01

    Full Text Available Francesca Assogna,1 Sabrina Fagioli,1 Luca Cravello,1 Giuseppe Meco,2 Mariangela Pierantozzi,3 Alessandro Stefani,3 Francesca Imperiale,2 Carlo Caltagirone,1,3 Francesco E Pontieri,4 Gianfranco Spalletta11I.R.C.C.S. Santa Lucia Foundation, Rome, Italy; 2Department of Neurology and Psychiatry (Parkinson’s Centre and Research Centre of Social Diseases (CIMS, University “Sapienza”, Rome, Italy; 3Department of Neuroscience, University “Tor Vergata”, Rome, Italy; 4Department of Neuroscience, Mental Health and Sensory Systems, University “Sapienza”, Movement Disorder Unit, Sant’Andrea Hospital, Rome, ItalyBackground: Patients with neurological and non-neurological medical illnesses very often complain of depressive symptoms that are associated with cognitive and functional impairments. We compared the profile of depressive symptoms in Parkinson’s disease (PD patients with that of control subjects (CS suffering from non-neurological medical illnesses.Methods: One-hundred PD patients and 100 CS were submitted to a structured clinical interview for identification of major depressive disorder (MDD and minor depressive disorder (MIND, according to the Diagnostic and Statistical Manual of Mental Disorders, 4th edition, text revision (DSM-IV-TR, criteria. The Hamilton Depression Rating Scale (HDRS and the Beck Depression Inventory (BDI were also administered to measure depression severity.Results: When considering the whole groups, there were no differences in depressive symptom frequency between PD and CS apart from worthlessness/guilt, and changes in appetite reduced rates in PD. Further, total scores and psychic and somatic subscores of HDRS and BDI did not differ between PD and CS. After we separated PD and CS in those with MDD, MIND, and no depression (NODEP, comparing total scores and psychic/somatic subscores of HDRS and BDI, we found increased total depression severity in NODEP PD and reduced severity of the psychic symptoms of

  17. Barriers to care for patients with neurologic disease in rural Zambia.

    Birbeck, G L

    2000-03-01

    The awesome burden of treatable yet untreated neurologic disease in the developing world presents a humanitarian crisis to those of us with neurologic expertise from more privileged situations. Although increased economic resources are critically needed, a shortage of personnel to care for these patients is as great a problem. It is neither feasible nor desirable to propose training neurologists to work in these regions. However, COs could be selected to receive additional training and return to their home regions to serve as resources for referrals and as community educators. Such a training program would not require massive financial commitments. A handful of dedicated neurologists could conceivably accomplish this in 6- to 8-week training sessions. Ideally, educational materials, such as posters and pamphlets in both English and the native language of the various regions, would be provided at no cost. Existing textbooks in neurology are written for physicians and often focus on diagnostic evaluations and therapies far beyond the services available in developing countries. A text for practical use by COs and community health workers that discusses the application of available medicines and therapies for common neurologic problems would be invaluable. Similar books exist that address general medical and obstetrical problems (for example, Where There Is No Doctor: A Village Health Care Handbook). Where There Is No Neurologist could be developed as a primary teaching tool and a valuable reference for COs with neurologic expertise. Neuroscience researchers, clinical neurologists, and neurology residents from industrialized countries have much to offer and to gain by working in the Third World. Research to monitor the incidence and resource utilization of emerging problems such as stroke is needed to influence public policy. The economic burden and lost productivity caused by neurologic disease in this part of the world has not been appreciated or explored. Disease

  18. Overstimulation of the inhibitory nervous system plays a role in the pathogenesis of neuromuscular and neurological diseases: a novel hypothesis.

    Tuk, Bert

    2016-01-01

    Based upon a thorough review of published clinical observations regarding the inhibitory system, I hypothesize that this system may play a key role in the pathogenesis of a variety of neuromuscular and neurological diseases. Specifically, excitatory overstimulation, which is commonly reported in neuromuscular and neurological diseases, may be a homeostatic response to inhibitory overstimulation. Involvement of the inhibitory system in disease pathogenesis is highly relevant, given that most approaches currently being developed for treating neuromuscular and neurological diseases focus on reducing excitatory activity rather than reducing inhibitory activity.

  19. Clinical application of multi-shot diffusion EPI in neurological disease

    Ishihara, Tetsuya; Hirata, Koichi; Kubo, Jin; Yamazaki, Kaoru [Dokkyo Univ., Mibu, Tochigi (Japan). School of Medicine; Sato, Toshihiko

    1998-05-01

    Using the multi-shot EPI method we investigated the clinical application of diffusion weighted imaging (DWI) in the diagnosis of neurological disease. The multi-shot method provided better susceptibility artifact-free DWI than the single-shot method particularly in the region of the posterior cranial fossa. DWI using the multi-shot EPI method readily shows the pyramidal tract extending from the internal capsule to the brainstems which is inaccessible by the conventional single-shot EPI method, and providing three-dimensional and distinct images of pyramidal tract changes in amyotrophic lateral sclerosis or cerebral infarction with pyramidal tract disturbance. Our findings suggest that the use of DWI with the multi-shot EPI method would provide a technique for the easy diagnosis and evaluation of various neurological diseases. (author)

  20. Newer insights to the neurological diseases among biblical characters of old testament

    Mathew Stephen

    2010-01-01

    Full Text Available Many people over the years have studied the Bible from a medical point of view offering diagnoses for the symptoms and signs that appear to have afflicted numerous individuals in the Bible. We review the biblical characters in the Old Testament and offer newer insights to their neurological diseases. We first look at the battle between Goliath and David. Interestingly, Goliath probably suffered from acromegaly. We propose autism as a diagnosis for Samson which would precede the first known case of autism by centuries. Isaac was a diabetic, and he probably had autonomic neuropathy. Few verses from the books of I Samuel, Psalms, and Ezekiel reveal symptoms suggestive of stroke. Jacob suffered from sciatica, and the child of the Shunnamite woman in II Kings had a subarachnoid hemorrhage. These instances among others found in the Old Testament of the Bible offer newer insights on the history of current neurological diseases.

  1. Stem Cells in Large Animal Models of Retinal and Neurological Disease

    2012-01-01

    papers that focus on stem and progenitor cells from the central nervous system (both brain and retina ) of nonrodent mammals, or cells modified to resemble...FEB 2012 2. REPORT TYPE N/A 3. DATES COVERED - 4. TITLE AND SUBTITLE Stem cells in large animal models of retinal and neurological disease...Prescribed by ANSI Std Z39-18 Hindawi Publishing Corporation Stem Cells International Volume 2012, Article ID 460504, 2 pages doi:10.1155/2012/460504

  2. Depressive symptoms in Parkinson’s disease and in non-neurological medical illnesses

    Assogna F; Fagioli S; Cravello L; Meco G; Pierantozzi M; Stefani A.; Imperiale F; Caltagirone C; Pontieri FE; Spalletta G

    2013-01-01

    Francesca Assogna,1 Sabrina Fagioli,1 Luca Cravello,1 Giuseppe Meco,2 Mariangela Pierantozzi,3 Alessandro Stefani,3 Francesca Imperiale,2 Carlo Caltagirone,1,3 Francesco E Pontieri,4 Gianfranco Spalletta11I.R.C.C.S. Santa Lucia Foundation, Rome, Italy; 2Department of Neurology and Psychiatry (Parkinson’s Centre) and Research Centre of Social Diseases (CIMS), University “Sapienza”, Rome, Italy; 3Department of Neuroscience, University “Tor Vergata&rdq...

  3. Recent evidence for an expanded role of the kynurenine pathway of tryptophan metabolism in neurological diseases.

    Lovelace, Michael D; Varney, Bianca; Sundaram, Gayathri; Lennon, Matthew J; Lim, Chai K; Jacobs, Kelly; Guillemin, Gilles J; Brew, Bruce J

    2017-01-01

    The kynurenine pathway (KP) of tryptophan metabolism has emerged in recent years as a key regulator of the production of both neuroprotective (e.g. kynurenic and picolinic acid, and the essential cofactor NAD+) and neurotoxic metabolites (e.g. quinolinic acid, 3-hydroxykynurenine). The balance between the production of the two types of metabolites is controlled by key rate-limiting enzymes such as indoleamine-2,3-dioxygenase (IDO-1), and in turn, molecular signals such as interferon-γ (IFN-γ), which activate the KP metabolism of tryptophan by this enzyme, as opposed to alternative pathways for serotonin and melatonin production. Dysregulated KP metabolism has been strongly associated with neurological diseases in recent years, and is the subject of increasing efforts to understand how the metabolites are causative of disease pathology. Concurrent with these endeavours are drug development initiatives to use inhibitors to block certain enzymes in the pathway, resulting in reduced levels of neurotoxic metabolites (e.g. quinolinic acid, an excitotoxin and N-Methyl-d-Aspartate (NMDA) receptor agonist), while in turn enhancing the bioavailability of the neuroprotective metabolites such as kynurenic acid. Neurodegenerative diseases often have a substantial autoimmune or inflammatory component; hence a greater understanding of how KP metabolites influence the inflammatory cascade is required. Additionally, challenges exist in diseases like multiple sclerosis (MS) and motor neurone disease (MND), which do not have reliable biomarkers. Clinical diagnosis can often be prolonged in order to exclude other diseases, and often diagnosis occurs at an advanced state of disease pathology, which does not allow a lengthy time for patient assessment and intervention therapies. This review considers the current evidence for involvement of the KP in several neurological diseases, in biomarkers of disease and also the parallels that exist in KP metabolism with what is known in other

  4. Management of disease-modifying treatments in neurological autoimmune diseases of the central nervous system

    Salmen, A; Gold, R; Chan, A

    2014-01-01

    The therapeutic armamentarium for autoimmune diseases of the central nervous system, specifically multiple sclerosis and neuromyelitis optica, is steadily increasing, with a large spectrum of immunomodulatory and immunosuppressive agents targeting different mechanisms of the immune system. However, increasingly efficacious treatment options also entail higher potential for severe adverse drug reactions. Especially in cases failing first-line treatment, thorough evaluation of the risk–benefit profile of treatment alternatives is necessary. This argues for the need of algorithms to identify patients more likely to benefit from a specific treatment. Moreover, paradigms to stratify the risk for severe adverse drug reactions need to be established. In addition to clinical/paraclinical measures, biomarkers may aid in individualized risk–benefit assessment. A recent example is the routine testing for anti-John Cunningham virus antibodies in natalizumab-treated multiple sclerosis patients to assess the risk for the development of progressive multi-focal leucoencephalopathy. Refined algorithms for individualized risk assessment may also facilitate early initiation of induction treatment schemes in patient groups with high disease activity rather than classical escalation concepts. In this review, we will discuss approaches for individiualized risk–benefit assessment both for newly introduced agents as well as medications with established side-effect profiles. In addition to clinical parameters, we will also focus on biomarkers that may assist in patient selection. Other Articles published in this series Paraneoplastic neurological syndromes. Clinical and Experimental Immunology 2014, 175: 336–48. Disease-modifying therapy in multiple sclerosis and chronic inflammatory demyelinating polyradiculoneuropathy: common and divergent current and future strategies. Clinical and Experimental Immunology 2014, 175: 359–72. Monoclonal antibodies in treatment of multiple

  5. Hyponatremia in acute brain disease: the cerebral salt wasting syndrome.

    Betjes, Michiel G.H.

    2002-02-01

    Hyponatremia in acute brain disease is a common occurrence, especially after an aneurysmal subarachnoid hemorrhage. Originally, excessive natriuresis, called cerebral salt wasting, and later the syndrome of inappropriate antidiuretic hormone secretion (SIADH), were considered to be the causes of hyponatremia. In recent years, it has become clear that most of these patients are volume-depleted and have a negative sodium balance, consistent with the original description of cerebral salt wasting. Elevated plasma concentrations of atrial or brain natriuretic peptide have been identified as the putative natriuretic factor. Hyponatremia and volume depletion may aggravate neurological symptoms, and timely treatment with adequate replacement of water and NaCl is essential. The use of fludrocortisone to increase sodium reabsorption by the renal tubules may be an alternative approach.

  6. Neutron activation analysis of the central nervous system tissues in neurological diseases

    Yasui, Masayuki; Ota, Kiichiro [Wakayama Medical Coll. (Japan); Sasajima, Kazuhisa

    1994-07-01

    As the diseases due to excessive metals in living bodies and the metals of their causes, Minamata disease due to Hg, itai-itai disease due to Cd, dialysis brain disease due to Al, hemochromatosis due to Fe, Wilson disease due to Cu and so on have been known. Also as the neural diseases, in which the possibility that metals take part in them is presumed, there are amyotrophic lateral sclerosis, Alzheimer disease, Parkinson disease, Parkinsonism dementia and so on. In order to know the causes of the diseases due to excessive metals in living bodies and neurological diseases, the authors have measured Cu, Ca, Al, Mn, Zn and Fe in central nervous system tissues by activation analysis nondestructive method. The cases investigated were 4 cases of hepatocerebral diseases, 6 cases of ALS, 4 cases of Parkinson disease, 4 cases of Parkinsonism dementia, 4 cases of multiple sclerosis and 5 cases without CNS disease for the control. The method of measurement is described. The results for respective diseases are reported. Cu and Fe are in the relation of mirror images, and Cu formed Cu-superoxide dismutase (SOD) similarly to Zn and Mn as SOD carrier metals, and protects living bodies and CNS from oxidative stress. (K.I.).

  7. An Acute Hemorrhagic Infectious Disease:Ebola Virus Disease

    JIAO Lei; XU An-hua; FENG Chao; QIU Qian-qian; TANG Qi-ling; LIU Xiao-huan

    2014-01-01

    Ebola virus disease (EVD) is an acute hemorrhagic infectious disease caused by ebola virus, with high infectivity and fatality rate. At present, it mainly occurs in areas of Central Africa and West Africa and no effective vaccine and antiviral drugs are available for the clinical treatment.

  8. Epilepsy and other neurological diseases in the parents of children with infantile autism. A case control study

    Mouridsen, S.E.; Rich, B.; Isager, T.

    2008-01-01

    fathers the proportion was 5.7% vs 9.7%. No single neurological disease was significantly more frequent among parents of persons with IA. Our study lent support to the notion that epilepsy and other neurological diseases are not part of the broader IA phenotype Udgivelsesdato: 2008/3......In order to study the broader phenotype of infantile autism (IA) we compared the rates and types of epilepsy and other neurological diseases in the parents of 111 consecutively admitted patients with IA with a matched control group of parents of 330 children from the general population. All...

  9. Exposure to lipophilic chemicals as a cause of neurological impairments, neurodevelopmental disorders and neurodegenerative diseases.

    Zeliger, Harold I

    2013-09-01

    Many studies have associated environmental exposure to chemicals with neurological impairments (NIs) including neuropathies, cognitive, motor and sensory impairments; neurodevelopmental disorders (NDDs) including autism and attention deficit hyperactivity disorder (ADHD); neurodegenerative diseases (NDGs) including Alzheimer's disease, Parkinson's disease and amyotrophic lateral sclerosis (ALS). The environmental chemicals shown to induce all these diseases include persistent organic pollutants (POPs), the plastic exudates bisphenol A and phthalates, low molecular weight hydrocarbons (LMWHCs) and polynuclear aromatic hydrocarbons (PAHs). It is reported here that though these chemicals differ widely in their chemical properties, reactivities and known points of attack in humans, a common link does exist between them. All are lipophilic species found in serum and they promote the sequential absorption of otherwise non-absorbed toxic hydrophilic species causing these diseases.

  10. Risk of neurological diseases among survivors of electric shocks: a nationwide cohort study, Denmark, 1968-2008.

    Grell, Kathrine; Meersohn, Andrea; Schüz, Joachim; Johansen, Christoffer

    2012-09-01

    Several studies suggest a link between electric injuries and neurological diseases, where electric shocks may explain elevated risks for neuronal degeneration and, subsequently, neurological diseases. We conducted a retrospective cohort study on the risk of neurological diseases among people in Denmark who had survived an electric accident in 1968-2008. The cohort included 3,133 people and occurrences of neurological diseases were determined by linkage to the nationwide population-based Danish National Register of Patients. The numbers of cases observed at first hospital contact in the cohort were compared with the respective rates of first hospital contacts for neurological diseases in the general population. We observed significantly increased risks for peripheral nerve diseases (standardized hospitalization ratio (SHR), 1.66; 95% confidence interval (CI), 1.22-2.22), for migraine (SHR, 1.80; 95% CI, 1.23-2.54), for vertigo (SHR, 1.60; 95% CI, 1.22-2.05), and for epilepsy (SHR, 1.45; 95% CI, 1.11-1.85). Only small numbers of cases of other neurological diseases were found, making the risk estimates unstable. These findings suggest an association between a single electric shock and increased risks for peripheral nerve diseases, migraines, vertigo, and epilepsy, but confirmation of these observations is needed.

  11. Epilepsy and other neurological diseases in the parents of children with infantile autism. A case control study

    Mouridsen, Svend Erik; Rich, Bente; Isager, Torben

    2008-01-01

    In order to study the broader phenotype of infantile autism (IA) we compared the rates and types of epilepsy and other neurological diseases in the parents of 111 consecutively admitted patients with IA with a matched control group of parents of 330 children from the general population. All parti...... fathers the proportion was 5.7% vs 9.7%. No single neurological disease was significantly more frequent among parents of persons with IA. Our study lent support to the notion that epilepsy and other neurological diseases are not part of the broader IA phenotype.......In order to study the broader phenotype of infantile autism (IA) we compared the rates and types of epilepsy and other neurological diseases in the parents of 111 consecutively admitted patients with IA with a matched control group of parents of 330 children from the general population. All...

  12. Retrospective study of the clinical effects of acupuncture on cervical neurological diseases in dogs

    Liu, Ching Ming; Chang, Fang Chia

    2016-01-01

    This study was conducted to evaluate new acupuncture protocols for the clinical treatment of cervical spinal cord diseases in 19 dogs. Three treatment options containing Jing-jiaji (cervical jiaji) were developed to treat neck pain, hemiparesis, and tetraparesis depending on the severity. The interval between the neurological disease onset and treatment (duration of signs), time to improvement after treatment, and recovery time were compared in dogs by body weight, age, and dry needle acupuncture (AP) with or without electro-AP (EAP). The duration of signs was longer in dogs weighing greater than 10 kg than in those weighing less than 10 kg (p Acupuncture with Jing-jiaji was effective in cervical spinal cord diseases in different sized dogs and in middle-aged and senior dogs. This report standardized AP treatment containing Jing-jiaji for canine cervical problems and evaluated its effects. The newly standardized AP methodology offers clinical practitioners an effective way to improve the outcomes of cervical neurological diseases in dogs. PMID:26645331

  13. Functional Neurons Generated from T Cell-Derived Induced Pluripotent Stem Cells for Neurological Disease Modeling

    Takuya Matsumoto

    2016-03-01

    Full Text Available Modeling of neurological diseases using induced pluripotent stem cells (iPSCs derived from the somatic cells of patients has provided a means of elucidating pathogenic mechanisms and performing drug screening. T cells are an ideal source of patient-specific iPSCs because they can be easily obtained from samples. Recent studies indicated that iPSCs retain an epigenetic memory relating to their cell of origin that restricts their differentiation potential. The classical method of differentiation via embryoid body formation was not suitable for T cell-derived iPSCs (TiPSCs. We developed a neurosphere-based robust differentiation protocol, which enabled TiPSCs to differentiate into functional neurons, despite differences in global gene expression between TiPSCs and adult human dermal fibroblast-derived iPSCs. Furthermore, neurons derived from TiPSCs generated from a juvenile patient with Parkinson's disease exhibited several Parkinson's disease phenotypes. Therefore, we conclude that TiPSCs are a useful tool for modeling neurological diseases.

  14. microRNA involvement in developmental and functional aspects of the nervous system and in neurological diseases

    Christensen, Mette; Schratt, Gerhard M

    2009-01-01

    and early differentiation as well as in later stages of neuronal development, such as dendritogenesis and synaptic plasticity. A link between microRNAs and neurological diseases, such as neurodegeneration or synaptic dysfunction, is becoming increasingly clear. This review summarizes the current knowledge...... of the function of microRNAs in the developing and adult nervous system and their potential contribution to the etiology of neurological diseases....

  15. Treatment of Acute Pelvic Inflammatory Disease

    Richard L. Sweet

    2011-01-01

    Full Text Available Pelvic inflammatory disease (PID, one of the most common infections in nonpregnant women of reproductive age, remains an important public health problem. It is associated with major long-term sequelae, including tubal factor infertility, ectopic pregnancy, and chronic pelvic pain. In addition, treatment of acute PID and its complications incurs substantial health care costs. Prevention of these long-term sequelae is dependent upon development of treatment strategies based on knowledge of the microbiologic etiology of acute PID. It is well accepted that acute PID is a polymicrobic infection. The sexually transmitted organisms, Neisseria gonorrhoeae and Chlamydia trachomatis, are present in many cases, and microorganisms comprising the endogenous vaginal and cervical flora are frequently associated with PID. This includes anaerobic and facultative bacteria, similar to those associated with bacterial vaginosis. Genital tract mycoplasmas, most importantly Mycoplasma genitalium, have recently also been implicated as a cause of acute PID. As a consequence, treatment regimens for acute PID should provide broad spectrum coverage that is effective against these microorganisms.

  16. The mTOR signalling cascade: paving new roads to cure neurological disease.

    Crino, Peter B

    2016-07-01

    Defining the multiple roles of the mechanistic (formerly 'mammalian') target of rapamycin (mTOR) signalling pathway in neurological diseases has been an exciting and rapidly evolving story of bench-to-bedside translational research that has spanned gene mutation discovery, functional experimental validation of mutations, pharmacological pathway manipulation, and clinical trials. Alterations in the dual contributions of mTOR - regulation of cell growth and proliferation, as well as autophagy and cell death - have been found in developmental brain malformations, epilepsy, autism and intellectual disability, hypoxic-ischaemic and traumatic brain injuries, brain tumours, and neurodegenerative disorders. mTOR integrates a variety of cues, such as growth factor levels, oxygen levels, and nutrient and energy availability, to regulate protein synthesis and cell growth. In line with the positioning of mTOR as a pivotal cell signalling node, altered mTOR activation has been associated with a group of phenotypically diverse neurological disorders. To understand how altered mTOR signalling leads to such divergent phenotypes, we need insight into the differential effects of enhanced or diminished mTOR activation, the developmental context of these changes, and the cell type affected by altered signalling. A particularly exciting feature of the tale of mTOR discovery is that pharmacological mTOR inhibitors have shown clinical benefits in some neurological disorders, such as tuberous sclerosis complex, and are being considered for clinical trials in epilepsy, autism, dementia, traumatic brain injury, and stroke.

  17. Bullous pemphigoid in a leg affected with hemiparesia: a possible relation of neurological diseases with bullous pemphigoid?

    Foureur, N; Descamps, V; Lebrun-Vignes, B; Picard-Dahan, C; Grossin, M; Belaich, S; Crickx, B

    2001-01-01

    We report a typical case of bullous pemphigoid (BP) associated with a neurological disorder and study a possible link between neurological disorders and BP. An 84-year-old hemiplegic woman presented with unilateral BP on the hemiparetic side. BP was confirmed by histological and immunofluorescence data. The medical records of the previous 46 consecutive patients with BP were retrospectively analyzed (average age: 79; median age: 85). Thirty of the 46 patients with BP had neurological disorders. These disorders included dementia, epilepsy, multiple sclerosis, cerebral stroke, Parkinson's disease, gonadotropic adenoma, trembling, dyskinesia, lumbar spinal stenosis. In a control group of the 46 consecutive oldest patients (older than 71; average age: 82,5; median age: 80) with another skin disease referred during the previous two-year-period to our one-day-unit only, 13 patients had a neurological disorder. This study demonstrates that there is a high prevalence of neurological disorders in patients with BP (p = 0.0004). A prospective case control study with neurological examination and psychometrical evaluation is warranted to confirm these data. We speculate that neuroautoimmunity associated with the aging process or neurological disorders may be involved in pemphigoid development via an autoimmune response against dystonin which shares homology with bullous pemphigoid antigen 1. Bullous pemphigoid could be considered to be a marker of neurological disorder.

  18. Epigenetic Modifications in Neurological Diseases: Natural Products as Epigenetic Modulators a Treatment Strategy.

    Gangisetty, Omkaram; Murugan, Sengottuvelan

    2016-01-01

    Epigenetic modifications, including DNA methylation, covalent histone modifications, and small noncoding RNAs, play a key role in regulating the gene expression. This regulatory mechanism is important in cellular differentiation and development. Recent advances in the field of epigenetics extended the role of epigenetic mechanisms in controlling key biological processes such as genome imprinting and X-chromosome inactivation. Aberrant epigenetic modifications are associated with the development of many diseases. The role of epigenetic modifications in various neurodegenerative disorders including Alzheimer's disease, Parkinson's disease, Huntington disease, epilepsy, and multiple sclerosis is rapidly emerging. The use of epigenetic modifying drugs to treat these diseases has been the interest in recent years. A number of natural products having diverse mechanism of action are used for drug discovery. For many years, natural compounds have been used to treat various neurodegenerative diseases, but the use of such compounds as epigenetic modulators to reverse or treat neurological diseases are not well studied. In this chapter, we mainly focus on how various epigenetic modifications play a key role in neurodegenerative diseases, their mechanism of action, and how it acts as a potential therapeutic target for epigenetic drugs to treat these diseases will be discussed.

  19. Chagas' Disease: an acute transfusional case report

    Dalva Marli Valério Wanderley

    1988-12-01

    Full Text Available Report of a case of acute transfusional Chagas'disease in a four-year-old child with a previous diagnosis of acute lymphocytic leukemia, transmitted in São Paulo, the Capital of São Paulo State, Brazil. Epidemiological investigation disclosed the donor's serological positivity and his previous residence in an area where Chagas' disease is endemic. The importance of adequate sorological screening in blood donors is evident. It should be stressed that this is the first case notified to the Superintendência de Controle de Endemias (SUCEN (Superintendency for the Endemy Control of the State Secretariat of Health, São Paulo, for the last five years.

  20. Acute recurrent pancreatitis: An autoimmune disease?

    Raffaele Pezzilli

    2008-01-01

    In this review article,we will briefly describe the main characteristics of autoimmune pancreatitis and then we will concentrate on our aim,namely,evaluating the clinical characteristics of patients having recurrence of pain from the disease.In fact,the open question is to evaluate the possible presence of autoimmune pancreatitis in patients with an undefined etiology of acute pancreatitis and for this reason we carried out a search in the literature in order to explore this issue.In cases of recurrent attacks of pain in patients with "idiopathic"pancreatitis,we need to keep in mind the possibility that our patients may have autoimmune pancreatitis.Even though the frequency of this disease seems to be quite low,we believe that in the future,by increasing our knowledge on the subject,we will be able to diagnose an ever-increasing number of patients having acute recurrence of pain from autoimmune pancreatitis.

  1. Replication Validity of Initial Association Studies: A Comparison between Psychiatry, Neurology and Four Somatic Diseases

    Dumas-Mallet, Estelle; Button, Katherine; Boraud, Thomas; Munafo, Marcus; Gonon, François

    2016-01-01

    Context There are growing concerns about effect size inflation and replication validity of association studies, but few observational investigations have explored the extent of these problems. Objective Using meta-analyses to measure the reliability of initial studies and explore whether this varies across biomedical domains and study types (cognitive/behavioral, brain imaging, genetic and “others”). Methods We analyzed 663 meta-analyses describing associations between markers or risk factors and 12 pathologies within three biomedical domains (psychiatry, neurology and four somatic diseases). We collected the effect size, sample size, publication year and Impact Factor of initial studies, largest studies (i.e., with the largest sample size) and the corresponding meta-analyses. Initial studies were considered as replicated if they were in nominal agreement with meta-analyses and if their effect size inflation was below 100%. Results Nominal agreement between initial studies and meta-analyses regarding the presence of a significant effect was not better than chance in psychiatry, whereas it was somewhat better in neurology and somatic diseases. Whereas effect sizes reported by largest studies and meta-analyses were similar, most of those reported by initial studies were inflated. Among the 256 initial studies reporting a significant effect (p<0.05) and paired with significant meta-analyses, 97 effect sizes were inflated by more than 100%. Nominal agreement and effect size inflation varied with the biomedical domain and study type. Indeed, the replication rate of initial studies reporting a significant effect ranged from 6.3% for genetic studies in psychiatry to 86.4% for cognitive/behavioral studies. Comparison between eight subgroups shows that replication rate decreases with sample size and “true” effect size. We observed no evidence of association between replication rate and publication year or Impact Factor. Conclusion The differences in reliability

  2. Intravenous thrombolytic treatment experiences in patients with acute ischemic stroke at the University of Kocatepe, Neurology Clinics

    Serdar Oruç

    2015-12-01

    Full Text Available INTRODUCTION: This study aimed to discuss the results of the intravenous thrombolytic treatment (IV-tPA to acute ischemic stroke patients, in the light of the literature. METHODS: We performed our study with forty acute ischemic stroke patients who were receiving the IV-tPA in the intensive care unit of our neurology clinic between 2011 and 2015.. The demographic, clinical and radiological data were collected retrospectively. The intracranial hemorrhage detected within 3 months after discharge and neurological status at the end of the 3rd month were evaluated by using modified Rankin scale (MRS and National Institutes of Health Stroke Scale (NIHSS scores. The symptom-to-needle time, Alberta stroke programe early computed tomography score (ASPECT and initial and follow-up scores of NIHSS were analyzed. RESULTS: Fifteen patients were female, twenty-five were male, and the mean age was 66.45±10.56. The initial mean NIHSS score was 13±4.33, whereas it was 4,10±3,37at 3rd month. The initial mean ASPECT score was 8.23±1.20. Symptomatic intracranial hemorrhage was detected in 1 patient and asymptomatic intracranial hemorrhage was detected in 6. The mean symptom-to-needle time was 139,0±48,1 minutes. The neurological disability of 13 patients ( %32.5 were fully recovered at the end of the 3rd month, while 7 patients were died. (% 17,5 The initial NIHSS and ASPECT scores were significantly different between group of patients with a MRS score between 0-2 and between 3-6 (p=0.03 and p=0.006; respectively, while the symptom-to-needle time was not different (p=0.79. DISCUSSION AND CONCLUSION: The results of the current study are in accordance with previous studies in the literature. These results have shown that the IV-tPA treatment is efficient and safe treatment modality in acute ischemic stroke, and reduces disability at the end of the 3rd month.

  3. Fatal acute Chagas Disease in a Chimpanzee

    2009-08-01

    protozoan parasite belonging to the order Kinetoplastida, family Trypanosomatidae. Arthropod vectors (Reduviidae, assassin bugs or cone nosed bugs or...Laranja FS. Experimental Chagas’ disease in rhesus monkeys. I. Clinical, parasitological , hematological and anatomo- pathological studies in the acute...of infection and immunity from mother to young. Parasitology . 1972; 65:1–9. [PubMed: 4626452] 21. Miles MA, Marsden PD, Pettitt LE, Draper CC, Watson

  4. Lyme disease presenting as isolated acute urinary retention caused by transverse myelitis: an electrophysiological and urodynamical study.

    Olivares, J P; Pallas, F; Ceccaldi, M; Viton, J M; Raoult, D; Planche, D; Delarque, A

    1995-12-01

    Several neurological manifestations of Lyme disease, both central and peripheral, have been described. Reported here is a case of acute transverse myelitis related to a Lyme neuroborreliosis that presented with isolated acute urinary retention and no lower-extremity impairment. This case, documented by urodynamic and electrophysiological investigations, partially resolved after 6 weeks of intravenous ceftriaxone, affording the removal of the indwelling catheter. Alpha blocker therapy was needed for 3 months, until the complete normalisation of urodynamic and electrophysiological records. This case study indicates that whenever urinary retention is encountered associated with acute transverse myelitis or alone, the patient should be investigated for Lyme disease.

  5. Using phosphate supplementation to reverse hypophosphatemia and phosphate depletion in neurological disease and disturbance.

    Håglin, Lena

    2016-06-01

    Hypophosphatemia (HP) with or without intracellular depletion of inorganic phosphate (Pi) and adenosine triphosphate has been associated with central and peripheral nervous system complications and can be observed in various diseases and conditions related to respiratory alkalosis, alcoholism (alcohol withdrawal), diabetic ketoacidosis, malnutrition, obesity, and parenteral and enteral nutrition. In addition, HP may explain serious muscular, neurological, and haematological disorders and may cause peripheral neuropathy with paresthesias and metabolic encephalopathy, resulting in confusion and seizures. The neuropathy may be improved quickly after proper phosphate replacement. Phosphate depletion has been corrected using potassium-phosphate infusion, a treatment that can restore consciousness. In severe ataxia and tetra paresis, complete recovery can occur after adequate replacement of phosphate. Patients with multiple risk factors, often with a chronic disease and severe HP that contribute to phosphate depletion, are at risk for neurologic alterations. To predict both risk and optimal phosphate replenishment requires assessing the nutritional status and risk for re-feeding hypophosphatemia. The strategy for correcting HP depends on the severity of the underlying disease and the goal for re-establishing a phosphate balance to limit the consequences of phosphate depletion.

  6. Involuntary emotional expression disorder - new/old disease in psychiatry and neurology.

    Presecki, Paola; Mimica, Ninoslav

    2007-09-01

    Involuntary emotional expression disorder (IEED) is underrecognized by clinicians, misdiagnosed as depression or bipolar disorder and undertreated, because clinicians are unfamiliar with the disorder. An important clinical consideration for IEED is that of distinguishing mood from affect. IEED describes a syndrome of relatively stereotypical episodes of uncontrollable crying and/or laughing, resulting from lesions of multiple types, in multiple brain regions, without an apparent stimulus to trigger such responses. This syndrome is common among a number of neurological diseases like patients with a stroke or traumatic brain injury (TBI), patients with amyotrophic lateral sclerosis (ALS), multiple sclerosis (MS), as well as dementias such as Alzheimer;s disease (AD), and motor disorders such as Parkinson;s disease (PD). The neuropathological cause and neurochemistry of the disorder remains unclear. There is general agreement that IEED is the result of an injury to the neurological pathways that control the expression of emotions. Adequate treatment can reduce the frequency and improve the quality of life of patients and caregivers.

  7. Introduction to Focus Issue: Rhythms and Dynamic Transitions in Neurological Disease: Modeling, Computation, and Experiment

    Kaper, Tasso J., E-mail: tasso@bu.edu; Kramer, Mark A., E-mail: mak@bu.edu [Department of Mathematics and Statistics, Boston University, Boston, Massachusetts 02215 (United States); Rotstein, Horacio G., E-mail: horacio@njit.edu [Department of Mathematical Sciences, New Jersey Institute of Technology, Newark, New Jersey 07102 (United States)

    2013-12-15

    Rhythmic neuronal oscillations across a broad range of frequencies, as well as spatiotemporal phenomena, such as waves and bumps, have been observed in various areas of the brain and proposed as critical to brain function. While there is a long and distinguished history of studying rhythms in nerve cells and neuronal networks in healthy organisms, the association and analysis of rhythms to diseases are more recent developments. Indeed, it is now thought that certain aspects of diseases of the nervous system, such as epilepsy, schizophrenia, Parkinson's, and sleep disorders, are associated with transitions or disruptions of neurological rhythms. This focus issue brings together articles presenting modeling, computational, analytical, and experimental perspectives about rhythms and dynamic transitions between them that are associated to various diseases.

  8. Introduction to Focus Issue: Rhythms and Dynamic Transitions in Neurological Disease: Modeling, Computation, and Experiment

    Kaper, Tasso J.; Kramer, Mark A.; Rotstein, Horacio G.

    2013-12-01

    Rhythmic neuronal oscillations across a broad range of frequencies, as well as spatiotemporal phenomena, such as waves and bumps, have been observed in various areas of the brain and proposed as critical to brain function. While there is a long and distinguished history of studying rhythms in nerve cells and neuronal networks in healthy organisms, the association and analysis of rhythms to diseases are more recent developments. Indeed, it is now thought that certain aspects of diseases of the nervous system, such as epilepsy, schizophrenia, Parkinson's, and sleep disorders, are associated with transitions or disruptions of neurological rhythms. This focus issue brings together articles presenting modeling, computational, analytical, and experimental perspectives about rhythms and dynamic transitions between them that are associated to various diseases.

  9. Acute onset anarthria without hepatic manifestation: a rare presentation of Wilson disease.

    Verma, Rajesh; Bhandari, Aveg; Tiwari, Navin; Chaudhari, Tejendra S

    2013-08-20

    Wilson disease (WD) is one of the few inherited but treatable disorder mainly affecting the liver and brain resulting in severe disability or death if left untreated. Hence, it is important to keep a high index of suspicion for diagnosing this clinical entity in appropriate clinical settings. The clinical presentation can be quite variable and they may present solely with neurological features sans hepatic symptoms. Such neurological manifestations usually follow subacute to chronic course. Acute onset anarthria as the heralding and predominant presenting feature has been rarely reported in the literature. We reported a case of a 12-year-old girl who presented with acute onset anarthria and dystonia of 1-month duration. On further evaluation, a diagnosis of WD was made. The patient showed partial improvement after she was started on copper chelating agents and anticholinergics.

  10. Clinical NMR imaging of the brain in children: normal and neurologic disease

    Johnson, M.A, (Hammersmith Hospital, London, England); Pennock, J.M.; Bydder, G.M.; Steiner, R.E.; Thomas, D.J.; Hayward, R.; Bryant, D.R.T.; Payne, J.A.; Levene, M.I.; Whitelaw, A.; Dubowitz, L.M.S.; Dubowitz, V.

    1983-11-01

    The results of initial clinical nuclear magnetic resonance imaging of the brain in eight normal and 52 children with a wide variety of neurologic diseases were reviewed. The high level of gray-white matter contrast available with inversion-recovery sequences provided a basis for visualizing normal myelination as well as delays or deficits in this process. The appearances seen in cases of parenchymal hemorrhage, cerebral infarction, and proencephalic cysts are described. Ventricular enlargement was readily identified and marginal edema was demonstrated with spin-echo sequences. Abnormalities were seen in cerebral palsy, congenital malformations, Hallervorden-Spatz disease, aminoaciduria, and meningitis. Space-occupying lesions were identified by virtue of their increased relaxation times and mass effects. Nuclear magnetic resonance imaging has considerable potential in pediatric neuroradiologic practice, in some conditions supplying information not available by computed tomography or sonography.

  11. Serum brain-derived neurotrophic factor levels in different neurological diseases.

    Ventriglia, Mariacarla; Zanardini, Roberta; Bonomini, Cristina; Zanetti, Orazio; Volpe, Daniele; Pasqualetti, Patrizio; Gennarelli, Massimo; Bocchio-Chiavetto, Luisella

    2013-01-01

    Consistent evidence indicates the involvement of the brain-derived neurotrophic factor (BDNF) in neurodegenerative disorders such as Alzheimer's disease (AD) and Parkinson's disease (PD). In the present study, we compared serum BDNF in 624 subjects: 266 patients affected by AD, 28 by frontotemporal dementia (FTD), 40 by Lewy body dementia (LBD), 91 by vascular dementia (VAD), 30 by PD, and 169 controls. Our results evidenced lower BDNF serum levels in AD, FTD, LBD, and VAD patients (P benzodiazepines (P = 0.020). In conclusion, our results support the role of BDNF alterations in neurodegenerative mechanisms common to different forms of neurological disorders and underline the importance of including drug treatment in the analyses to avoid confounding effects.

