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Sample records for acute neurological diseases

  1. [Demyelinating diseases in children with acute neurological symptoms].

    Olofsson, Isa Amalie; Skov, Liselotte; Miranda, Maria Jose

    2015-12-01

    Demyelinating diseases in children is a broad group of illnesses, which affect the central nervous system. Demyelinating diseases can be monophasic or chronic and comprise acute disseminated encephalomyelitis, optic neuritis, transverse myelitis, multiple sclerosis and neuromyelitis optica. Demyelinating diseases are rare, but it is important for the physician to recognize these diseases, as well as to understand the differential diagnoses. This review summarizes the current knowledge of demyelinating disorders in children, focusing on an approach to diagnosis and management. PMID:26651911

  2. Neurologic Diseases

    The brain, spinal cord, and nerves make up the nervous system. Together they control all the workings of the body. When something goes wrong ... develops, such as spina bifida Degenerative diseases, where nerve cells are ... to the spinal cord and brain Seizure disorders, such as epilepsy ...

  3. Borna disease virus induces acute fatal neurological disorders in neonatal gerbils without virus- and immune-mediated cell destructions

    Borna disease virus (BDV) is a noncytolytic, neurotropic RNA virus that is known to cause neurological disturbances in various animal species. Our previous experiment demonstrated that neonate gerbils develop an acute fatal neurological disease following infection with BDV , Virology 282, 65-76). The study suggested that BDV directly causes functional damage of neuronal cells resulting in the lethal disorder in neonatal gerbils. To extend this finding, we examined whether BDV can induce neurological diseases in the absence of virus- and immune-mediated cell destruction, by using cyclosporine A (CsA)-treated neonatal gerbils. Although CsA completely suppressed specific antibody production and brain inflammation in the infected gerbil brains, the fatal neurological disorder was not inhibited by the treatment. Furthermore, we demonstrated that CsA treatment significantly decreased brain levels of cytokines, except interleukin (IL)-1β, in the infected gerbils. These results suggested that BDV replication, as well as brain cytokines, at least IL-1β, rapidly induces fatal disturbances in gerbil brain. We demonstrate here that BDV exhibits a unique neuropathogenesis in neonatal gerbil that may be pathologically and immunologically different from those in two other established rodent models, rats and mice. With this novel rodent model of virus infection it should be possible not only to examine acute neurological disturbances without severe neuroanatomical and immunopathological alterations but also to analyze molecular and cellular damage by virus replication in the central nervous system

  4. Creativity and neurological disease.

    Acosta, Lealani Mae Y

    2014-08-01

    Although humans have long valued creativity, the generation of such innovation is still incompletely understood. Looking at the healthy brain, researchers have localized certain parts for a basic understanding of these mechanisms. By researching the brain affected by neurological disease, scientists have observed unique manifestations of creativity, such as in frontotemporal lobar degeneration, Alzheimer's disease, Parkinson's disease and parkinsonian spectrum disorders, and stroke, which help clarify these creative underpinnings. Incorporating both healthy and disease models of cerebral functioning, neurological and neuroscientific research from recent years has built on established theories and expanded current knowledge. PMID:24938215

  5. Astroglia in neurological diseases

    Verkhratsky, Alexei; Rodríguez Arellano, Jose Julio; Parpura, V.

    2013-01-01

    Roč. 8, č. 2 (2013), s. 149-158. ISSN 1479-6708 R&D Projects: GA ČR(CZ) GAP304/11/0184; GA ČR GA309/09/1696 Institutional support: RVO:68378041 Keywords : amyotrophic lateral sclerosis * Alzheimer's disease * Alexander disease Subject RIV: FH - Neurology

  6. Astroglia in neurological diseases

    VERKHRATSKY, ALEXEI; Rodríguez, José J.; Parpura, Vladimir

    2013-01-01

    Astroglia encompass a subset of versatile glial cells that fulfill a major homeostatic role in the mammalian brain. Since any brain disease results from failure in brain homeostasis, astroglial cells are involved in many, if not all, aspects of neurological and/or psychiatric disorders. In this article, the roles of astrocytes as homeostatic cells in healthy and diseased brains are surveyed. These cells can mount the defence response to the insult of the brain, astrogliosis, when and where th...

  7. Pyroptosis and neurological diseases

    Zhen Xie

    2014-09-01

    Full Text Available Pyroptosis is a new process of programmed cell death, which has been discovered and confirmed in recent years. Its cardinal features include activation of caspase-1 and a massive release of inflammatory cytokines (interleukin (IL-1β, IL-18, etc. The morphological characteristics, occurrence and regulatory mechanisms of the pyroptosis greatly, differ from other cell death mechanisms such as apoptosis and necrosis. It has already been proven that pyroptosis participates and plays an important role in a wide range of neuronal diseases. Here, we review the current understanding of the pyroptosis and its roles in neurological diseases.