  12. A novel porcine model of ataxia telangiectasia reproduces neurological features and motor deficits of human disease.

    Beraldi, Rosanna; Chan, Chun-Hung; Rogers, Christopher S; Kovács, Attila D; Meyerholz, David K; Trantzas, Constantin; Lambertz, Allyn M; Darbro, Benjamin W; Weber, Krystal L; White, Katherine A M; Rheeden, Richard V; Kruer, Michael C; Dacken, Brian A; Wang, Xiao-Jun; Davis, Bryan T; Rohret, Judy A; Struzynski, Jason T; Rohret, Frank A; Weimer, Jill M; Pearce, David A

    2015-11-15

    Ataxia telangiectasia (AT) is a progressive multisystem disorder caused by mutations in the AT-mutated (ATM) gene. AT is a neurodegenerative disease primarily characterized by cerebellar degeneration in children leading to motor impairment. The disease progresses with other clinical manifestations including oculocutaneous telangiectasia, immune disorders, increased susceptibly to cancer and respiratory infections. Although genetic investigations and physiological models have established the linkage of ATM with AT onset, the mechanisms linking ATM to neurodegeneration remain undetermined, hindering therapeutic development. Several murine models of AT have been successfully generated showing some of the clinical manifestations of the disease, however they do not fully recapitulate the hallmark neurological phenotype, thus highlighting the need for a more suitable animal model. We engineered a novel porcine model of AT to better phenocopy the disease and bridge the gap between human and current animal models. The initial characterization of AT pigs revealed early cerebellar lesions including loss of Purkinje cells (PCs) and altered cytoarchitecture suggesting a developmental etiology for AT and could advocate for early therapies for AT patients. In addition, similar to patients, AT pigs show growth retardation and develop motor deficit phenotypes. By using the porcine system to model human AT, we established the first animal model showing PC loss and motor features of the human disease. The novel AT pig provides new opportunities to unmask functions and roles of ATM in AT disease and in physiological conditions.

  13. The role of hyperhomocysteinemia in neurological features associated with coeliac disease.

    Ferretti, Alessandro; Parisi, Pasquale; Villa, Maria Pia

    2013-10-01

    Although a range of neurological and psychiatric disorders are widely reported to be associated with coeliac patients, their pathogenesis remains unclear. Some such disorders are believed to be secondary to vitamin deficiency due to malabsorption, others to immune mechanisms. We hypothesise that hyperhomocysteinemia might, by damaging the blood-brain barrier, expose neuronal tissue to all neuro-irritative metabolites, such as homocysteine itself, a neurotoxic excitatory and proconvulsant amino acid. Neurons respond to these stimuli through hyperexcitability, thereby predisposing subjects to neurological disorders such as epilepsy and headache. Furthermore, persisting endothelial damage may cause blood extravasation and subsequent deposition of calcium salts. We suggest that this might be the pathogenesis of the CEC syndrome, which is characterized by the association of coeliac disease, epilepsy and cerebral calcifications. Indeed, homocysteine plays a well-known role in cardiovascular endothelial dysfunction, with high serum and cerebrospinal fluid levels often being reported in coeliac patients. Moreover, data in the literature show a strong, growing association of homocysteine with epilepsy and migraine in non-coeliac subjects. Despite these findings, homocysteine has never been held directly responsible for neuronal functional features (neuronal hyperexcitability underlying epilepsy and migraine) and structural brain damage (expressed as cerebral calcification) in coeliac patients. Damage to the blood-brain barrier might also facilitate immune reactions against neuronal tissue to a considerable extent. This hypothesis combines the two afore-mentioned theories (vitamin deficiency due to malabsorption and immune mechanisms). We also wish to point out that no studies have yet investigated the prevalence of neuronal hyperexcitability and subclinical electroencephalic abnormalities in children and adults with newly-diagnosed coeliac disease before the introduction of

  14. Maternal stress induces epigenetic signatures of psychiatric and neurological diseases in the offspring.

    Fabiola C R Zucchi

    Full Text Available The gestational state is a period of particular vulnerability to diseases that affect maternal and fetal health. Stress during gestation may represent a powerful influence on maternal mental health and offspring brain plasticity and development. Here we show that the fetal transcriptome, through microRNA (miRNA regulation, responds to prenatal stress in association with epigenetic signatures of psychiatric and neurological diseases. Pregnant Long-Evans rats were assigned to stress from gestational days 12 to 18 while others served as handled controls. Gestational stress in the dam disrupted parturient maternal behaviour and was accompanied by characteristic brain miRNA profiles in the mother and her offspring, and altered transcriptomic brain profiles in the offspring. In the offspring brains, prenatal stress upregulated miR-103, which is involved in brain pathologies, and downregulated its potential gene target Ptplb. Prenatal stress downregulated miR-145, a marker of multiple sclerosis in humans. Prenatal stress also upregulated miR-323 and miR-98, which may alter inflammatory responses in the brain. Furthermore, prenatal stress upregulated miR-219, which targets the gene Dazap1. Both miR-219 and Dazap1 are putative markers of schizophrenia and bipolar affective disorder in humans. Offspring transcriptomic changes included genes related to development, axonal guidance and neuropathology. These findings indicate that prenatal stress modifies epigenetic signatures linked to disease during critical periods of fetal brain development. These observations provide a new mechanistic association between environmental and genetic risk factors in psychiatric and neurological disease.

  15. [Acute neurological disclosure of B12 avitaminosis induced by folic acid administration].

    Ammar, N; Martinez Almoyna, L; Husson, H; De Broucker, T

    2005-04-01

    A paradigmatic case of acute combined spinal cord degeneration and delirium due to inappropriate administration of folic acid in the context of chronic cobalamin deficiency is described. Rapid improvement was obtained with immediate cessation of folate administration and parenteral cobalamin supplementation. Folic acid and cobalamin prescription rules are recalled. Pathophysiological hypotheses tentatively explaining the neurotoxicity of folic acid in case of vitamin B12 deficiency are summarized.

  16. Crohn's disease and acute pancreatitis. A review of literature.

    Jasdanwala, Sarfaraz; Babyatsky, Mark

    2015-03-01

    Crohn's disease, a transmural inflammatory bowel disease, has many well-known extra-intestinal manifestations and complications. Although acute pancreatitis has a higher incidence in patients with Crohn's disease as compared to the general population, acute pancreatitis is still relatively uncommon in patients with Crohn's disease. Patients with Crohn's disease are at an approximately fourfold higher risk than the general population to develop acute pancreatitis. The risk of developing acute pancreatitis is higher in females as compared to males. Acute pancreatitis can occur at any age with higher incidence reported in patients in their 20s and between 40-50 years of age. The severity and prognosis of acute pancreatitis in patients with Crohn's disease is the same as in general population. Acute pancreatitis can occur before onset of intestinal Crohn's disease, this presentation being more common in children than adults. It can also occur as the presenting symptom. However, most commonly it occurs after intestinal symptoms have manifest with a mean time interval between the initial presentation and development of acute pancreatitis being 2 years. There are several etiological factors contributing to acute pancreatitis in patients with Crohn's disease. It is not clear whether acute pancreatitis is a direct extra-intestinal manifestation of Crohn's disease; however, majority of the cases of acute pancreatitis in patients with Crohn's disease are due to GS and medications. Drugs used for the treatment of Crohn's disease that have been reported to cause acute pancreatitis include 5-ASA agents, azathioprine and 6 mercaptopurine, metornidazole and corticosteroids. Recent evidence has emerged correlating both type 1 and 2 autoimmune pancreatitis with Crohn's disease. Understanding the association between the two disease entities is key to effectively manage patients with Crohn's disease and acute pancreatitis.

  17. Disseminated necrotizing myeloencephalitis: a herpes-associated neurological disease of horses.

    Little, P B; Thorsen, J

    1976-01-01

    Equine viral rhinopneumonitis type I virus was isolated from spinal cord and brain of a paraparetic horse with disseminated necrotizing myeloencephalitis. Necrotic arteriolitis,nonsuppurative necrotizing myeloencephalitis and Gasserian ganglioneuritis were present. On record were 12 more cases of horses with similar lesions. The horses had been ataxic or paretic for up to several weeks. A field survey indicated that 14 of 24 horses with acute myelitic signs developed them after recent exposure to respiratory disease.

  18. Health implications of creatine: can oral creatine supplementation protect against neurological and atherosclerotic disease?

    Wyss, Markus; Schulze, Andreas

    2002-01-01

    Major achievements made over the last several years have highlighted the important roles of creatine and the creatine kinase reaction in health and disease. Inborn errors of metabolism have been identified in the three main steps involved in creatine metabolism: arginine:glycine amidinotransferase (AGAT), S-adenosyl-L-methionine:N-guanidinoacetate methyltransferase (GAMT), and the creatine transporter. All these diseases are characterized by a lack of creatine and phosphorylcreatine in the brain, and by (severe) mental retardation. Similarly, knockout mice lacking the brain cytosolic and mitochondrial isoenzymes of creatine kinase displayed a slightly increased creatine concentration, but no phosphorylcreatine in the brain. These mice revealed decreased weight gain and reduced life expectancy, disturbed fat metabolism, behavioral abnormalities and impaired learning capacity. Oral creatine supplementation improved the clinical symptoms in both AGAT and GAMT deficiency, but not in creatine transporter deficiency. In addition, creatine supplementation displayed neuroprotective effects in several animal models of neurological disease, such as Huntington's disease, Parkinson's disease, or amyotrophic lateral sclerosis. All these findings pinpoint to a close correlation between the functional capacity of the creatine kinase/phosphorylcreatine/creatine system and proper brain function. They also offer a starting-point for novel means of delaying neurodegenerative disease, and/or for strengthening memory function and intellectual capabilities.Finally, creatine biosynthesis has been postulated as a major effector of homocysteine concentration in the plasma, which has been identified as an independent graded risk factor for atherosclerotic disease. By decreasing homocysteine production, oral creatine supplementation may, thus, also lower the risk for developing, e.g., coronary heart disease or cerebrovascular disease. Although compelling, these results require further

  19. [Acute encephalic manifestations in Senegalese children with sickle cell disease].

    Diagne, I; Diagne-Guèye, N R; Fall, L; Ndiaye, O; Camara, B; Diouf, S; Signate-Sy, H; Kuakuvi, N

    2001-01-01

    The course of sickle cell disease (SCD) may be complicated by neurologic events, mainly bactérial meningitidis and stroke. We retrospectively studied all cases with acute encephalic manifestations (AEM) in a cohort of 461 children and adolescents with SCD followed at Albert Royer Children Hospital of Dakar (Senegal) from january 1991 to december 2000 (ten years). Among them 438 had sickle cell anemia (SCA), 19 SC disease and 4 S-beta thalassemia (3 S-beta+, 1 S-beta0). Seven patients, all with SCA, presented antecedents of AEM revealed by flacid and proportionnal hemiplegia evoking stroke. Prevalence of these AEM was 1.5 per cent among patients with SCD and 1.6 per cent among those with SCA. They were 4 girls and 3 boys (sex ratio = 0.75) aged 4 to 8.5 years when occurred the first accident. We observed no clinical or biological distinctive characteristic of SCA in these patients compared to those without crebrovascular accident. Recurrence was observed once in a boy after a 12 months interval and twice in a girl after 20 and 60 months intervals successively. No transfusionnal program was applied to prevent recurrent stroke because of insufficient conditions for long-term transfusion. Stroke appears to be rare in senegalese children with SCD. However it poses in our context the major problem of applicability of transfusionnal program which constitute the only therapy universally recognised to be effective to prevent recurrence. Nevertheless hydroxyurea could be a satisfactory alternative.

  20. Clarithromycin-induced acute interstitial nephritis and minimal change disease

    Russell, Wendy; Smith, William

    2009-01-01

    Drug associated acute interstitial nephritis and minimal change disease has been well documented but the simultaneous presentation of both is rare and has not been reported with clarithromycin. We describe a case of simultaneous acute tubulointerstitial nephritis and minimal change disease induced by clarithromycin. The patient had acute kidney injury, nephrotic syndrome, eosinophilic pneumonitis and a maculopapular skin rash. The role of steroid therapy in acute interstitial nephritis is con...

  1. West Nile virus lineage 2 as a cause of zoonotic neurological disease in humans and horses in southern Africa.

    Venter, Marietjie; Swanepoel, Robert

    2010-10-01

    West Nile virus (WNV) is widely distributed in South Africa, but since a few cases of neurological disease have been reported from this region, endemic lineage 2 strains were postulated to be of low virulence. Several cases of nonfatal encephalitis in humans as well as fatal cases in a foal, dog, and ostrich chicks have, however, been associated with lineage 2 WNV in South Africa. The pathogenesis of lineage 2 WNV strains was investigated using mouse neuroinvasive experiments, gene expression experiments, and genome sequence comparisons which indicated that lineage 2 strains that are highly pathogenic exist. To determine whether cases of WNV were being missed in South Africa, horses with fever and neurological disease were investigated. Several cases of WNV were identified, all associated with severe neurological disease, 85% of which had to be euthanized or died. All cases positive by RT-PCR were shown to belong to lineage 2 WNV by DNA sequencing and phylogenetic analysis. Two cases of occupational infection were investigated, including a case of zoonotic transmission to a veterinarian who performed an autopsy on one of the horses as well as a laboratory infection after a needle stick injury with a neuroinvasive lineage 2 strain. Both resulted in neurological disease. Cytokine expression was investigated in the second case to assess the immunopathogenesis of WNV. Collectively, these studies suggest that lineage 2 WNV may be significantly under estimated as a cause of neurological disease in South Africa.

  2. Clinical features, MRI brain, and MRS abnormalities of drug-naïve neurologic Wilson′s disease

    Satyabrata Pulai

    2014-01-01

    Full Text Available Background: Magnetic resonance imaging (MRI helps in the diagnosis of neurologic Wilson′s disease (WD. The literature regarding MR spectroscopy (MRS and diffusion-weighted imaging (DWI in WD is limited. Objectives: To evaluate the clinical features and neuroimaging findings in drug-naοve neurologic WD and to find correlation between clinical stage and disease duration with different imaging findings. Materials and Methods: The study subjects included consecutive and follow-up neurologic WD patients attending movement disorder clinic. The initial clinical and MRI features before commencement of chelation therapy were noted. Of 78 patients, 34 underwent DWI study and MRS was done in 38 patients and in 32 control subjects. Results: Dystonia, dysarthria, tremor, and behavioral abnormality were common presenting features. All patients had MRI abnormality with major affection of basal ganglia. The clinical severity and anatomical extent of MRI abnormalities were positively correlated (P < 0.001; r s = 0.709. Presence of diffusion restriction was inversely related to duration of disease (P < 0.001; r s = 0.760. WD patients had reduced N-acetylaspartate/creatine (Cr and choline (Cho/Cr ratio (P < 0.001 as compared with control subjects in MRS study. Conclusion: Dystonia, dysarthria and tremor are common neurological features of WD. In this study, MRI abnormalities were positively correlated with disease severity; diffusion restriction was inversely correlated with the duration of the disease process. MRS was also a sensitive tool for diagnosing patient of neurologic WD.

  3. Neurologic Complications Associated with Sjögren’s Disease: Case Reports and Modern Pathogenic Dilemma

    Michele Colaci

    2014-01-01

    Full Text Available Objectives. Sjögren’s syndrome (SS may be complicated by some neurological manifestations, generally sensory polyneuropathy. Furthermore, involvement of cranial nerves was described as rare complications of SS. Methods. We reported 2 cases: the first one was a 40-year-old woman who developed neuritis of the left optic nerve as presenting symptom few years before the diagnosis of SS; the second was a 54-year-old woman who presented a paralysis of the right phrenic nerve 7 years after the SS onset. An exhaustive review of the literature on patients with cranial or phrenic nerve involvements was also carried out. Results. To the best of our knowledge, our second case represents the first observation of SS-associated phrenic nerve mononeuritis, while optic neuritis represents the most frequent cranial nerve involvement detectable in this connective tissue disease. Trigeminal neuropathy is also frequently reported, whereas neuritis involving the other cranial nerves is quite rare. Conclusions. Cranial nerve injury is a harmful complication of SS, even if less commonly recorded compared to peripheral neuropathy. Neurological manifestations may precede the clinical onset of SS; therefore, in patients with apparently isolated cranial nerve involvement, a correct diagnosis of the underlying SS is often delayed or overlooked entirely; in these instances, standard clinicoserological assessment is recommendable.

  4. A neuropsychological comparison of siblings with neurological versus hepatic symptoms of Wilson's Disease.

    Arguedas, Deborah; Stewart, Jeanette; Hodgkinson, Suzanne; Batchelor, Jennifer

    2015-01-01

    Wilson's Disease (WD) (also known as hepatolenticular degeneration) is a rare inherited autosomal recessive disorder of abnormal copper metabolism, with an estimated prevalence of approximately 1 in 30,000. The clinical features associated with WD are highly varied. However, subtypes generally reflect neurological, hepatic, and psychiatric symptoms. The present case study reports two brothers with a recent diagnosis of WD. Neurological symptoms and cognitive deficits were exhibited in one brother (BL) in the form of extrapyramidal features, while the other brother (AL) only exhibited hepatic symptoms. Extensive neuropsychological testing was conducted on both siblings to compare cognitive profiles. Results for BL indicated significantly impaired motor functioning and information processing speed, which impacted him significantly at school. Aspects of executive dysfunction were also apparent in addition to reduced visual and verbal memory, working memory, and attention. Results for AL revealed evidence of verbal memory difficulties and aspects of executive dysfunction. Comparison is made of the distinct and common cognitive characteristics of the cases presented in terms of implications for early intervention and management of cognitive difficulties.

  5. A case of acute viral hepatitis interfering with acute fatty liver disease of pregnancy

    Abdulkadir Turgut

    2013-03-01

    Full Text Available Acute hepatitis A is a rarely seen infection during pregnancy.In terms of clinical and laboratory findings, it can beinterfere with acute fatty liver disease which can be quitemortal during pregnancy. Since liver function tests are elevatedin both conditions, hepatitis A infection should alsobe kept in mind in differential diagnosis. We present a 30year-old pregnant woman with 35 weeks of gestation whopresented to our clinic with a suspection of acute fattyliver disease but finally diagnosed as acute hepatitis A infection.J Clin Exp Invest 2013; 4 (1: 123-125Key words: Hepatitis A, pregnancy, acute fatty liver disease

  6. Using Support Vector Machine to identify imaging biomarkers of neurological and psychiatric disease: a critical review.

    Orrù, Graziella; Pettersson-Yeo, William; Marquand, Andre F; Sartori, Giuseppe; Mechelli, Andrea

    2012-04-01

    Standard univariate analysis of neuroimaging data has revealed a host of neuroanatomical and functional differences between healthy individuals and patients suffering a wide range of neurological and psychiatric disorders. Significant only at group level however these findings have had limited clinical translation, and recent attention has turned toward alternative forms of analysis, including Support-Vector-Machine (SVM). A type of machine learning, SVM allows categorisation of an individual's previously unseen data into a predefined group using a classification algorithm, developed on a training data set. In recent years, SVM has been successfully applied in the context of disease diagnosis, transition prediction and treatment prognosis, using both structural and functional neuroimaging data. Here we provide a brief overview of the method and review those studies that applied it to the investigation of Alzheimer's disease, schizophrenia, major depression, bipolar disorder, presymptomatic Huntington's disease, Parkinson's disease and autistic spectrum disorder. We conclude by discussing the main theoretical and practical challenges associated with the implementation of this method into the clinic and possible future directions.

  7. Acute renal dysfunction in liver diseases

    2007-01-01

    Renal dysfunction is common in liver diseases, either as part of multiorgan involvement in acute illness or secondary to advanced liver disease. The presence of renal impairment in both groups is a poor prognostic indicator. Renal failure is often multifactorial and can present as pre-renal or intrinsic renal dysfunction. Obstructive or post renal dysfunction only rarely complicates liver disease. Hepatorenal syndrome (MRS) is a unique form of renal failure associated with advanced liver disease or cirrhosis, and is characterized by functional renal impairment without significant changes in renal histology. Irrespective of the type of renal failure, renal hypoperfusion is the central pathogenetic mechanism, due either to reduced perfusion pressure or increased renal vascular resistance. Volume expansion, avoidance of precipitating factors and treatment of underlying liver disease constitute the mainstay of therapy to prevent and reverse renal impairment. Splanchnic vasoconstrictor agents, such as terlipressin, along with volume expansion, and early placement of transjugular intrahepatic portosystemic shunt (TIPS) may be effective in improving renal function in HRS. Continuous renal replacement therapy (CRRT) and molecular absorbent recirculating system (MARS) in selected patients may be life saving while awaiting liver transplantation.

  8. Identification and validation of clinical predictors for the risk of neurological involvement in children with hand, foot, and mouth disease in Sarawak

    del Sel Sylvia

    2009-01-01

    Full Text Available Abstract Background Human enterovirus 71 (HEV71 can cause Hand, foot, and mouth disease (HFMD with neurological complications, which may rapidly progress to fulminant cardiorespiratory failure, and death. Early recognition of children at risk is the key to reduce acute mortality and morbidity. Methods We examined data collected through a prospective clinical study of HFMD conducted between 2000 and 2006 that included 3 distinct outbreaks of HEV71 to identify risk factors associated with neurological involvement in children with HFMD. Results Total duration of fever ≥ 3 days, peak temperature ≥ 38.5°C and history of lethargy were identified as independent risk factors for neurological involvement (evident by CSF pleocytosis in the analysis of 725 children admitted during the first phase of the study. When they were validated in the second phase of the study, two or more (≥ 2 risk factors were present in 162 (65% of 250 children with CSF pleocytosis compared with 56 (30% of 186 children with no CSF pleocytosis (OR 4.27, 95% CI2.79–6.56, p rd or later day of febrile illness, the sensitivity, specificity, PPV and NPV of ≥ 2 risk factors predictive of CSF pleocytosis were 75%(57/76, 59%(27/46, 75%(57/76 and 59%(27/46, respectively. Conclusion Three readily elicited clinical risk factors were identified to help detect children at risk of neurological involvement. These risk factors may serve as a guide to clinicians to decide the need for hospitalization and further investigation, including cerebrospinal fluid examination, and close monitoring for disease progression in children with HFMD.

  9. Brain Dynamics: Methodological Issues and Applications in Psychiatric and Neurologic Diseases

    Pezard, Laurent

    The human brain is a complex dynamical system generating the EEG signal. Numerical methods developed to study complex physical dynamics have been used to characterize EEG since the mid-eighties. This endeavor raised several issues related to the specificity of EEG. Firstly, theoretical and methodological studies should address the major differences between the dynamics of the human brain and physical systems. Secondly, this approach of EEG signal should prove to be relevant for dealing with physiological or clinical problems. A set of studies performed in our group is presented here within the context of these two problematic aspects. After the discussion of methodological drawbacks, we review numerical simulations related to the high dimension and spatial extension of brain dynamics. Experimental studies in neurologic and psychiatric disease are then presented. We conclude that if it is now clear that brain dynamics changes in relation with clinical situations, methodological problems remain largely unsolved.

  10. IgG-index predicts neurological morbidity in patients with infectious central nervous system diseases

    Deisenhammer Florian

    2010-07-01

    Full Text Available Abstract Background Prognosis assessment of patients with infectious and neoplastic disorders of the central nervous system (CNS may still pose a challenge. In this retrospective cross-sectional study the prognostic value of basic cerebrospinal fluid (CSF parameters in patients with bacterial meningitis, viral meningoencephalitis and leptomeningeal metastases were evaluated. Methods White blood cell count, CSF/serum glucose ratio, protein, CSF/serum albumin quotient and Immunoglobulin indices for IgG, IgA and IgM were analyzed in 90 patients with bacterial meningitis, 117 patients with viral meningoencephalitis and 36 patients with leptomeningeal metastases in a total of 480 CSF samples. Results In the initial spinal tap, the IgG-index was the only independent predictor for unfavorable outcome (GOS Conclusion The present study suggests that in infectious CNS diseases an elevated IgG-Index might be an additional marker for the early identification of patients at risk for neurological morbidity.

  11. Neurological disease mutations compromise a C-terminal ion pathway in the Na(+)/K(+)-ATPas

    Poulsen, Hanne; Khandelia, Himanshu; Morth, Jens Preben

    2010-01-01

    The Na(+)/K(+)-ATPase pumps three sodium ions out of and two potassium ions into the cell for each ATP molecule that is split, thereby generating the chemical and electrical gradients across the plasma membrane that are essential in, for example, signalling, secondary transport and volume...... potassium is released the proton will also return to the cytoplasm, thus allowing an overall asymmetric stoichiometry of the transported ions. The C terminus controls the gate to the pathway. Its structure is crucial for pump function, as demonstrated by at least eight mutations in the region that cause...... severe neurological diseases. This novel model for ion transport by the Na(+)/K(+)-ATPase is established by electrophysiological studies of C-terminal mutations in familial hemiplegic migraine 2 (FHM2) and is further substantiated by molecular dynamics simulations. A similar ion regulation is likely...

  12. Neurological disease mutations compromise a C-terminal ion pathway in the Na(+)/K(+)-ATPase

    Poulsen, Hanne; Khandelia, Himanshu; Morth, J Preben

    2010-01-01

    The Na(+)/K(+)-ATPase pumps three sodium ions out of and two potassium ions into the cell for each ATP molecule that is split, thereby generating the chemical and electrical gradients across the plasma membrane that are essential in, for example, signalling, secondary transport and volume...... potassium is released the proton will also return to the cytoplasm, thus allowing an overall asymmetric stoichiometry of the transported ions. The C terminus controls the gate to the pathway. Its structure is crucial for pump function, as demonstrated by at least eight mutations in the region that cause...... severe neurological diseases. This novel model for ion transport by the Na(+)/K(+)-ATPase is established by electrophysiological studies of C-terminal mutations in familial hemiplegic migraine 2 (FHM2) and is further substantiated by molecular dynamics simulations. A similar ion regulation is likely...

  13. The global burden of mental, neurological and substance use disorders: an analysis from the Global Burden of Disease Study 2010.

    Harvey A Whiteford

    Full Text Available The Global Burden of Disease Study 2010 (GBD 2010, estimated that a substantial proportion of the world's disease burden came from mental, neurological and substance use disorders. In this paper, we used GBD 2010 data to investigate time, year, region and age specific trends in burden due to mental, neurological and substance use disorders.For each disorder, prevalence data were assembled from systematic literature reviews. DisMod-MR, a Bayesian meta-regression tool, was used to model prevalence by country, region, age, sex and year. Prevalence data were combined with disability weights derived from survey data to estimate years lived with disability (YLDs. Years lost to premature mortality (YLLs were estimated by multiplying deaths occurring as a result of a given disorder by the reference standard life expectancy at the age death occurred. Disability-adjusted life years (DALYs were computed as the sum of YLDs and YLLs.In 2010, mental, neurological and substance use disorders accounted for 10.4% of global DALYs, 2.3% of global YLLs and, 28.5% of global YLDs, making them the leading cause of YLDs. Mental disorders accounted for the largest proportion of DALYs (56.7%, followed by neurological disorders (28.6% and substance use disorders (14.7%. DALYs peaked in early adulthood for mental and substance use disorders but were more consistent across age for neurological disorders. Females accounted for more DALYs in all mental and neurological disorders, except for mental disorders occurring in childhood, schizophrenia, substance use disorders, Parkinson's disease and epilepsy where males accounted for more DALYs. Overall DALYs were highest in Eastern Europe/Central Asia and lowest in East Asia/the Pacific.Mental, neurological and substance use disorders contribute to a significant proportion of disease burden. Health systems can respond by implementing established, cost effective interventions, or by supporting the research necessary to develop

  14. Aging potentiates the acute and chronic neurological symptoms of pyrithiamine-induced thiamine deficiency in the rodent.

    Pitkin, S R; Savage, L M

    2001-03-15

    The present study aimed to assess the role of advanced age in the development and manifestation of thiamine deficiency using an animal model of Wernicke-Korsakoff syndrome (WKS). Interactions between pyrithiamine-induced thiamine deficiency (PTD) and age were examined relative to working memory impairment and neuropathology in Fischer 344 rats. Young (2-3 months) and aged (22-23 months) F344 rats were assigned to one of two treatment conditions: PTD or pair-fed control (PF). Rats in the former group were further divided into three groups according to duration of PTD treatment. Working memory was assessed with an operant matching-to-position (MTP) task; after testing, animals were sacrificed and both gross and immunocytochemical measures of brain pathology were obtained. Aged rats exhibited acute neurological disturbances during the PTD treatment regime earlier than did young rats, and also developed more extensive neuropathology with a shorter duration of PTD. Aged rats displayed increased brain shrinkage (smaller frontal cortical and callosal thickness) as well as enhanced astrocytic activity in the thalamus and a decrease in ChAT-positive cell numbers in the medial septum; the latter two measures of neuropathology were potentiated by PTD. In both young and aged rats, and to a greater degree in the latter group, PTD reduced thalamic volume. Behaviorally, aged rats displayed impaired choice accuracy on the delayed MTP task. Regardless of age, rats with lesions centered on the internal medullary lamina of the thalamus also displayed impaired choice accuracy. Moreover, increased PTD treatment duration led to increased response times on the delayed MTP task. These results suggest that aging does indeed potentiate the neuropathology associated with experimental thiamine deficiency, supporting an age coupling hypothesis of alcohol-related neurological disorders.

  15. Artesunate-mefloquine combination therapy in acute Plasmodium falciparum malaria in young children: a field study regarding neurological and neuropsychiatric safety

    Hatz Christoph

    2010-10-01

    Full Text Available Abstract Background Mefloquine-artesunate combination therapy for uncomplicated falciparum malaria is one of the treatments used in African children. Data concerning neurological safety in adults and children treated with mefloquine and artesunate combination therapy is well documented in Asia. Safety data for neurological and neuropsychiatric side effects of mefloquine and artesunate combination therapy in African children are scarce, although WHO recommends this therapy in Africa. Methods A phase IV, open label, single arm study was conducted among African children between 10 and 20 kg with acute uncomplicated falciparum malaria. They were treated over three consecutive days with a paediatric fixed-dose combination of artesunate (50 mg/d and mefloquine (125 mg/d. Parasitological, clinical and neurological examinations and standardized questions about neuropsychiatric symptoms were carried out on days 0, 4, 7, 28 and 63. The primary objective was to assess the neurological and neuropsychiatric safety of artesunate-mefloquine combination therapy in young children. Results From December 2007 to March 2009, 220 children with uncomplicated Plasmodium falciparum malaria were treated with artesunate and mefloquine. 213 children were analysed according to study protocol. 50 neurological and neuropsychiatric adverse events occurred in 28 patients. Eleven drug-related neurological and neuropsychiatric adverse events occurred in eight patients. Sleeping disorders were present in 2.3%, neurological disorders in 1.4%, neuropsychiatric disorders in 1% and eating disorders in 0.5% of the patients. Adverse events were of mild to moderate intensity and resolved spontaneously. Conclusion African children showed a low percentage of self-limited neurological and neuropsychiatric adverse events, confirming studies on neurological safety in Asian children treated with artesunate and mefloquine. Sleeping disorders were most frequently observed.

  16. Considerations on Intervention Goal and Efficacy Evaluation of Traditional Chinese Medicine in the Treatment of Neurological Diseases

    XIE Ren-ming; DU Bao-xin; HUANG Yan; ZHOU Dao-you; WANG yong-yan; HUANG pei-xin

    2007-01-01

    In the last several years, traditional Chinese medicine (TCM) has made much progress in the treatment of neurological diseases. The living space of TCM in neurological diseases lies in refractory diseases, aging and chronic diseases caused by multiple factors as well as sub-health state and chronic fatigue state. The effect model of TCM mainly consists of whole effect, self-organization,self-stable model, holographic effect and butterfly effect. The effective point of TCM in neurological diseases lies mainly in end-points and health-related events. Moreover, TCM has advantages in the evaluation of symptoms, syndrome and quality of life (QOL). Some key indexes should be included when evaluating the efficacy of TCM in neurological diseases. Meanwhile, the advantages of TCM such as end-points, health-related events and QOL should be highlighted. Multi-subject researching methods could be adopted to make a comprehensive evaluation of subjective and objective indexes.The clinical evidence on the TCM efficacy evaluation may come from RCTs, and other types of designs can also be considered.

  17. Functional Performance and Associations between Performance Tests and Neurological Assessment Differ in Men and Women with Parkinson’s Disease

    Kadri Medijainen

    2015-01-01

    Full Text Available Background. Neurological assessment of a patient with Parkinson’s disease (PD is expected to reflect upon functional performance. As women are known to report more limitations even for same observed functional performance level, present study was designed to examine whether associations between neurological assessments and functional performance differ across genders. Methods. 14 men and 14 women with PD participated. Functional performance was assessed by measuring walking speeds on 10-meter walk test (10MWT and by performing timed-up-and-go-test (TUG. Neurological assessment included Hoehn and Yahr Scale (HY, Movement Disorders Society Unified Parkinson’s Disease Rating Scale (MDS-UPDRS, Schwab and England Activities of Daily Living Scale (S-E, and Mini Mental State Examination (MMSE. Results. In women with PD, Kendall’s tau-b correlation analyses revealed significant correlations between functional performance tests and neurological assessment measures, with the exception in MMSE. No corresponding associations were found for men, although they demonstrated better functional performance, as expected. Conclusion. Men in similar clinical stage of the PD perform better on functional tests than women. Disease severity reflects upon functional performance differently in men and women with PD. Results indicate that when interpreting the assessment results of both functional performance and neurological assessment tests, the gender of the patient should be taken into consideration.

  18. Noninvasive radioelectric asymmetric conveyor brain stimulation treatment improves balance in individuals over 65 suffering from neurological diseases: pilot study

    Margotti ML

    2012-02-01

    Full Text Available Vania Fontani1, Salvatore Rinaldi1, Alessandro Castagna1, Matteo Lotti Margotti21Department of Neuro Psycho Physio Pathology, Rinaldi Fontani Institute, Florence, Italy; 2Department of Information Technology and Statistical Analysis, Rinaldi Fontani Institute, Florence, ItalyPurpose: In the elderly population, problems with walking and balance are very common. These problems seriously affect the quality of life of the elderly. When gait and balance problems are caused by neurological disease, these problems can be more serious and difficult to handle. The aim of this pilot study was to verify the effect of a noninvasive radioelectric conveyor asymmetric brain stimulation protocol, named neuropostural optimization (NPO, to improve balance in neurological elderly.Patients and methods: Twelve patients suffering from various neurological diseases participated in this study. They were assessed with the Romberg test, which was performed on a computerized stabilometric platform before, immediately following, and 72 hours after NPO was used to improve balance.Results: The results showed that a stabilization of balance was recorded in all subjects a few minutes after administration of NPO. This stabilization increased 72 hours after treatment.Conclusion: The results show that NPO could be a valuable therapeutic approach to improve sensory-motor strategies and neurological control of balance in elderly patients suffering from various neurological diseases.Keywords: Romberg test, instability, imbalance, gait, REAC, neuropostural optimization

  19. Acute glyphosate-surfactant poisoning with neurological sequels and fatal outcome

    Potrebić Olivera

    2009-01-01

    Full Text Available Introduction. Clinical picture of severe glyphosatesurfactant poisoning is manifested by gastroenteritis, respiratory disturbances, altered mental status, hypotension refractory to the treatment, renal failure, shock. Single case report indicated possible neurotoxic sequels of glyphosatesurfactant exposure with white matter lesions and development of Parkinsonism. We described a patient with massive white matter damage which led to vigil coma and lethal outcome. Case report. A 56-year old woman ingested about 500 mL of herbicide containing glyphosate isopropylamine salt. The most prominent manifestation of poisoning included hypotension, coma, hyperkaliemia, respiratory and renal failure. The patient was treated in intensive care unit by symptomatic and supportive therapy including mechanical ventilation and hemodialysis. The patient survived the acute phase of poisoning, but she developed vigil coma. Nuclear magnetic imagining revealed extensive bilateral lesions of the brain stem white matter and pons. Conclusion. The outcome of reported poisoning may be the consequence of glyphosate-surfactant neurotoxic effect or/and ischemia, especially in the episodes of marked hypotension during hemodialysis. Considering recommendation of early hemodialysis as the treatment of choice, even before renal failure development, we point out the importance of careful planning of dialysis modality in hemodynamically instable patient and recommend continuous dialysis methods.

  20. [Peroxisomal neurologic diseases and Refsum disease: very long chain fatty acids and phytanic acid as diagnostic markers].

    Molzer, B; Stöckler, S; Bernheimer, H

    1992-01-01

    Peroxisomal disorders are genetic metabolic diseases with generalized, multiple, or single functional disturbances of the peroxisome. According to the extent of the functional disturbances 3 groups of diseases can be differentiated: disorders with generalized loss of peroxisomal functions (Zellweger syndrome, ZS; neonatal adrenoleukodystrophy, NALD; infantile Refsum's disease), disorders with multiple enzymatic defects (e.g. rhizomelic chondrodysplasia punctata), and disorders with a single enzymatic defect in the peroxisome, the most important being adrenoleukodystrophy/adrenomyeloneuropathy (ALD/AMN). Adult Refsum's disease, a genetic neurological disorder with phytanic acid accumulation, is due to a mitochondrial enzyme deficiency, but is often considered together with peroxisomal diseases because of phytanic acid (PHYT) accumulation in most peroxisomal diseases. The main clinical and pathological criteria of the major disorders and the biochemical parameters of their differentiation are presented. Elevated levels of very long chain fatty acids (VLCFA) and/or PHYT are the primary diagnostic markers for all peroxisomal disorders and adult Refsum's disease, respectively. Our investigations disclosed 30 ALD/AMN hemizygotes, 16 ALD/AMN heterozygotes, 8 cases of ZS/NALD and 7 patients with adult Refsum's disease. In addition, 15 cases of peroxisomal disorders were confirmed by biochemical investigations in autopsy material. With regard to peroxisomal disorders, therapeutic concepts exist only for ALD/AMN: corticosteroid substitution for adrenal insufficiency, dietary treatment, and bone marrow transplantation (BMT). Adult Refsum's disease can be treated successfully by dietary therapy. In case of dietary treatment and BMT, assay of VLCFA and/or PHYT is important for the biochemical evaluation of these therapies.

  1. Neurologic Complications of Pre-eclampsia

    Zeeman, Gerda G.

    2009-01-01

    Pre-eclampsia is mainly responsible for the world's large maternal mortality rates, mostly due to acute cerebral complications. This review provides insight into the pathogenesis of the neurologic complications of hypertensive disease in pregnancy. In addition, practical relevance for clinical care

  2. Progress in Pediatrics in 2012: choices in allergy, endocrinology, gastroenterology, hematology, infectious diseases, neurology, nutrition and respiratory tract illnesses.

    Caffarelli, Carlo; Santamaria, Francesca; Vottero, Alessandra; Bernasconi, Sergio

    2013-05-08

    In this review, we summarize the progresses in allergy, endocrinology, gastroenterology, hematology, infectious diseases, neurology, nutrition and respiratory tract illnesses that have been published in The Italian Journal of Pediatrics in 2012. The induction of Treg activity by probiotics might be effective for promoting tolerance towards food allergens. Nasal cytology is useful in patients with rhinitis for diagnosing chronic non-allergic non-infectious diseases. Atopic eczema is associated both with an aberrant skin matrix and impaired systemic immune response. Therefore, isolated topical treatment may have suboptimal effect. Diagnostic work-up of exercise-induced anaphylaxis, including exercise challenge test, is necessary to reach a diagnosis. Studies may support a role for nutrition on prevention of asthma and cardiovascular diseases. Clinicians need to early identify adolescent menstrual abnormalities to minimize sequelae, and to promote health information. In Multiple Endocrine Neoplasia type 2B investigations include acetylcholinesterase study of rectal mucosa followed by the molecular analysis of RET mutation. Low adherence to gluten-free diet and osteopenia are common problems in children with diabetes mellitus type 1 and celiac disease. In infantile colic, laboratory tests are usually unnecessary and the treatment is based on reassurance. Prevalence of obesity and stunting is elucidated by several studies. Evidences are growing that dietetic measures are needed to prevent obesity in children with acute leukemia. Treatment studies for infectious diseases show promise for probiotics along with standard triple therapy in children with Helicobacter pilori infection, while zinc has no effect on pneumonia. Educational programs about the proper management of the febrile child are warranted. A new hour-specific total serum bilirubin nomogram has been shown to be able to predict newborns without hyperbilirubinemia after 48 to 72 hours of life. Newborns with

  3. Bartonella vinsonii subsp. berkhoffii and Bartonella henselae bacteremia in a father and daughter with neurological disease

    Woods Christopher W

    2010-04-01

    Full Text Available Abstract Background Bartonella vinsonii subsp. berkhoffii is an important, emerging, intravascular bacterial pathogen that has been recently isolated from immunocompetent patients with endocarditis, arthritis, neurological disease and vasoproliferative neoplasia. Vector transmission is suspected among dogs and wild canines, which are the primary reservoir hosts. This investigation was initiated to determine if pets and family members were infected with one or more Bartonella species. Methods PCR and enrichment blood culture in Bartonella alpha Proteobacteria growth medium (BAPGM was used to determine infection status. Antibody titers to B. vinsonii subsp. berkhoffii genotypes I-III and B. henselae were determined using a previously described indirect fluorescent antibody test. Two patients were tested sequentially for over a year to assess the response to antibiotic treatment. Results Intravascular infection with B. vinsonii subsp. berkhoffii genotype II and Bartonella henselae (Houston 1 strain were confirmed in a veterinarian and his daughter by enrichment blood culture, followed by PCR and DNA sequencing. Symptoms included progressive weight loss, muscle weakness, lack of coordination (the father and headaches, muscle pain and insomnia (the daughter. B. vinsonii subsp. berkhoffii genotype II was also sequenced from a cerebrospinal fluid BAPGM enrichment culture and from a periodontal swab sample. After repeated courses of antibiotics, post-treatment blood cultures were negative, there was a decremental decrease in antibody titers to non-detectable levels and symptoms resolved in both patients. Conclusions B. vinsonii subsp. berkhoffii and B. henselae are zoonotic pathogens that can be isolated from the blood of immunocompetent family members with arthralgias, fatigue and neurological symptoms. Therapeutic elimination of Bartonella spp. infections can be challenging, and follow-up testing is recommended. An increasing number of arthropod

  4. Neurological, psychological, and cognitive disorders in patients with chronic kidney disease on conservative and replacement therapy

    Lai, Silvia; Mecarelli, Oriano; Pulitano, Patrizia; Romanello, Roberto; Davi, Leonardo; Zarabla, Alessia; Mariotti, Amalia; Carta, Maria; Tasso, Giorgia; Poli, Luca; Mitterhofer, Anna Paola; Testorio, Massimo; Frassetti, Nicla; Aceto, Paola; Galani, Alessandro; Lai, Carlo

    2016-01-01

    Abstract Chronic kidney disease (CKD) is a highly prevalent condition in the world. Neurological, psychological, and cognitive disorders, related to CKD, could contribute to the morbidity, mortality, and poor quality of life of these patients. The aim of this study was to assess the neurological, psychological, and cognitive imbalance in patients with CKD on conservative and replacement therapy. Seventy-four clinically stable patients affected by CKD on conservative therapy, replacement therapy (hemodialysis (HD), peritoneal dialysis (PD)), or with kidney transplantation (KT) and 25 healthy controls (HC), matched for age and sex were enrolled. Clinical, laboratory, and instrumental examinations, as renal function, inflammation and mineral metabolism indexes, electroencephalogram (EEG), psychological (MMPI-2, Sat P), and cognitive tests (neuropsychological tests, NPZ5) were carried out. The results showed a significant differences in the absolute and relative power of delta band and relative power of theta band of EEG (P = 0.008, P 2D3) (P 2D3, intact parathyroid hormone (iPTH), phosphorus, and cynical and hysterical personality, are correlated with higher relative power of delta (P = 0.016) and theta band (P = 0.016). Moreover, all NPZ5 scores showed a significant difference between the means of nephropathic patients and the means of the HC, and a positive correlation with eGFR, serum nitrogen, CRP, iPTH, and vitamin D. In CKD patients, simple and noninvasive instruments, as EEG, and cognitive-psychological tests, should be performed and careful and constant monitoring of renal risk factors, probably involved in neuropsychological complications (inflammation, disorders of mineral metabolism, electrolyte disorders, etc.), should be carried out. Early identification and adequate therapy of neuropsychological, and cognitive disorders, might enable a better quality of life and a major compliance with a probable reduction in the healthcare costs. PMID

  5. Brain MRI and SPECT in the diagnosis of early neurological involvement in Wilson's disease

    Piga, Mario; Satta, Loredana; Serra, Alessandra; Loi, Gianluigi [Policlinico Universitario, University of Cagliari, Nuclear Medicine, Department of Medical Science, Monserrato, Cagliari (Italy); Murru, Alessandra; Demelia, Luigi [Policlinico Universitario, University of Cagliari, Gastroenterology, Department of Medical Science, Monserrato, Cagliari (Italy); Sias, Alessandro [Policlinico Universitario, University of Cagliari, Radiology, Department of Medical Science, Monserrato, Cagliari (Italy); Marrosu, Francesco [Policlinico Universitario, University of Cagliari, Neurology, Department of Medical Science, Monserrato, Cagliari (Italy)

    2008-04-15

    To evaluate the impact of brain MRI and single-photon emission computed tomography (SPECT) in early detection of central nervous system abnormalities in patients affected by Wilson's disease (WD) with or without neurological involvement. Out of 25 consecutive WD patients, 13 showed hepatic involvement, ten hepatic and neurological manifestations, and twp hepatic, neurological, and psychiatric symptoms, including mainly movement disorders, major depression, and psychosis. Twenty-four healthy, age-gender matched subjects served as controls. All patients underwent brain MRI and {sup 99m}Tc-ethyl-cysteinate dimer (ECD) SPECT before starting specific therapy. Voxel-by-voxel analyses were performed using statistical parametric mapping to compare differences in {sup 99m}Tc-ECD brain uptake between the two groups. Brain MRI showed T2-weighted hyperintensities in seven patients (28%), six of whom were affected by hepatic and neurological forms. Brain perfusion SPECT showed pathological data in 19 patients (76%), revealing diffuse or focal hypoperfusion in superior frontal (Brodmann area (BA) 6), prefrontal (BA 9), parietal (BA 40), and occipital (BA 18, BA 39) cortices in temporal gyri (BA 37, BA 21) and in caudatus and putamen. Moreover, hepatic involvement was detected in nine subjects; eight presented both hepatic and neurological signs, while two exhibited WD-correlated hepatic, neurological, and psychiatric alterations. All but one patient with abnormal MRI matched with abnormal ECD SPECT. Pathologic MRI findings were obtained in six out of ten patients with hepatic and neurological involvement while abnormal ECD SPECT was revealed in eight patients. Both patients with hepatic, neurological, and psychiatric involvement displayed abnormal ECD SPECT and one displayed an altered MRI. These findings suggest that ECD SPECT might be useful in detecting early brain damage in WD, not only in the perspective of assessing and treating motor impairment but also in evaluating

  6. Acute rheumatic fever and rheumatic heart disease.

    Carapetis, Jonathan R; Beaton, Andrea; Cunningham, Madeleine W; Guilherme, Luiza; Karthikeyan, Ganesan; Mayosi, Bongani M; Sable, Craig; Steer, Andrew; Wilson, Nigel; Wyber, Rosemary; Zühlke, Liesl

    2016-01-14

    Acute rheumatic fever (ARF) is the result of an autoimmune response to pharyngitis caused by infection with group A Streptococcus. The long-term damage to cardiac valves caused by ARF, which can result from a single severe episode or from multiple recurrent episodes of the illness, is known as rheumatic heart disease (RHD) and is a notable cause of morbidity and mortality in resource-poor settings around the world. Although our understanding of disease pathogenesis has advanced in recent years, this has not led to dramatic improvements in diagnostic approaches, which are still reliant on clinical features using the Jones Criteria, or treatment practices. Indeed, penicillin has been the mainstay of treatment for decades and there is no other treatment that has been proven to alter the likelihood or the severity of RHD after an episode of ARF. Recent advances - including the use of echocardiographic diagnosis in those with ARF and in screening for early detection of RHD, progress in developing group A streptococcal vaccines and an increased focus on the lived experience of those with RHD and the need to improve quality of life - give cause for optimism that progress will be made in coming years against this neglected disease that affects populations around the world, but is a particular issue for those living in poverty.

  7. Noncoding RNAs and RNA editing in brain development, functional diversification, and neurological disease.

    Mehler, Mark F; Mattick, John S

    2007-07-01

    The progressive maturation and functional plasticity of the nervous system in health and disease involve a dynamic interplay between the transcriptome and the environment. There is a growing awareness that the previously unexplored molecular and functional interface mediating these complex gene-environmental interactions, particularly in brain, may encompass a sophisticated RNA regulatory network involving the twin processes of RNA editing and multifaceted actions of numerous subclasses of non-protein-coding RNAs. The mature nervous system encompasses a wide range of cell types and interconnections. Long-term changes in the strength of synaptic connections are thought to underlie memory retrieval, formation, stabilization, and effector functions. The evolving nervous system involves numerous developmental transitions, such as neurulation, neural tube patterning, neural stem cell expansion and maintenance, lineage elaboration, differentiation, axonal path finding, and synaptogenesis. Although the molecular bases for these processes are largely unknown, RNA-based epigenetic mechanisms appear to be essential for orchestrating these precise and versatile biological phenomena and in defining the etiology of a spectrum of neurological diseases. The concerted modulation of RNA editing and the selective expression of non-protein-coding RNAs during seminal as well as continuous state transitions may comprise the plastic molecular code needed to couple the intrinsic malleability of neural network connections to evolving environmental influences to establish diverse forms of short- and long-term memory, context-specific behavioral responses, and sophisticated cognitive capacities.

  8. Serum Brain-Derived Neurotrophic Factor Levels in Different Neurological Diseases

    Mariacarla Ventriglia

    2013-01-01

    Full Text Available Consistent evidence indicates the involvement of the brain-derived neurotrophic factor (BDNF in neurodegenerative disorders such as Alzheimer's disease (AD and Parkinson’s disease (PD. In the present study, we compared serum BDNF in 624 subjects: 266 patients affected by AD, 28 by frontotemporal dementia (FTD, 40 by Lewy body dementia (LBD, 91 by vascular dementia (VAD, 30 by PD, and 169 controls. Our results evidenced lower BDNF serum levels in AD, FTD, LBD, and VAD patients (P<0.001 and a higher BDNF concentration in patients affected by PD (P=0.045. Analyses of effects of pharmacological treatments suggested significantly higher BDNF serum levels in patients taking mood stabilizers/antiepileptics (P=0.009 and L-DOPA (P<0.001 and significant reductions in patients taking benzodiazepines (P=0.020. In conclusion, our results support the role of BDNF alterations in neurodegenerative mechanisms common to different forms of neurological disorders and underline the importance of including drug treatment in the analyses to avoid confounding effects.

  9. α-Synuclein in the colon and premotor markers of Parkinson disease in neurologically normal subjects.

    Kim, Joong-Seok; Park, In-Seok; Park, Hyung-Eun; Kim, Su-Young; Yun, Jung A; Jung, Chan Kwon; Sung, Hye-Young; Lee, Jin-Kwon; Kang, Won-Kyung

    2017-01-01

    Extranigral non-motor signs precede the first motor manifestations of Parkinson's disease by many years in some patients. The presence of α-synuclein deposition within colon tissues in patients with Parkinson's disease can aid in identifying early neuropathological changes prior to disease onset. In the present study, we evaluated the roles of non-motor symptoms and signs and imaging biomarkers of nigral neuronal changes and α-synuclein accumulation in the colon. Twelve subjects undergoing colectomy for primary colon cancer were recruited for this study. Immunohistochemical staining for α-synuclein in normal and phosphorylated forms was performed in normally appearing colonic tissue. We evaluated 16 candidate premotor risk factors in this study cohort. Among them, ten subjects showed positive immunostaining with normal- and phosphorylated-α-synuclein. An accumulation of premotor markers in each subject was accompanied with positive normal- and phosphorylated-α-synuclein immunostaining, ranging from 2 to 7 markers per subject, whereas the absence of Lewy bodies in the colon was associated with relative low numbers of premotor signs. A principal component analysis and a cluster analysis of these premotor markers suggest that urinary symptoms were commonly clustered with deposition of peripheral phosphorylated-α-synuclein. Among other premotor marker, color vision abnormalities were related to non-smoking. This mathematical approach confirmed the clustering of premotor markers in preclinical stage of Parkinson's disease. This is the first report showing that α-synuclein in the colon and other premotor markers are related to each other in neurologically normal subjects.

  10. Neurologic involvement in Behcet disease. Case report; Morbus Behcet mit neurologischer Beteiligung. Ein Fallbericht

    Boehner, C. [Erlangen-Nuernberg Univ., Erlangen (Germany). Inst. fuer Diagnostische Radiologie; Fellner, F. [Erlangen-Nuernberg Univ., Erlangen (Germany). Inst. fuer Diagnostische Radiologie; Reinhardt, F. [Erlangen-Nuernberg Univ., Erlangen (Germany). Neurologische Klinik mit Poliklinik; Eberhardt, K.E.W. [Erlangen-Nuernberg Univ., Erlangen (Germany). Abt. fuer Neuroradiologie

    1997-05-01

    Morbus Behcet is a very infrequent multi-system disease caused by an immunological vasculitis affecting the arteries and veins. The disease is primarily known in Israel, Turkey, Italy, Great Britain and Japan. It may become manifest as erythema nodosum, polyarthritis, thrombophlebitis, occlusive arterial disease, pulmonary infarction, ulcerous colitis, portal hypertension, or via neurological symptoms (collateral symptoms), and it is not possible to make a clear prognosis. The most frequent signs of the neuro-Behcet syndrome, as in the case reported, is a meningoencephalitis. MRI is an essential modality for diagnosis and follow-up of the disease. In this case, the findings included meningeal irritations as well as an abduction deficit indicating involvement of the brain stem of type 1. As described in the literature, pathologic findings have been obtained by spinal fluid tests, showing increased cell number and proteins and pleocytosis of the granulocytes. (Orig./vhe) [Deutsch] Der Morbus Behcet ist eine seltene Multisystemerkrankung in Form einer immunvermittelten Vaskulitis, die Arterien und Venen betrifft. Die Erkrankung findet man ueberwiegend in Israel, der Tuerkei, Italien, Grossbritannien und Japan. Die Erkrankung ist multisystemisch, kann sich als Erythema nodosum, Polyarthritis, Thrombophlebitis, arterielle Verschlusskrankheit, Lungeninfarkt, ulzeroese Colitis, portale Hypertension oder mit neurologischen Symptomen (Nebensymptome) manifestieren, und basiert auf einer immunvermittelten Vaskulitis mit unklarer Prognose. Am haeufigsten manifestiert sich das Neuro-Behcet-Syndrom, wie beim untersuchten Patienten, in Form einer Meningoenzephalitis. Hierbei fand sich neben meningealen Reizsymptomen ein Abduktionsdefizit als Zeichen der Hirnstammbeteiligung im Sinne eines Typ 1. Wie in der Literatur beschrieben, zeigte sich ein pathologischer Liquorbefund mit Zellzahl- und Proteinerhoehung sowie granulozytaerer Pleozytose. Beim Neuro-Behcet, wie bei den anderen

  11. Consensus Statement on medication use in multiple sclerosis by the Spanish Society of Neurology's study group for demyelinating diseases.

    García-Merino, A; Fernández, O; Montalbán, X; de Andrés, C; Oreja-Guevara, C; Rodríguez-Antigüedad, A; Arbizu, T

    2013-01-01

    Treatments for multiple sclerosis therapy are rapidly evolving. It is believed that new drugs will be approved in the near future, thereby changing current indications for treatment. In this context, the Spanish Society of Neurology's study group on demyelinating diseases, which evaluates medication use in MS, has decided to draw up a consensus statement on the current indications and guidelines for multiple sclerosis treatment.

  12. Epilepsy and Other Neurological Diseases in the Parents of Children with Infantile Autism. A Case Control Study

    Mouridsen, Svend Erik; Rich, Bente; Isager, Torben

    2008-01-01

    In order to study the broader phenotype of infantile autism (IA) we compared the rates and types of epilepsy and other neurological diseases in the parents of 111 consecutively admitted patients with IA with a matched control group of parents of 330 children from the general population. All participants were screened through the nationwide Danish…

  13. [Neurologic and psychiatric syndromes of rare para-rheumatic diseases (author's transl)].

    Gottwald, W

    1977-03-01

    In Wegener's granulomatosis peripheral nerves are more often involved than central nervous structures; functional psychoses are secondary e.g. to renal complications. Neurologic-psychiatric symptoms are varied and result from local spread from the upper respiratory tract with separate granulomata and vasculitis. Striped muscle is involved in about 10% of cases. Specific vegetative disturbances of function do not appear to occur. This is also true for Granuloma gangrenescens. Its multicentric inflammatory, ulcerating and necrotic foci involve midline structures and often start at the anterior neuroporus. Becet's disease, however, shows clear characteristic localisation, symmetry of various dermatologic phenomena in the extremities and the almost obligatory involvement of mouth, stomach and the genitals and their surroundings. This one may assume to be due to a mesencephalic parasympathicotonic reaction as the basis for the occurrence of perioral and acro-syndroms after Fischer-Brügge and Sunder-plassmann. Some similarity with the localization of other collagenous diseases is evident. The anterior basal ganglia represent a favored focus where e.g. initial, imcomplete and readily recurrent processes, possibly of an exsudative nature, may start off neurovascular changes of limited areas without necessarily producing clinically detectable signs of the usual neurologic-psychiatric type. In Behcet's disease, rather as in scleroderma, the nervous system appears to be decisively involved. About one third of the cases show changes in the nervous system ("Neuro-Behcet" in the usual sense) with little peripheral signs and rare myositis. But Behcet's disease may also-like erythematodes or progressive sclerodermia-simulate a disseminated encephalomyelitis. The pathogenetic factors of Reiter's syndrome appear to be closely related. The relatively constant main symptoms are also localized and permit the conclusion that they depend on vasomotorically linked innervation of the

  14. [Current status of the predictive genetic testing for hereditary neurological diseases in Shinshu University Hospital].

    Tanaka, Keiko; Sekijima, Yoshiki; Yoshida, Kunihiro; Mizuuchi, Asako; Yamashita, Hiromi; Tamai, Mariko; Ikeda, Shu-ichi; Fukushima, Yoshimitsu

    2013-01-01

    The current status of predictive genetic testing for late-onset hereditary neurological diseases in Japan is largely unknown. In this study, we analyzed data from 73 clients who visited the Division of Clinical and Molecular Genetics, Shinshu University Hospital, for the purpose of predictive genetic testing. The clients consisted of individuals with family histories of familial amyloid polyneuropathy (FAP; n=30), Huntington's disease (HD; n=16), spinocerebellar degeneration (SCD; n=14), myotonic dystrophy type 1 (DM1; n=9), familial amyotrophic lateral sclerosis type 1 (ALS1; n=3), and Alzheimer's disease (AD; n=1). Forty-nine of the 73 (67.1%) clients were in their twenties or thirties. Twenty-seven of the 73 (37.0%) clients visited a medical institution within 3 months after becoming aware of predictive genetic testing. The most common reason for requesting predictive genetic testing was a need for certainty or to reduce uncertainty and anxiety. The decision-making about marriage and having a child was also a main reason in clients in the twenties and thirties. The numbers of clients who actually underwent predictive genetic testing was 22 of 30 (73.3%) in FAP, 3 of 16 (18.8%) in HD, 6 of 10 (60.0%) in SCD, 7 of 9 (77.8%) in DM1, and 0 of 3 (0%) in ALS1 (responsible gene of the disease was unknown in 4 SCD patients and an AD patient). The percentage of test usage was lower in untreatable diseases such as HD and SCD than that in FAP, suggesting that many clients changed their way of thinking on the significance of testing through multiple genetic counseling sessions. In addition, it was obvious that existence of disease-modifying therapy promoted usage of predictive genetic testing in FAP. Improvement of genetic counseling system to manage predictive genetic testing is necessary, as consultation concerning predictive genetic testing is the main motivation to visit genetic counseling clinic in many at-risk clients.

  15. THE RHEOLOGICAL PROPERTIES OF BLOOD IN THE MOST ACUTE STAGE OF ISCHEMIC STROKE AND THEIR RELATION TO THE SEVERITY OF NEUROLOGICAL IMPAIRMENT

    M. N. Azhermacheva

    2013-01-01

    Full Text Available The study evaluated the rheological parameters of blood: blood viscosity, plasma viscosity, hematocrit, red blood cell aggregation and deformability. The severity of the patients was assessed by clinical scales:Glasgowcoma scale, the scale NIHSS, Barthel index. The study found that in the acute phase of ischemic stroke increased blood viscosity by increasing red blood cell aggregation and reduced erythrocyte deformability. The increase in the viscosity of the blood in acute ischemic stroke is accompanied by increased severity of neurological disorders.

  16. Acid-sensing ion channels (ASICs: therapeutic targets for neurological diseases and their regulation

    Hae-Jin Kweon

    2013-06-01

    Full Text Available Extracellular acidification occurs not only in pathologicalconditions such as inflammation and brain ischemia, but alsoin normal physiological conditions such as synaptic transmission.Acid-sensing ion channels (ASICs can detect a broadrange of physiological pH changes during pathological andsynaptic cellular activities. ASICs are voltage-independent,proton-gated cation channels widely expressed throughout thecentral and peripheral nervous system. Activation of ASICs isinvolved in pain perception, synaptic plasticity, learning andmemory, fear, ischemic neuronal injury, seizure termination,neuronal degeneration, and mechanosensation. Therefore,ASICs emerge as potential therapeutic targets for manipulatingpain and neurological diseases. The activity of these channelscan be regulated by many factors such as lactate, Zn2+, andPhe-Met-Arg-Phe amide (FMRFamide-like neuropeptides byinteracting with the channel’s large extracellular loop. ASICsare also modulated by G protein-coupled receptors such asCB1 cannabinoid receptors and 5-HT2. This review focuses onthe physiological roles of ASICs and the molecularmechanisms by which these channels are regulated. [BMBReports 2013; 46(6: 295-304

  17. The Effect of tDCS on Cognition and Neurologic Recovery of Rats with Alzheimer's Disease.

    Yu, Seong Hun; Park, Seong Doo; Sim, Ki Chel

    2014-02-01

    [Purpose] This study examined the effect of the application of transcranial direct current stimulation (tDCS) on neurologic recovery and cognitive function of rats with Alzheimer-like dementia induced by scopolamine injections. [Subjects] To create a cognition dysfunction model, intraperitoneal injection of scopolamine was given to Sprague-Dawley rats that subsequently received tDCS for 4 weeks. [Methods] Changes in motor behavior were evaluated by conducting an open field test. Acetylcholine content in the cerebral cortex and hippocampus was examined for a biochemical assessment. [Results] With respect to changes in motor behavior, group II showed the most meaningful difference after scopolamine injection, followed by group III. In the biochemical assessment, the results of the examination of acetylcholine content in the tissue of the cerebral cortex and the hippocampus on the 14th and 28th days, respectively, showed the most significant increase in group II, followed by group III. [Conclusion] The above findings confirm that tDCS application after the onset of cognitive dysfunction caused by Alzheimer's disease leads to a positive effect on motor behavior and biochemical changes, and this effect is maintained over a specific period of time.

  18. Circulating cell-free microRNA as biomarkers for screening, diagnosis and monitoring of neurodegenerative diseases and other neurologic pathologies

    Kira S Sheinerman

    2013-09-01

    Full Text Available Many neurodegenerative diseases, such as Alzheimer’s disease, Parkinson disease, vascular and frontotemporal dementias, as well as other chronic neurological pathologies, are characterized by slow development with a long asymptomatic period followed by a stage with mild clinical symptoms. As a consequence, these serious pathologies are diagnosed late in the course of a disease, when massive death of neurons has already occurred and effective therapeutic intervention is problematic. Thus, the development of screening tests capable of detecting neurodegenerative diseases during early, preferably asymptomatic, stages is a high unmet need. Since such tests are to be used for screening of large populations, they should be non-invasive and relatively inexpensive. Further, while subjects identified by screening tests can be further tested with more invasive and expensive methods, e.g. analysis of cerebrospinal fluid or imaging techniques, to be of practical utility screening tests should have high sensitivity and specificity. In this review, we discuss advantages and disadvantages of various approaches to developing screening tests based on analysis of circulating cell-free miRNA. Applications of circulating miRNA-based tests for diagnosis of acute and chronic brain pathologies, for research of normal brain aging, and for disease and treatment monitoring are also discussed.

  19. CORRELATION OF NEUROLOGIC DYSFUNCTION WITH CT-SCAN BRAIN FINDINGS AND CAROTID DOPPLER STUDY IN ACUTE ISCHAEMIC STROKE

    Bharat L

    2014-12-01

    Full Text Available BACKGROUND AND OBJECTIVES: stroke is one of the most common debilitating diseases with a huge burden related to both mortality and morbidity, ischemic stroke is far common compared to haemorrhagic stroke and also associated with significant carotid stenosis. Hence this present study is carried out to evaluate all the aspects of this disease. OBJECTIVES: 1. To correlate the nature of lesion, site of lesion, and severity of lesion on clinical grounds with CT-Scan findings. 2. To find the prevalence of Carotid Artery Stenosis in Acute Ischaemic Stroke patients. 3. To find if there is any association between Carotid Artery Stenosis and risk factors such as Diabetes mellitus, Hypertension, Hyperlipidemia, Smoking and Age.

  20. Epidemiology of neurological disorders in India: review of background, prevalence and incidence of epilepsy, stroke, Parkinson's disease and tremors.

    Gourie-Devi, M

    2014-01-01

    Growth and development of neuroepidemiology in India during the last four decades has been documented highlighting the historical milestones. The prevalence rates of the spectrum of neurological disorders from different regions of the country ranged from 967-4,070 with a mean of 2394 per 100,000 population, providing a rough estimate of over 30 million people with neurological disorders (excluding neuroinfections and traumatic injuries). Prevalence and incidence rates of common disorders including epilepsy, stroke, Parkinson's disease and tremors determined through population-based surveys show considerable variation across different regions of the country. The need for a standardized screening questionnaire, uniform methodology for case ascertainment and diagnosis is an essential requiste for generating robust national data on neurological disorders. Higher rates of prevalence of neurological disorders in rural areas, 6-8 million people with epilepsy and high case fatality rates of stroke (27-42%) call for urgent strategies to establish outreach neurology services to cater to remote and rural areas, develop National Epilepsy Control Program and establish stroke units at different levels of health care pyramid.

  1. Clarithromycin-induced acute interstitial nephritis and minimal change disease.

    Russell, Wendy; Smith, William

    2009-10-01

    Drug associated acute interstitial nephritis and minimal change disease has been well documented but the simultaneous presentation of both is rare and has not been reported with clarithromycin. We describe a case of simultaneous acute tubulointerstitial nephritis and minimal change disease induced by clarithromycin. The patient had acute kidney injury, nephrotic syndrome, eosinophilic pneumonitis and a maculopapular skin rash. The role of steroid therapy in acute interstitial nephritis is controversial but is accepted as beneficial in minimal change nephrotic syndrome. Steroid therapy in our patient resulted in complete clinical resolution.

  2. Neurological, psychological, and cognitive disorders in patients with chronic kidney disease on conservative and replacement therapy.

    Lai, Silvia; Mecarelli, Oriano; Pulitano, Patrizia; Romanello, Roberto; Davi, Leonardo; Zarabla, Alessia; Mariotti, Amalia; Carta, Maria; Tasso, Giorgia; Poli, Luca; Mitterhofer, Anna Paola; Testorio, Massimo; Frassetti, Nicla; Aceto, Paola; Galani, Alessandro; Lai, Carlo

    2016-11-01

    Chronic kidney disease (CKD) is a highly prevalent condition in the world. Neurological, psychological, and cognitive disorders, related to CKD, could contribute to the morbidity, mortality, and poor quality of life of these patients. The aim of this study was to assess the neurological, psychological, and cognitive imbalance in patients with CKD on conservative and replacement therapy.Seventy-four clinically stable patients affected by CKD on conservative therapy, replacement therapy (hemodialysis (HD), peritoneal dialysis (PD)), or with kidney transplantation (KT) and 25 healthy controls (HC), matched for age and sex were enrolled. Clinical, laboratory, and instrumental examinations, as renal function, inflammation and mineral metabolism indexes, electroencephalogram (EEG), psychological (MMPI-2, Sat P), and cognitive tests (neuropsychological tests, NPZ5) were carried out.The results showed a significant differences in the absolute and relative power of delta band and relative power of theta band of EEG (P = 0.008, P therapy, and Grade 2-3 in KT patients. The scales of MMPI-2 hysteria and paranoia, are significantly correlated with creatinine, eGFR, serum nitrogen, CRP, 1,25-(OH)2D3, intact parathyroid hormone (iPTH), phosphorus, and cynical and hysterical personality, are correlated with higher relative power of delta (P = 0.016) and theta band (P = 0.016). Moreover, all NPZ5 scores showed a significant difference between the means of nephropathic patients and the means of the HC, and a positive correlation with eGFR, serum nitrogen, CRP, iPTH, and vitamin D.In CKD patients, simple and noninvasive instruments, as EEG, and cognitive-psychological tests, should be performed and careful and constant monitoring of renal risk factors, probably involved in neuropsychological complications (inflammation, disorders of mineral metabolism, electrolyte disorders, etc.), should be carried out. Early identification and adequate therapy of neuropsychological

  3. Role of Oxidative Stress in the Worsening of Neurologic Wilson Disease Following Chelating Therapy.

    Kalita, Jayantee; Kumar, Vijay; Ranjan, Abhay; Misra, Usha K

    2015-12-01

    Patients with neurologic Wilson disease (NWD) may worsen on treatment, but there is no study evaluating the role of oxidative stress. We report the role of plasma glutathione (GSH), total antioxidant capacity (TAC) and malondialdehyde (MDA) in the worsening of NWD following treatment. Fifty-one treatment-naïve NWD patients were subjected to detailed clinical evaluation. The severity of NWD was noted, and dystonia was measured by Burke-Fahn-Marsden (BFM) score. Their hematological, serum chemistry, ultrasound abdomen and cranial MRI changes were noted. Plasma GSH, TAC and MDA, serum free copper (Cu) and 24-h urinary Cu were measured at admission and at 3 and 6 months after treatment. The patients were considered worsened if there was one or more grade deterioration in severity scale, >10 % deterioration in BFM score or appearance of new neurologic signs. The median age of the patients was 11 (5-37) years, and 12 were females. Following treatment, 25 patients improved, 12 worsened, and 14 had stationary course. The worsened group at 3 months had lower GSH (1.99 ± 0.17 vs. 2.30 ± 0.30 mg/dl; P = 0.004) and TAC (1.59 ± 0.12 vs. 1.82 ± 0.17 mmol Trolox equivalent/L; P = 0.001) and higher MDA (5.24 ± 0.22 vs. 4.34 ± 0.46 nmol/ml; P < 0.001) levels compared to the improved group. These changes were associated with increased serum free Cu (41.81 ± 3.31 vs. 35.62 ± 6.40 µg/dl; P = 0.02) and 24-h urinary Cu (206.42 ± 41.61 vs. 121.99 ± 23.72 µg/24 h; P < 0.001) in the worsened compared to the improved group. All the patients having worsening were on penicillamine. Worsening following chelating treatment in NWD may be due to oxidative stress which is induced by increased serum free Cu. These results may have future therapeutic implication and needs further study.

  4. Caenorhabditis elegans as an experimental tool for the study of complex neurological diseases: Parkinson's disease, Alzheimer's disease and autism spectrum disorder.

    Calahorro, Fernando; Ruiz-Rubio, Manuel

    2011-12-01

    The nematode Caenorhabditis elegans has a very well-defined and genetically tractable nervous system which offers an effective model to explore basic mechanistic pathways that might be underpin complex human neurological diseases. Here, the role C. elegans is playing in understanding two neurodegenerative conditions, Parkinson's and Alzheimer's disease (AD), and a complex neurological condition, autism, is used as an exemplar of the utility of this model system. C. elegans is an imperfect model of Parkinson's disease because it lacks orthologues of the human disease-related genes PARK1 and LRRK2 which are linked to the autosomal dominant form of this disease. Despite this fact, the nematode is a good model because it allows transgenic expression of these human genes and the study of the impact on dopaminergic neurons in several genetic backgrounds and environmental conditions. For AD, C. elegans has orthologues of the amyloid precursor protein and both human presenilins, PS1 and PS2. In addition, many of the neurotoxic properties linked with Aβ amyloid and tau peptides can be studied in the nematode. Autism spectrum disorder is a complex neurodevelopmental disorder characterised by impairments in human social interaction, difficulties in communication, and restrictive and repetitive behaviours. Establishing C. elegans as a model for this complex behavioural disorder is difficult; however, abnormalities in neuronal synaptic communication are implicated in the aetiology of the disorder. Numerous studies have associated autism with mutations in several genes involved in excitatory and inhibitory synapses in the mammalian brain, including neuroligin, neurexin and shank, for which there are C. elegans orthologues. Thus, several molecular pathways and behavioural phenotypes in C. elegans have been related to autism. In general, the nematode offers a series of advantages that combined with knowledge from other animal models and human research, provides a powerful

  5. Research advances in treatment of neurological and psychological diseases by acupuncture at the Acupuncture Meridian Science Research Center

    Bombi Lee

    2014-06-01

    Full Text Available Acupuncture is an ancient therapeutic intervention that can be traced back at least 2100 years and is emerging worldwide as one of the most widely used therapies in the field of complementary and alternative medicine. Due to limitations associated with Western medicine's focus on the treatment of diseases rather than on their causes, interests are shifting to complementary and alternative medicines. The Acupuncture and Meridian Science Research Center (AMSRC was established in 2005 to elucidate the neurophysiological mechanisms of acupuncture for neurological diseases based on multidisciplinary research supported by the Korean Ministry of Science and Technology. In the AMSRC, resultant research articles have shown that acupuncture can improve neurological and psychological problems, including Parkinson's disease, pain, and depression, in animal models. Basic research studies suggest its effectiveness in treating various problems such as depression, drug addiction, epilepsy, ischemia, dementia, Parkinson's disease, and pain. We strongly believe that these effects, evident from the AMSRC research results, can play leading roles in the use of acupuncture for treating neurological diseases, based on collaboration among various academic fields such as neurophysiology, molecular genetics, and traditional Korean medicine.

  6. Anti-B-Cell Therapies in Autoimmune Neurological Diseases: Rationale and Efficacy Trials.

    Alexopoulos, Harry; Biba, Angie; Dalakas, Marinos C

    2016-01-01

    B cells have an ever-increasing role in the etiopathology of a number of autoimmune neurological disorders, acting as antibody-producing cells and, most importantly, as sensors, coordinators, and regulators of the immune response. B cells, among other functions, regulate the T-cell activation process through their participation in antigen presentation and production of cytokines. The availability of monoclonal antibodies or fusion proteins against B-cell surface molecules or B-cell trophic factors bestows a rational approach for treating autoimmune neurological disorders, even when T cells are the main effector cells. This review summarizes basic aspects of B-cell biology, discusses the role(s) of B cells in neurological autoimmunity, and presents anti-B-cell drugs that are either currently on the market or are expected to be available in the near future for treating neurological autoimmune disorders.

  7. Application of Array-Based Comparative Genomic Hybridization to Pediatric Neurologic Diseases

    2013-01-01

    Purpose Array comparative genomic hybridization (array-CGH) is a technique used to analyze quantitative increase or decrease of chromosomes by competitive DNA hybridization of patients and controls. This study aimed to evaluate the benefits and yield of array-CGH in comparison with conventional karyotyping in pediatric neurology patients. Materials and Methods We included 87 patients from the pediatric neurology clinic with at least one of the following features: developmental delay, mental r...

  8. Nested PCR for rapid detection of mumps virus in cerebrospinal fluid from patients with neurological diseases.

    Poggio, G P; Rodriguez, C; Cisterna, D; Freire, M C; Cello, J

    2000-01-01

    In this study, we have developed a reverse transcription (RT)-nested polymerase chain reaction (n-PCR) for the detection of mumps virus RNA in cerebrospinal fluid (CSF) from patients with neurological infections. A specific 112-bp fragment was amplified by this method with primers from the nucleoprotein of the mumps virus genome. The mumps virus RT-n-PCR was capable of detecting 0.001 PFU/ml and 0.005 50% tissue culture infective dose/ml. This method was found to be specific, since no PCR product was detected in each of the CSF samples from patients with proven non-mumps virus-related meningitis or encephalitis. Mumps virus RNA was detected in all 18 CSF samples confirmed by culture to be infected with mumps virus. Positive PCR results were obtained for the CSF of 26 of 28 patients that were positive for signs of mumps virus infection (i.e., cultivable virus from urine or oropharyngeal samples or positivity for anti-mumps virus immunoglobulin M) but without cultivable virus in their CSF. Overall, mumps virus RNA was detected in CSF of 96% of the patients with a clinical diagnosis of viral central nervous system (CNS) disease and confirmed mumps virus infection, while mumps virus was isolated in CSF of only 39% of the patients. Furthermore, in a retrospective study, we were able to detect mumps virus RNA in 25 of 55 (46%) CSF samples from patients with a clinical diagnosis of viral CNS disease and negative laboratory evidence of viral infection including mumps virus infection. The 25 patients represent 12% of the 236 patients who had a clinical diagnosis of viral CNS infections and whose CSF was examined at our laboratory for a 2-year period. The findings confirm the importance of mumps virus as a causative agent of CNS infections in countries with low vaccine coverage rates. In summary, our study demonstrates the usefulness of the mumps virus RT-n-PCR for the diagnosis of mumps virus CNS disease and suggests that this assay may soon become the "gold standard" test

  9. Mitochondrial dysfunction in psychiatric and neurological diseases: cause(s), consequence(s), and implications of antioxidant therapy.

    Kasote, Deepak M; Hegde, Mahabaleshwar V; Katyare, Surendra S

    2013-01-01

    Mitochondrial dysfunction is at the base of development and progression of several psychiatric and neurologic diseases with different etiologies. MtDNA/nDNA mutational damage, failure of endogenous antioxidant defenses, hormonal malfunction, altered membrane permeability, metabolic dysregulation, disruption of calcium buffering capacity and ageing have been found to be the root causes of mitochondrial dysfunction in psychatric and neurodegenerative diseases. However, the overall consequences of mitochondrial dysfunction are only limited to increase in oxidative/nitrosative stress and cellular energy crises. Thus far, extensive efforts have been made to improve mitochondrial function through specific cause-dependent antioxidant therapy. However, owing to complex genetic and interlinked causes of mitochondrial dysfunction, it has not been possible to achieve any common, unique supportive antioxidant therapeutic strategy for the treatment of psychiatric and neurologic diseases. Hence, we propose an antioxidant therapeutic strategy for management of consequences of mitochondrial dysfunction in psychiatric and neurologic diseases. It is expected that this will not only reduces oxidative stress, but also promote anaerobic energy production.

  10. Crosstalk of metabolic factors and neurogenic signaling in adult neurogenesis: Implication of metabolic regulation for mental and neurological diseases.