  8. Neurologic Complications of Celiac Disease

    J Gordon Millichap

    2004-06-01

    Full Text Available Patients with celiac disease (CD [n=l 11] and controls (n=211 were questioned regarding neurologic disorders, their charts were reviewed, and they received neurologic evaluations, including brain imaging or EEG if indicated, in a study of neurologic complications of CD at Carmel Medical Center, Technion-Israel Institute of Technology, Haifa, Israel.

  9. 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.

  10. Neurologic manifestations of Kanzaki disease.

    Umehara, F; Matsumuro, K; Kurono, Y; Arimura, K; Osame, M; Kanzaki, T

    2004-05-11

    We describe the neurologic findings in a patient with alpha-N-acetylgalactosaminidase deficiency (Kanzaki disease). Clinical and electrophysiologic studies revealed sensory-motor polyneuropathy, and sural nerve pathology showed decreased density of myelinated fibers with axonal degeneration. The patient had mildly impaired intellectual function with abnormal brain MRI and sensory-neuronal hearing impairment with repeated episodes of vertigo attacks. These findings suggest that Kanzaki disease may develop neurologic complications in the CNS and peripheral nervous system. PMID:15136691

  11. Neurological complications of acute multifocal placoid pigment epitheliopathy.

    Brownlee, W J; Anderson, N E; Sims, J; Pereira, J A

    2016-09-01

    Acute multifocal placoid pigment epitheliopathy (AMPPE) is an autoimmune chorioretinal disease that can be complicated by neurological involvement. There is limited information on this potentially treatable condition in the neurological literature. The objective of this patient series is to describe the neurological complications of AMPPE. We retrospectively identified patients with neurological complications of AMPPE seen at Auckland Hospital between 2008 and 2013 and summarised cases in the literature between 1976 and 2013. We identified five patients with neurological complications of AMPPE at Auckland Hospital and 47 reported patients. These patients demonstrated a spectrum of neurological involvement including isolated headache, stroke or transient ischaemic attack, seizures, venous sinus thrombosis, optic neuritis, sensorineural hearing loss and peripheral vestibular disorder. We propose criteria to define AMPPE with neurological complications. A cerebrospinal fluid (CSF) lymphocytosis in a patient with isolated headache may predict the development of cerebrovascular complications of AMPPE. Patients with cerebrovascular complications of AMPPE have a poor prognosis with high rates of death and neurological disability among survivors. Predictors of poor outcome in those who develop neurological complications of AMPPE are a relapsing course, generalised seizures and multifocal infarction on MRI. All patients with neurological complications of AMPPE, including headache alone, should be investigated with an MRI brain and CSF examination. Patients with focal neurological symptoms should receive intravenous (IV) methylprednisolone followed by a tapering course of oral steroids for at least 3months. Patients with AMPPE and an isolated headache with a CSF pleocytosis should be treated with oral steroids. PMID:27183958

  12. [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

  13. Neurological manifestations in Fabry's disease

    Møller, Anette Torvin; Jensen, Troels Staehelin

    2007-01-01

    . 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...... treatment is available to prevent irreversible organ damage. Females with Fabry's disease who present with pain have often been ignored and misdiagnosed because of the disorder's X-linked inheritance. This Review will stress the importance of recognizing neurological symptoms for the diagnosis of Fabry...

  14. 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 radicu...

  15. 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.

  16. Neurologic Manifestations of Childhood Rheumatic Diseases

    Shiari, Reza

    2012-01-01

    How to Cite this Article: Shiari R. Neurologic Manifestations of Childhood Rheumatic Diseases.  Iran J Child Neurol Autumn 2012; 6(4): 1-7.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 dis...

  17. 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.

  18. 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.

  19. Neurological Complications of Lyme Disease

    ... may begin with flu-like symptoms such as fever, chills, swollen lymph nodes, headaches, fatigue, muscle aches, and joint pain. Neurological complications most often occur in the second stage ... such as fever, stiff neck, and severe headache. Other problems, which ...

  20. 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.

  1. Astaxanthin as a Potential Neuroprotective Agent for Neurological Diseases

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

    2015-01-01

    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...