    Gao, Chong; Wang, Qi; Chung, Sookja K; Shen, Jiangang

    2017-02-07

    Metabolic disorders like diabetes and obesity are commonly companied with neurological diseases and psychiatric disorders. Accumulating evidences indicated that cellular metabolic factors affect adult neurogenesis and have modulating effects on neurodegenerative disorders and psychiatric diseases. Adult neurogenesis contains multiple steps including proliferation of neural stem cells, lineage commitments of neural progenitor cells, maturation into functional neurons, and integration into neuronal network. Many intrinsic and extrinsic factors produced from neural stem/progenitor cells and their microenvironment or neurogenic niche take roles in modulating neurogenesis and contribute to the brain repair and functional recoveries in many neurological diseases and psychiatric disorders. In this article, we review current progress about how different growth factors, neurotrophin, neurotransmitters and transcriptional factors work on regulating neurogenic process. In particular, we emphasize the roles of the cellular metabolic factors, such as insulin/IGF signaling, incretins, and lipid metabolic signaling molecules in modulating adult neurogenesis, and discuss their impacts on neurological behaviors. We propose that the metabolic factors could be the new therapeutic targets for adult neurogenesis. Plus, the metabolism-regulating drugs have the potentials for treatment of neurodegenerative diseases and mental disorders.

  11. Managing Acute Complications Of Sickle Cell Disease In Pediatric Patients.

    Subramaniam, Sathyaseelan; Chao, Jennifer H

    2016-11-01

    Sickle cell disease is a chronic hematologic disease with a variety of acute, and often recurring, complications. Vaso-occlusive crisis, a unique but common presentation in sickle cell disease, can be challenging to manage. Acute chest syndrome is the leading cause of death in patients with sickle cell disease, occurring in more than half of patients who are hospitalized with a vaso-occlusive crisis. Uncommon diagnoses in children, such as stroke, priapism, and transient red cell aplasia, occur more frequently in patients with sickle cell disease and necessitate a degree of familiarity with the disease process and its management. Patients with sickle cell trait generally have a benign course, but are also subject to serious complications. This issue provides a current review of evidence-based management of the most common acute complications of sickle cell disease seen in pediatric patients in the emergency department.

  12. Modeling Human Neurological and Neurodegenerative Diseases: From Induced Pluripotent Stem Cells to Neuronal Differentiation and Its Applications in Neurotrauma

    Bahmad, Hisham; Hadadeh, Ola; Chamaa, Farah; Cheaito, Katia; Darwish, Batoul; Makkawi, Ahmad-Kareem; Abou-Kheir, Wassim

    2017-01-01

    With the help of several inducing factors, somatic cells can be reprogrammed to become induced pluripotent stem cell (iPSCs) lines. The success is in obtaining iPSCs almost identical to embryonic stem cells (ESCs), therefore various approaches have been tested and ultimately several ones have succeeded. The importance of these cells is in how they serve as models to unveil the molecular pathways and mechanisms underlying several human diseases, and also in its potential roles in the development of regenerative medicine. They further aid in the development of regenerative medicine, autologous cell therapy and drug or toxicity screening. Here, we provide a comprehensive overview of the recent development in the field of iPSCs research, specifically for modeling human neurological and neurodegenerative diseases, and its applications in neurotrauma. These are mainly characterized by progressive functional or structural neuronal loss rendering them extremely challenging to manage. Many of these diseases, including Parkinson's disease (PD), Huntington's disease (HD), Amyotrophic lateral sclerosis (ALS) and Alzheimer's disease (AD) have been explored in vitro. The main purpose is to generate patient-specific iPS cell lines from the somatic cells that carry mutations or genetic instabilities for the aim of studying their differentiation potential and behavior. This new technology will pave the way for future development in the field of stem cell research anticipating its use in clinical settings and in regenerative medicine in order to treat various human diseases, including neurological and neurodegenerative diseases. PMID:28293168

  13. Overstimulation of the inhibitory nervous system plays a role in the pathogenesis of neuromuscular and neurological diseases: a novel hypothesis [version 2; referees: 2 approved

    Bert Tuk

    2016-08-01

    Full Text Available Based upon a thorough review of published clinical observations regarding the inhibitory system, I hypothesize that this system may play a key role in the pathogenesis of a variety of neuromuscular and neurological diseases. Specifically, excitatory overstimulation, which is commonly reported in neuromuscular and neurological diseases, may be a homeostatic response to inhibitory overstimulation. Involvement of the inhibitory system in disease pathogenesis is highly relevant, given that most approaches currently being developed for treating neuromuscular and neurological diseases focus on reducing excitatory activity rather than reducing inhibitory activity.

  14. ACUTE ATAXIA, TAKING PLACE AFTER ACUTE RESPIRATORY VIRAL INFECTION IN 2 Y. O. GIRL, AS A DEBUT NEUROLOGIC SIGN OF THE ANGELMAN SYNDROME

    E. B. Voropanova

    2015-01-01

    Full Text Available Angleman syndrome (АS – is a chromosomal syndrome, which is manifested through atypical autism with feeble minding, epilepsy, outrage of the speech development, movement disorders, ataxia, as well as special (happy behavior of patients, combined with outbursts of laugh. The disease is caused by the mutation of 15q11.2–13 maternal locus or by the gene of UBE3A ubiquitinated complex. Such genes regulate the functional activity of hippocampus neurons, of olfactory bulbs, of the parastriate cortex, of the tentorium. We demonstrate the atypical AS case, which clinical presentation developed after acute respiratory viral infection with febrile temperature. The disease started with episodes of acute ataxia, interrupting daily activities of the child. Step by step the speech development was regressing – several words have fallen out,leaving the space for babbling sounds. Also appeared stereotypic movements of upper extremities (bending of arms in elbow joints, its retraction and joggling of hands, unmotivated laugh. Due to the nonrelevant starting presentation in the acute period following conditions were differentially diagnosed: 1 opsoclonus-myoclonus syndrome; 2 cerebral circulation diseases; 3 epilepsy with absences and atonic attacks; 4 paroxysmal dyskenisias and ataxias; 5 start of the neurodegenerative disease; 6 early childhood autism. Results of laboratory research allowed to exclude opsoclonus-myoclonus, the magnetic and resonance tomography and vessels research allowed to exclude the cerebrovascular pathology. Changes, revealed in the course of the videoelectroencephalographic monitoring, as well as anamnesis data (clinical symptoms after fever allowed to narrow the diagnostic search; AS suspected. Provided the combination of ataxia with movement disorders, it was decided to carry out not molecular & genetic, but also micromatrix analysis, in order to exclude the channelopathy, as well as other genetic reasons. The method of

  15. MRI in acute neuropathic Gaucher's disease

    Chang, Y.C. [Department of Paediatrics, Chang Gung Memorial Children' s Hospital in Kaohsiung, Taiwan (Taiwan); Huang, C.C. [National Cheng Kung University Hospital, Taiwan (Taiwan); Chen, C.Y. [Department of Diagnostic Radiology, Tri-Service General Hospital and National Defence Medical Centre, Taipei (Taiwan); Zimmerman, R.A. [Department of Radiology, Children' s Hospital of Philadelphia (United States)

    2000-01-01

    We present the cranial MRI findings in a 6-month-old girl with biopsy-proven acute neuropathic Gaucher's disease, which include unilateral cerebral atrophy and dural thickening with contrast enhancement. (orig.)

  16. ACUTE RESPIRATORY DISEASE AS THE DEBUT OF SYSTEMIC LUPUS ERYTHEMATOSUS

    A. Yu. Ischenko

    2015-01-01

    Full Text Available Systemic lupus erythematosus — a chronic autoimmune disease that is often associated with infectious processes. The paper presents two clinical cases of systemic lupus erythematosus , debuted with acute respiratory infection.

  17. NEUROLOGICAL DISORDER

    2003-01-01

    16.1 Intoxication and drug related disease2003140 The neuromuscular transmission effects induced by pralidoxime chloride on rats with acute iso-carbophos poisoning.JU Zhichang(琚志昌),et al. Instil Nutr Food Hyg, Beijing Center Dis Prev & Contr, Bei-

  18. Enterovirus 71-associated hand, foot and mouth diseases with neurologic symptoms, a university hospital experience in Korea, 2009

    Hye Kyung Cho

    2010-05-01

    Full Text Available Purpose : Hand-foot-mouth disease (HFMD is a common viral illness in children, which is usually mild and self-limiting. However, in recent epidemics of HFMD in Asia, enterovirus 71 (EV71 has been recognized as a causative agent with severe neurological symptoms with or without cardiopulmonary involvement. HFMD was epidemic in Korea in the spring of 2009. Severe cases with complications including death have been reported. The clinical characteristics in children with neurologic manifestations of EV71 were studied in Ewha Womans University Mokdong Hospital. Methods : Examinations for EV71 were performed from the stools, respiratory secretion or CSF of children who presented neurologic symptoms associated with HFMD by realtime PCR. Clinical and radiologic data of the patients were collected and analyzed. Results : EV71 was isolated from the stool of 16 patients but not from respiratory secretion or CSF. Among the 16 patients, meningitis (n=10 was the most common manifestation, followed by Guillain-Barr&eacute; syndrome (n=3, meningoencephalitis (n=2, poliomyelitis-like paralytic disease (n=1, and myoclonus (n=1. Gene analysis showed that most of them were caused by EV71 subgenotype C4a, which was prevalent in China in 2008. Conclusion : Because EV71 causes severe complications and death in children, a surveillance system to predict upcoming outbreaks should be established and maintained and adequate public health measures are needed to control disease.

  19. Acute myeloid leukaemia as a cause of acute ischaemic heart disease

    van Haelst, P.L.; Schot, Bart; Hoendermis, E.S.; van den Berg, M.P.

    2006-01-01

    Ischaemic heart disease is almost invariably the result of atherosclerotic degeneration of the coronary arteries. However, other causes of ischaemic heart disease should always be considered. Here we describe two patients with a classic presentation of ischaemic heart disease resulting from acute le

  20. Atmospheric pressure does not influence acute diverticular disease

    Velayos, Benito; Pons-Renedo, Fernando; Feranández-Salazar, Luis; Muñoz, María Fe; Olmo, Lourdes del; Almaraz Gómez, Ana; Beltrán de Heredia, Juan; Hernández-González, José Manuel

    2013-01-01

    Producción Científica The article offers information on a study which examines the influence of atmospheric pressure on the development of acute diverticular disease. The value of atmospheric pressure and its daily trends in 2012 was collected to prove whether atmospheric pressure influence this disease by raising intra-diverticular pressure in days with higher atmospheric pressure. The study involved patients with acute diverticulitis who underwent computed tomography.

  1. Acute Demyelinating Disease after Oral Therapy with Herbal Extracts

    Alex Kostianovsky

    2011-06-01

    Full Text Available Central nervous system demyelinating processes such as multiple sclerosis and acute disseminated encephalomyelitis constitute a group of diseases not completely understood in their physiopathology. Environmental and toxic insults are thought to play a role in priming autoimmunity. The aim of the present report is to describe a case of acute demyelinating disease with fatal outcome occurring 15 days after oral exposure to herbal extracts.

  2. Acute Exacerbation of Chronic Obstructive Pulmonary Disease: Cardiovascular Links

    Cheryl R. Laratta

    2014-01-01

    Full Text Available Chronic obstructive pulmonary disease (COPD is a chronic, progressive lung disease resulting from exposure to cigarette smoke, noxious gases, particulate matter, and air pollutants. COPD is exacerbated by acute inflammatory insults such as lung infections (viral and bacterial and air pollutants which further accelerate the steady decline in lung function. The chronic inflammatory process in the lung contributes to the extrapulmonary manifestations of COPD which are predominantly cardiovascular in nature. Here we review the significant burden of cardiovascular disease in COPD and discuss the clinical and pathological links between acute exacerbations of COPD and cardiovascular disease.

  3. Controlling the Regional Identity of hPSC-Derived Neurons to Uncover Neuronal Subtype Specificity of Neurological Disease Phenotypes

    Kent Imaizumi

    2015-12-01

    Full Text Available The CNS contains many diverse neuronal subtypes, and most neurological diseases target specific subtypes. However, the mechanism of neuronal subtype specificity of disease phenotypes remains elusive. Although in vitro disease models employing human pluripotent stem cells (PSCs have great potential to clarify the association of neuronal subtypes with disease, it is currently difficult to compare various PSC-derived subtypes. This is due to the limited number of subtypes whose induction is established, and different cultivation protocols for each subtype. Here, we report a culture system to control the regional identity of PSC-derived neurons along the anteroposterior (A-P and dorsoventral (D-V axes. This system was successfully used to obtain various neuronal subtypes based on the same protocol. Furthermore, we reproduced subtype-specific phenotypes of amyotrophic lateral sclerosis (ALS and Alzheimer’s disease (AD by comparing the obtained subtypes. Therefore, our culture system provides new opportunities for modeling neurological diseases with PSCs.

  4. Clinical use of gadobutrol for contrast-enhanced magnetic resonance imaging of neurological diseases

    Cheng KT

    2012-02-01

    Full Text Available Kenneth T Cheng1, Hannah Y Cheng2, Kam Leung31Department of Radiology and Imaging Sciences, Clinical Center, National Institutes of Health, Bethesda, MD, USA; 2Freelance Technical Writer, New Orleans, LA, USA; 3National Center for Biotechnology Information, National Library of Medicine, National Institutes of Health, Bethesda, MD, USAAbstract: Contrast-enhanced magnetic resonance imaging (CE-MRI is an important clinical tool for diagnosing neurological diseases. The appropriate use of a suitable MRI contrast agent or contrast pharmaceutical is essential for CE-MRI to produce desirable diagnostic images. Currently, there are seven contrast agents (CAs or pharmaceuticals approved for clinical imaging of the central nervous system (CNS in the US, Europe, or Japan. All of the clinically approved CAs are water-soluble gadolinium-based contrast agents (GBCAs which do not penetrate the CNS blood–brain barrier (BBB. These agents are used for imaging CNS areas without a BBB, or various pathologies, such as tumors and infection that break down the BBB and allow CAs to enter into the surrounding parenchyma. Clinically, GBCAs are most useful for detecting primary and secondary cerebral neoplastic lesions. Among these CNS GBCAs, gadobutrol (Gd-BT-DO3A, Gadovist™ is a neutral, nonionic, macrocyclic compound that showed promising results from clinical trials of CNS imaging. In comparison with other GBCAs, Gd-BT-DO3A has relatively high in vitro kinetic stability and r1 relaxivity. Gd-BT-DO3A has been recently approved by the US Food and Drug Administration (FDA in 2011 for CNS imaging. A review of available literature shows that Gd-BT-DO3A exhibits similar safety and clinical efficacy profiles to other GBCAs. Gd-BT-DO3A has the distinguishing feature that it is the only clinical agent commercially available in a formulation of 1.0 M concentration with a relatively higher in vitro T1 shortening per unit volume than other clinical GBCAs which are only

  5. Does surgical treatment within 4 hours after trauma have an influence on neurological remission in patients with acute spinal cord injury?

    Biglari B

    2016-08-01

    Full Text Available Bahram Biglari,1 Christopher Child,2 Timur Mert Yildirim,2 Tyler Swing,2 Tim Reitzel,1 Arash Moghaddam2 1Department of Paraplegiology and Technical Orthopedics, BG Trauma Centre, Ludwigshafen, Germany; 2Heidelberg Trauma Research Group, Center for Orthopedics, Trauma Surgery and Spinal Cord injury, Heidelberg University Hospital, Heidelberg, Germany Background: The proper timing for surgery in patients with acute spinal cord injury is controversial. This study was conducted to detect if there is an advantage in early (within the first 4 hours after trauma compared to late (between 4 and 24 hours after trauma surgery on neurological outcome.Methods: In this single institution prospective cohort study, data were analyzed from 51 spinal cord injured patients with an average age of 43.4 (±19.2 years. The influence of early (29 patients within the first 4 hours as opposed to late (22 patients between 4 and 24 hours decompression was evaluated by comparing data for neurological outcome. Patients of the study collectively suffered acute spinal fractures from C2 to L3 (cervical 39.2%, thoracic 29.4%, and lumbal 21.6% or nonosseous lesions (9.8%. American Spinal Injury Association (ASIA Impairment Scale (AIS grades were assessed at time of admission and 6 months after trauma or longer depending on the time of release. Surgical treatment included early stabilization and decompression within 24 hours.Results: No significant difference between improved neurological function, measured with the AIS, and an early or late surgery time can be seen (P=0.402. Furthermore, binary logistic regression shows no significant difference between sex or age, and AIS improvement as possible confounders.Conclusion: In our study, all patients with spinal cord injury were treated with spine stabilization and decompression within the first 24 hours after trauma. Surgical decompression within the first 4 hours after trauma was not associated with improved neurological outcome

  6. Acute Kidney Disease After Liver and Heart Transplantation.

    Rossi, Ana P; Vella, John P

    2016-03-01

    After transplantation of nonrenal solid organs, an acute decline in kidney function develops in the majority of patients. In addition, a significant number of nonrenal solid organ transplant recipients develop chronic kidney disease, and some develop end-stage renal disease, requiring renal replacement therapy. The incidence varies depending on the transplanted organ. Acute kidney injury after nonrenal solid organ transplantation is associated with prolonged length of stay, cost, increased risk of death, de novo chronic kidney disease, and end-stage renal disease. This overview focuses on the risk factors for posttransplant acute kidney injury after liver and heart transplantation, integrating discussion of proteinuria and chronic kidney disease with emphasis on pathogenesis, histopathology, and management including the use of mechanistic target of rapamycin inhibition and costimulatory blockade.

  7. Maple Syrup Urine Disease (MSUD detected in neurologic disorders Iraqi children

    Adel A. Kareem

    2016-09-01

    Conclusion In the absence of newborn screening, MSUD is not uncommon in neurologically disorder patients where MSUD was still diagnosed clinically, but delayed. The importance of clinical awareness and accurate biochemical analysis were the key tools for diagnosis and the necessity for a comprehensive national newborn screening program.

  8. A cross-sectional evaluation of home health service in patients with chronic neurologic diseases in a province of Turkey.

    Senadim, Songul; Cabalar, Murat; Gedik, Habip; Kasim, Ali Bayram; Bulut, Anıl; Yayla, Vildan; Erdogdu, Zeynep

    2016-03-01

    In this study, we aimed to compare patients' characteristics, comorbid risk factors, medical supplies, and caregivers' demographics between stroke patients and patients with other chronic neurological diseases receiving home health services. In our study, between November 2013 and March 2014, chronic neurological disease (CND) patients having home health services were enrolled in the study. During patient visits, patients were assessed by the questionnaire comprising the modified Rankin scale (mRS), Barthel index, Zarit caregiver burden scale, and mini nutritional assessment (MNA). Stroke patients were classified as Group I, and the other neurologic diseases as Group II. A total of 202 patients including stroke patients (n = 112), dementia (n = 64), Parkinson's disease (n = 17), motor neuron disease (n = 4), brain cancer (n = 2), cerebral palsy (n = 1), multiple sclerosis (n = 1), and head trauma (n = 1) answered the questionnaire. The mean age of Group I (61K:51E) was 76.6 ± 9.1 years; the Group II (28M:62F) was 80.9 ± 12.3 years. The mean age of Group I was significantly lower than Group II (p = 0.005) and the number of male patients in Group I was significantly higher (p = 0.001). The educational status between the two groups was not significantly different in terms of duration of illness and addiction. There was no difference between the two groups in terms of Zarit caregiver burden scale, Barthel index, and mRS. The presence of malnutrition (MNA nutritional support and providing appropriate nutritional support in other CND patients are expected to increase the life quality.

  9. Relationship between intracellular Na+ concentration and reduced Na+ affinity in Na+,K+-ATPase mutants causing neurological disease

    Toustrup-Jensen, Mads Schak; Einholm, Anja P.; Schack, Vivien;

    2014-01-01

    The neurological disorders familial hemiplegic migraine type 2 (FHM2), alternating hemiplegia of childhood (AHC), and rapid-onset dystonia parkinsonism (RDP) are caused by mutations of Na+,K+-ATPase α2- and α3-isoforms, expressed in glial and neuronal cells, respectively. Although these disorders...... are distinct, they overlap in phenotypical presentation. Two Na+,K+-ATPase mutations, extending the C-terminus by either 28 residues ("+28" mutation) or an extra tyrosine ("+Y"), are associated with FHM2 and RDP, respectively. We describe here functional consequences of these and other neurological disease......, addressing the question to what extent they cause a change of the intracellular Na+ and K+ concentrations ([Na+]i and [K+]i) in COS cells. C-terminal extension mutants generally showed dramatically reduced Na+ affinity without disturbance of K+ binding, as did other RDP mutants. No phosphorylation from ATP...

  10. Ultrasonographic Findings of Extratesticular Diseases Causing Acute Scrotal Disorders

    Chung, Jae Joon; Lee, Tack; Chang, So Yong; Kim, Myeong Jin; Yoo, Hyung Sik; Lee, Jong Tae [Yonsei University College of Medicine, Seoul (Korea, Republic of)

    1996-12-15

    To evaluate the kinds of extratesticular diseases causing acute scrotal disorders by emergent sonography of the scrotum. Scrotal sonography in sixty-five patients, with age ranging from 5months to 82 years (mean : 27.3 years), with acute scrotal pain and swelling, was prospectively carried out by either a 10 or 7.5 MHz transducer. We evaluated the size and echogenicity of the epididymis, the presence of extratesticular solid mass or cyst, testicular involvement by extratesticular diseases, calcification, hydrocele and scrotal wall thickening. The most common cause of acute scrotal disorders was acute epididymitis (n= 50), followed by acute epididymo-orchitis (n = 4), mumps epididymo-orchitis (n = 2), enlarged epididymis secondary to testicular torsion (n = 2), infected hydrocele (n = 2), epididymal cyst (n = 2), rupture of varicocele (n = 1), angioneurotic edema (n = 1), and sperm granuloma (n = 1). Hydrocele was seen in 20 cases, and epididymal calcification was noted in 6 cases. Emergent scrotal sonography was useful for correct diagnosis and proper treatment in patients with acute scrotal disorders, especially in the differentiation of the acute epididymitis from other intrascrotal diseases

  11. Utility of Induced Pluripotent Stem Cells for the Study and Treatment of Genetic Diseases: Focus on Childhood Neurological Disorders.

    Barral, Serena; Kurian, Manju A

    2016-01-01

    The study of neurological disorders often presents with significant challenges due to the inaccessibility of human neuronal cells for further investigation. Advances in cellular reprogramming techniques, have however provided a new source of human cells for laboratory-based research. Patient-derived induced pluripotent stem cells (iPSCs) can now be robustly differentiated into specific neural subtypes, including dopaminergic, inhibitory GABAergic, motorneurons and cortical neurons. These neurons can then be utilized for in vitro studies to elucidate molecular causes underpinning neurological disease. Although human iPSC-derived neuronal models are increasingly regarded as a useful tool in cell biology, there are a number of limitations, including the relatively early, fetal stage of differentiated cells and the mainly two dimensional, simple nature of the in vitro system. Furthermore, clonal variation is a well-described phenomenon in iPSC lines. In order to account for this, robust baseline data from multiple control lines is necessary to determine whether a particular gene defect leads to a specific cellular phenotype. Over the last few years patient-derived neural cells have proven very useful in addressing several mechanistic questions related to central nervous system diseases, including early-onset neurological disorders of childhood. Many studies report the clinical utility of human-derived neural cells for testing known drugs with repurposing potential, novel compounds and gene therapies, which then can be translated to clinical reality. iPSCs derived neural cells, therefore provide great promise and potential to gain insight into, and treat early-onset neurological disorders.

  12. A 25-Year-Old Man with Exudative Retinal Detachments and Infiltrates without Hematological or Neurological Findings Found to Have Relapsed Precursor T-Cell Acute Lymphoblastic Leukemia

    Jordan S. Johnson

    2015-09-01

    Full Text Available Background: Precursor T-cell acute lymphoblastic leukemia (pre-T-ALL may cause ocular pathologies such as cotton-wool spots, retinal hemorrhage, and less commonly, retinal detachment or leukemic infiltration of the retina itself. However, these findings are typically accompanied by the pathognomonic hematological signs of acute leukemia. CasePresentation: In this case report and review of the literature, we describe a particularly unusual case of a 25-year-old man who presented to our hospital with bilateral exudative retinal detachments associated with posterior pole thickening without any hematological or neurological findings. The patient, who had a history of previously treated pre-T-ALL in complete remission, was found to have leukemia cell infiltration on retinal biopsy. Conclusion: Our case underscores the fact that the ophthalmologist may be the first provider to detect the relapse of previously treated leukemia, and that ophthalmic evaluation is critical for detecting malignant ocular infiltrates.

  13. Neurological deterioration in the acute phase of minor ischemic stroke is an independent predictor of poor outcomes at 1 year: results from the China National Stroke Registry (CNSR)

    JU Yi; ZHAO Xing-quan; WANG Chun-xue; WANG Yi-long; LIU Gai-fen; WANG Yong-jun

    2013-01-01

    Background The risk of clinical deterioration still exists in the acute phase despite the fact that patients with minor stroke may display less severe symptoms.The impact of this clinical deterioration on long-term outcomes is unknown.We characterized the clinical features of neurological deterioration (ND) in the acute phase of minor ischemic stroke (MIS) and investigated its impact on mid-and long-term outcomes.Methods This was a multi-centered,prospective clinical study involving patients with MIS (the National Institutes of Health Stroke Scale,NIHSS <3) recruited from the China National Stroke Registry.Patients were included who had been hospitalized within 24 hours of stroke onset.Baseline characteristics,complication rates during hospitalization,etiology of stroke,as well as 3-,6-,and 12-month post-stroke outcomes were compared between patients with and without ND during the acute phase.Results A number of 368 (15.2%) out of 2424 patients included in the study exhibited ND in the acute phase.Compared to patients without ND,patients with ND had longer hospital stay,increased rate of baseline diabetes,and multiple complications.Multivariate Logistic regression indicated that ND in acute phase was an independent factor predictive of increased dependence (adjusted odds ratio =5.20,95% Cl,3.51-7.70,P <0.001) at 12-month post-stroke.Conclusions The risk of ND in the acute phase is high in patients with MIS.ND in the acute phase is an independent predictor for poor outcomes at 12 months post-stroke onset.

  14. Acute colonic disease: How to image in emergency

    Romano, Stefania [Department of Diagnostic Imaging, A. Cardarelli Hospital, Viale Cardarelli 9, 80131 Naples (Italy)]. E-mail: stefromano@libero.it; Lombardo, Patrizia [Department of Diagnostic Imaging, A. Cardarelli Hospital, Viale Cardarelli 9, 80131 Naples (Italy); Cinque, Teresa [Department of Diagnostic Imaging, A. Cardarelli Hospital, Viale Cardarelli 9, 80131 Naples (Italy); Tortora, Giovanni [Department of Diagnostic Imaging, A. Cardarelli Hospital, Viale Cardarelli 9, 80131 Naples (Italy); Romano, Luigia [Department of Diagnostic Imaging, A. Cardarelli Hospital, Viale Cardarelli 9, 80131 Naples (Italy)

    2007-03-15

    The diseases affecting the large intestine represent a diagnostic problem in adult patients with acute abdomen, especially when clinical symptoms are not specific. The role of the diagnostic imaging is to help clinicians and surgeons in differential diagnosis for an efficient early and prompt therapy to perform. This review article summarizes the imaging spectrum of findings of colonic acute disease, from mechanical obstruction to inflammatory diseases and perforation, offering keys to problem solving in doubtful cases as well as discussing regarding the more indicated imaging method to use in emergency, particularly MDCT.

  15. Risk of psychiatric and neurological diseases in patients with workplace mobbing experience in Germany: a retrospective database analysis

    Kostev, Karel

    2014-05-01

    Full Text Available [english] Introduction: The number of mobbing experiences recorded has increased during recent years and it has now been established as global phenomenon among the working population. The goal of our study was to analyze the incidence of certain neurologic and psychiatric diseases as a consequence of mobbing as compared with a control group and to examine the possible influence of previous diseases that occurred within one year before the first mobbing documentation on the incidence of mobbing.Material & methods: We used a large database (IMS Disease Analyzer, Germany to collect data from general practitioners in Germany from 01/2003 until 12/2012. Based on age, gender, and health insurance, patients with experience of mobbing were matched with a control group of patients who had not reported workplace mobbing and who were being treated by the same physicians. At first, diseases that occurred within one year before the bullying experience took place (“index date” were noted and compared to a control group of similar composition in terms of gender, age, and health insurance. Subsequently, the prevalence of depression, anxiety, somatoform disorders, and sleep disorders following experiences of mobbing were determined. After adjustment to take into account the odds of bullying, the ratios of these diseases were assessed using a logistic regression model.Results: The study population consisted of n=2,625 patients and n=2,625 controls, of which 33% were men. The number of cases of bullying documented rose continuously from 2003 to 2011 and remained high in 2012. Those who would later become victims of mobbing demonstrated a considerably higher prevalence of diseases in general – these diseases were not confined to the neurologic-psychiatric spectrum. Following experiences of bullying, depression, anxiety, somatoform disorders, and sleep disorders were significantly more prevalent than in the control group (for all, p<0.05. Similarly, odds

  16. [Education and training in neurology: update].

    Yanagisawa, Nobuo

    2010-11-01

    Progress in basic neurosciences and advances in technology in the last decades have contributed to clarification of neural mechanisms in behavior or cognition in health and disease. They have elaborated diagnosis and treatment of nervous diseases remarkably. Needs in neurologists in both primary and specific medical services are rapidly increasing, with aging society and progresses in medical care in Japan. Attraction of neurology for students and junior residents is a great concern of Japanese Society of Neurology. In the undergraduate education, recent achievement in basic neurosciences including neurogenetics, molecular cytology, physio-pathology and imaging technique should be taught comprehensively. In the early postgraduate course for two years, neurology is either elective or obligatory depending on the curriculum of training institutions. Work at the stroke care unit is strongly recommended in the course of emergency service, which is mandatory. Experiences in acute infectious diseases, in various stages of neurodegenerative diseases, in collaboration with other specialist doctors for systemic diseases including metabolic or collagen diseases, in collaboration with other medical personnel in care of dementia are all included in advanced stages of postgraduate education before board examination. In summary, studies for practical services as well as clinical researches, teaching of symptoms and signs based on neural functions, and socio-economical issues for chronic nervous diseases in aged society are important in the education in neurology.

  17. Modeling oscillatory dynamics in brain microcircuits as a way to help uncover neurological disease mechanisms: A proposal

    Skinner, F. K. [Toronto Western Research Institute, University Health Network, Krembil Discovery Tower, Toronto Western Hospital, 60 Leonard Street, 7th floor, 7KD411, Toronto, Ontario M5T 2S8 (Canada); Department of Medicine (Neurology), University of Toronto, 200 Elizabeth Street, Toronto, Ontario M5G 2C4 (Canada); Department of Physiology, University of Toronto Medical Sciences Building, 3rd Floor, 1 King' s College Circle, Toronto, Ontario M5S 1A8 (Canada); Ferguson, K. A. [Toronto Western Research Institute, University Health Network, Krembil Discovery Tower, Toronto Western Hospital, 60 Leonard Street, 7th floor, 7KD411, Toronto, Ontario M5T 2S8 (Canada); Department of Physiology, University of Toronto Medical Sciences Building, 3rd Floor, 1 King' s College Circle, Toronto, Ontario M5S 1A8 (Canada)

    2013-12-15

    There is an undisputed need and requirement for theoretical and computational studies in Neuroscience today. Furthermore, it is clear that oscillatory dynamical output from brain networks is representative of various behavioural states, and it is becoming clear that one could consider these outputs as measures of normal and pathological brain states. Although mathematical modeling of oscillatory dynamics in the context of neurological disease exists, it is a highly challenging endeavour because of the many levels of organization in the nervous system. This challenge is coupled with the increasing knowledge of cellular specificity and network dysfunction that is associated with disease. Recently, whole hippocampus in vitro preparations from control animals have been shown to spontaneously express oscillatory activities. In addition, when using preparations derived from animal models of disease, these activities show particular alterations. These preparations present an opportunity to address challenges involved with using models to gain insight because of easier access to simultaneous cellular and network measurements, and pharmacological modulations. We propose that by developing and using models with direct links to experiment at multiple levels, which at least include cellular and microcircuit, a cycling can be set up and used to help us determine critical mechanisms underlying neurological disease. We illustrate our proposal using our previously developed inhibitory network models in the context of these whole hippocampus preparations and show the importance of having direct links at multiple levels.

  18. Isolation of saint louis encephalitis virus from a horse with neurological disease in Brazil.

    Roberta Rosa

    2013-11-01

    Full Text Available St. Louis encephalitis virus (SLEV is a causative agent of encephalitis in humans in the Western hemisphere. SLEV is a positive-sense RNA virus that belongs to the Flavivirus genus, which includes West Nile encephalitis virus, Japanese encephalitis virus, Dengue virus and other medically important viruses. Recently, we isolated a SLEV strain from the brain of a horse with neurological signs in the countryside of Minas Gerais, Brazil. The SLEV isolation was confirmed by reverse-transcription RT-PCR and sequencing of the E protein gene. Virus identity was also confirmed by indirect immunofluorescence using commercial antibodies against SLEV. To characterize this newly isolated strain in vivo, serial passages in newborn mice were performed and led to hemorrhagic manifestations associated with recruitment of inflammatory cells into the central nervous system of newborns. In summary this is the first isolation of SLEV from a horse with neurological signs in Brazil.

  19. Potential of caveolae in the therapy of cardiovascular and neurological diseases

    Gemma eNavarro; Kjell eFuxe; Dasiel Oscar Borroto-Escuela; Rafael eFranco

    2014-01-01

    Caveolae are membrane micro-domains enriched in cholesterol, sphingolipids and caveolins, which are transmembrane proteins with a hairpin-like structure. Caveolae participate in receptor-mediated trafficking of cell surface receptors and receptor-mediated signaling. Furthermore, caveolae participate in clathrin-independent endocytosis of membrane receptors. On the one hand, caveolins are involved in vascular and cardiac dysfunction. Also, neurological abnormalities in caveolin-1 knockout mice...

  20. An Inside Job: How Endosomal Na+/H+ Exchangers Link to Autism and Neurological Disease

    Kondapalli, Kalyan C.; Hari ePrasad; Rajini eRao

    2014-01-01

    Autism imposes a major impediment to childhood development and a huge emotional and financial burden on society. In recent years, there has been rapidly accumulating genetic evidence that links the eNHE, a subset of Na+/H+ exchangers that localize to intracellular vesicles, to a variety of neurological conditions including autism, attention deficit hyperactivity disorder (ADHD), intellectual disability, and epilepsy. By providing a leak pathway for protons pumped by the V-ATPase, eNHE determi...

  1. Neurology and neurologic practice in China.

    Shi, Fu-Dong; Jia, Jian-Ping

    2011-11-29

    In the wake of dramatic economic success during the past 2 decades, the specialized field of neurology has undergone a significant transformation in China. With an increase in life expectancy, the problems of aging and cognition have grown. Lifestyle alterations have been associated with an epidemiologic transition both in the incidence and etiology of stroke. These changes, together with an array of social issues and institution of health care reform, are creating challenges for practicing neurologists throughout China. Notable problems include overcrowded, decrepit facilities, overloaded physician schedules, deteriorating physician-patient relationships, and an insufficient infrastructure to accommodate patients who need specialized neurologic care. Conversely, with the creation of large and sophisticated neurology centers in many cities across the country, tremendous opportunities exist. Developments in neurologic subspecialties enable delivery of high-quality care. Clinical and translational research based on large patient populations as well as highly sophisticated technologies are emerging in many neurologic centers and pharmaceutical companies. Child neurology and neurorehabilitation will be fast-developing subdisciplines. Given China's extensive population, the growth and progress of its neurology complex, and its ever-improving quality control, it is reasonable to anticipate that Chinese neurologists will contribute notably to unraveling the pathogenic factors causing neurologic diseases and to providing new therapeutic solutions.

  2. Neurological complications and risk factors of cardiopulmonary failure of EV-A71-related hand, foot and mouth disease.

    Long, Lili; Xu, Lin; Xiao, Zhenghui; Hu, Shixiong; Luo, Ruping; Wang, Hua; Lu, Xiulan; Xu, Zhiyue; Yao, Xu; Zhou, Luo; Long, Hongyu; Gong, Jiaoe; Song, Yanmin; Zhao, Li; Luo, Kaiwei; Zhang, Mengqi; Feng, Li; Yang, Liming; Sheng, Xiaoqi; Fan, Xuegong; Xiao, Bo

    2016-03-22

    From 2010 to 2012, large outbreaks of EV-A71-related- hand foot and mouth disease (HFMD) occurred annually in China. Some cases had neurological complications and were closely associated with fatal cardiopulmonary collapse, but not all children with central nervous system (CNS) involvement demonstrated a poor prognosis. To identify which patients and which neurological complications are more likely to progress to cardiopulmonary failure, we retrospectively studied 1,125 paediatric inpatients diagnosed with EV-A71-related HFMD in Hunan province, including 1,017 cases with CNS involvement. These patients were divided into cardiopulmonary failure (976 people) group and group without cardiopulmonary failure (149 people). A logistic regression analysis was used to compare the clinical symptoms, laboratory test results, and neurological complications between these two groups. The most significant risk factors included young age, fever duration ≥3 days, coma, limb weakness, drowsiness and ANS involvement. Patients with brainstem encephalitis and more CNS-involved regions were more likely to progress to cardiopulmonary failure. These findings can help front-line clinicians rapidly and accurately determine patient prognosis, thus rationally distributing the limited medical resources and implementing interventions as early as possible.

  3. Lithium-induced minimal change disease and acute kidney injury

    Parul Tandon; Natalie Wong; Zaltzman, Jeffrey S.

    2015-01-01

    Context: Lithium carbonate is a psychiatric medication commonly used in the treatment of bipolar disorder. It has been implicated in inducing nephrogenic diabetes inspidus, chronic tubulointerstitial nephropathy, and acute tubular necrosis. We describe a case of lithium-induced minimal change disease (MCD) and acute kidney injury (AKI). Case Report: A 32-year-old female with a medical history of bipolar disorder treated with chronic lithium therapy presented with anasarca, fatigue, and tremor...

  4. Perioperative and late outcomes of laparoscopic fundoplication for neurologically impaired children with gastro-esophageal reflux disease

    WONG Kenneth KY; LIU Xue-lai

    2012-01-01

    Background Persistent gastro-esophageal reflux (GER) due to various pathological factors often results in overt clinical symptoms and signs,which is termed as gastro-esophageal reflux disease (GERD).Affected children usually present with failure to thrive,recurrent pneumonia or apnea.Many neurologically impaired children have symptoms related to GERD.Although laparoscopic fundoplication has been established to be an effective treatment modality for children with GERD,data on its role and long-term efficacy for neurologically impaired patients remain sparse.The aim of this study was to review the results of such patients who underwent laparoscopic fundoplication.Methods A retrospective review was performed from 1998 to 2009.All children with neurological impairment who had laparoscopic fundoplication were included.Results Fifty-nine GERD patients (male=32,female=27; mean age 6 years) were identified.All subjects showed symptoms of frequent emesis; 32 of them had history of hematemesis (54.2%); 54 had feeding difficulty; 35 (59.3%) had associated respiratory symptoms,including recurrent pneumonia.Gastrostomy was performed concurrently in 39 cases.There was no conversion to open procedure nor was there intra-operative complications and operative mortality.Emesis or hematemesis was controlled adequately in all.However,respiratory symptoms were not controlled in 10 patients (16.9%),and five of them required further respiratory assistance including nasal airway tube and tracheostomy.Clinical recurrence of GERD was not observed in any subject.Twelve patients died during follow-up (range from 3 months to 9 years) due to severe respiratory complications,cardiac arrest,and brain tumor.Conclusions Laparoscopic fundoplication is an excellent procedure for controlling clinically significant symptoms in neurological impaired patients with GERD.Further studies are required to assess the improvement of the quality of life in such patients.

  5. Elaboration of a clinical and paraclinical score to estimate the probability of herpes simplex virus encephalitis in patients with febrile, acute neurologic impairment.

    Gennai, S; Rallo, A; Keil, D; Seigneurin, A; Germi, R; Epaulard, O

    2016-06-01

    Herpes simplex virus (HSV) encephalitis is associated with a high risk of mortality and sequelae, and early diagnosis and treatment in the emergency department are necessary. However, most patients present with non-specific febrile, acute neurologic impairment; this may lead clinicians to overlook the diagnosis of HSV encephalitis. We aimed to identify which data collected in the first hours in a medical setting were associated with the diagnosis of HSV encephalitis. We conducted a multicenter retrospective case-control study in four French public hospitals from 2007 to 2013. The cases were the adult patients who received a confirmed diagnosis of HSV encephalitis. The controls were all the patients who attended the emergency department of Grenoble hospital with a febrile acute neurologic impairment, without HSV detection by polymerase chain reaction (PCR) in the cerebrospinal fluid (CSF), in 2012 and 2013. A multivariable logistic model was elaborated to estimate factors significantly associated with HSV encephalitis. Finally, an HSV probability score was derived from the logistic model. We identified 36 cases and 103 controls. Factors independently associated with HSV encephalitis were the absence of past neurological history (odds ratio [OR] 6.25 [95 % confidence interval (CI): 2.22-16.7]), the occurrence of seizure (OR 8.09 [95 % CI: 2.73-23.94]), a systolic blood pressure ≥140 mmHg (OR 5.11 [95 % CI: 1.77-14.77]), and a C-reactive protein probability score was calculated summing the value attributed to each independent factor. HSV encephalitis diagnosis may benefit from the use of this score based upon some easily accessible data. However, diagnostic evocation and probabilistic treatment must remain the rule.

  6. Association between acute pancreatitis and peptic ulcer disease

    Kang-Moon Lee; Chang-Nyol Paik; Woo Chul Chung; Jin Mo Yang

    2011-01-01

    AIM:To evaluate the relationship between peptic ulcer disease (PUD) and acute pancreatitis.METHODS:A cohort of 78 patients with acute pancreatitis were included in this study.The presence of PUD and the Helicobacter pylori (H.pylori ) status were assessed by an endoscopic method.The severity of acute pancreatitis was assessed using Ranson's score, the Acute Physiology and Chronic Health Evaluation (APACHE) Ⅱ score,computed tomography severity index and the clinical data during hospitalization,all of which were compared between the patients with and without PUD.The risk factors for PUD were also evaluated. RESULTS:Among 78 patients,41 patients (52.6%) with acute pancreatitis suffered from PUD,but only 13 (31.7%) patients with PUD were infected by H.pylori .On univariate analysis,male gender,an etiology of alcohol-induced pancreatitis,a history of smoking or alcohol consumption, elevated triglyceride and C-reactive protein levels, and high APACHE Ⅱ score were significantly associated with PUD.However,on multivariate logistic regression analysis,the APACHE Ⅱ score (odds ratio:7.69; 95% confidence interval:1.78-33.33; P < 0.01) was found to be the only independent risk factor for PUD.CONCLUSION:Patients with acute pancreatitis are liable to suffer from PUD.PUD is associated with severe acute pancreatitis according to the APACHE Ⅱ score, and treatment for PUD should be considered for patients with severe acute pancreatitis.

  7. Effect of long-term low dose of aspirin on severity of disease following onset of acute cerebral infarction

    Jun Xu; Lili Cao; Xiaomei Deng; Enji Han

    2006-01-01

    BACKGROUND: Aspirin can decrease the incidence risk of high-risk crowdgroup of cerebral infarction, but there are still controversy if it might decrease the degree of disease in degree of patients with acute cerebral infarction.OBJECTIVE: To observe the effect of lower dose of aspirin during taking for a long time on disease degree of disease following onset of acute cerebral infarction.DESIGN: Grouping according to the admission time and 1:1 paired observation.SETTING: Department of Neurology, Qilu Hospital of Shandong University.PARTICIPANTS: The participants in present study were 321 patients with acute cerebral infarction who received treatments in the Department of Neurology, Qilu Hospital of Shandong University from January 1999 to June 2000. There were 190 male and 131 female ,with mean (65±11 )years of age. Inclusive criteria: ① A focal neurological disturbance occurred suddenly and had lasted for more than 24 hours, patients were admitted within 3 days after onset of disease; ② A computed tomography of the brain was performed and excluded hemorrhage in all patients; ③ The patients were proved internal carotid occlusions by clinical features and image findings; ④ The functions of limbs were normal (before the first stroke) or almost normal (before the second stroke). Exclusive criteria: ①The patients who had have cardiogenic cerebral embolism; ②The patients who had taken warfarin orally and other platelet agglutination drugs.METHODS: ①All the patients were divided into 2 groups according to whether they had taken aspirin before: aspirin-treated group (n=110) and blank control group (n=211). There were 70 male and 40 female in aspirin-treated group, with average(65±10) years of age.All patients had taken 50-100 mg/d aspirin for 6 months to 10 years before onset. There were 120 male and 91 female in blank control group, with average (65±13)years of age. Patients received a clinical scoring within 3 days and similar therapeutic measures (such

  8. A case of neuro-Behcet's disease: comparison of neurological symptoms with PET, SPECT, and MRI findings

    Kim, Jin Wook; An, Min; Kim, So Yon; Kim, Young Jung; Cho, Min Koo; Lee, Gwon Jun; Lim, Sang Mun; Hong, Sung Woon; Choi, Chang Woon [Korea Cancer Center, Seoul (Korea, Republic of)

    1998-08-01

    We describe a 27-year-old man who developed gait disturbance and dysarthria 2 years after the onset of cardinal symptoms of Behect's disease. Position emission tomography with {sup 18}F-fluorodeoxyglucose revealed severe hypometabolism in the cerebellum, in accordance with cerebellar symptoms and signs of the patients. However, single-photon emission tomography with {sup 99m}Tc-HMPAO and {sup 99m}Tc-ECD did not disclose significant perfusion abnormalities in the brain. Routine brain magnetic resonance imaging did not show signal abnormalities. The findings of imaging studies compared with neurological manifestations of the patient are discussed.

  9. Follow-up nationwide survey on predictive genetic testing for late-onset hereditary neurological diseases in Japan.

    Tanaka, Keiko; Sekijima, Yoshiki; Yoshida, Kunihiro; Tamai, Mariko; Kosho, Tomoki; Sakurai, Akihiro; Wakui, Keiko; Ikeda, Shu-ichi; Fukushima, Yoshimitsu

    2013-08-01

    A follow-up nationwide survey on predictive genetic testing for late-onset neurological diseases in Japan was conducted. A questionnaire was sent to 89 institutional members of the Japan's National Liaison Council for Clinical Sections of Medical Genetics, and was returned by 60 (67.4%). A total of 301 clients with an interest in predictive testing were accumulated from April 2006 to March 2011. The greatest interest was shown for spinocerebellar degeneration (SCD, n=110), followed by myotonic dystrophy type 1 (DM1, n=69), Huntington's disease (HD, n=52) and familial amyloid polyneuropathy (FAP, n=35). The ratios of clients who actually underwent predictive testing were: SCD, 21.8%; DM1, 39.1%; HD, 26.9%; and FAP, 74.3%, indicating that predictive testing was conducted very cautiously for untreatable neurological diseases in Japan. Clinical geneticists were predominantly involved in genetic counseling, whereas the participation of non-medical doctor (non-MD) staff, including nurses, clinical psychologists and genetic counselors, was not common. Lack of non-MD counseling staff was one of the most serious issues in conducting predictive testing, which has not been improved since the previous survey performed in 2006. Institutional arrangements, such as revision of medical insurance system regarding genetic testing and counseling, might be necessary to resolve this issue.

  10. Headache and focal neurologic signs following exposure to spicy aroma as an initial presentation of moyamoya disease

    Bilal A. Siddiqui, A.B.

    2014-12-01

    Full Text Available Moyamoya disease is a condition of the cerebrovascular system that involves stenosis of the intracranial internal carotid arteries as well as their proximal branches, often leading to stroke in affected patients. Here we describe the case of a patient with headache and focal neurologic signs following exposure to a spicy aroma, who initially had a negative vascular work-up and a preliminary diagnosis of a complex migraine syndrome. She subsequently developed infarction of the left frontal lobe, and imaging studies revealed the diagnosis of moyamoya disease. She was treated with an encephalodurosynangiosis procedure, with notable improvement. This case highlights the importance of considering moyamoya disease in the differential diagnosis of patients presenting with headaches with aura.

  11. The acute haemolytic syndrome in Wilson's disease--a review of 22 patients.

    Walshe, J M

    2013-11-01

    An analysis of 321 case notes of patients with Wilson's disease seen between 1955 and 2000 and one case seen in 1949 has revealed that 22 patients presented with a haemolytic crisis. This study was not a specific research project but a retrospective analysis of 321 patients with Wilson's disease seen between 1949 and 2000. All investigations were carried out in the best interests of diagnosis and management of patients referred to my clinic. The delay in diagnosis in 18 cases resulted in progression to severe hepatic disease in 14 cases and to neurological disease in 4 cases. One patient had no symptoms at the time her sister's illness was diagnosed as Wilson's disease. In a second patient, with liver disease, the diagnosis was also made when a sister was found to have Wilson's disease. There was a female to male ratio of 15:7. The average age of onset was 12.6 years and the incidence 6.9%. Delay in diagnosis resulted in nine deaths. Three patients, late in the series, were admitted in the acute phase, two female and one male; of these two responded to chelation therapy, the third required liver transplantation. Haemolysis appeared to be extravascular, and possible mechanisms of the haemolysis are discussed.

  12. Neurology and international organizations.

    Mateen, Farrah J

    2013-07-23

    A growing number of international stakeholders are engaged with neurologic diseases. This article provides a brief overview of important international stakeholders in the practice of neurology, including global disease-specific programs, United Nations agencies, governmental agencies with international influence, nongovernmental organizations, international professional organizations, large private donors, private-public partnerships, commercial interests, armed forces, and universities and colleges. The continued engagement of neurologists is essential for the growing number of international organizations that can and should incorporate neurologic disease into their global agendas.

  13. Nested PCR for Rapid Detection of Mumps Virus in Cerebrospinal Fluid from Patients with Neurological Diseases

    Poggio, Gustavo Palacios; Rodriguez, Claudia; Cisterna, Daniel; Freire, María Cecilia; Cello, Jerónimo

    2000-01-01

    In this study, we have developed a reverse transcription (RT)-nested polymerase chain reaction (n-PCR) for the detection of mumps virus RNA in cerebrospinal fluid (CSF) from patients with neurological infections. A specific 112-bp fragment was amplified by this method with primers from the nucleoprotein of the mumps virus genome. The mumps virus RT–n-PCR was capable of detecting 0.001 PFU/ml and 0.005 50% tissue culture infective dose/ml. This method was found to be specific, since no PCR pro...

  14. Acute rheumatic fever and rheumatic heart disease in indigenous populations.

    Steer, Andrew C; Carapetis, Jonathan R

    2009-12-01

    Acute rheumatic fever and rheumatic heart disease are diseases of socioeconomic disadvantage. These diseases are common in developing countries and in Indigenous populations in industrialized countries. Clinicians who work with Indigenous populations need to maintain a high index of suspicion for the potential diagnosis of acute rheumatic fever, particularly in patients presenting with joint pain. Inexpensive medicines, such as aspirin, are the mainstay of symptomatic treatment of rheumatic fever; however, antiinflammatory treatment has no effect on the long-term rate of progression or severity of chronic valvular disease. The current focus of global efforts at prevention of rheumatic heart disease is on secondary prevention (regular administration of penicillin to prevent recurrent rheumatic fever), although primary prevention (timely treatment of streptococcal pharyngitis to prevent rheumatic fever) is also important in populations in which it is feasible.

  15. Progressive neurological disease induced by tacrolimus in a renal transplant recipient: Case presentation

    Hanna Michael G

    2006-03-01

    Full Text Available Abstract Background Tacrolimus and cyclosporine, both calcineurin inhibitors, can cause neurological side effects. While mild symptoms such as tremor are well recognised, severe complications including seizures and encephalopathy are poorly documented following renal transplantation. Case presentation We report a 42 year old man who received a cadaver renal transplant. He received tacrolimus and prednisolone. The course was uneventful for 6 weeks when he became intermittently confused, with unsteady gait and slurred speech. Following a grand mal convulsion he was admitted. He had no focal neurological signs, cerebrospinal fluid was normal; electroencephalogram was consistent with temporal lobe partial epilepsy. The magnetic resonance imaging of brain showed widespread changes with multiple areas of low signal intensity in brain stem and cerebral hemispheres. He was readmitted 3 weeks later after further fits, despite anti-convulsant therapy. He was psychotic with visual hallucinations, and rapidly became obtunded. Although his tacrolimus blood concentration had been kept in the normal range, his symptoms improved dramatically when the tacrolimus was stopped. Conclusion Severe central nervous system toxicity from calcineurin inhibitors has been rarely reported in renal transplantation and we found only one report of tacrolimus-induced toxicity in an adult. We believe the condition is frequently undiagnosed. It is a very important diagnosis not to miss as the remedy is simple and failure may result in unnecessary brain biopsy, as well as irreversible injury.

  16. Cerebral Amyloidal Angiopathy--a disease with implications for neurology and psychiatry.

    Gahr, Maximilian; Nowak, Dennis A; Connemann, Bernhard J; Schönfeldt-Lecuona, Carlos

    2013-06-26

    Cerebral Amyloidal Angiopathy (CAA), which occurs sporadically in most cases but can also occur hereditarily, belongs to the group amyloidoses and is characterized by the deposition and accumulation of beta-amyloid (Aβ) in smaller arterial vessels of the brain. The deposition of Aβ leads to degenerative changes in the cerebral vessel system (thickening of the vessel wall, microaneurysm, constriction of vascular lumen, dissection), which favour the development of the clinical symptomatology most often associated with CAA. Besides haemorrhages, cerebral ischaemia, transient neurological symptoms, leukoencephalopathy as well as cognitive decline and even dementia may appear in connection with CAA. A definite diagnosis of CAA can only be made on the basis of a pathological assessment, even though diagnostic findings of cerebral neuroimaging and clinical symptoms allow the diagnosis of a probable CAA. At present, no causal therapy options are available. Although CAA is placed within the range of neurological illnesses, psychiatric symptoms such as cognitive impairment, personality change or behavioural problems as well as depression are plausible clinical manifestations of CAA and may even dominate the clinical picture. Apart from epidemiological, pathogenetical, clinical and diagnostical aspects, possible psychiatric implications of CAA are discussed in the review article.

  17. A new clinical tool for assessing numerical abilities in neurological diseases: Numerical Activities of Daily Living

    Carlo eSemenza

    2014-06-01

    Full Text Available The aim of this study was to build an instrument, the Numerical Activities of Daily Living (NADL, designed to identify the specific impairments in numerical functions that may cause problems in everyday life. These impairments go beyond what can be inferred from the available scales evaluating activities of daily living in general, and are not adequately captured by measures of the general deterioration of cognitive functions as assessed by standard clinical instruments like the MMSE and MoCA. We assessed a control group (n = 148 and a patient group affected by a wide variety of neurological conditions (n = 175, with NADL along with IADL, MMSE, and MoCA. The NADL battery was found to have satisfactory construct validity and reliability, across a wide age range. This enabled us to calculate appropriate criteria for impairment that took into account age and education. It was found that neurological patients tended to overestimate their abilities as compared to the judgment made by their caregivers, assessed with objective tests of numerical abilities.

  18. An Inside Job: How Endosomal Na+/H+ Exchangers Link to Autism and Neurological Disease

    Kalyan C. Kondapalli

    2014-06-01

    Full Text Available Autism imposes a major impediment to childhood development and a huge emotional and financial burden on society. In recent years, there has been rapidly accumulating genetic evidence that links the eNHE, a subset of Na+/H+ exchangers that localize to intracellular vesicles, to a variety of neurological conditions including autism, attention deficit hyperactivity disorder, intellectual disability and epilepsy. By providing a leak pathway for protons pumped by the V-ATPase, eNHE determine luminal pH and regulate cation (Na+, K+ content in early and recycling endosomal compartments. Loss-of-function mutations in eNHE cause hyperacidification of endosomal lumen, as a result of imbalance in pump and leak pathways. Two isoforms, NHE6 and NHE9 are highly expressed in brain, including hippocampus and cortex. Here, we summarize evidence for the importance of luminal cation content and pH on processing, delivery and fate of cargo and on the surface expression and function of membrane receptors and neurotransmitter transporters, drawing upon insights from model organisms and mammalian cells. These studies lead to cellular models of eNHE activity in pre- and post-synaptic neurons and astrocytes, where they could impact synapse development and plasticity. The study of eNHE has provided new insight on the mechanism of autism and other debilitating neurological disorders and opened up new possibilities for therapeutic intervention.

  19. An inside job: how endosomal Na(+)/H(+) exchangers link to autism and neurological disease.

    Kondapalli, Kalyan C; Prasad, Hari; Rao, Rajini

    2014-01-01

    Autism imposes a major impediment to childhood development and a huge emotional and financial burden on society. In recent years, there has been rapidly accumulating genetic evidence that links the eNHE, a subset of Na(+)/H(+) exchangers that localize to intracellular vesicles, to a variety of neurological conditions including autism, attention deficit hyperactivity disorder (ADHD), intellectual disability, and epilepsy. By providing a leak pathway for protons pumped by the V-ATPase, eNHE determine luminal pH and regulate cation (Na(+), K(+)) content in early and recycling endosomal compartments. Loss-of-function mutations in eNHE cause hyperacidification of endosomal lumen, as a result of imbalance in pump and leak pathways. Two isoforms, NHE6 and NHE9 are highly expressed in brain, including hippocampus and cortex. Here, we summarize evidence for the importance of luminal cation content and pH on processing, delivery and fate of cargo. Drawing upon insights from model organisms and mammalian cells we show how eNHE affect surface expression and function of membrane receptors and neurotransmitter transporters. These studies lead to cellular models of eNHE activity in pre- and post-synaptic neurons and astrocytes, where they could impact synapse development and plasticity. The study of eNHE has provided new insight on the mechanism of autism and other debilitating neurological disorders and opened up new possibilities for therapeutic intervention.

  20. [Palliative care in neurology].

    Provinciali, Leandro; Tarquini, Daniela; De Falco, Fabrizio A; Carlini, Giulia; Zappia, Mario; Toni, Danilo

    2015-07-01

    Palliative care in neurology is characterized by the need of taking into account some distinguishing features which supplement and often differ from the general palliative approach to cancer or to severe organ failures. Such position is emphasized by a new concept of palliative assistance which is not limited to the "end of life" stage, as it was the traditional one, but is applied along the entire course of progressive, life-limiting, and disabling conditions. There are various reasons accounting for a differentiation of palliative care in neurology and for the development of specific expertise; the long duration of the advanced stages of many neurological diseases and the distinguishing features of some clinical problems (cognitive disorders, psychic disorders, etc.), in addition to the deterioration of some general aspects (nutrition, etc.), make the general criteria adopted for cancer, severe respiratory, hepatic or renal failures and heart failure inadequate. The neurological diseases which could benefit from the development of a specific palliative approach are dementia, cerebrovascular diseases, movement disorders, neuromuscular diseases, severe traumatic brain injury, brain cancers and multiple sclerosis, as well as less frequent conditions. The growing literature on palliative care in neurology provides evidence of the neurological community's increasing interest in taking care of the advanced and terminal stages of nervous system diseases, thus encouraging research, training and updating in such direction. This document aims to underline the specific neurological requirements concerning the palliative assistance.

  1. Acute tubulointerstitial nephritis complicating Legionnaires' disease: a case report

    Daumas Aurélie

    2012-04-01

    Full Text Available Abstract Introduction Legionnaires' disease is recognized as a multi-systemic illness. Afflicted patients may have pulmonary, renal, gastrointestinal tract and central nervous system complications. However, renal insufficiency is uncommon. The spectrum of renal involvement may range from a mild and transient elevation of serum creatinine levels to anuric renal failure requiring dialysis and may be linked to several causes. In our present case report, we would like to draw attention to the importance of the pathological documentation of acute renal failure by reporting a case of a patient with acute tubulointerstitial nephritis complicating Legionnaires' disease. Case presentation A 55-year-old Caucasian man was admitted to our hospital for community-acquired pneumonia complicated by acute renal failure. Legionella pneumophila serogroup type 1 was diagnosed. Although the patient's respiratory illness responded to intravenous erythromycin and ofloxacin therapy, his renal failure worsened, he became anuric, and hemodialysis was started. A renal biopsy was performed, which revealed severe tubulointerstitial nephritis. After initiation of steroid therapy, his renal function improved dramatically. Conclusions This case highlights the importance of kidney biopsies in cases where acute renal failure is a complicating factor in Legionnaires' disease. If the presence of acute tubulointerstitial nephritis can be confirmed, it will likely respond favorably to steroidal treatment and thus irreversible renal damage and chronic renal failure will be avoided.

  2. Acute Diarrhoeal Diseases Among Preschool Children in Western Maharashtra, India.

    Mahesh B Tondare , Vaishali V Raje, Satish V Kakade , Madhavi V Rayate

    2014-01-01

    Full Text Available "Background: Malnutrition and infectious diseases both occur in the same unfortunate children and together they play a major role in causing the high morbidity and mortality in them. Out of all the childhood illnesses, acute respiratory tract infections, diarrhoeal diseases and malnutrition are the principle causes of illness and death in the developing countries. Acute Diarrhoeal diseases (ADD’s are reported to be the 2nd leading cause of child morbidity and mortality. Objectives: To study the attack rate of Acute Diarrhoeal Disease among pre-school children and to study the socio-demographic variables of pre-school children suffering from Acute Diarrhoeal Disease. Methods: A Longitudinal study was conducted among preschool children (3-5years who were selected from Private pre-primary school of urban area and followed for the period of one year. Mother/guardian/teacher was interviewed by using pre-tested proforma during this period. Results: About 56% of children found suffering from ADD with 0.6 episodes per children per year among private pre-primary school. Higher proportions of ADD affected children were residing in nuclear type of family, belonging to middle socio-economic class, mothers were literate & housewives, born with order >2 compared to non ADD affected children. Conclusion: Maximum number of children from private pre-primary schools suffered with nearly one attack of Acute Diarrhoeal Disease with maternal illiteracy and working mothers found favorable factors. Immunization coverage, EBF and proper weaning play a very important role in prevention of infections."

  3. Acute Rheumatic Fever: Global Persistence of a Preventable Disease.

    Bono-Neri, Francine

    2016-10-21

    The persistence of acute rheumatic fever continues to be seen globally. Once thought to be eradicated in various parts of the world, the disease came back with a vengeance secondary to a lack of diligence on the part of providers. Today, the global burden of group A streptococcal infection, the culprit of the numerous sequelae manifested in acute rheumatic fever, is considerable. Although a completely preventable disease, rheumatic fever continues to exist. It is a devastating disease that involves long-term, multisystem treatment and monitoring for patients who were unsuccessful at eradicating the precipitating group A streptococcal infection. Prevention is the key to resolving the dilemma of the disease's global burden, yet the method to yield its prevention still remains unknown. Thus, meticulous attention to implementing proper treatment is the mainstay and remains a top priority.

  4. Postpartum Acute Pulmonary Oedema with Sub clinical Rheumatic Heart Disease

    2015-01-01

    Acute dyspnea with pulmonary oedema in postpartum is uncommon but life-threatening event. Contributing factors for pulmonary oedema include, administration of tocolytics, underlying cardiac disease, iatrogenic fluid overload and preeclampsia acounting 0.08% of pregnancies. Pulmonary embolism, amniotic fluid embolism, pneumonia, aspiration and pulmonary oedema are some of the potentially devastating conditions that should be considered by the attending physician.

  5. Acute type II cryoglobulinaemic vasculitis mimicking atherosclerotic peripheral vascular disease.

    Saeed, A

    2012-01-31

    Atherosclerotic peripheral vascular disease is a common presenting cause for digital ischaemia in life long smokers. Acute severe Type II Cryoglobulinaemic vasculitis is a rare yet important cause, which may present with similar clinical features and which if undiagnosed may be rapidly fatal. Following the instigation of therapy with intravenous methylprednisolone and cyclophosphamide this patient made an excellent recovery.

  6. [Acute atrioventricular block in chronic Lyme disease].

    Wagner, Vince; Zima, Endre; Gellér, László; Merkely, Béla

    2010-09-26

    The tick bite transmitted Lyme disease is one of the most common antropozoonosis, about 10 000 new infections are reported in Hungary each year. The progress and clinical presentation can vary, and carditis can occur in later stages. A serologically verified Lyme disease caused third degree atrioventricular block in young male presenting with presyncope. Based on the tick-bites mentioned a few weeks prior to hospital admission, Lyme carditis was considered with the administration of antibiotics and monitor observation. Typical skin lesions were not recognized and laboratory findings showed no pathology. An electrophysiological study recorded a predominant supra-His atrioventricular block. Total regression of conduction could be detected later and the serological tests established an underlying Lyme disease. Currently no definite treatment recommendation is available for the potentially reversible Lyme carditis. The tick bite seemed to be the key on our way to diagnosis; however, serological tests proved the disease to be older than one year. A detailed medical history and serological tests are essential in identifying the cause and pacemaker implantation can be avoided.

  7. Difficulties with neurological prognostication in a young woman with delayed-onset generalised status myoclonus after cardiac arrest due to acute severe asthma

    Arvind Rajamani

    2011-01-01

    Full Text Available Neurological prognostication in cardiac arrest survivors is difficult, especially when the primary etiology is respiratory arrest. Prognostic factors designed to have zero false-positive rates to robustly confirm poor outcome are usually inadequate to rule out poor outcomes (i.e., high specificity and low sensitivity. One of the least understood prognosticators is generalised status myoclonus (GSM, with case reports confusing GSM, isolated myoclonic jerks and post-hypoxic intention myoclonus (Lance Adams syndrome [LAS]. With several prognostic indicators (including status myoclonus having been validated in the pre-hypothermia era, their current relevance is debatable. New modalities such as brain magnetic resonance imaging (MRI and continuous electroencephalography are being evaluated. We describe here a pregnant woman resuscitated from a cardiac arrest due to acute severe asthma, and an inability to reach a consensus based on published guidelines, with a brief overview of myoclonus, LAS and the role of MRI brain in assisting prognostication.

  8. [Acute epidural hematoma of the posterior fossa in a case of von Willebrand's disease].

    Takenaka, N; Mine, T; Ikeda, E; Iwai, H; Kusano, S

    1988-01-01

    A rare case of acute epidural hematoma of the posterior fossa associated with von Willebrand's disease is reported. A 9-year-old boy fell down and hit his occipital region against a floor. Soon after he came home and slept, but three hours later he began to vomit and became drowsiness. He visited our hospital and his Glasgow Coma Scale showed 13 points. CT scan on admission showed acute epidural hematoma of left posterior fossa and contusional hematoma in the right temporal lobe. The bleeding time was over 18 minutes. He had been suspected to be suffering from von Willebrand's disease two years ago. Then fresh blood, fresh frozen plasma and anti-hemophilic globulin were prepared. Ten hours after injury, the operation was begun. Fresh epidural hematoma existed as a clot beyond transverse sinus. During the procedure of dural tenting suture, diffuse bleeding from bone, muscle, subcutaneous tissue and dura occurred and it was difficult to stop the bleeding. By using fresh blood and anti-hemophilic globulin, the bleeding was controlled, and then the operation was achieved. In the postoperative course a new epidural hematoma was found in the left temporal region and a new but asymptomatic retinal hemorrhage was found in his right eye. He was discharged without any neurological deficits 25 days after operation.

  9. Cerebrospinal fluid and serum cytokine profiling to detect immune control of infectious and inflammatory neurological and psychiatric diseases.

    Maxeiner, Horst-Guenter; Marion Schneider, E; Kurfiss, Sina-Tatjana; Brettschneider, Johannes; Tumani, Hayrettin; Bechter, Karl

    2014-09-01

    The present study aimed at profiling inflammatory cytokines for neurological and psychiatric diseases. A total of 86 patients with meningitis, multiple sclerosis, tension-type headache, idiopathic facial nerve palsy (IFNP), affective and schizophrenic disorders were tested for both, serum and cerebrospinal fluid (CSF) using a multiplexed cytokine ELISA for IFN-γ, TNF-α, IL-1β, IL-2, IL-4, IL-5, IL-8/CXCL8, IL-10, IL12p70, IL-13 and IL-17. Cases with viral and bacterial meningitis had unequivocally higher cytokine concentrations in the CSF when compared with serum. Bacterial meningitis was unique by extremely elevated IL-17, TNF-α and IL-1β, indicating a plethora of inflammatory pathways, selectively activated in the CSF. In relapsing multiple sclerosis, IFN-γ and IL-10 were elevated in both, serum and CSF, but IL-12p70, IL-5, IL-13, and TNF-α were more prominent in serum than in CSF. Qualitatively similar biomarker patterns were detected in patients with idiopathic facial nerve palsy and tension-type cephalgia. Affective and schizophrenic disorders clearly present with an inflammatory phenotype in the CSF and also serum, the cytokines determined were in general higher in schizophrenia. Except IFN-γ, schizophrenic patients had higher IL-12p70 and a trend of higher IL-10 and IL-13 in serum suggesting a more prominent TH2-type counter regulatory immune response than in affective disorders. These differences were also mirrored in the CSF. Elevated IL-8 appears to be the most sensitive marker for inflammation in the CSF of all diseases studied, whereas TNF-α was restricted to peripheral blood. With the exception of IL-8, all but viral and bacterial meningitis, studied, displayed higher means of elevated lymphokine concentrations in the serum than in the CSF. This observation supports the concept of immunological crosstalk between periphery and intrathecal immunity in neurological and psychiatric diseases.

  10. The role of nanotechnology and nano and micro-electronics in monitoring and control of cardiovascular diseases and neurological disorders

    Varadan, Vijay K.

    2007-04-01

    Nanotechnology has been broadly defined as the one for not only the creation of functional materials and devices as well as systems through control of matter at the scale of 1-100 nm, but also the exploitation of novel properties and phenomena at the same scale. Growing needs in the point-of-care (POC) that is an increasing market for improving patient's quality of life, are driving the development of nanotechnologies for diagnosis and treatment of various life threatening diseases. This paper addresses the recent development of nanodiagnostic sensors and nanotherapeutic devices with functionalized carbon nanotube and/or nanowire on a flexible organic thin film electronics to monitor and control of the three leading diseases namely 1) neurodegenerative diseases, 2) cardiovascular diseases, and 3) diabetes and metabolic diseases. The sensors developed include implantable and biocompatible devices, light weight wearable devices in wrist-watches, hats, shoes and clothes. The nanotherapeutics devices include nanobased drug delivery system. Many of these sensors are integrated with the wireless systems for the remote physiological monitoring. The author's research team has also developed a wireless neural probe using nanowires and nanotubes for monitoring and control of Parkinson's disease. Light weight and compact EEG, EOG and EMG monitoring system in a hat developed is capable of monitoring real time epileptic patients and patients with neurological and movement disorders using the Internet and cellular network. Physicians could be able to monitor these signals in realtime using portable computers or cell phones and will give early warning signal if these signals cross a pre-determined threshold level. In addition the potential impact of nanotechnology for applications in medicine is that, the devices can be designed to interact with cells and tissues at the molecular level, which allows high degree of functionality. Devices engineered at nanometer scale imply a

  11. Virtual reality interface devices in the reorganization of neural networks in the brain of patients with neurological diseases.

    Gatica-Rojas, Valeska; Méndez-Rebolledo, Guillermo

    2014-04-15

    Two key characteristics of all virtual reality applications are interaction and immersion. Systemic interaction is achieved through a variety of multisensory channels (hearing, sight, touch, and smell), permitting the user to interact with the virtual world in real time. Immersion is the degree to which a person can feel wrapped in the virtual world through a defined interface. Virtual reality interface devices such as the Nintendo® Wii and its peripheral nunchuks-balance board, head mounted displays and joystick allow interaction and immersion in unreal environments created from computer software. Virtual environments are highly interactive, generating great activation of visual, vestibular and proprioceptive systems during the execution of a video game. In addition, they are entertaining and safe for the user. Recently, incorporating therapeutic purposes in virtual reality interface devices has allowed them to be used for the rehabilitation of neurological patients, e.g., balance training in older adults and dynamic stability in healthy participants. The improvements observed in neurological diseases (chronic stroke and cerebral palsy) have been shown by changes in the reorganization of neural networks in patients' brain, along with better hand function and other skills, contributing to their quality of life. The data generated by such studies could substantially contribute to physical rehabilitation strategies.

  12. Circulating cell-free microRNA as biomarkers for screening, diagnosis and monitoring of neurodegenerative diseases and other neurologic pathologies

    Sheinerman, Kira S.; Umansky, Samuil R.

    2013-01-01

    Many neurodegenerative diseases, such as Alzheimer's disease, Parkinson disease, vascular and frontotemporal dementias, as well as other chronic neurological pathologies, are characterized by slow development with a long asymptomatic period followed by a stage with mild clinical symptoms. As a consequence, these serious pathologies are diagnosed late in the course of a disease, when massive death of neurons has already occurred and effective therapeutic intervention is problematic. Thus, the ...

  13. Moyamoya disease in a child with previous acute necrotizing encephalopathy

    Kim, Taik-Kun; Cha, Sang Hoon; Chung, Kyoo Byung; Kim, Jung Hyuck; Kim, Baek Hyun; Chung, Hwan Hoon [Department of Diagnostic Radiology, Korea University College of Medicine, Ansan Hospital, 516 Kojan-Dong, Ansan City, Kyungki-Do 425-020 (Korea); Eun, Baik-Lin [Department of Pediatrics, Korea University College of Medicine, Seoul (Korea)

    2003-09-01

    A previously healthy 24-day-old boy presented with a 2-day history of fever and had a convulsion on the day of admission. MRI showed abnormal signal in the thalami, caudate nuclei and central white matter. Acute necrotising encephalopathy was diagnosed, other causes having been excluded after biochemical and haematological analysis of blood, urine and CSF. He recovered, but with spastic quadriparesis. At the age of 28 months, he suffered sudden deterioration of consciousness and motor weakness of his right limbs. MRI was consistent with an acute cerebrovascular accident. Angiography showed bilateral middle cerebral artery stenosis or frank occlusion with numerous lenticulostriate collateral vessels consistent with moyamoya disease. (orig.)

  14. An Acoustic Study of the Relationships among Neurologic Disease, Dysarthria Type, and Severity of Dysarthria

    Kim, Yunjung; Kent, Raymond D.; Weismer, Gary

    2011-01-01

    Purpose: This study examined acoustic predictors of speech intelligibility in speakers with several types of dysarthria secondary to different diseases and conducted classification analysis solely by acoustic measures according to 3 variables (disease, speech severity, and dysarthria type). Method: Speech recordings from 107 speakers with…

  15. Progress in pediatrics in 2013: choices in allergology, endocrinology, gastroenterology, hypertension, infectious diseases, neonatology, neurology, nutrition and respiratory tract illnesses.

    Caffarelli, Carlo; Santamaria, Francesca; Vottero, Alessandra; Dascola, Carlotta Povesi; Mirra, Virginia; Sperli, Francesco; Bernasconi, Sergio

    2014-07-12

    This review will provide new information related to pathophysiology and management of specific diseases that have been addressed by selected articles published in the Italian Journal of Pediatrics in 2013, focusing on allergology, endocrinology, gastroenterology, hypertension, infectious diseases, neonatology, neurology, nutrition and respiratory tract illnesses in children. Recommendations for interpretation of skin prick test to foods in atopic eczema, management of allergic conjunctivitis, hypertension and breastfeeding in women treated with antiepileptic drugs and healthy breakfast have been reported. Epidemiological studies have given emphasis to high incidence of autoimmune disorders in patients with Turner syndrome, increasing prevalence of celiac disease, frequency of hypertension in adolescents, incidence and risk factor for retinopathy of prematurity. Advances in prevention include elucidation of the role of probiotics in reducing occurrence of allergies and feeding intolerance, and events of foetal life that influence later onset of diseases. Mechanistic studies suggested a role for vitamin D deficiency in asthma and type 1 diabetes and for reactivation of Varicella-Zoster virus in aseptic meningitis. Regarding diagnosis, a new mean for the diagnosis of hyperbilirubinaemia in newborns, a score for recognition of impaired nutritional status and growth and criteria for early Dyke-Davidoff-Masson Syndrome have been suggested. New therapeutic approaches consist of use of etanercept for reducing insulin dose in type 1 diabetes, probiotics in atopic eczema, and melatonin in viral infections.

  16. Demyelinizing neurological disease after treatment with tumor necrosis factor alpha-inhibiting agents in a rheumatological outpatient clinic

    Theibich, Ali; Dreyer, Lene; Magyari, Melinda;

    2014-01-01

    Biological treatment with inhibitors of the pro-inflammatory cytokine TNF-alpha has dramatically improved the disease course of several chronic rheumatologic conditions. Adverse events (AEs) are primarily infections and hypersensitivity reactions. Demyelinizing neurological symptoms resembling...... multiple sclerosis (MS) have been described as a rare AE. During about 10-year use of anti TNF-alpha, the Danish Medicines Agency has recorded eight cases of MS like AEs. The objective of this study was to estimate the incidence of demyelinizing AEs both in the central and peripheral nervous system after...... treatment with anti TNF-alpha in a cohort of patients from a large rheumatologic outpatient clinic in Copenhagen. In a 4-year period from January 2008 to December 2011, approximately 550 patients annually were undergoing treatment with anti TNF-alpha inhibitors in our department. We collected data on all...

  17. Clinical effects of comprehensive therapy of early psychological intervention and rehabilitation training on neurological rehabilitation of patients with acute stroke

    Duo-Yu Wu; Min Guo; Yun-Suo Gao; Yan-Hai Kang; Jun-Cheng Guo; Xiang-Ling Jiang; Feng Chen; Tao Liu

    2012-01-01

    Objective: To evaluate the clinical effects of comprehensive therapy of psychological intervention and rehabilitation training on the mental health of the patients with acute stroke. Methods: A total of 120 patients with acute stroke were randomly divided into trial group and control group. Both groups were given the corresponding drug therapy, medical basic nursing and convention nursing. Besides, psychological intervention and comprehensive rehabilitation training were added to the trial group. SCL-90, Europ stroke scales (ESS) score were assessed with each patient on day 3 for the first time and on day 21 for the second time;Barthel index was assessed on the day 90. Results: After psychological intervention, SCL-90 declined significantly in the trial group comparing with the control group, there were signicant differences in the somatization, obsession, depression, anxiety, fear, ESS score, Barthel index and other psychological factors between the trial group and control group (P<0.05). Conclusions:Comprehensive therapy of early psychological intervention and rehabilitation training can significantly improve the mental health, limb movement function, stress ability and activity of daily living on the patients with acute stroke.

  18. Perspectives on cognitive domains, H3 receptor ligands and neurological disease.

    Hancock, Arthur A; Fox, Gerard B

    2004-10-01

    Histamine H(3) receptor agonists and antagonists have been evaluated in numerous in vitro and in vivo animal models to better understand how H(3) receptors modulate neurotransmitter function in the central nervous system. Likewise, behavioural models have explored the hypothesis that changes in neurotransmitter release could enhance cognitive function in human diseases. This review examines the reported effects of H(3) receptor ligands and how they influence cognitive behaviour. These data are interpreted on the basis of different cognitive domains that are relevant to neuropsychiatric diseases. Because of the diversity of H(3) receptors, their function and their influence on neurotransmitter systems, considerable promise exists for H(3) ligands to treat diseases in which aspects of learning and memory are impaired. However, because of the complexities of the histaminergic system and H(3) receptors and the lack of clinical data so far, proof of principle for use in human disease remains to be established.