  2. 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

  3. Astrocytes: a central element in neurological diseases

    M. Pekny; M. Pekna; A. Messing; C. Steinhäuser; J.M. Lee; V. Parpura; E.M. Hol; M.V. Sofroniew; A. Verkhratsky

    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

  4. 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

  5. Mitochondrial Biology and Neurological Diseases.

    Arun, Siddharth; Liu, Lei; Donmez, Gizem

    2016-01-01

    Mitochondria are extremely active organelles that perform a variety of roles in the cell including energy production, regulation of calcium homeostasis, apoptosis, and population maintenance through fission and fusion. Mitochondrial dysfunction in the form of oxidative stress and mutations can contribute to the pathogenesis of various neurodegenerative diseases such as Parkinson's (PD), Alzheimer's (AD), and Huntington's diseases (HD). Abnormalities of Complex I function in the electron transport chain have been implicated in some neurodegenerative diseases, inhibiting ATP production and generating reactive oxygen species that can cause major damage to mitochondria. Mutations in both nuclear and mitochondrial DNA can contribute to neurodegenerative disease, although the pathogenesis of these conditions tends to focus on nuclear mutations. In PD, nuclear genome mutations in the PINK1 and parkin genes have been implicated in neurodegeneration [1], while mutations in APP, PSEN1 and PSEN2 have been implicated in a variety of clinical symptoms of AD [5]. Mutant htt protein is known to cause HD [2]. Much progress has been made to determine some causes of these neurodegenerative diseases, though permanent treatments have yet to be developed. In this review, we discuss the roles of mitochondrial dysfunction in the pathogenesis of these diseases. PMID:26903445

  6. Regenerative cellular therapies for neurologic diseases.

    Levy, Michael; Boulis, Nicholas; Rao, Mahendra; Svendsen, Clive N

    2016-05-01

    The promise of stem cell regeneration has been the hope of many neurologic patients with permanent damage to the central nervous system. There are hundreds of stem cell trials worldwide intending to test the regenerative capacity of stem cells in various neurological conditions from Parkinson׳s disease to multiple sclerosis. Although no stem cell therapy is clinically approved for use in any human disease indication, patients are seeking out trials and asking clinicians for guidance. This review summarizes the current state of regenerative stem cell transplantation divided into seven conditions for which trials are currently active: demyelinating diseases/spinal cord injury, amyotrophic lateral sclerosis, stroke, Parkinson׳s disease, Huntington׳s disease, macular degeneration and peripheral nerve diseases. This article is part of a Special Issue entitled SI: PSC and the brain. PMID:26239912

  7. 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…

  8. Diagnostic imaging of acute neurologic symptoms in children

    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.)

  9. Toward precision medicine in neurological diseases.

    Tan, Lin; Jiang, Teng; Tan, Lan; Yu, Jin-Tai

    2016-03-01

    Technological development has paved the way for accelerated genomic discovery and is bringing precision medicine into view. The goal of precision medicine is to deliver optimally targeted and timed interventions tailored to an individual's molecular drivers of disease. Neurological diseases are promisingly suited models for precision medicine because of the rapidly expanding genetic knowledge base, phenotypic classification, the development of biomarkers and the potential modifying treatments. Moving forward, it is crucial that through these integrated research platforms to provide analysis both for accurate personal genome analysis and gene and drug discovery. Here we describe our vision of how precision medicine can bring greater clarity to the clinical and biological complexity of neurological diseases. PMID:27127757

  10. Caval variations in neurologically diseased patients

    The import of the cavum variation and its prevalence rate in healthy individuals is still not clear, likewise in neurologically diseased patients. To evaluate the frequency and pattern of caval variations in neurologically diseased patients. The presence or absence of the cavum septum pellucidum (CSP), cavum vergae (CV), or cavum velum interpositum (CVI) was reviewed from successive cranial computerized tomography (CT) images of patients who were aged 6 months and above. Two hundred and seventeen cranial CT images were reviewed. At least a cavum variation was noted in 130 (59.9%) of the CT scan images reviewed. The CV, CVI, and CSP were noted in 86 (39.6%), 53 (24.4%), and 50 images (23%), respectively. Caval multiplicity was noted in 102 patients (47%). There was no significant difference in the rate of occurrence of cavum variations in patients with congenital brain diseases and acquired brain conditions (P = 0.484), neither was there a significant difference in the frequency of cavum variation in children aged older than 6 months compared to adults (P = 0.101). Cava variations are relatively common in neurological brain diseases. Patients with congenital brain diseases did not have a higher frequency of cava variation when compared with those that had acquired lesions. The most common type of cavum variation noted in this study was the vergae variety, while the CSP is the rarest

  11. 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

  12. RNA Regulation in Neurologic Disease and Cancer

    Darnell, Robert B

    2010-01-01

    The paraneoplastic neurologic diseases (PNDs) are brain degenerations that develop in the setting of clinically inapparent cancers. PNDs arise when common cancers express brain proteins, triggering an anti-tumor immune response and tumor immunity. Research on these brain-cancer proteins has revealed a new world of neuron-specific RNA binding proteins whose functions may be aberrantly used by tumor cells. Efforts to gain insight into their function has led to the development of new methods and...