  19. Recent developments in epigenetics of acute and chronic kidney diseases.

    Reddy, Marpadga A; Natarajan, Rama

    2015-08-01

    The growing epidemic of obesity and diabetes, the aging population as well as prevalence of drug abuse has led to significant increases in the rates of the closely associated acute and chronic kidney diseases, including diabetic nephropathy. Furthermore, evidence shows that parental behavior and diet can affect the phenotype of subsequent generations via epigenetic transmission mechanisms. These data suggest a strong influence of the environment on disease susceptibility and that, apart from genetic susceptibility, epigenetic mechanisms need to be evaluated to gain critical new information about kidney diseases. Epigenetics is the study of processes that control gene expression and phenotype without alterations in the underlying DNA sequence. Epigenetic modifications, including cytosine DNA methylation and covalent post-translational modifications of histones in chromatin, are part of the epigenome, the interface between the stable genome and the variable environment. This dynamic epigenetic layer responds to external environmental cues to influence the expression of genes associated with disease states. The field of epigenetics has seen remarkable growth in the past few years with significant advances in basic biology, contributions to human disease, as well as epigenomics technologies. Further understanding of how the renal cell epigenome is altered by metabolic and other stimuli can yield novel new insights into the pathogenesis of kidney diseases. In this review, we have discussed the current knowledge on the role of epigenetic mechanisms (primarily DNAme and histone modifications) in acute and chronic kidney diseases, and their translational potential to identify much needed new therapies.

  20. Factors influencing cerebrospinal fluid and plasma HIV-1 RNA detection rate in patients with and without opportunistic neurological disease during the HAART era

    Aleixo Agdemir W

    2007-12-01

    Full Text Available Abstract Background In the central nervous system, HIV replication can occur relatively independent of systemic infection, and intrathecal replication of HIV-1 has been observed in patients with HIV-related and opportunistic neurological diseases. The clinical usefulness of HIV-1 RNA detection in the cerebrospinal fluid (CSF of patients with opportunistic neurological diseases, or the effect of opportunistic diseases on CSF HIV levels in patients under HAART has not been well defined. We quantified CSF and plasma viral load in HIV-infected patients with and without different active opportunistic neurological diseases, determined the characteristics that led to a higher detection rate of HIV RNA in CSF, and compared these two compartments. Methods A prospective study was conducted on 90 HIV-infected patients submitted to lumbar puncture as part of a work-up for suspected neurological disease. Seventy-one patients had active neurological diseases while the remaining 19 did not. Results HIV-1 RNA was quantified in 90 CSF and 70 plasma samples. The HIV-1 RNA detection rate in CSF was higher in patients with neurological diseases, in those with a CD4 count lower than 200 cells/mm3, and in those not receiving antiretroviral therapy, as well as in patients with detectable plasma HIV-1 RNA. Median viral load was lower in CSF than in plasma in the total population, in patients without neurological diseases, and in patients with toxoplasmic encephalitis, while no significant difference between the two compartments was observed for patients with cryptococcal meningitis and HIV-associated dementia. CSF viral load was lower in patients with cryptococcal meningitis and neurotoxoplasmosis under HAART than in those not receiving HAART. Conclusion Detection of HIV-1 RNA in CSF was more frequent in patients with neurological disease, a CD4 count lower than 200 cells/mm3 and detectable plasma HIV-1. Median HIV-1 RNA levels were generally lower in CSF than in

  1. DYNC1H1 mutations associated with neurological diseases compromise processivity of dynein-dynactin-cargo adaptor complexes.

    Hoang, Ha Thi; Schlager, Max A; Carter, Andrew P; Bullock, Simon L

    2017-02-28

    Mutations in the human DYNC1H1 gene are associated with neurological diseases. DYNC1H1 encodes the heavy chain of cytoplasmic dynein-1, a 1.4-MDa motor complex that traffics organelles, vesicles, and macromolecules toward microtubule minus ends. The effects of the DYNC1H1 mutations on dynein motility, and consequently their links to neuropathology, are not understood. Here, we address this issue using a recombinant expression system for human dynein coupled to single-molecule resolution in vitro motility assays. We functionally characterize 14 DYNC1H1 mutations identified in humans diagnosed with malformations in cortical development (MCD) or spinal muscular atrophy with lower extremity predominance (SMALED), as well as three mutations that cause motor and sensory defects in mice. Two of the human mutations, R1962C and H3822P, strongly interfere with dynein's core mechanochemical properties. The remaining mutations selectively compromise the processive mode of dynein movement that is activated by binding to the accessory complex dynactin and the cargo adaptor Bicaudal-D2 (BICD2). Mutations with the strongest effects on dynein motility in vitro are associated with MCD. The vast majority of mutations do not affect binding of dynein to dynactin and BICD2 and are therefore expected to result in linkage of cargos to dynein-dynactin complexes that have defective long-range motility. This observation offers an explanation for the dominant effects of DYNC1H1 mutations in vivo. Collectively, our results suggest that compromised processivity of cargo-motor assemblies contributes to human neurological disease and provide insight into the influence of different regions of the heavy chain on dynein motility.

  2. Development of a patient reported outcome measure for fatigue in motor neurone disease: the Neurological Fatigue Index (NFI-MND

    Gibbons Chris J

    2011-11-01

    Full Text Available Abstract Background The objective of this research was to develop a disease-specific measure for fatigue in patients with motor neurone disease (MND by generating data that would fit the Rasch measurement model. Fatigue was defined as reversible motor weakness and whole-body tiredness that was predominantly brought on by muscular exertion and was partially relieved by rest. Methods Qualitative interviews were undertaken to confirm the suitability of a previously identified set of 52 neurological fatigue items as relevant to patients with MND. Patients were recruited from five U.K. MND clinics. Questionnaires were administered during clinic or by post. A sub-sample of patients completed the questionnaire again after 2-4 weeks to assess test-retest validity. Exploratory factor analyses and Rasch analysis were conducted on the item set. Results Qualitative interviews with ten MND patients confirmed the suitability of 52 previously identified neurological fatigue items as relevant to patients with MND. 298 patients consented to completing the initial questionnaire including this item set, with an additional 78 patients completing the questionnaire a second time after 4-6 weeks. Exploratory Factor Analysis identified five potential subscales that could be conceptualised as representing: 'Energy', 'Reversible muscular weakness' (shortened to 'Weakness', 'Concentration', 'Effects of heat' and 'Rest'. Of the original five factors, two factors 'Energy' and 'Weakness' met the expectations of the Rasch model. A higher order fatigue summary scale, consisting of items from the 'Energy' and 'Weakness' subscales, was found to fit the Rasch model and have acceptable unidimensionality. The two scales and the higher order summary scale were shown to fulfil model expectations, including assumptions of unidimensionality, local independency and an absence of differential item functioning. Conclusions The Neurological Fatigue Index for MND (NFI-MND is a simple, easy

  3. Feasibility and efficacy of thrombolysis in acute ischemic stroke: A study from National Institute of Neurological and Allied Sciences, Kathmandu, Nepal

    Thapa, Lekhjung; Shrestha, Shikher; Shrestha, Pratyush; Bhattarai, Suman; Gongal, D N; Devkota, U P

    2016-01-01

    Background: Stroke is the major cause of morbidity and mortality worldwide. The number of stroke patients receiving recombinant tissue plasminogen activator (rt-PA), also known as Alteplase, in the developing world is extremely low. We aim to study the feasibility and efficacy of thrombolysis for the 1st time in our country. Materials and Methods: In this retrospective study (July 2012-August 2015), acute ischemic stroke patients who were thrombolyzed within 3 h of stroke onset were included. Their demographic profiles, clinical profiles, risk factors, type of thrombolytic used, and outcomes were systematically recorded and analyzed. Results: A total of 9 patients were thrombolyzed. The mean time from the onset of stroke symptoms to first dose of rt-PA (onset to treatment) was 1.2 h. Six patients had good neurological outcome as measured by modified Rankin Scale (mRS). The median mRS at discharge was 3. Thrombolysis-related post treatment complication was noted in 44.4%, of which nonfatal intracranial bleed occurred only in 2 patients (22.2%). None of the patients receiving intravenous tenecteplase had thrombolysis-related complications, and none of the patients had fatal intracranial bleed. Conclusion: This study clearly demonstrates the beginning of a feasible and effective thrombolysis in the treatment of acute ischemic stroke in Nepal. PMID:26933345

  4. Feasibility and efficacy of thrombolysis in acute ischemic stroke: A study from National Institute of Neurological and Allied Sciences, Kathmandu, Nepal

    Lekhjung Thapa

    2016-01-01

    Full Text Available Background: Stroke is the major cause of morbidity and mortality worldwide. The number of stroke patients receiving recombinant tissue plasminogen activator (rt-PA, also known as Alteplase, in the developing world is extremely low. We aim to study the feasibility and efficacy of thrombolysis for the 1st time in our country. Materials and Methods: In this retrospective study (July 2012-August 2015, acute ischemic stroke patients who were thrombolyzed within 3 h of stroke onset were included. Their demographic profiles, clinical profiles, risk factors, type of thrombolytic used, and outcomes were systematically recorded and analyzed. Results: A total of 9 patients were thrombolyzed. The mean time from the onset of stroke symptoms to first dose of rt-PA (onset to treatment was 1.2 h. Six patients had good neurological outcome as measured by modified Rankin Scale (mRS. The median mRS at discharge was 3. Thrombolysis-related post treatment complication was noted in 44.4%, of which nonfatal intracranial bleed occurred only in 2 patients (22.2%. None of the patients receiving intravenous tenecteplase had thrombolysis-related complications, and none of the patients had fatal intracranial bleed. Conclusion: This study clearly demonstrates the beginning of a feasible and effective thrombolysis in the treatment of acute ischemic stroke in Nepal.

  5. Practical approach to management of respiratory complications in neurological disorders.

    Mangera, Zaheer; Panesar, Gurkirat; Makker, Himender

    2012-01-01

    Patients with certain neurological diseases are at increased risk of developing chest infections as well as respiratory failure due to muscular weakness. In particular, patients with certain neuromuscular disorders are at higher risk. These conditions are often associated with sleep disordered breathing. It is important to identify patients at risk of respiratory complications early in the course of their disease, although patients with neuromuscular disorders often present in the acute setting with respiratory involvement. This review of the respiratory complications of neurological disorders, with a particular focus on neuromuscular disorders, explores why this happens and looks at how to recognize, investigate, and manage these patients effectively.

  6. Hodgkin's Lymphoma: A Review of Neurologic Complications

    Sean Grimm

    2011-01-01

    Full Text Available Hodgkin's lymphoma is a hematolymphoid neoplasm, primarily of B cell lineage, that has unique histologic, immunophenotypic, and clinical features. Neurologic complications of Hodgkin's Lymphoma can be separated into those that result directly from the disease, indirectly from the disease, or from its treatment. Direct neurologic dysfunction from Hodgkin's Lymphoma results from metastatic intracranial spinal disease, epidural metastases causing spinal cord/cauda equina compression, leptomeningeal metastases, or intradural intramedullary spinal cord metastases. Indirect neurologic dysfunction may be caused by paraneoplastic disorders (such as paraneoplastic cerebellar degeneration or limbic encephalitis and primary angiitis of the central nervous system. Hodgkin's lymphoma treatment typically includes chemotherapy or radiotherapy with potential treatment-related complications affecting the nervous system. Neurologic complications resulting from mantle-field radiotherapy include the “dropped head syndrome,” acute brachial plexopathy, and transient ischemic attacks/cerebral infarcts. Chemotherapy for Hodgkin's lymphoma may cause cerebral infarction (due to emboli from anthracycline-induced cardiomyopathy and peripheral neuropathy.

  7. Specific circulating immune complexes in acute chagas' disease

    Ricardo Corral

    1987-02-01

    Full Text Available The presence of circulating immune complexes formed by IgM and IgG (CIC-IgM and CIC-IgG was investigated, using antigen-specific enzyme-immunoassays (ELISA, in 30 patients with acute Chagas' disease who showed parasitemia and inoculation chagoma. Control population consisted of patients with chronic T. cruzi infection (30, acute toxoplasmosis 10, leishmaniasis (8, rheumatoid arthritis (3 and healthy individuals with negative serology for Chagas* disease (30. Acute chagasic patients were 100% CIC-IgG and 96.66% CIC-IgM positive whereas immunofluorescence tests yielded 90% and 86.66% of positivity for specific IgG and IgM antibodies, respectively. Chronic patients were 68% CIC-IgG and 0% CIC-IgM positive. The 30 negative and the 21 cross-reaction controls proved negative for ELISA (CIC-IgM and CIC-IgG. The high sensitivity of ELISA assays would allow early immunologic diagnosis, as well as prompt treatment, of acute T. cruzi infection, thus eliminating the problem of the false-positive and false-negative results which affects traditional methods for detection of circulating antibodies.

  8. Genes that affect brain structure and function identified by rare variant analyses of Mendelian neurologic disease

    Karaca, Ender; Harel, Tamar; Pehlivan, Davut; Jhangiani, Shalini N.; Gambin, Tomasz; Akdemir, Zeynep Coban; Gonzaga-Jauregui, Claudia; Erdin, Serkan; Bayram, Yavuz; Campbell, Ian M.; Hunter, Jill V.; Atik, Mehmed M.; Van Esch, Hilde; Yuan, Bo; Wiszniewski, Wojciech; Isikay, Sedat; Yesil, Gozde; Yuregir, Ozge O.; Bozdogan, Sevcan Tug; Aslan, Huseyin; Aydin, Hatip; Tos, Tulay; Aksoy, Ayse; De Vivo, Darryl C.; Jain, Preti; Geckinli, B. Bilge; Sezer, Ozlem; Gul, Davut; Durmaz, Burak; Cogulu, Ozgur; Ozkinay, Ferda; Topcu, Vehap; Candan, Sukru; Cebi, Alper Han; Ikbal, Mevlit; Gulec, Elif Yilmaz; Gezdirici, Alper; Koparir, Erkan; Ekici, Fatma; Coskun, Salih; Cicek, Salih; Karaer, Kadri; Koparir, Asuman; Duz, Mehmet Bugrahan; Kirat, Emre; Fenercioglu, Elif; Ulucan, Hakan; Seven, Mehmet; Guran, Tulay; Elcioglu, Nursel; Yildirim, Mahmut Selman; Aktas, Dilek; Alikaşifoğlu, Mehmet; Ture, Mehmet; Yakut, Tahsin; Overton, John D.; Yuksel, Adnan; Ozen, Mustafa; Muzny, Donna M.; Adams, David R.; Boerwinkle, Eric; Chung, Wendy K.; Gibbs, Richard A.; Lupski, James R

    2015-01-01

    Development of the human nervous system involves complex interactions between fundamental cellular processes and requires a multitude of genes, many of which remain to be associated with human disease. We applied whole exome sequencing to 128 mostly consanguineous families with neurogenetic disorders that often included brain malformations. Rare variant analyses for both single nucleotide variant (SNV) and copy number variant (CNV) alleles allowed for identification of 45 novel variants in 43 known disease genes, 41 candidate genes, and CNVs in 10 families, with an overall potential molecular cause identified in >85% of families studied. Among the candidate genes identified, we found PRUNE, VARS, and DHX37 in multiple families, and homozygous loss of function variants in AGBL2, SLC18A2, SMARCA1, UBQLN1, and CPLX1. Neuroimaging and in silico analysis of functional and expression proximity between candidate and known disease genes allowed for further understanding of genetic networks underlying specific types of brain malformations. PMID:26539891

  9. Genetic counseling issues in predictive genetic testing for familial adult-onset neurologic diseases.

    Burson, C M; Markey, K R

    2001-09-01

    Genetic counseling is important in any genetic testing situation in order to address the various issues related to obtaining a genetic diagnosis. Presymptomatic testing for adult-onset neurodegenerative disease, in particular, presents a complex counseling scenario. It is imperative to discuss the potential impact of test results on patients' family dynamics, insurability and employability, family planning, and future health in addition to ascertaining a complete understanding of recurrence, inheritance, and testing parameters. The Huntington disease presymptomatic testing protocol is well-defined and has been used for more than 10 years. These guidelines, which protect both patient and provider, can now be applied to other diseases as further presymptomatic testing capabilities are realized.

  10. The role of vitamin D in the brain and related neurological diseases

    Mustafa Yılmaz

    2013-09-01

    Full Text Available Vitamin D is a steroid hormone that is produced photochemicallyin epidermis. It is known that vitamin D involvedin the regulation of bone mineralization and calcium-phosphorus balance. However, in recent studies havesuggested that vitamin D may have a significant impactin the development of the cell proliferation, differentiation,neurotransmission, neuroplasticity, neurotropic andneuroprotective effects in central nervous system (CNS.For the reason of the effects, it can be considered as aneurosteroid was reported. It was discussed that the levelof vitamin D may be associated to neurodegenerativediseases such as Parkinson’s disease, Alzheimer’s disease,multiple sclerosis (MS, amyotrophic lateral sclerosis(ALS. The role of vitamin D and the mechanisms ofthese diseases will be discussed in the review. J Clin ExpInvest 2013; 4 (3: 411-415Key words: Vitamin D, neurosteroid, brain, neurologicdiseases

  11. A Darwinian approach to Huntington's disease: subtle health benefits of a neurological disorder.

    Eskenazi, Benjamin R; Wilson-Rich, Noah S; Starks, Philip T

    2007-01-01

    Huntington's disease (HD) is a neurodegenerative disorder that, unlike most autosomal dominant disorders, is not being selected against. One explanation for the maintenance of the mutant HD allele is that it is transparent to natural selection because disease symptoms typically occur subsequent to an individual's peak reproductive years. While true, this observation does not explain the population-level increase in HD. The increase in HD is at least partly the result of enhanced fitness: HD+ individuals have more offspring than unaffected relatives. This phenomenon has previously been explained as the result of elevated promiscuity of HD+ individuals. For this to be true, disease symptoms must be expressed during the otherwise asymptomatic peak reproductive years and promiscuity must increase offspring production; however, neither prediction is supported by data. Instead, new data suggest that the mutant HD allele bestows health benefits on its carriers. HD+ individuals show elevated levels of the tumor suppressor protein p53 and experience significantly less cancer than unaffected siblings. We hypothesize that the mutant HD allele elevates carriers' immune activity and thus HD+ individuals are, on average, healthier than HD- individuals during reproductive years. As health and reproductive output are positively related, data suggest a counterintuitive relationship: health benefits may lead to an increased prevalence of Huntington's disease.

  12. New Perspectives in Nuclear Neurology for the Evaluation of Parkinson's Disease

    Benadiba, Marcel; Luurtsema, Gert; Tumas, Vitor; Buchpigel, Carlos Alberto; Busatto, Geraldo F.

    2013-01-01

    The pathophysiology of Parkinson's disease (PD) has not yet been completely elucidated. However, during the past few years, significant progress has been made in understanding the intra- and extracellular mechanisms by which proteins such as alpha-synuclein and neuroinflammatory molecules may displa

  13. Type 2 Gaucher Disease (Acute Infantile Gaucher Disease or Neuropathic Type

    Mohammad Mehdi TAGHDIRI

    2012-12-01

    Full Text Available How to Cite this Article: Taghdiri MM. Type 2 Gaucher Disease (Acute Infantile Gaucher Disease or Neuropathic Type. Iran J Child Neurol Autumn 2012; 6:4 (suppl. 1:12. Pls see PDF. 

  14. Role of anaerobes in acute pelvic inflammatory disease

    2003-01-01

    Pouch of Douglas aspirates were collected from 50 women with history and examination suggestive of acute pelvic inflammatory disease (PID) and 20 healthy women admitted for tubal ligation served as control. A total of 57 microorganisms were isolated from 37 patients out of 50 in study group. Of 37 positive cultures 21(56.7%) were monomicrobial and 16(43.2%) were polymicrobial. Most common symptom in study group was lower abdominal pain (90%), vaginal discharge (70%...

  15. Neurological Findings in Myeloproliferative Neoplasms

    Semra Paydas

    2013-04-01

    Full Text Available Myeloproliferative neoplasms (MPN arise from genetic deficiencies at the level of pluripotent stem cells. Each of these neoplasms is a clonal stem cell disorder with specific phenotypic, genetic and clinical properties. Age is one of the most important factors in the development of symptoms and complications associated with MPNs.High white blood cell counts in chronic myelocytic leukemia also known as leukocytosis may lead to central nervous system findings. Tumors developing outside the bone marrow named as extramedullary myeloid tumors (EMMT could be detected at the initial diagnosis or during the prognosis of the disease, which may cause neurological symptoms due to pressure of leukemic cell mass on various tissues along with spinal cord. Central nervous system involvement and thrombocytopenic hemorrhage may lead to diverse neurological symptoms and findings.Transient ischemic attack and thrombotic stroke are the most common symptoms in polycythemia vera. Besides thrombosis and hemorrage, transformation to acute leukemia can cause neurological symptoms and findings. Transient ischemic attack, thrombotic stroke and specifically hemorrage can give rise to neurological symptoms similar to MPN in essential thrombocytosis.Extramedullary hematopoiesis refers to hematopoietic centers arise in organ/tissues other than bone marrow in myelofibrosis. Extramedullar hematopoietic centers may cause intracranial involvement, spinal cord compression, seizures and hydrocephalia. Though rare, extramedullary hematopoiesis can be detected in cranial/spinal meninges, paraspinal tissue and intracerebral regions. Extramedullary hematopoiesis has been reported in peripheral neurons, choroid plexus, pituitary, orbits, orbital and lacrimal fossa and in sphenoidal sinuses. [Cukurova Med J 2013; 38(2.000: 157-169

  16. Aberrant functional connectome in neurologically asymptomatic patients with end-stage renal disease.

    Xiaofen Ma

    Full Text Available This study aimed to investigate the topological organization of intrinsic functional brain networks in patients with end-stage renal disease (ESRD.Resting-state functional MRI data were collected from 22 patients with ESRD (16 men, 18-61 years and 29 age- and gender-matched healthy controls (HCs, 19 men, 32-61 years. Whole-brain functional networks were obtained by calculating the interregional correlation of low-frequency fluctuations in spontaneous brain activity among 1,024 parcels that cover the entire cerebrum. Weighted graph-based models were then employed to topologically characterize these networks at different global, modular and nodal levels.Compared to HCs, the patients exhibited significant disruption in parallel information processing over the whole networks (P < 0.05. The disruption was present in all the functional modules (default mode, executive control, sensorimotor and visual networks although decreased functional connectivity was observed only within the default mode network. Regional analysis showed that the disease disproportionately weakened nodal efficiency of the default mode components and tended to preferentially affect central or hub-like regions. Intriguingly, the network abnormalities correlated with biochemical hemoglobin and serum calcium levels in the patients. Finally, the functional changes were substantively unchanged after correcting for gray matter atrophy in the patients.Our findings provide evidence for the disconnection nature of ESRD's brain and therefore have important implications for understanding the neuropathologic substrate of the disease from disrupted network organization perspective.

  17. The biochemistry of ketogenesis and its role in weight management, neurological disease and oxidative stress.

    McPherson, Peter Andrew C; McEneny, Jane

    2012-03-01

    Ketogenesis is the branch of mammalian metabolism concerned with the synthesis of ketone bodies. In this process, the small, water-soluble compounds acetoacetate, D-3-β-hydroxybutyrate and propanone are produced by the liver in response to reduced glucose availability. Although ketone bodies are always present at a low level in healthy individuals, dietary manipulation and certain pathological conditions can increase the levels of these compounds in vivo. In some instances, such as in refractory epilepsy, high levels of ketone bodies can be beneficial-in this instance, by exerting an anticonvulsant effect. Conversely, if the levels of ketones rise to supraphysiological levels, as can occur in diabetes mellitus, a state of ketoacidosis can occur, which has serious consequences for cellular function. More recently, research has identified a possible link between ketogenesis and free radical-mediated pathologies, highlighting the potential application of ketogenic diets to the treatment of conditions such as Alzheimer's disease. Overall, an understanding of ketone body metabolism and its links to human disease may prove to be vital in developing new regimens for the treatment of human disease.

  18. The severity of Minamata disease declined in 25 years: temporal profile of the neurological findings analyzed by multiple logistic regression model.

    Uchino, Makoto; Hirano, Teruyuki; Satoh, Hiroshi; Arimura, Kimiyoshi; Nakagawa, Masanori; Wakamiya, Jyunji

    2005-01-01

    Minamata disease (MD) was caused by ingestion of seafood from the methylmercury-contaminated areas. Although 50 years have passed since the discovery of MD, there have been only a few studies on the temporal profile of neurological findings in certified MD patients. Thus, we evaluated changes in neurological symptoms and signs of MD using discriminants by multiple logistic regression analysis. The severity of predictive index declined in 25 years in most of the patients. Only a few patients showed aggravation of neurological findings, which was due to complications such as spino-cerebellar degeneration. Patients with chronic MD aged over 45 years had several concomitant diseases so that their clinical pictures were complicated. It was difficult to differentiate chronic MD using statistically established discriminants based on sensory disturbance alone. In conclusion, the severity of MD declined in 25 years along with the modification by age-related concomitant disorders.

  19. Aspectos neurológicos da moléstia de chagas Neurological aspects of Chagas disease

    Fritz Köberle

    1967-09-01

    Full Text Available Carlos Chagas related in more than two 200 cases, what he called "nervous forms" of trypanosomiasis, that is neurological manifestations from central origin (idiotism, infantilism, pseudo-bulbar paralysis, aphasia, cerebellar ataxia, atetosis, espostic or paralytic diplegia, disbasia. At that time Chagas expressed his concepts as follows: "In relation to the frequency of trypanosomiasis nervous forms we have performed many observations which allow us to state that this disease is the one which causes the largest number of organic affections of the central nervous system, in human pathology". We are plenty convinced by Chagas's statement. By experiments on animals of laboratory we have very often noticed a rather varied neurological symptomatology, being worth point out identical syndromes to those observed by Chagas. Our autopsy material non-rarely include chronic Chagas cases presenting a most varied symtomatology. Among them we have named only three cases of discerebral nanism, a rather rare affection in other parts of the world and relatively frequent in our material. The fact which we have demonstrated, i.e., a relatively great decreasing of number of nervous cells in the peripheral system could happen in the central nervous system as well. Provided that there are only two quantitative works on neuron number diminishing in the central nervous system in mice and rats we decline to go into further details about central neuropathies in man. We emphasized the necessity to perform researches on this field by means of intimate collaboration between clinicians and pathologists, as the only way to confirm on scientific basis all that was observed by the panoramic and genial vision of Carlos Chagas.

  20. Genomics in Neurological Disorders

    Guangchun Han; Jiya Sun; Jiajia Wang; Zhouxian Bai; Fuhai Song; Hongxing Lei

    2014-01-01

    Neurological disorders comprise a variety of complex diseases in the central nervous system, which can be roughly classified as neurodegenerative diseases and psychiatric disorders. The basic and translational research of neurological disorders has been hindered by the difficulty in accessing the pathological center (i.e., the brain) in live patients. The rapid advancement of sequencing and array technologies has made it possible to investigate the disease mechanism and biomarkers from a systems perspective. In this review, recent progresses in the discovery of novel risk genes, treatment targets and peripheral biomarkers employing genomic technologies will be dis-cussed. Our major focus will be on two of the most heavily investigated neurological disorders, namely Alzheimer’s disease and autism spectrum disorder.

  1. Stroke in patients with sickle cell disease: clinical and neurological aspects Acidente cerebrovascular em pacientes com anemia falciforme: aspectos clínicos e neurológicos

    Carolina Camargo de Oliveira

    2008-03-01

    Full Text Available The aim of this study was to characterize a group of patients (n=8 with sickle cell disease (SCD and ischemic stroke concerning the clinical, neurological, imaging and progressive aspects. Data were collected from records and completed with an interview of patients and their parents. In this study there were 8 patients with ages ranging from 10 to 23 years old; SCD diagnosis was given between one and two years of age with clinical features of fatigue and anemia. The stroke was ischemic in all individuals and the first cerebrovascular event occurred before 6 years of age; 3 patients had recurrence of stroke despite prophylactic blood transfusion therapy and both cerebral hemispheres were affected in 4 patients. Clinical and neurological current features observed were: acute pain crises, sialorrhea, mouth breathing, motor, and neuropsychological impairments resulting from cortical-subcortical structure lesions.O objetivo deste estudo foi caracterizar um grupo de sujeitos (n=8 com antecedentes de anemia falciforme (AF e acidente vascular cerebral (AVC isquêmico, dos pontos de vista clínico, neurológico, radiológico e evolutivo, reavaliados através de exame neurológico e neuropsicológico. A partir de prontuários dos sujeitos com diagnóstico comprovado de AF e AVC, coletamos dados, complementados por entrevista com pacientes e responsáveis. Foram avaliados 8 pacientes; atualmente com idades entre 10 e 23 anos; diagnóstico da AF entre um e dois anos; quadro clínico de fraqueza e anemia. Em todos, o AVC foi isquêmico e o primeiro evento na maioria ocorreu antes dos 6 anos de idade; houve recorrência do AVC em 3, apesar da profilaxia com transfusão sanguínea; ambos os hemisférios afetados em 4; no quadro clínico e neurológico atual constatamos crises dolorosas, sialorréia, respiração oral e importante comprometimento motor e neuropsicológico, resultantes de lesões estruturais cortico-subcorticais.

  2. Intravenous high-dose immunotherapy: practical recommendations for use in the treatment of neurological disimmune diseases

    N. A. Suponeva

    2015-01-01

    Full Text Available Current publication summarizes main indications and benefits of intravenous high-dose immunotherapy (IHI in the treatment of various autoimmune diseases of the peripheral nervous system. Available products of intravenous immunoglobulin (IVIG on the Russian market are reviewed. Tactics for choosing optimal medication for IHI based on its effectiveness and safety are analyzed. Dosage calculation and way of administration of IVIG are described, beeing of a high practical value in neurologist’s daily work.

  3. Anti-NMDA receptor encephalitis: a neurological disease in psychiatric disguise.

    Sharma, Bhawna; Handa, Rahul; Prakash, Swayam; Nagpal, Kadam; Gupta, Pankaj

    2014-02-01

    Anti-NMDA receptor encephalitis was first described in 2005 when psychiatric features, memory loss and altered consciousness were found in four women with ovarian teratoma. We report a case of anti-NMDA receptor encephalitis in a 16-year-old female who presented with psychiatric features followed by autonomic dysfunction and orofacial dyskinesias that showed drastic improvement to intravenous immunoglobulin. As many patients of anti-NMDAR encephalitis initially present with psychiatric features, it is important for psychiatrists to have high index of suspicion for this disease and thus avoid the delay in diagnosing this treatable condition which may be otherwise fatal.

  4. Progressive neurologic and somatic disease in a novel mouse model of human mucopolysaccharidosis type IIIC

    Sara Marcó

    2016-09-01

    Full Text Available Mucopolysaccharidosis type IIIC (MPSIIIC is a severe lysosomal storage disease caused by deficiency in activity of the transmembrane enzyme heparan-α-glucosaminide N-acetyltransferase (HGSNAT that catalyses the N-acetylation of α-glucosamine residues of heparan sulfate. Enzyme deficiency causes abnormal substrate accumulation in lysosomes, leading to progressive and severe neurodegeneration, somatic pathology and early death. There is no cure for MPSIIIC, and development of new therapies is challenging because of the unfeasibility of cross-correction. In this study, we generated a new mouse model of MPSIIIC by targeted disruption of the Hgsnat gene. Successful targeting left LacZ expression under control of the Hgsnat promoter, allowing investigation into sites of endogenous expression, which was particularly prominent in the CNS, but was also detectable in peripheral organs. Signs of CNS storage pathology, including glycosaminoglycan accumulation, lysosomal distension, lysosomal dysfunction and neuroinflammation were detected in 2-month-old animals and progressed with age. Glycosaminoglycan accumulation and ultrastructural changes were also observed in most somatic organs, but lysosomal pathology seemed most severe in liver. Furthermore, HGSNAT-deficient mice had altered locomotor and exploratory activity and shortened lifespan. Hence, this animal model recapitulates human MPSIIIC and provides a useful tool for the study of disease physiopathology and the development of new therapeutic approaches.

  5. The SOS pilot study: a RCT of routine oxygen supplementation early after acute stroke--effect on recovery of neurological function at one week.

    Christine Roffe

    Full Text Available UNLABELLED: Mild hypoxia is common after stroke and associated with poor long-term outcome. Oxygen supplementation could prevent hypoxia and improve recovery. A previous study of routine oxygen supplementation showed no significant benefit at 7 and 12 months. This pilot study reports the effects of routine oxygen supplementation for 72 hours on oxygen saturation and neurological outcomes at 1 week after a stroke. METHODS: Patients with a clinical diagnosis of acute stroke were recruited within 24 h of hospital admission between October 2004 and April 2008. Participants were randomized to oxygen via nasal cannulae (72 h or control (room air, oxygen given only if clinically indicated. Clinical outcomes were assessed by research team members at 1 week. Baseline data for oxygen (n = 148 and control (n = 141 did not differ between groups. RESULTS: The median (interquartile range National Institutes of Health Stroke Scale (NIHSS score for the groups at baseline was 6 (7 and 5 (7 respectively. The median Nocturnal Oxygen Saturation during treatment was 1.4% (0.3 higher in the oxygen than in the control group (p<0.001 during the intervention. At 1 week, the median NIHSS score had reduced by 2 (3 in the oxygen and by 1 (2 in the control group. 31% of participants in the oxygen group and 14% in the control group had an improvement of ≥4 NIHSS points at 1 week doubling the odds of improvement in the oxygen group (OR: 2.9. CONCLUSION: Our data show that routine oxygen supplementation started within 24 hours of hospital admission with acute stroke led to a small, but statistically significant, improvement in neurological recovery at 1 week. However, the difference in NIHSS improvement may be due to baseline imbalance in stroke severity between the two groups and needs to be confirmed in a larger study and linked to longer-term clinical outcome. TRIAL REGISTRATION: Controlled-Trials.com ISRCTN12362720; European Clinical Trials Database 2004-001866-41.

  6. A novel unbiased proteomic approach to detect the reactivity of cerebrospinal fluid in neurological diseases.

    Menon, Krishnakumar N; Steer, David L; Short, Martin; Petratos, Steven; Smith, Ian; Bernard, Claude C A

    2011-06-01

    Neurodegenerative diseases, such as multiple sclerosis represent global health issues. Accordingly, there is an urgent need to understand the pathogenesis of this and other central nervous system disorders, so that more effective therapeutics can be developed. Cerebrospinal fluid is a potential source of important reporter molecules released from various cell types as a result of central nervous system pathology. Here, we report the development of an unbiased approach for the detection of reactive cerebrospinal fluid molecules and target brain proteins from patients with multiple sclerosis. To help identify molecules that may serve as clinical biomarkers for multiple sclerosis, we have biotinylated proteins present in the cerebrospinal fluid and tested their reactivity against brain homogenate as well as myelin and myelin-axolemmal complexes. Proteins were separated by two-dimensional gel electrophoresis, blotted onto membranes and probed separately with biotinylated unprocessed cerebrospinal fluid samples. Protein spots that reacted to two or more multiple sclerosis-cerebrospinal fluids were further analyzed by matrix assisted laser desorption ionization-time-of-flight time-of-flight mass spectrometry. In addition to previously reported proteins found in multiple sclerosis cerebrospinal fluid, such as αβ crystallin, enolase, and 14-3-3-protein, we have identified several additional molecules involved in mitochondrial and energy metabolism, myelin gene expression and/or cytoskeletal organization. These include aspartate aminotransferase, cyclophilin-A, quaking protein, collapsin response mediator protein-2, ubiquitin carboxy-terminal hydrolase L1, and cofilin. To further validate these findings, the cellular expression pattern of collapsin response mediator protein-2 and ubiquitin carboxy-terminal hydrolase L1 were investigated in human chronic-active MS lesions by immunohistochemistry. The observation that in multiple sclerosis lesions phosphorylated collapsin

  7. The neurological effects of ghrelin in brain diseases: Beyond metabolic functions.

    Jiao, Qian; Du, Xixun; Li, Yong; Gong, Bing; Shi, Limin; Tang, Tingting; Jiang, Hong

    2017-02-01

    Ghrelin, a peptide released by the stomach that plays a major role in regulating energy metabolism, has recently been shown to have effects on neurobiological behaviors. Ghrelin enhances neuronal survival by reducing apoptosis, alleviating inflammation and oxidative stress, and accordingly improving mitochondrial function. Ghrelin also stimulates the proliferation, differentiation and migration of neural stem/progenitor cells (NS/PCs). Additionally, the ghrelin is benefit for the recovery of memory, mood and cognitive dysfunction after stroke or traumatic brain injury. Because of its neuroprotective and neurogenic roles, ghrelin may be used as a therapeutic agent in the brain to combat neurodegenerative disease. In this review, we highlight the pre-clinical evidence and the proposed mechanisms underlying the role of ghrelin in physiological and pathological brain function.

  8. Neurology in Asia.

    Tan, Chong-Tin

    2015-02-10

    Asia is important as it accounts for more than half of the world population. The majority of Asian countries fall into the middle income category. As for cultural traditions, Asia is highly varied, with many languages spoken. The pattern of neurologic diseases in Asia is largely similar to the West, with some disease features being specific to Asia. Whereas Asia constitutes 60% of the world's population, it contains only 20% of the world's neurologists. This disparity is particularly evident in South and South East Asia. As for neurologic care, it is highly variable depending on whether it is an urban or rural setting, the level of economic development, and the system of health care financing. To help remedy the shortage of neurologists, most counties with larger populations have established training programs in neurology. These programs are diverse, with many areas of concern. There are regional organizations serving as a vehicle for networking in neurology and various subspecialties, as well as an official journal (Neurology Asia). The Asian Epilepsy Academy, with its emphasis on workshops in various locations, EEG certification examination, and fellowships, may provide a template of effective regional networking for improving neurology care in the region.

  9. Cerebellar Purkinje cells incorporate immunoglobulins and immunotoxins in vitro: implications for human neurological disease and immunotherapeutics

    Rose John W

    2009-10-01

    Full Text Available Abstract Background Immunoglobulin G (IgG antibodies reactive with intracellular neuronal proteins have been described in paraneoplastic and other autoimmune disorders. Because neurons have been thought impermeable to immunoglobulins, however, such antibodies have been considered unable to enter neurons and bind to their specific antigens during life. Cerebellar Purkinje cells - an important target in paraneoplastic and other autoimmune diseases - have been shown in experimental animals to incorporate a number of molecules from cerebrospinal fluid. IgG has also been detected in Purkinje cells studied post mortem. Despite the possible significance of these findings for human disease, immunoglobulin uptake by Purkinje cells has not been demonstrated in living tissue or studied systematically. Methods To assess Purkinje cell uptake of immunoglobulins, organotypic cultures of rat cerebellum incubated with rat IgGs, human IgG, fluorescein-conjugated IgG, and rat IgM were studied by confocal microscopy in real time and following fixation. An IgG-daunorubicin immunotoxin was used to determine whether conjugation of pharmacological agents to IgG could be used to achieve Purkinje cell-specific drug delivery. Results IgG uptake was detected in Purkinje cell processes after 4 hours of incubation and in Purkinje cell cytoplasm and nuclei by 24-48 hours. Uptake could be followed in real time using IgG-fluorochrome conjugates. Purkinje cells also incorporated IgM. Intracellular immunoglobulin did not affect Purkinje cell viability, and Purkinje cells cleared intracellular IgG or IgM within 24-48 hours after transfer to media lacking immunoglobulins. The IgG-daunomycin immunotoxin was also rapidly incorporated into Purkinje cells and caused extensive, cell-specific death within 8 hours. Purkinje cell death was not produced by unconjugated daunorubicin or control IgG. Conclusion Purkinje cells in rat organotypic cultures incorporate and clear host (rat and non

  10. Impaired functional default mode network in patients with mild neurological Wilson's disease.

    Han, Yongsheng; Cheng, Hewei; Toledo, Jon B; Wang, Xun; Li, Bo; Han, Yongzhu; Wang, Kai; Fan, Yong

    2016-09-01

    Wilson's disease (WD) is an autosomal recessive metabolic disorder characterized by cognitive, psychiatric and motor signs and symptoms that are associated with structural and pathological brain abnormalities, in addition to liver changes. However, functional brain connectivity pattern of WD patients remains largely unknown. In the present study, we investigated functional brain connectivity pattern of WD patients using resting state functional magnetic resonance imaging. Particularly, we studied default mode network (DMN) using posterior cingulate cortex (PCC) based seed functional connectivity analysis and graph theoretic functional brain network analysis tools, and investigated the relationship between the DMN's functional connectivity pattern of WD patients and their attention functions examined using the attention network test (ANT). Our results demonstrated that WD patients had altered DMN's functional connectivity and lower local and global network efficiency compared with normal controls (NCs). In addition, the functional connectivity between left inferior temporal cortex and right lateral parietal cortex was correlated with altering function, one of the attention functions, across WD and NC subjects. These findings indicated that the DMN's functional connectivity was altered in WD patients, which might be correlated with their attention dysfunction.

  11. Postpartum Acute Pulmonary Oedema with Sub clinical Rheumatic Heart Disease.

    R, Padmaja; Gande, Sri Krishna Padma Challa Rao

    2015-02-01

    Acute dyspnea with pulmonary oedema in postpartum is uncommon but life-threatening event. Contributing factors for pulmonary oedema include, administration of tocolytics, underlying cardiac disease, iatrogenic fluid overload and preeclampsia acounting 0.08% of pregnancies. Pulmonary embolism, amniotic fluid embolism, pneumonia, aspiration and pulmonary oedema are some of the potentially devastating conditions that should be considered by the attending physician. Here, we report a case of postpartum acute pulmonary oedema referred to causality after an emergency caesarean section in a private hospital. No matter what the underlying pathology, prompt administration and appropriate resuscitation is always the first priority. Only after the patient has been stabilized attention must be turned to diagnosis and specific treatment. A diagnosis of severe Mitral Stenosis, probably of rheumatic origin was made after stabilizing the patient.

  12. Acute Warfarin Toxicity as Initial Manifestation of Metastatic Liver Disease

    Varalaxmi Bhavani Nannaka

    2016-01-01

    Full Text Available Near complete infiltration of the liver secondary to metastasis from the head and neck cancer is a rare occurrence. The prognosis of liver failure associated with malignant infiltration is extremely poor; the survival time of patients is extremely low. We present a case of acute warfarin toxicity as initial manifestation of metastatic liver disease. Our patient is a 64-year-old woman presenting with epigastric pain and discomfort, found to have unrecordable International Normalized Ratio. She rapidly deteriorated with acute respiratory failure requiring mechanical ventilation, profound shock requiring high dose vasopressor infusion, severe coagulopathy, worsening liver enzymes with worsening of lactic acidosis and severe metabolic abnormalities, and refractory to aggressive supportive care and died in less than 48 hours. Autopsy revealed that >90% of the liver was replaced by tumor masses.

  13. Neurological Response to cART vs. cART plus Integrase Inhibitor and CCR5 Antagonist Initiated during Acute HIV.

    Victor G Valcour

    Full Text Available To compare central nervous system (CNS outcomes in participants treated during acute HIV infection with standard combination antiretroviral therapy (cART vs. cART plus integrase inhibitor and CCR5 antagonist (cART+.24-week randomized open-label prospective evaluation.Participants were evaluated then randomized to initiate cART (efavirenz, tenofovir, and either emtricitabine or lamivudine vs. cART+ (cART plus raltegravir and maraviroc during acute HIV and re-evaluated at 4, 12 and 24 weeks. We examined plasma and CSF cytokines, HIV RNA levels, neurological and neuropsychological findings, and brain MRS across groups and compared to healthy controls.At baseline, 62 participants were in Fiebig stages I-V. Randomized groups were similar for mean age (27 vs. 25, p = 0.137, gender (each 94% male, plasma log10 HIV RNA (5.4 vs. 5.6, p = 0.382, CSF log10 HIV RNA (2.35 vs. 3.31, p = 0.561, and estimated duration of HIV (18 vs. 17 days, p = 0.546. Randomized arms did not differ at 24 weeks by any CNS outcome. Combining arms, all measures concurrent with antiretroviral treatment improved, for example, neuropsychological testing (mean NPZ-4 of -0.408 vs. 0.245, p<0.001 and inflammatory markers by MRS (e.g. mean frontal white matter (FWM choline of 2.92 vs. 2.84, p = 0.045 at baseline and week 24, respectively. Plasma neopterin (p<0.001 and interferon gamma-induced protein 10 (IP-10 (p = 0.007 remained elevated in participants compared to controls but no statistically significant differences were seen in CSF cytokines compared to controls, despite individual variability among the HIV-infected group.A 24-week course of cART+ improved CNS related outcomes, but was not associated with measurable differences compared to standard cART.

  14. Neurological Response to cART vs. cART plus Integrase Inhibitor and CCR5 Antagonist Initiated during Acute HIV

    Valcour, Victor G.; Spudich, Serena S.; Sailasuta, Napapon; Phanuphak, Nittaya; Lerdlum, Sukalaya; Fletcher, James L. K.; Kroon, Eugene D. M. B.; Jagodzinski, Linda L.; Allen, Isabel E.; Adams, Collin L.; Prueksakaew, Peeriya; Slike, Bonnie M.; Hellmuth, Joanna M.; Kim, Jerome H.; Ananworanich, Jintanat

    2015-01-01

    Objective To compare central nervous system (CNS) outcomes in participants treated during acute HIV infection with standard combination antiretroviral therapy (cART) vs. cART plus integrase inhibitor and CCR5 antagonist (cART+). Design 24-week randomized open-label prospective evaluation. Method Participants were evaluated then randomized to initiate cART (efavirenz, tenofovir, and either emtricitabine or lamivudine) vs. cART+ (cART plus raltegravir and maraviroc) during acute HIV and re-evaluated at 4, 12 and 24 weeks. We examined plasma and CSF cytokines, HIV RNA levels, neurological and neuropsychological findings, and brain MRS across groups and compared to healthy controls. Results At baseline, 62 participants were in Fiebig stages I-V. Randomized groups were similar for mean age (27 vs. 25, p = 0.137), gender (each 94% male), plasma log10 HIV RNA (5.4 vs. 5.6, p = 0.382), CSF log10 HIV RNA (2.35 vs. 3.31, p = 0.561), and estimated duration of HIV (18 vs. 17 days, p = 0.546). Randomized arms did not differ at 24 weeks by any CNS outcome. Combining arms, all measures concurrent with antiretroviral treatment improved, for example, neuropsychological testing (mean NPZ-4 of -0.408 vs. 0.245, p<0.001) and inflammatory markers by MRS (e.g. mean frontal white matter (FWM) choline of 2.92 vs. 2.84, p = 0.045) at baseline and week 24, respectively. Plasma neopterin (p<0.001) and interferon gamma-induced protein 10 (IP-10) (p = 0.007) remained elevated in participants compared to controls but no statistically significant differences were seen in CSF cytokines compared to controls, despite individual variability among the HIV-infected group. Conclusions A 24-week course of cART+ improved CNS related outcomes, but was not associated with measurable differences compared to standard cART. PMID:26555069

  15. A Stochastic Multiscale Model That Explains the Segregation of Axonal Microtubules and Neurofilaments in Neurological Diseases.

    Chuan Xue

    2015-08-01

    Full Text Available The organization of the axonal cytoskeleton is a key determinant of the normal function of an axon, which is a long thin projection of a neuron. Under normal conditions two axonal cytoskeletal polymers, microtubules and neurofilaments, align longitudinally in axons and are interspersed in axonal cross-sections. However, in many neurotoxic and neurodegenerative disorders, microtubules and neurofilaments segregate apart from each other, with microtubules and membranous organelles clustered centrally and neurofilaments displaced to the periphery. This striking segregation precedes the abnormal and excessive neurofilament accumulation in these diseases, which in turn leads to focal axonal swellings. While neurofilament accumulation suggests an impairment of neurofilament transport along axons, the underlying mechanism of their segregation from microtubules remains poorly understood for over 30 years. To address this question, we developed a stochastic multiscale model for the cross-sectional distribution of microtubules and neurofilaments in axons. The model describes microtubules, neurofilaments and organelles as interacting particles in a 2D cross-section, and is built upon molecular processes that occur on a time scale of seconds or shorter. It incorporates the longitudinal transport of neurofilaments and organelles through this domain by allowing stochastic arrival and departure of these cargoes, and integrates the dynamic interactions of these cargoes with microtubules mediated by molecular motors. Simulations of the model demonstrate that organelles can pull nearby microtubules together, and in the absence of neurofilament transport, this mechanism gradually segregates microtubules from neurofilaments on a time scale of hours, similar to that observed in toxic neuropathies. This suggests that the microtubule-neurofilament segregation can be a consequence of the selective impairment of neurofilament transport. The model generates the

  16. Efficacy and safety comparison of DL-3-n-butylphthalide and Cerebrolysin: Effects on neurological and behavioral outcomes in acute ischemic stroke.

    Xue, Li-Xia; Zhang, Ting; Zhao, Yu-Wu; Geng, Zhi; Chen, Jing-Jiong; Chen, Hao

    2016-05-01

    Cerebrolysin and DL-3-n-butylphthalide (NBP) have each shown neuroprotective efficacy in preclinical models of acute ischemic stroke (AIS) and passed clinical trials as therapeutic drugs for AIS. The present study was a clinical trial to assess and compare the efficacy and safety of NBP and Cerebrolysin in the reduction of neurological and behavioral disability following AIS. A randomized, double-blind trial was conducted with enrolment of 60 patients within 12 h of AIS. In addition to routine treatment, patients were randomly assigned to receive a 10-day intravenous administration of NBP, Cerebrolysin or placebo. National Institutes of Health Stroke Scale (NIHSS) and Barthel Index (BI) scores were used to evaluate the efficacy of the treatment in the patients with AIS at 11 and 21 days after the initiation of therapy. Adverse events were also analyzed among the three groups. After 10 days of treatment with NBP or Cerebrolysin, the NIHSS and BI scores at day 21 showed statistical differences compared with those in the placebo group (PCerebrolysin groups were higher than those in the placebo group at days 11 and 21 (PCerebrolysin. The results indicate that NBP may be more effective than Cerebrolysin in improving short-term outcomes following AIS. This trial is registered at ClinicalTrials.gov with clinical trial identifier number NCT02149875.

  17. [Neurological diseases and SPECT--analysis using easy Z-score imaging system (eZIS)].

    Matsuda, Hiroshi

    2007-05-01

    We developed a method for automated diagnosis of brain perfusion SPECT and designated this method as an easy Z-score imaging system (eZIS). In this software program, voxel-by-voxel Z-score analysis after voxel normalization to global mean or cerebellar values; Z-score = ( [control mean] - [individual value] )/ (control SD) is performed. These Z-score maps are displayed by overlay on tomographic sections and by projection with averaged Z-score of 14mm thickness to surface rendering of the anatomically standardized MRI template. Anatomical standardization of SPECT images into a stereotactic space is performed using statistical parametric mapping (SPM) 2. This program has an advantage of capability of incorporation of SPM results into automated analysis of Z-score values as a volume of interest (VOI). A specific VOI can be determined by group comparison of SPECT images for patients with a neuropsychiatric disease with those for healthy volunteers using SPM. Even if a center can construct a normal database with good quality comprising a large number of healthy volunteers, other centers have not been able to use this normal database because of differences between the used gamma cameras, collimators and physical correction algorithms. Since SPECT exhibits greater variations in image quality among different centers than PET, conversion of SPECT images may be necessary for sharing a normal database. In this eZIS software, we incorporated a newly developed program for making it possible to share a normal database in SPECT studies. A Hoffman 3-dimensional brain phantom experiment was conducted to determine systematic differences between SPECT scanners. SPECT images for the brain phantom were obtained using two different scanners. Dividing these two phantom images after anatomical standardization by SPM created a 3-dimensional conversion map. The use of a conversion map obtained from SPECT images of the same phantom provided very similar SPECT data despite extreme differences

  18. Adult neurology training during child neurology residency.

    Schor, Nina F

    2012-08-21

    As it is currently configured, completion of child neurology residency requires performance of 12 months of training in adult neurology. Exploration of whether or not this duration of training in adult neurology is appropriate for what child neurology is today must take into account the initial reasons for this requirement and the goals of adult neurology training during child neurology residency.

  19. Color Doppler US in the acute scrotal disease

    Cha, Yoo Mi; Yang, Dal Mo; Kang, Sook Wook; Kim, Hyung Sik; Lee, Young Seok; Kim, Hyeon Hoe [Chung Ang Gil Hospital, Incheon (Korea, Republic of)

    1993-12-15

    To evaluate the utility of Color doppler US in the patients with acute scrotal pain, we retrospectively analyzed 37 patients referred for Color Doppler US of the scrotum. The diagnosis was confirmed by means of appropriate response to antibiotic treatment (31 cases) or surgery (6 cases). Thirty one of 37 patients were diagnosed as inflammatory disease (24 cases of epididymitis, 7 cases of epididymo-orchitis). Twenty three of 24cases of epididymitis had increased epididymal flow, while 6 of 7 cases of epididymo-orchitis had increased epididymal and testicular flow. Five patients were confirmed as testicular torsion, and in all cases nointratesticular blood flow was identified on the symptomatic side. In one case of torsion of appendix testis, epididymis was enlarged and there were increased signals suggesting epididymitis on Color Doppler US, but was confirmed by surgery as torsion of appendix testis. Therefore, the differentiation between torsion and inflammatory disease was possible by using Color Doppler US of the scrotum in 34 of 37 cases in our study. On the basis of our results, we may conclude that Color Doppler US can simultaneously display blood flow and detailed anatomic images, and function as an effective means of evaluating patients with acute scrotal disease

  20. Lithium-induced minimal change disease and acute kidney injury

    Parul Tandon

    2015-01-01

    Full Text Available Context: Lithium carbonate is a psychiatric medication commonly used in the treatment of bipolar disorder. It has been implicated in inducing nephrogenic diabetes inspidus, chronic tubulointerstitial nephropathy, and acute tubular necrosis. We describe a case of lithium-induced minimal change disease (MCD and acute kidney injury (AKI. Case Report: A 32-year-old female with a medical history of bipolar disorder treated with chronic lithium therapy presented with anasarca, fatigue, and tremors. Work-up revealed supra-therapeutic lithium levels, hypoalbuminemia, and significant proteinuria. The patient was treated conservatively with fluids and discontinuation of lithium therapy. Subsequently, she developed significant AKI and persistent proteinuria. She underwent a renal biopsy that demonstrated effacement of podocyte foot processes consistent with lithium-induced MCD. This was treated with corticosteroids, which decreased the proteinuria and resolved all the patient′s symptoms. Conclusion: Lithium-induced MCD is a rare disease that affects patients of all ages. It is often associated with therapeutic lithium and is typically resolved with discontinuation of lithium. In some cases, concurrent AKI may result due to vascular obstruction from hyperalbuminuria and associated renal interstitial edema. Corticosteroids may be needed to reduce the proteinuria and prevent progression to chronic kidney disease. As such, patients on lithium therapy may benefit from monitoring of glomerular function via urinalysis to prevent the onset of nephrotic syndrome.

  1. Epidemiology of neurological disorders in India: Review of background, prevalence and incidence of epilepsy, stroke, Parkinson′s disease and tremors

    M Gourie-Devi

    2014-01-01

    Full Text Available Growth and development of neuroepidemiology in India during the last four decades has been documented highlighting the historical milestones. The prevalence rates of the spectrum of neurological disorders from different regions of the country ranged from 967-4,070 with a mean of 2394 per 100000 population, providing a rough estimate of over 30 million people with neurological disorders (excluding neuroinfections and traumatic injuries. Prevalence and incidence rates of common disorders including epilepsy, stroke, Parkinson′s disease and tremors determined through population-based surveys show considerable variation across different regions of the country. The need for a standardized screening questionnaire, uniform methodology for case ascertainment and diagnosis is an essential requiste for generating robust national data on neurological disorders. Higher rates of prevalence of neurological disorders in rural areas, 6-8 million people with epilepsy and high case fatality rates of stroke (27-42% call for urgent strategies to establish outreach neurology services to cater to remote and rural areas, develop National Epilepsy Control Program and establish stroke units at different levels of health care pyramid.

  2. Neutron activation analysis in the central nervous system tissues of neurological diseases and rats maintained on minerally unbalanced diets

    Yasui, Masayuki; Ota, Kiichiro [Wakayama Medical Coll. (Japan); Sasajima, Kazuhisa

    1995-02-01

    Epidemiological surveys on Guam have suggested that low calcium (Ca), magnesium (Mg) and high Al and Mn in river, soil and drinking water may be implicated in the pathogenesis of PD. Experimentally, low Ca-Mg diets with or without added Al have been found to accelerate Al deposition in the CNS of rats and monkeys. Although excessive deposition of Mn produces neurotoxic action similar to Al in CNS tissues, the mechanism of Mn deposition coupled with Al loading in the presence of low Ca-Mg intake is not yet known. In this animal study, the deposition and metal-metal interaction of both Al and Mn in the CNS, visceral organs and bones of rats fed unbalanced mineral diets were analyzed. Male Wistar rats, weighing 200 g, were maintained for 90 days on the following diets: (A) standard diet, (B) low Ca diet, (C) low Ca-Mg diet, (D) low Ca-Mg diet with high Al. Al and Mn content were determined in the frontal cortex, spinal cord, kidney, muscle, abdominal aorta, femur and lumbar spine using neutron activation analysis (NAA). Intake of low Ca and Mg with added Al in rats led to the high concentrations of Mn and Al in bones and in the frontal cortex. It is likely that unbalanced mineral diets and metal-metal interactions may lead to the unequal distribution of Al and Mn in bones and ultimately in the CNS inducing CNS degeneration. On the other hand, concentrations of copper (Cu), calcium (Ca) and aluminum (Al) for 26 subanatomical regions of the CNS were measured by neutron activation analysis (NAA) in two cases of Wilson`s disease, two of portal systemic encephalopathy, six pathologically verified cases of ALS, four of Parkinson`s disease and five neurologically normal controls. Also zinc (Zn) and iron (Fe) concentrations were measured by NAA for frontal and occipital lobes of parkinsonism-dementia. (author).

  3. Acute Limb Ischemia and Coronary Artery Disease in a Case of Kimura’s Disease

    Heo, Woon; Jun, Hee Jae; Kang, Do Kyun; Min, Ho-Ki; Hwang, Youn-Ho; Kim, Ji Yong; Nam, Kyung Han

    2017-01-01

    Kimura disease (KD) is an immune-mediated chronic inflammatory disease of unknown etiology. KD has many complications associated with hypereosinophilia, including various forms of allergic reactions and eosinophilic lung disease. Additionally, hypereosinophilia is associated with hypercoagulability, which may lead to thromboembolic events. A 36-year-old man with KD presented with acute limb ischemia and coronary artery occlusion. He underwent thrombectomy, partial endarterectomy of both popliteal arteries, and coronary artery stent insertion. KD is a systemic disease that affects many organs and presents with thromboembolism and vasculitis. In a patient with KD, physicians should evaluate the vascular system, including the coronary arteries. PMID:28382271

  4. Relationship Between Periodontal Disease and Acute Myocardial Infarction

    M Zamirian

    2008-07-01

    Full Text Available Background: Conventional risk factors for coronary artery disease and myocardial infarction do not explain all of the clinical and epidemiological features of the disease. Periodontal disease is a common bacterial and destructive disorder of oral tissues. Many studies demonstrate close association between chronic periodontitis and development of generalized inflammation, vascular endothelial injury, and atherosclesis. Periodontal disease has been convincingly emerging as an important independent risk factor for ischemic heart disease. A case - control study was carried out to assess the prevalence of periodontitis in patients with Acute myocardial Infarction (AMI and evaluate the possible relationship between AMI and chronic periodontitis. Patients and Methods: A number of 160 patients, aged 35 to 70 years old, enrolled in the study. Eighty patients (43 men, 37 women were examined four days after hospitalization due to AMI. Control group consisted of 80 persons (38 men, 42 women with normal coronary angiography. The following periodontal parameters were examined: Plaque index (PI, gingiral index (GI, bleeding on probing (BOP, probing depth (PD, clinical attachment loss (CAL and number of sites with CAL.Results: The case, compared to control showed significantly worse results for some periodontal variables studied: The mean of PD and PD > 3 mm, CAL, and number of sites with CAL, had worse results compared to control despite similar oral hygiene and frequency of brushing. The confounding factors for the present study were found to be hypertension and diabetes. Conclusion: The association between periodontitis and acute myocardial infarction was significant after adjusting for conventional risk factors for AMI.

  5. Epidemiología de los enterovirus asociados a enfermedades neurológicas Epidemiology of enterovirus associated with neurologic diseases

    Daniel M. Cisterna

    2007-04-01

    . Echovirus 4 (E4, E9, E30 and E17 were identified from meningitis outbreaks. Coxsackievirus A2 (CAV2, CBV2, CBV5, E7, E11, E19, E24, E29 and enterovirus 71 were recovered only from sporadic cases. Three different serotypes were identified in encephalitis patients: E4, E7 and E24. A total of 28 different serotypes of non-polio enteroviruses were detected from acute flaccid paralysis cases. The information here presented contributes to improving our knowledge about enteroviruses epidemiology in Argentina and their relationship with different neurological diseases. This study provides valuable data that could be useful to further research.

  6. Migraine- and dystonia-related disease-mutations of Na+/K+-ATPases: Relevance of behavioral studies in mice to disease symptoms and neurological manifestations in humans

    Bøttger, Pernille; Doganli, Canan; Lykke-Hartmann, Karin

    2012-01-01

    The two autosomal dominantly inherited neurological diseases: familial hemiplegic migraine type 2 (FHM2) and familial rapid-onset of dystonia-parkinsonism (Familial RDP) are caused by in vivo mutations of specific alpha subunits of the sodium–potassium pump (Na+/K+-ATPase). Intriguingly, patients...... with classical FHM2 and RDP symptoms additionally suffer from other manifestations, such as epilepsy/seizures and developmental disabilities. Recent studies of FHM2 and RDP mouse models provide valuable tools for dissecting the vital roles of the Na+/K+-ATPases, and we discuss their relevance to the complex...... patient symptoms and manifestations. Thus, it is interesting that mouse models targeting a specific -isoform cause different, although still comparable, phenotypes consistent with classical symptoms and other manifestations observed in FHM2 and RDP patients. This review highlights that use of mouse models...

  7. Pathogenic mechanisms of Acute Graft versus Host Disease

    Ferrara James L.M.

    2002-01-01

    Full Text Available Graft-versus-host-disease (GVHD is the major complication of allogeneic Bone Marrow Transplant (BMT. Older BMT recipients are a greater risk for acute GVHD after allogeneic BMT, but the causes of this association are poorly understood. Using well-characterized murine BMT models we have explored the mechanisms of increased GVHD in older mice. GVHD mortality and morbidity, and pathologic and biochemical indices were all worse in old recipients. Donor T cell responses were significantly increased in old recipients both in vivo and in vitro when stimulated by antigen-presenting cells (APCs from old mice. In a haploidential GVHD model, CD4+ donor T cells mediated more severe GVHD in old mice. We confirmed the role of aged APCs in GVHD using bone marrow chimera recipient created with either old or young bone marrow. APCs from these mice also stimulated greater responses from allogeneic cells in vitro. In a separate set of experiments we evaluated whether alloantigen expression on host target epithelium is essential for tissue damage induced by GVHD. Using bone marrow chimeras recipients in which either MHC II or MHC I alloantigen was expressed only on APCs, we found that acute GVHD does not require alloantigen expression on host target epithelium and that neutralization of tumor necrosis factor-alpha and interleukin-1 prevents acute GVHD. These results pertain to CD4-mediated GVHD and to a lesser extent in CD8-mediated GVHD, and confirm the central role of most APCs as well as inflammatory cytokines.

  8. Role of anaerobes in acute pelvic inflammatory disease

    Saini S

    2003-01-01

    Full Text Available Pouch of Douglas aspirates were collected from 50 women with history and examination suggestive of acute pelvic inflammatory disease (PID and 20 healthy women admitted for tubal ligation served as control. A total of 57 microorganisms were isolated from 37 patients out of 50 in study group. Of 37 positive cultures 21(56.7% were monomicrobial and 16(43.2% were polymicrobial. Most common symptom in study group was lower abdominal pain (90%, vaginal discharge (70% and irregular bleeding (40% and 30% patients had history of intrauterine contraceptive device (IUCD implantation. The predominant aerobic isolates were Escherichia coli, Coagulase Negative Staphylococcus (CONS, Staphylococcus aureus, Klebsiella pneumoniae while common anaerobes were Bacteroides fragilis, Prevotella melaninogenica, Fusobacterium nucleatum and Peptostreptococcus spp. Our study shows that cefotaxime, cefuroxime and gentamicin may be used for gram negative aerobic bacilli; cloxacillin, cephaloridine and erythromycin for aerobic gram positive cocci and amikacin and ceftazidime for Pseudomonas aeruginosa. Thus for optimum therapy of acute PID it is beneficial to keep in mind major conceptual changes and therapeutic realities that have influenced current understanding of acute PID and have affected the choice of therapy.

  9. Role of anaerobes in acute pelvic inflammatory disease.

    Saini, S; Gupta, N; Batra, G; Arora, D R

    2003-01-01

    Pouch of Douglas aspirates were collected from 50 women with history and examination suggestive of acute pelvic inflammatory disease (PID) and 20 healthy women admitted for tubal ligation served as control. A total of 57 microorganisms were isolated from 37 patients out of 50 in study group. Of 37 positive cultures 21(56.7%) were monomicrobial and 16(43.2%) were polymicrobial. Most common symptom in study group was lower abdominal pain (90%), vaginal discharge (70%) and irregular bleeding (40%) and 30% patients had history of intrauterine contraceptive device (IUCD) implantation. The predominant aerobic isolates were Escherichia coli, Coagulase Negative Staphylococcus (CONS), Staphylococcus aureus, Klebsiella pneumoniae while common anaerobes were Bacteroides fragilis, Prevotella melaninogenica, Fusobacterium nucleatum and Peptostreptococcus spp. Our study shows that cefotaxime, cefuroxime and gentamicin may be used for gram negative aerobic bacilli; cloxacillin, cephaloridine and erythromycin for aerobic gram positive cocci and amikacin and ceftazidime for Pseudomonas aeruginosa. Thus for optimum therapy of acute PID it is beneficial to keep in mind major conceptual changes and therapeutic realities that have influenced current understanding of acute PID and have affected the choice of therapy.

  10. Inhibition of human glutamine synthetase by L-methionine-S,R-sulfoximine-relevance to the treatment of neurological diseases.

    Jeitner, Thomas M; Cooper, Arthur J L

    2014-12-01

    At high concentrations, the glutamine synthetase inhibitor L-methionine-S,R-sulfoximine (MSO) is a convulsant, especially in dogs. Nevertheless, sub-convulsive doses of MSO are neuroprotective in rodent models of hyperammonemia, acute liver disease, and amyotrophic lateral sclerosis and suggest MSO may be clinically useful. Previous work has also shown that much lower doses of MSO are required to produce convulsions in dogs than in primates. Evidence from the mid-20th century suggests that humans are also less sensitive. In the present work, the inhibition of recombinant human glutamine synthetase by MSO is shown to be biphasic-an initial reversible competitive inhibition (K i 1.19 mM) is followed by rapid irreversible inactivation. This K i value for the human enzyme accounts, in part, for relative insensitivity of primates to MSO and suggests that this inhibitor could be used to safely inhibit glutamine synthetase activity in humans.

  11. Behcet's disease with neurological involvement: a case report%伴有神经系统症状的白塞病1例

    陈威; 王恩普; 田青; 王华

    2007-01-01

    白塞病是一种慢性、系统性疾病.其特征为复发性口腔、生殖器溃疡,眼部炎症同时伴有皮肤、关节的异常.病程中偶可见神经系统受累.我们报告1例病程中出现神经系统症状的白塞病的患者病例.白塞病有可能被误诊为神经系统肿瘤.%Behcet's Disease is a chronic systemic disorder characterized by recurrent oral and genital ulcerations, ocular inflammation, having manifestations related to skin and joints. Neurologic signs can also be observed seldomly during the course of the disease. In this report a case of Behcet's disease with neurological involvement was presented.

  12. 自噬在神经系统疾病中的作用研究进展%The Role of Autophagy in Neurological Disease

    王莉; 刘媛; 伍亚民

    2015-01-01

    自噬是广泛存在于真核细胞内,通过溶酶体清除自身受损细胞器和异常蛋白的过程。在神经系统中,从神经退行性疾病到神经创伤,自噬都发挥了重要作用。深入研究神经细胞自噬发生及其分子调控机制,对全面了解自噬在神经系统疾病中的作用有重要意义。%Autophagy as a degradative process that widely exists in eukaryotic cells can clear intracellular damaged organelles and protein through lysosomes. It was found in recent studies that autophagy play an important role in neurological disease, both neurodegenerative and neurotraumatic diseases. Comprehensive understanding of autophagy effect and molecular mechanism in different types of neurological diseases can provide new strategies for disease prevention and the treatment. This review mainly focused on the inlfuence of autophagy on neurological diseases.

  13. Presymptomatic testing for Huntington's disease: a world wide survey. The World Federation of Neurology Research Group on Huntington's Disease.

    1993-01-01

    World wide data on presymptomatic testing for Huntington's disease using closely linked DNA markers show that 1479 persons at risk received completed test results up to the end of 1991. Testing has been carried out in 19 countries, with at least 88 centres involved, and numbers have levelled off after a peak in 1990. Only 5% of those at risk have been tested in six countries with the longest established programmes. Continued monitoring of international data will be of value in assessing the s...

  14. Correlation between neurologic defect and blood pressure regulation in acute phase of ischemic stroke caused by hypertension%高血压缺血性脑卒中神经功能缺损与急性期 血压调控的相关分析

    刘江; 翟明; 丁里; 朱之庄

    2001-01-01

    @@Background:Various managements were employed for acute phase of ischemic stroke caused by hypertension.Regulation of hypertension in acute phase was closely associated with prognosis of ischemic stroke . Objective:To study relationship between neurologic function defect and blood pressure regulation in acute phase of ischemic stroke caused by hypertension.

  15. Pulmonary Surfactants for Acute and Chronic Lung Diseases (Part II

    O. A. Rozenberg

    2014-01-01

    Full Text Available Part 2 of the review considers the problem of surfactant therapy for acute respiratory distress syndrome (ARDS in adults and young and old children. It gives information on the results of surfactant therapy and prevention of ARDS in patients with severe concurrent trauma, inhalation injuries, complications due to complex expanded chest surgery, or severe pneumonias, including bilateral pneumonia in the presence of A/H1N1 influenza. There are data on the use of a surfactant in obstetric care and prevention of primary graft dysfunction during lung transplantation. The results of longterm use of surfactant therapy in Russia, suggesting that death rates from ARDS may be substantially reduced (to 20% are discussed. Examples of surfactant therapy for other noncritical lung diseases, such as permanent athelectasis, chronic obstructive pulmonary diseases, and asthma, as well tuberculosis, are also considered.

  16. Acute Chagas disease in El Salvador 2000-2012 - Need for surveillance and control

    Emi Sasagawa; Ana Vilma Guevara de Aguilar; Marta Alicia Hernández de Ramírez; José Eduardo Romero Chévez; Jun Nakagawa; Rafael Antonio Cedillos; Kiyoshi Kita

    2014-01-01

    Several parasitological studies carried out in El Salvador between 2000-2012 showed a higher frequency of acute cases of Chagas disease than that in other Central American countries. There is an urgent need for improved Chagas disease surveillance and vector control programs in the provinces where acute Chagas disease occurs and throughout El Salvador as a whole.

  17. Acute erythroid leukemia: autopsy report of a rare disease

    Cristiane Rúbia Ferreira

    2011-12-01

    Full Text Available Acute erythroid leukemia (AEL is a rare subtype of acute myeloid leukemia(AML, characterized by predominant erythroid proliferation. The 2008 WorldHealth Organization (WHO classification of AML defined two AEL subtypes:erythroleukaemia (EL, in which erythroid precursors account for 50% or moreof all nucleated bone marrow cells and myeloblasts account for 20% or more ofthe nonerythroid cell population; and pure erythroid leukemia (PEL, in whicherythroid precursors account for 80% or more of all nucleated bone marrowcells. We report the case of an elderly female patient with wasting syndromeand pancytopenia without evidence of blasts in peripheral blood. A diagnosisof PEL was established on the basis of bone marrow biopsy findings. Thepatient died on postadmission day 20, and an autopsy was performed. Wereclassified the disease as EL on the basis of the autopsy findings, whichincluded myeloblasts accounting for more than 20% of the nonerythroid cellsin the bone marrow, as well as leukemic infiltration and myeloid metaplasia insolid organs, such as the liver, spleen, kidneys, adrenal glands, and abdominallymph nodes. A rare disease, AEL accounts for less than 5% of all AMLs and ispractically a diagnosis of exclusion. Autopsy reports of AEL are extremely rarein the literature. We demonstrate that in the case reported here, leukemia cellstended to infiltrate solid organs with myeloid metaplasia. Our findings alsoshow that a larger neoplastic bone marrow sample is crucial to the correctdiagnosis of EL, which is based on morphological and quantitative criteria.

  18. [Diagnosis of Alzheimer's disease in Brazil: cognitive and functional evaluation. Recommendations of the Scientific Department of Cognitive Neurology and Aging of the Brazilian Academy of Neurology].

    Nitrini, Ricardo; Caramelli, Paulo; Bottino, Cássio Machado de Campos; Damasceno, Benito Pereira; Brucki, Sonia Maria Dozzi; Anghinah, Renato

    2005-09-01

    The educational and cultural heterogeneity of the Brazilian population leads to peculiar characteristics regarding the diagnosis of Alzheimer's disease (AD). This consensus had the objective of recommending evidence-based guidelines for the clinical diagnosis of AD in Brazil. Studies on the diagnosis of AD published in Brazil were systematically evaluated in a thorough research of PUBMED and LILACS databases. For global cognitive evaluation, the Mini-Mental State Examination was recommended; for memory evaluation: delayed recall subtest of CERAD or of objects presented as drawings; attention: trail-making or digit-span; language: Boston naming, naming test from ADAS-Cog or NEUROPSI; executive functions: verbal fluency or clock-drawing; conceptualization and abstraction: similarities from CAMDEX or NEUROPSI; construction: drawings from CERAD. For functional evaluation, IQCODE, or Pfeffer Questionnaire or Bayer Scale for Activities of Daily Living was recommended. The panel concluded that the combined use of cognitive and functional evaluation based on interview with informant is recommended.

  19. Identification of a Common Epitope between Enterovirus 71 and Human MED25 Proteins Which May Explain Virus-Associated Neurological Disease

    Peihu Fan

    2015-03-01

    Full Text Available Enterovirus 71 (EV71 is a major causative pathogen of hand, foot and mouth disease with especially severe neurologic complications, which mainly account for fatalities from this disease. To date, the pathogenesis of EV71 in the central neurons system has remained unclear. Cytokine-mediated immunopathogenesis and nervous tissue damage by virus proliferation are two widely speculated causes of the neurological disease. To further study the pathogenesis, we identified a common epitope (co-epitope between EV71 VP1 and human mediator complex subunit 25 (MED25 highly expressed in brain stem. A monoclonal antibody (2H2 against the co-epitope was prepared, and its interaction with MED25 was examined by ELISA, immunofluorescence assay and Western blot in vitro and by live small animal imaging in vivo. Additionally, 2H2 could bind to both VP1 and MED25 with the affinity constant (Kd of 10−7 M as determined by the ForteBio Octet System. Intravenously injected 2H2 was distributed in brain stem of mice after seven days of EV71 infection. Interestingly, 2H2-like antibodies were detected in the serum of EV71-infected patients. These findings suggest that EV71 infection induces the production of antibodies that can bind to autoantigens expressed in nervous tissue and maybe further trigger autoimmune reactions resulting in neurological disease.

  20. Frequency and Pathological Phenotype of Bovine Astrovirus CH13/NeuroS1 Infection in Neurologically-Diseased Cattle: Towards Assessment of Causality

    Senija Selimovic-Hamza

    2017-01-01

    Full Text Available Next-generation sequencing (NGS has opened up the possibility of detecting new viruses in unresolved diseases. Recently, astrovirus brain infections have been identified in neurologically diseased humans and animals by NGS, among them bovine astrovirus (BoAstV CH13/NeuroS1, which has been found in brain tissues of cattle with non-suppurative encephalitis. Only a few studies are available on neurotropic astroviruses and a causal relationship between BoAstV CH13/NeuroS1 infections and neurological disease has been postulated, but remains unproven. Aiming at making a step forward towards assessing the causality, we collected brain samples of 97 cases of cattle diagnosed with unresolved non-suppurative encephalitis, and analyzed them by in situ hybridization and immunohistochemistry, to determine the frequency and neuropathological distribution of the BoAstV CH13/NeuroS1 and its topographical correlation to the pathology. We detected BoAstV CH13/NeuroS1 RNA or proteins in neurons throughout all parts of the central nervous system (CNS in 34% of all cases, but none were detected in cattle of the control group. In general, brain lesions had a high correlation with the presence of the virus. These findings show that a substantial proportion of cattle with non-suppurative encephalitis are infected with BoAstV CH13/NeuroS1 and further substantiate the causal relationship between neurological disease and astrovirus infections.

  1. Frequency and Pathological Phenotype of Bovine Astrovirus CH13/NeuroS1 Infection in Neurologically-Diseased Cattle: Towards Assessment of Causality

    Selimovic-Hamza, Senija; Boujon, Céline L.; Hilbe, Monika; Oevermann, Anna; Seuberlich, Torsten

    2017-01-01

    Next-generation sequencing (NGS) has opened up the possibility of detecting new viruses in unresolved diseases. Recently, astrovirus brain infections have been identified in neurologically diseased humans and animals by NGS, among them bovine astrovirus (BoAstV) CH13/NeuroS1, which has been found in brain tissues of cattle with non-suppurative encephalitis. Only a few studies are available on neurotropic astroviruses and a causal relationship between BoAstV CH13/NeuroS1 infections and neurological disease has been postulated, but remains unproven. Aiming at making a step forward towards assessing the causality, we collected brain samples of 97 cases of cattle diagnosed with unresolved non-suppurative encephalitis, and analyzed them by in situ hybridization and immunohistochemistry, to determine the frequency and neuropathological distribution of the BoAstV CH13/NeuroS1 and its topographical correlation to the pathology. We detected BoAstV CH13/NeuroS1 RNA or proteins in neurons throughout all parts of the central nervous system (CNS) in 34% of all cases, but none were detected in cattle of the control group. In general, brain lesions had a high correlation with the presence of the virus. These findings show that a substantial proportion of cattle with non-suppurative encephalitis are infected with BoAstV CH13/NeuroS1 and further substantiate the causal relationship between neurological disease and astrovirus infections. PMID:28106800

  2. Cannabinoids in neurology – Brazilian Academy of Neurology

    Sonia M. D. Brucki

    2015-04-01

    Full Text Available The use of cannabidiol in some neurological conditions was allowed by Conselho Regional de Medicina de São Paulo and by Agência Nacional de Vigilância Sanitária (ANVISA. Specialists on behalf of Academia Brasileira de Neurologia prepared a critical statement about use of cannabidiol and other cannabis derivatives in neurological diseases.

  3. β-Carboline Alkaloids and Essential Tremor: Exploring the Environmental Determinants of One of the Most Prevalent Neurological Diseases

    Elan D. Louis

    2010-01-01

    Full Text Available Essential tremor (ET is among the most prevalent neurological diseases, yet its etiology is not well understood. Susceptibility genotypes undoubtedly underlie many ET cases, although no genes have been identified thus far. Environmental factors are also likely to contribute to the etiology of ET. Harmane (1-methyl-9H-pyrido[3,4-β]indole is a potent, tremor-producing β-carboline alkaloid, and emerging literature has provided initial links between this neurotoxin and ET. In this report, we review this literature. Two studies, both in New York, have demonstrated higher blood harmane levels in ET cases than controls and, in one study, especially high levels in familial ET cases. Replication studies of populations outside of New York and studies of brain harmane levels in ET have yet to be undertaken. A small number of studies have explored several of the biological correlates of exposure to harmane in ET patients. Studies of the mechanisms of this putative elevation of harmane in ET have explored the role of increased dietary consumption, finding weak evidence of increased exogenous intake in male ET cases, and other studies have found initial evidence that the elevated harmane in ET might be due to a hereditarily reduced capacity to metabolize harmane to harmine (7-methoxy-1-methyl-9H-pyrido[3,4-β]-indole. Studies of harmane and its possible association with ET have been intriguing. Additional studies are needed to establish more definitively whether these toxic exposures are associated with ET and are of etiological importance.

  4. Acute onset anarthria without hepatic manifestation: a rare presentation of Wilson disease

    Verma, Rajesh; Bhandari, Aveg; Tiwari, Navin; Chaudhari, Tejendra S

    2013-01-01

    Wilson disease (WD) is one of the few inherited but treatable disorder mainly affecting the liver and brain resulting in severe disability or death if left untreated. Hence, it is important to keep a high index of suspicion for diagnosing this clinical entity in appropriate clinical settings. The clinical presentation can be quite variable and they may present solely with neurological features sans hepatic symptoms. Such neurological manifestations usually follow subacute to chronic course. A...

  5. Detection of acute hepatopancreatic necrosis disease (AHPND) in Mexico.

    Nunan, Linda; Lightner, Donald; Pantoja, Carlos; Gomez-Jimenez, Silvia

    2014-08-21

    Acute hepatopancreatic necrosis disease (AHPND), which has also been referred to as early mortality syndrome (EMS), initially emerged as a destructive disease of cultured shrimp species in Asia in 2009. The pathogen associated with the disease, Vibrio parahaemolyticus, subsequently spread to the Western Hemisphere and emerged in Mexico in early 2013. The spread to the Western Hemisphere is a major concern to shrimp producers in the region. To date, the only peer-reviewed published method for determining whether mortalities are due to AHPND is through histological examination. A novel PCR detection method was employed to assess samples from Mexico in order to confirm the presence of the pathogen in this country. This manuscript details the detection methods used to confirm the presence of AHPND in Mexico. Both immersion and per os challenge studies were used to expose the Penaeus vannamei to the bacteria in order to induce the disease. Histological analysis confirmed AHPND status following the challenge studies. Also provided are the details of the molecular test by PCR that was used for screening candidate V. parahaemolyticus isolates. A rapid PCR assay for detection of AHPND may help with early detection and help prevent the spread of AHPND to other countries.

  6. Predicción clínica de aspiración en pacientes con patología neurológica aguda CLINICAL PREDICTION OF ASPIRATION IN ACUTE NEUROLOGICAL PATIENTS

    Iván Caviedes S

    2005-01-01

    Full Text Available La disfunción glótica es una entidad prevalente en enfermos con patología neurológica aguda: compromete aproximadamente entre el 45 al 51% de los pacientes que padecen un ataque cerebrovascular, incrementando su mortalidad en una relación de 3/1. Su complicación principal es el síndrome aspirativo, que involucra diversas especialidades, entre ellas enfermedades respiratorias y neurología. El propósito de este estudio ha sido evaluar la fonación húmeda, el test del vaso de agua y la auscultación cervical como indicadores clínicos de aspiración frente a la certificación nasolaringoscópica, considerada patrón estándar de evaluación al lado de la cama del enfermo. Evaluamos prospectivamente durante un año a pacientes neurológicos agudos hospitalizados en una unidad de tratamiento intensivo. La fonación húmeda, el test del vaso de agua y la auscultación cervical demostraron sensibilidades de 66,7, 88,9 y 77,8%, con una especificidad respectiva de 85,2, 59,3 y 77,8%. Los valores predictivos positivo fueron de 60, 42,1 y 53,8%, con valores predictivos negativo de 88,5, 94,1 y 91,3% respectivamente. Nuestros resultados permiten afirmar que el monitoreo clínico de aspiración es un instrumento sencillo y valioso, que puede ser realizado al lado de la cama del enfermoGlottic dysfunction is a frequent condition in patients with acute neurological diseases: the incidence in stroke patients is between 45 - 51% increasing mortality three times. The principal complication is aspiration, which demands the involvement of pulmonary physicians and neurologists. The purpose of this study is to evaluate wet voice, water swallow test and cervical auscultation as clinical predictors of aspiration using endoscopical observation as a gold standard. During a period of one year we have prospectively evaluated these tests in acute neurological patients hospitalized in an intensive care unit. Wet voice, 3 oz water swallow test and cervical

  7. MRI findings of enterovirus 71-infected hand, foot and mouth disease complicated with neurological impairment%EV71病毒感染手足口病合并神经系统损害的MRI研究进展

    谢歆昕(综述); 陈旺生; 陈锋; 李建军(审校)

    2016-01-01

    Hand, foot and mouth disease (HFMD) is an infectious disease caused by multiple enterovirus, which mostly happens in preschool children, and is mainly transmitted by routes of digestive tract, respiratory tract and close contact. The major clinical manifestations include fever, rash in mouth and extremities, herpes, and various neuro-logical complications in severe cases, such as encephalitis in brain stem, aseptic meningitis, acute flaccid paralysis (AFP), which result in high morbidity and mortality. Therefore, to diagnose HFMD with neurological complications ear-lier, to establish a treatment plan, and to assess the therapeutic effect are significant for reducing the mortality and mor-bidity. MRI is nowadays an effective method to investigate HFMD with neurological complications. The research prog-ress of MRI regarding HFMD complicated with neurological impairment were reviewed as follows.%手足口病(HFMD)是由多种肠道病毒引起的传染性疾病,好发于学龄前儿童。其主要传播途径为消化道、呼吸道以及密切接触;主要临床表现为发热,口腔和四肢末端的斑丘疹、疱疹,重者可引起多种神经系统并发症,如脑干脑炎、无菌性脑膜炎、急性驰缓性麻痹(AFP)等,致残率和病死率高。因此,早期诊断手足口病并发神经系统损害,制定治疗方案以及治疗效果的评估对降低重症手足口病病死率、致残率具有重要意义,而磁共振(MRI)是目前监测HFMD并发神经系统损害的有效手段。本文就手足口病并神经系统损害的MRI研究进展做一简要综述。

  8. History of neurologic examination books.

    Boes, Christopher J

    2015-04-01

    The objective of this study was to create an annotated list of textbooks dedicated to teaching the neurologic examination. Monographs focused primarily on the complete neurologic examination published prior to 1960 were reviewed. This analysis was limited to books with the word "examination" in the title, with exceptions for the texts of Robert Wartenberg and Gordon Holmes. Ten manuals met the criteria. Works dedicated primarily to the neurologic examination without a major emphasis on disease description or treatment first appeared in the early 1900s. Georg Monrad-Krohn's "Blue Book of Neurology" ("Blue Bible") was the earliest success. These treatises served the important purpose of educating trainees on proper neurologic examination technique. They could make a reputation and be profitable for the author (Monrad-Krohn), highlight how neurology was practiced at individual institutions (McKendree, Denny-Brown, Holmes, DeJong, Mayo Clinic authors), and honor retiring mentors (Mayo Clinic authors).

  9. [Acute hepatitis in a patient with adult onset Still disease].

    Gallo, M; Calvanese, A; Oscuro, F; Gallo, A; Caso, P; Annibale, E; Farinato, N

    1997-04-01

    Liver abnormalities in the course of Adult Onset Still's Disease (AOSD), both in form of hepatomegaly and elevation of hepatic enzymes, have been reported in up to three-quarts of the affected patients. These abnormalities may reflect disease activity or may be induced by drugs. Only in a few of this patients a liver biopsy was performed. However liver histology has shown, generally, non specific abnormalities or even normal pictures. We have recently observed a 47-year-old woman with a febrile illness started five months before, who after pertinent investigation was diagnosed as AOSD (according to criteria of Yamaguchi et al.). Apart from laboratory findings characteristic of an inflammatory disease, in absence of drug therapies the biochemical data showed raised levels of aspartate aminotransferase, alanine aminotransferase, alkaline phosphatase and aminoglutamil transferase. Serological tests for either viral hepatitis viruses (HAV, HBV, HCV) or other viruses were negative. Ultrasonographic examination of gallbladder and bile ducts did not find gallstones or other abnormalities. A liver biopsy was performed, which histopathologic examination showed moderate fatty methamorphosis with focal areas of hepatocellular swelling with minimal necrosis, mild Kuppfer cell hyperplasia, portal and sinusoidal infiltrates of mononuclear cells. This picture consisted with the diagnosis of an acute unspecific reactive hepatitis.

  10. Acute alithiasic cholecystitis: a not so rare disease

    Javier Blasco-Alonso

    2014-08-01

    Full Text Available Introduction: Acute acalculous cholecystitis (AAC occurs more frequently in critically ill patients, in the immediate postoperative period, after trauma or extensive burns. It has a high rate of morbidity and mortality. Ischemia, infection and vesicular stasis are determinants in its pathogenesis. Material and method: Retrospective study including all cases of AAC diagnosed in our pediatric intensive care unit between January 1997 and December 2012. Results: We included 7 patients, all associated with viral or bacterial infection. All of them suffered from abdominal pain, mainly localized in the right upper quadrant, jaundice and dark urine. Abdominal ultrasound showed thickening and hypervascularity of the gallbladder wall in all cases. The outcome was satisfactory without surgery in all patients. Conclusions: The clinical presentation is oligosymptomatic within severe systemic diseases. The AAC should be suspected in the appearance of any abdominal pain with jaundice/dark urine and hypertransaminasemia in patients suffering from critical or serious infections.

  11. CT appearance of acute inflammatory disease of the renal interstitium

    Gold, R.P. (New York Medical Coll., Valhalla); McClennan, B.L.; Rottenberg, R.R.

    1983-08-01

    Today, infection remains the most common disease of the urinary tract and constitutes almost 75% of patient problems requiring urologic evaluation. There have been several major factors responsible for our better understanding of the nature and pathophysiology of urinary tract infection. One has been quantitated urine bacteriology and another, the discovery that a significant part of the apparently healthy adult female population has asymptomatic bacteriuria. Abnormal conditions such as neurogenic bladder, bladder malignancy, prolonged catheter drainage and reflux, altered host resistance, diabetes mellitus, and urinary tract obstruction, as well as pregnancy, may either predispose to or be implicated in the pathogenesis of urinary tract infection. There is a wide range of conditions that result in acute renal inflammation and those under discussion affect primarily the interstitium. This term refers to the connective tissue elements separating the tubules in the cortex and medulla. Hence, the interstitial nephritides are to be distinguished from the glomerulonephritides and fall into two general etiologic categories: infectious and noninfectious.

  12. Acute Psychosis as Major Clinical Presentation of Legionnaires’ Disease

    Ricardo Coentre

    2016-01-01

    Full Text Available We report a case of a 61-year-old woman who presented with acute psychosis as a major manifestation of Legionnaires’ disease in the absence of other neuropsychiatric symptoms. Clinical history revealed dry cough and nausea. Observation showed fever and auscultation crackles in the lower lobe of the right lung. Laboratory testing demonstrated elevated C-reactive protein and lung chest radiograph showed patchy peribronchial and right lower lobe consolidation. Soon after admission, she started producing purulent sputum. Epidemiological data suggested Legionella pneumophila as possible cause of the clinical picture that was confirmed by urinary antigen detection and polymerase chain reaction of the sputum. She was treated with levofloxacin 750 mg/day for 10 days with complete remission of pulmonary and psychiatric symptoms. She has not had further psychotic symptoms.

  13. Vaccination and neurological disorders

    Anastasia Gkampeta

    2015-12-01

    Full Text Available Active immunization of children has been proven very effective in elimination of life threatening complications of many infectious diseases in developed countries. However, as vaccination-preventable infectious diseases and their complications have become rare, the interest focuses on immunization-related adverse reactions. Unfortunately, fear of vaccination-related adverse effects can led to decreased vaccination coverage and subsequent epidemics of infectious diseases. This review includes reports about possible side effects following vaccinations in children with neurological disorders and also published recommendations about vaccinating children with neurological disorders. From all international published data anyone can conclude that vaccines are safer than ever before, but the challenge remains to convey this message to society.

  14. POEMS syndrome with Guillan-Barre syndrome-like acute onset: a case report and review of neurological progression in 30 cases.

    Isose, S; Misawa, S; Kanai, K; Shibuya, K; Sekiguchi, Y; Nasu, S; Fujimaki, Y; Noto, Y; Nakaseko, C; Kuwabara, S

    2011-06-01

    POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes) syndrome is a rare cause of demyelinating neuropathy with monoclonal plasma cell proliferation, and POEMS neuropathy is usually chronically progressive. Herein, the authors report a 34-year-old woman with POEMS syndrome presenting as acute polyneuropathy. Within 2 weeks of disease onset, she became unable to walk with electrodiagnostic features of demyelination and was initially diagnosed as having Guillan-Barré syndrome. Other systemic features (oedema and skin changes) developed later, and an elevated serum level of vascular endothelial growth factor led to the diagnosis of POEMS syndrome. She received high-dose chemotherapy with autologous peripheral blood stem cell transplantation, resulting in good recovery. The authors also reviewed patterns and speed of progression of neuropathy in the 30 patients with POEMS syndrome; 22 (73%) of them were unable to walk independently with the median period of 9.5 months from POEMS onset (range 0.5-51 months). Whereas the speed of neuropathy progression varies considerably among patients, some POEMS patients can show acute or subacute polyneuropathy. The early diagnosis and treatment could result in rapid improvement as shown in the present patient.

  15. Moyamoya disease presenting as acute onset cortical blindness: a case report

    Maniram Dudi

    2016-03-01

    Full Text Available We report a case where acute onset cortical blindness is the mode of presentation in Moyamoya disease. Cortical blindness is very rare presenting symptom of Moyamoya disease. Progressive visual loss and homonymous anopsia has been described previously, but this case had acute visual loss.

  16. Moyamoya disease presenting as acute onset cortical blindness: a case report

    Maniram Dudi; Rajeev Bansal; Trilochan Srivastava; Sardana R.V.

    2016-01-01

    We report a case where acute onset cortical blindness is the mode of presentation in Moyamoya disease. Cortical blindness is very rare presenting symptom of Moyamoya disease. Progressive visual loss and homonymous anopsia has been described previously, but this case had acute visual loss.

  17. NEUROLOGIC MUSIC THERAPY TRAINING FOR MOBILITY AND STABILITY REHABILITATION WITH PARKINSON’S DISEASE – A PILOT STUDY.

    Anna A. Bukowska

    2016-01-01

    Full Text Available Idiopathic Parkinson’s Disease (PD is a progressive condition with gait disturbance and balance disorder as the main symptoms. Previous research studies focused on the application of Rhythmic Auditory Stimulation (RAS in PD gait rehabilitation. The key hypothesis of this pilot study, however, assumes the major role of the combination of all three Neurologic Music Therapy (NMT sensorimotor techniques in improving spatio-temporal gait parameters, and postural stability in the course of PD. The 55 PD-diagnosed subjects invited to the study were divided into two groups: 30 in the experimental and 25 in the control group. Inclusion criteria included Hoehn & Yahr stage 2 or 3, the ability to walk independently without any aid and stable pharmacological treatment for the duration of the experiment. In order to evaluate the efficacy of the chosen therapy procedure the following measures were applied: Optoelectrical 3D Movement Analysis System BTS Smart for gait, and Computerized Dynamic Posturography CQ Stab for stability and balance . All measures were conducted both before and after the therapy cycle. The subjects from the experimental group attended music therapy sessions 4 times a week for 4 weeks. Therapeutic Instrumental Music Performance (TIMP, Pattern Sensory Enhancement (PSE and Rhythmic Auditory Stimulation (RAS were used in every 45-minute session for practicing daily life activities, balance, pre-gait and gait pattern. Percussion instruments, the metronome and rhythmic music were the basis for each session. The subjects from the control group were asked to stay active and perform daily life activities between the measures. The research showed that the combination of the three NMT sensorimotor techniques can be used to improve gait and other rhythmical activities in PD rehabilitation.The results demonstrated significant improvement in the majority of the spatiotemporal gait parameters in the experimental group in comparison to the control

  18. A rare disease in the differential diagnosis of acute pancreatitis: acute brucellosis.

    Berber, Ilhami; Erkurt, Mehmet Ali; Yetkin, Funda; Unlu, Serkan; Yilmaz, Sami; Bentli, Recep; Bazna, Sezai

    2014-01-01

    Some infectious organisms may give rise to acute pancreatitis; brucellosis, however, extremely rarely leads to acute pancreatitis. A 40-year-old man was diagnosed with acute pancreatitis, the etiology of which was determined to be acute brucellosis. The patient was discharged without complications approximately 15 days after the initiation of trimethoprim-sulfamethoxazole and doxycycline treatment. Brucella infections may rarely be complicated by acute pancreatitis. Thus, brucellosis should be remembered in the etiology of acute pancreatitis in regions such as Turkey, where Brucella infections are endemic.

  19. Inebilizumab, a B Cell-Depleting Anti-CD19 Antibody for the Treatment of Autoimmune Neurological Diseases: Insights from Preclinical Studies

    Ding Chen

    2016-11-01

    Full Text Available Exaggerated or inappropriate responses by B cells are an important feature in many types of autoimmune neurological diseases. The recent success of B-cell depletion in the treatment of multiple sclerosis (MS has stimulated the development of novel B-cell-targeting therapies with the potential for improved efficacy. CD19 has emerged as a promising target for the depletion of B cells as well as CD19-positive plasmablasts and plasma cells. Inebilizumab (MEDI-551, an anti-CD19 antibody with enhanced antibody-dependent cell-mediated cytotoxicity against B cells, is currently being evaluated in MS and neuromyelitis optica. This review discusses the role of B cells in autoimmune neurological disorders, summarizes the development of inebilizumab, and analyzes the recent results for inebilizumab treatment in an autoimmune encephalitis mouse model. The novel insights obtained from these preclinical studies can potentially guide future investigation of inebilizumab in patients.

  20. Diagnostic criteria for acute liver failure due to Wilson disease

    Christoph Eisenbach; Olivia Sieg; Wolfgang Stremmel; Jens Encke; Uta Merle

    2007-01-01

    AIM: To describe the diagnostic criteria for acute liver failure due to Wilson disease (WD), which is an uncommon cause of acute liver failure (ALF).METHODS: We compared findings of patients presenting with ALF due to WD to those with ALF of other etiologies.RESULTS: Previously described criteria, such as low alkaline phosphatase activity, ratio of low alkaline phosphatase to total bilirubin or ratio of high aspartate aminotransferase (AST) to alanine aminotransferase (ALT), failed to identify patients with ALF due to WD. There were significant differences in low ALT and AST activities (53 ± 43 vs 1982 ± 938, P < 0.0001 and 87 ± 44 vs 2756 ± 2941, P = 0.037, respectively), low choline esterase activity (1.79 ± 1.2 vs 4.30 ± 1.2, P = 0.009), high urine copper concentrations (93.4 ± 144.0 vs 3.5 ± 1.8, P = 0.001) and low hemoglobin (7.0 ± 2.2 vs 12.6 ± 1.8, P < 0.0001) in patients with ALF caused by WD as compared with other etiologies. Interestingly, 4 of 7 patients with ALF due to WD survived without liver transplantation.CONCLUSION: In ALF, these criteria can help establish a diagnosis of WD. Where applicable, slit-lamp examination for presence of Kayser-Fleischer rings and liver biopsy for determination of hepatic copper concentration still remain important for the diagnosis of ALF due to WD. The need for liver transplantation should be evaluated carefully as the prognosis is not necessarily fatal.

  1. Urgencias neurológicas y guardias de Neurología The problem of neurological emergencies and the need for specific neurology shifts

    A. Gómez Ibáñez

    2008-01-01

    ? Neurological emergencies account for between 2.6% and 14% of medical emergencies. Stroke represents a third of all neurological emergencies, while the diagnoses of acute cerebrovascular disease, epilepsy and cephalea constitute 50% of all neurological care in the emergency department. On the basis of quality of care criteria and professional competence, the best care for patients with a neurological emergency is provided by a specialist in neurology. The implementation of specific neurology shifts, with a 24 hour physical presence, is associated with greater quality of care, better diagnostic and therapeutic orientation from the moment the patient arrives in emergency department, reduces unnecessary admissions, reduces costs and strengthens the neurology service.

  2. Endocrine disorders and the neurologic manifestations

    Yu, Jeesuk

    2014-01-01

    The nervous system and the endocrine system are closely interrelated and both involved intimately in maintaining homeostasis. Endocrine dysfunctions may lead to various neurologic manifestations such as headache, myopathy, and acute encephalopathy including coma. It is important to recognize the neurologic signs and symptoms caused by the endocrine disorders while managing endocrine disorders. This article provides an overview of the neurologic manifestations found in various endocrine disord...

  3. Review of Elephant Endotheliotropic Herpesviruses and Acute Hemorrhagic Disease.

    Long, Simon Y; Latimer, Erin M; Hayward, Gary S

    2016-01-01

    More than 100 young captive and wild Asian elephants are known to have died from a rapid-onset, acute hemorrhagic disease caused primarily by multiple distinct strains of two closely related chimeric variants of a novel herpesvirus species designated elephant endotheliotropic herpesvirus (EEHV1A and EEHV1B). These and two other species of Probosciviruses (EEHV4 and EEHV5) are evidently ancient and likely nearly ubiquitous asymptomatic infections of adult Asian elephants worldwide that are occasionally shed in trunk wash secretions. Although only a handful of similar cases have been observed in African elephants, they also have proved to harbor their own multiple and distinct species of Probosciviruses-EEHV2, EEHV3, EEHV6, and EEHV7-found in lung and skin nodules or saliva. For reasons that are not yet understood, approximately 20% of Asian elephant calves appear to be susceptible to the disease when primary infections are not controlled by normal innate cellular and humoral immune responses. Sensitive specific polymerase chain reaction (PCR) DNA blood tests have been developed, routine monitoring has been established, the complete large DNA genomes of each of the four Asian EEHV species have now been sequenced, and PCR gene subtyping has provided unambiguous evidence that this is a sporadic rather than epidemic disease that it is not being spread among zoos or other elephant housing facilities. Nevertheless, researchers have not yet been able to propagate EEHV in cell culture, determine whether or not human antiherpesvirus drugs are effective inhibitors, or develop serology assays that can distinguish between antibodies against the multiple different EEHV species.

  4. Rodent neonatal germinal matrix hemorrhage mimics the human brain injury, neurological consequences, and post-hemorrhagic hydrocephalus

    2012-01-01

    Germinal matrix hemorrhage (GMH) is the most common neurological disease of premature newborns. GMH causes neurological sequelae such as cerebral palsy, post-hemorrhagic hydrocephalus, and mental retardation. Despite this, there is no standardized animal model of spontaneous GMH using newborn rats to depict the condition. We asked whether stereotactic injection of collagenase type VII (0.3 U) into the ganglionic eminence of neonatal rats would reproduce the acute brain injury, gliosis, hydroc...

  5. Acute-phase reactants in periodontal disease: current concepts and future implications.

    Archana, Vilasan; Ambili, Ranjith; Nisha, Krishnavilasam Jayakumary; Seba, Abraham; Preeja, Chandran

    2015-05-01

    Periodontal disease has been linked to adverse cardiovascular events by unknown mechanisms. C-reactive protein is a systemic marker released during the acute phase of an inflammatory response and is a prognostic marker for cardiovascular disease, with elevated serum levels being reported during periodontal disease. Studies also reported elevated levels of various other acute-phase reactants in periodontal disease. It has been reported extensively in the literature that treatment of periodontal infections can significantly lower serum levels of C-reactive protein. Therefore, an understanding of the relationship between acute-phase response and the progression of periodontal disease and other systemic health complications would have a profound effect on the periodontal treatment strategies. In view of this fact, the present review highlights an overview of acute-phase reactants and their role in periodontal disease.

  6. Hypokalemic Rhabdomyolysis Induced Acute Renal Failure As a Presentation of Coeliac Disease

    Funda Sarı

    2012-03-01

    Full Text Available Adult coeliac disease commonly presents without classical symptoms as chronic diarrhea and weight loss. We describe the case of a 31-year-old woman with persistent life-threatening hypokalemia, acute renal failure, and acute quadriplegia due to diarrhea that had continued for one month. Although there are cases of coeliac disease diagnosed with hypokalemic rhabdomyolysis in the literature, none of the cases developed acute renal failure. This is the first case in the literature diagnosed with acute renal failure due to hypokalemic rhabdomyolysis as a presentation of coeliac disease. In acute renal failure cases that present with hypokalemic rhabdomyolysis due to severe diarrhea, coeliac disease should be considered as a differential diagnosis despite the negative antigliadin IgA antibody.

  7. Respiratory viruses in acute exacerbations of chronic obstructive pulmonary disease

    Koul, Parvaiz A; Mir, Hyder; Akram, Shabir; Potdar, Varsha; Chadha, Mandeep S

    2017-01-01

    Objective: Acute exacerbations of chronic obstructive pulmonary disease (AECOPD) cause significant morbidity, mortality, and an inexorable decline of lung function. Data from developed countries have shown viruses to be important causes of AECOPD, but data from developing countries like India are scant. We set out to determine the contribution of viruses in the causation of hospitalized patients with AECOPD. Methods: Twin nasopharyngeal/oropharyngeal swabs collected from 233 patients admitted with an acute AECOPD and tested for respiratory viruses including respiratory syncytial virus A and B, parainfluenza were (PIV) 1, 2, 3, and 4, human metapneumovirus (hMPV) A and B, influenza A and B, enterovirus, corona NL65, OC43, and 229E viruses, adenovirus 2 and 4, rhinovirus, and bocavirus, by duplex real time reverse-transcription polymerase chain reaction (qRT-PCR) using CDC approved primers and probes. Samples positive for influenza A were subtyped for A/H1N1pdm09 and A/H3N2 whereas influenza B samples were subtyped into B/Yamagata and B/Victoria subtypes, using primers and probes recommended by CDC, USA. Results: Respiratory viruses were detected in 46 (19.7%) cases, influenza A/H3N2 and rhinoviruses being the most common viruses detected. More than one virus was isolated in four cases consisting of hMPV-B + adeno-2 + Inf-B; rhino + H3N2, PIV-1 + rhino; and PIV-1+ hMPV-B in one case each. Ancillary supportive therapeutic measures included bronchodilators, antibiotics, steroids, and ventilation (noninvasive in 42 and invasive in 4). Antiviral therapy was instituted in influenza-positive patients. Three patients with A/H3N2 infection died during hospitalization. Conclusions: We conclude that respiratory viruses are important contributors to AECOPD in India. Our data calls for prompt investigation during an exacerbation for viruses to obviate inappropriate antibiotic use and institute antiviral therapy in viral disease amenable to antiviral therapy. Appropriate

  8. Correlation between serum fructosamine and hyperglycemia in patients with acute cerebrovascular disease

    Kaiqiu Chu; Pengpeng Liu; Lijuan Tan; Shuhua Zhou; Lisheng Ren

    2006-01-01

    BACKGROUND: Diabetes mellitus is one of the risk factors in patients with acute cerebral disease, and always leads to stroke or get it worse. There is often a high level of blood glucose in those patients with diabetes mellitus and cerebral disease, but it is hard to distinguish from both kinds of hyperglycemia. Serum fructosamine is said to be correlated with blood glucose.OBJECTIVE: To explore the relationship between serum fructosamine and blood glucose in patients with acute cerebrovascular disease.DESTGN: A case-controlled study.SETTINGS: Department of Clinical Laboratory, Health Department for Cadres and Department of Neurology of Affiliated Hospital, Qingdao University Medical College.PARTICIPANTS: Forty-eight inpatients and outpatients with cerebrovascular diseases were selected from the Department of Neurology, Affiliated Hospital of Qingdao University Medical College from December 2004 to April 2005. All the patients were confirmed with CT and MRI. There were 25 patients with diabetes mellitus secondary cerebrovascular diseases, who met the diagnostic standards of diabetes mellitus set by WHO,including 12 males and 13 females with an average of (60±8) years old, the course of diabetes mellitus ranged from 1 to 21 years.. The other 23 patients had no diabetes mellitus (without diabetes mellitus group), including 14 males and 9 females with an average of (62±6) years old. Meanwhile, another 50 healthy physical examinees in the hospital were selected as control group, including 26 males and 24 females with the average age of (62±5) years old. Informed content was obtained from all the participants.METHODS: Venous blood was drawn from all the participants, and content of blood glucose was assayed by means of glucose oxidase, and the concentration of serum fructosamine was determined by nitroblue tetrazolium colorimetric method. Comparison between groups was performed by the analysis of variance and q test, and the correlation was tested by linear

  9. Acute rheumatic fever and rheumatic heart disease among children--American Samoa, 2011-2012.

    Beaudoin, Amanda; Edison, Laura; Introcaso, Camille E; Goh, Lucy; Marrone, James; Mejia, Amelita; Van Beneden, Chris

    2015-05-29

    Acute rheumatic fever is a nonsuppurative, immune-mediated consequence of group A streptococcal pharyngitis (strep throat). Recurrent or severe acute rheumatic fever can cause permanent cardiac valve damage and rheumatic heart disease, which increases the risk for cardiac conditions (e.g., infective endocarditis, stroke, and congestive heart failure). Antibiotics can prevent acute rheumatic fever if administered no more than 9 days after symptom onset. Long-term benzathine penicillin G (BPG) injections are effective in preventing recurrent acute rheumatic fever attacks and are recommended to be administered every 3-4 weeks for 10 years or until age 21 years to children who receive a diagnosis of acute rheumatic fever. During August 2013, in response to anecdotal reports of increasing rates of acute rheumatic fever and rheumatic heart disease, CDC collaborated with the American Samoa Department of Health and the Lyndon B. Johnson Tropical Medical Center (the only hospital in American Samoa) to quantify the number of cases of pediatric acute rheumatic fever and rheumatic heart disease in American Samoa and to assess the potential roles of missed pharyngitis diagnosis, lack of timely prophylaxis prescription, and compliance with prescribed BPG prophylaxis. Using data from medical records, acute rheumatic fever incidence was calculated as 1.1 and 1.5 cases per 1,000 children aged ≤18 years in 2011 and 2012, respectively; 49% of those with acute rheumatic fever subsequently received a diagnosis of rheumatic heart disease. Noncompliance with recommended prophylaxis with BPG after physician-diagnosed acute rheumatic fever was noted for 22 (34%) of 65 patients. Rheumatic heart disease point prevalence was 3.2 cases per 1,000 children in August 2013. Establishment of a coordinated acute rheumatic fever and rheumatic heart disease control program in American Samoa, likely would improve diagnosis, treatment, and patient compliance with BPG prophylaxis.

  10. IMMUNOBIOLOGY OF ACUTE GRAFT-VERSUS-HOST DISEASE

    G. A. Efimov

    2015-01-01

    significant limitation for clinical applications of stem cell transplantation. Severe immunesuppression or depletion of mature donor T cells from the transplant leads to increased probability of relapse and weakens anti-infectious immunity. Hence, further search for alternative, more specific ways to prevent GVHD is required. This review will focus on the mechanisms of alloreactive T lymphocyte clone development and key pathogenetic stages of acute “graft versus host” disease.

  11. Facial Weakness, Otalgia, and Hemifacial Spasm: A Novel Neurological Syndrome in a Case-Series of 3 Patients With Rheumatic Disease.

    Birnbaum, Julius

    2015-10-01

    Bell palsy occurs in different rheumatic diseases, causes hemifacial weakness, and targets the motor branch of the 7th cranial nerve. Severe, persistent, and refractory otalgia having features of neuropathic pain (ie, burning and allodynic) does not characteristically occur with Bell palsy. Whereas aberrant regeneration of the 7th cranial nerve occurring after a Bell palsy may lead to a variety of clinical findings, hemifacial spasm only rarely occurs. We identified in 3 rheumatic disease patients (2 with Sjögren syndrome, 1 with rheumatoid arthritis) a previously unreported neurological syndrome of facial weakness, otalgia with neuropathic pain features, and hemifacial spasm. We characterized symptoms, examination findings, and response to therapy. All 3 patients experienced vertigo, as well as severe otalgia which persisted after mild facial weakness had completely resolved within 1 to 4 weeks. The allodynic nature of otalgia was striking. Two patients were rendered homebound, as even the barest graze of outdoor breezes caused intolerable ear pain. Patients developed hemifacial spasm either at the time of or within 3 months of facial weakness. Two patients had a polyphasic course, with recurrent episodes of facial weakness and increased otalgia. In all cases, otalgia and hemifacial spasm were unresponsive to neuropathic pain regimens, but responded in 1 case to intravenous immunoglobulin therapy. No patients had vesicles or varicella zoster virus in spinal-fluid studies. We have defined a novel neurological syndrome in 3 rheumatic disease patients, characterized by facial weakness, otalgia, and hemifacial spasm. As described in infectious disorders, the combination of otalgia, facial weakness, and 8th cranial nerve deficits suggests damage to the geniculate ganglia (ie, the sensory ganglia of the 7th cranial nerve), with contiguous involvement of other cranial nerves causing facial weakness and vertigo. However, the relapsing nature and association with

  12. EXTRAMEDULLARY DISEASE IN ACUTE PROMYELOCYTIC LEUKEMIA: TWO-IN-ONE DISEASE

    Giorgina Specchia

    2011-01-01

    Full Text Available

    In acute promyelocytic leukemia (APL, extramedullary disease (EMD is particularly rare and shows special clinical and biological features. It is estimated that about 3–5% of APL patients will suffer extramedullary relapse. The most common site of EMD in APL is the CNS.  At present, there are still many issues of EMD in APL needing further clarification, including pathogenesis, risk factors, prognosis and treatment. A better understanding of the biological mechanisms underlying EMD is important to be able to devise more effective CNS prophylaxis and induction-consolidation therapeutic strategies

  13. EXTRAMEDULLARY DISEASE IN ACUTE PROMYELOCYTIC LEUKEMIA: TWO-IN-ONE DISEASE

    Francesco Albano

    2011-12-01

    Full Text Available In acute promyelocytic leukemia (APL, extramedullary disease (EMD is particularly rare and shows special clinical and biological features. It is estimated that about 3–5% of APL patients will suffer extramedullary relapse. The most common site of EMD in APL is the CNS.  At present, there are still many issues of EMD in APL needing further clarification, including pathogenesis, risk factors, prognosis and treatment. A better understanding of the biological mechanisms underlying EMD is important to be able to devise more effective CNS prophylaxis and induction-consolidation therapeutic strategies

  14. Slowing down of recovery as generic risk marker for acute severity transitions in chronic diseases

    Olde Rikkert, M.G.M.; Dakos, V.; Buchman, T.; Boer, de R.; Glass, L.; Cramer, A.O.J.; Levin, S.; Nes, van E.H.; Sugihara, G.; Ferrari, M.D.; Tolner, E.A.; Leemput, van de I.A.

    2016-01-01

    OBJECTIVE:

    We propose a novel paradigm to predict acute attacks and exacerbations in chronic episodic disorders such as asthma, cardiac arrhythmias, migraine, epilepsy, and depression. A better generic understanding of acute transitions in chronic dynamic diseases is increasingly important

  15. Effects of chronic kidney disease on platelet response to antiplatelet therapy in acute myocardial infarction patients

    邓捷

    2012-01-01

    Objective To elucidate the effects of dual antiplatelet therapy on platelet response in acute myocardial infarction patients with chronic kidney disease. Methods From September 2011 to June 2012,a total of 195 acute myocardial infarction patients with drug eluting stent implanting were enrolled. Among them,133 cases had normal

  16. Shifts in the age distribution and from acute to chronic coronary heart disease hospitalizations

    Koopman, Carla; Bots, Michiel L.; Van Dis, Ineke; Vaartjes, Ilonca

    2016-01-01

    Background Shifts in the burden of coronary heart disease (CHD) from an acute to chronic illness have important public health consequences. Objective To assess age-sex-specific time trends in rates and characteristics of acute and chronic forms of CHD hospital admissions in the Netherlands. Methods

  17. Chagas' disease: study of congenital transmission in cases of acute maternal infection

    Moretti Edgardo

    2005-01-01

    Full Text Available We studied three pregnant women with acute chagasic infection. Two patients, infected in the third trimester of pregnancy, had uninfected children. The third patient, infected earlier, had an infected newborn. These results encourage research on risk factors of transmission and on medical decisions concerning pregnant women with acute Chagas' disease.

  18. {sup 1}H-MRS for the diagnosis of acute disseminated encephalomyelitis: insight into the acute-disease stage

    Ben Sira, Liat; Miller, Elka [Tel Aviv Sourasky Medical Center, Department of Radiology, Tel-Aviv (Israel); Artzi, Moran [Tel Aviv Sourasky Medical Center, Functional Brain Imaging Center, Tel-Aviv (Israel); Tel Aviv University, Sackler Faculty of Medicine, Tel Aviv (Israel); Fattal-Valevski, Aviva; Constantini, Shlomi [Tel Aviv University, Sackler Faculty of Medicine, Tel Aviv (Israel); Tel Aviv Medical Center, Paediatric Neurology Unit, The Paediatric Neurosurgery Department, Tel Aviv (Israel); Ben Bashat, Dafna [Tel Aviv Sourasky Medical Center, Functional Brain Imaging Center, Tel-Aviv (Israel)

    2010-01-15

    Acute disseminated encephalomyelitis (ADEM) is a demyelinating disorder of the central nervous system (CNS). Differentiating ADEM from other inflammatory disorders, such as multiple sclerosis, is not always conclusive using conventional MRI. To evaluate longitudinal magnetic resonance spectroscopy (MRS) changes that distinguish ADEM from other inflammatory disorders. MRI/MRS scans were performed in seven patients with ADEM during the acute and chronic phases of the disease. Partial recovery was detected between the acute and chronic phases in choline/creatine ratio. Major elevation of lipids and reduction in myo-inositol/creatine ratio was detected in all patients during the acute phase, followed by a reduction in lipids peak and elevation above normal in myo-inositol/creatine ratio during the chronic phase. Consistent and unique MRS changes in metabolite ratios between the acute and chronic presentations of the disease were found. To the best of our knowledge, these patterns have not been described in other inflammatory disorders and might assist in the early diagnosis of ADEM. (orig.)

  19. Anterior D-rod and titanium mesh fixation for acute mid-lumbar burst fracture with incomplete neurologic deficits A prospective study of 56 consecutive patients

    Zhe-yuan Huang

    2015-01-01

    Conclusion: Good mid term clinicoradiological results of anterior decompression with D-rod and titanium mesh fixation for suitable patients with mid-lumbar burst fractures with incomplete neurologic deficits can be achieved. The incident rate of complications was low. D-rod is a reliable implant and has some potential advantages in L4 vertebral fractures.

  20. Acute Myocardial Infarction: The First Manifestation of Ischemic Heart Disease and Relation to Risk Factors

    Manfroi Waldomiro Carlos

    2002-01-01

    Full Text Available OBJECTIVE: To assess the association between cardiovascular risk factors and acute myocardial infarction as the first manifestation of ischemic heart disease, correlating them with coronary angiographic findings. METHODS: We carried out a cross-sectional study of 104 patients with previous