  13. Acute intermittent porphyria presenting with neurological emergency: Review of six cases

    Kochar Dhanpat

    2007-01-01

    Full Text Available Acute intermittent porphyria presenting with short duration of gastrointestinal symptoms followed by rapidly progressive fulminant neurological syndrome during first attack is relatively uncommon. It is a neurological emergency and mimics many other psychiatric and medical disorders and can be fatal if it remains undiagnosed and untreated. Further, specific treatment in the form of Heme arginate is not universally available and very costly, so high clinical suspicion and early diagnosis and management of acute attack and prevention of further attacks are very important. We report a series of six cases who presented with convulsion and/or polyneuropathy early in the course of disease to highlight this fact.

  14. 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

  15. Neurological manifestations of Batch s disease

    To determine the prevalence, clinical manifestations, and laboratory features of Neuro-Behcets disease. This prospective study was carried out in the Behcets Research Clinic in Shiraz (south-west Iran) and included the patients referred from 1990-1999. The patients' clinical records, images, CSF analyses, and electrodiagnostic studies were reviewed. Eighteen (15 males and 3 females) out of 690 Behcet s patients (2.6%, 95% CI = 1.4-3.8%) were found to have neurological involvement. The mean +/- standard deviation age of these patients was 34.7 +/- 8.6 years. All fulfilled the criteria of the International Study Group of Behcet s Disease. Central nervous system involvement was more common than peripheral nervous system manifestations. Headache, weakness, tingling, and numbness were the most common symptoms. Hyperreflexia, upward plantar reflex, and somatosensory findings were the most frequent signs. Hemispheral and brainstem stroke-like syndromes and cerebral venous thrombosis were the major neurologic presentations. There were also cases of myelitic, pure meningoencephalitic, amyotrophic lateral sclerosis-like, multiple sclerosis-like, and Guillain Barre syndromes. Neuro-Behcets disease must be considered in the differential diagnosis of stroke in young adults, chronic meningitis, intracranial hypertension, multiple sclerosis, myelopathies, and peripheral neuropathies. (author)

  16. 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.

  17. Acute mountain sickness in athletes with neurological impairments

    Deepan C. Kamaraj, MD

    2013-04-01

    Full Text Available Acute mountain sickness (AMS is a symptom complex noticed commonly among high altitude travelers. The occurrence of AMS depends on multiple factors that have been studied extensively. However, AMS in individuals with neurological impairments has not been considered in detail. A total of 168 subjects, including active controls, inactive controls, and those with spinal cord injury (SCI, multiple sclerosis, and traumatic brain injury (TBI, were studied at the National Veterans Winter Sports Clinic in Snowmass, Colorado, from 2007 to 2009 for the occurrence of AMS. Lake Louise Score was used to quantify symptoms. A higher than anticipated occurrence of AMS (42.85% among the study population was noted, with significantly higher Lake Louis Scores among athletes with neurological impairments. Disability group, prior history of AMS, and prior occurrence of headache at high altitude could be used as predictors for the development of AMS symptoms. More research is warranted specifically targeting the interaction between factors affecting AMS and the pathophysiology of neurological impairments like SCI and TBI to further our understanding about prophylactic medications and treatments for AMS, especially because many military personnel with neurological impairments continue on Active Duty.

  18. Neuroimmunology I: Immunoregulation in neurological disease.

    Weiner, H L; Hauser, S L

    1982-05-01

    Aberrations in immune function that ultimately result in disease states may involve three aspects of immune regulation: (1) regulatory T cells, which both suppress and induce immune responses; (2) idiotype-antiidiotype networks, which serve as internal regulatory networks during generation of an immune response; and (3) immune response genes, which determine genetic differences in an individual's immune response. Three major diseases of the nervous system, multiple sclerosis, myasthenia gravis, and acute inflammatory polyneuropathy (Guillain-Barré syndrome), are classified as "autoimmune" in nature and may be due to underlying disorders of immunoregulation. In multiple sclerosis there is a loss of suppressor T cells in the peripheral blood during attacks, in myasthenia gravis there are thymic abnormalities and antibodies against the acetylcholine receptor, and in acute inflammatory polyneuropathy, macrophage-mediated destruction of peripheral nerve myelin occurs in the context of sensitized T cells and is usually associated with a preceding viral illness. In each of these diseases the following central questions must be answered: (1) against what antigen (or antigens) of the nervous system is the autoimmune response directed? (2) what is the mechanism of immune damage? and (3) what initiates, or triggers, the autoimmune response? PMID:6179458

  19. Neurological diseases as primary gliopathies: a reassessment of neurocentrism

    Albee Messing

    2012-04-01

    Full Text Available Diseases of the human brain are almost universally attributed to malfunction or loss of nerve cells. However, a considerable amount of work has, during the last decade, expanded our view on the role of astrocytes in CNS (central nervous system, and this analysis suggests that astrocytes contribute to both initiation and propagation of many (if not all neurological diseases. Astrocytes provide metabolic and trophic support to neurons and oligodendrocytes. Here, we shall endeavour a broad overviewing of the progress in the field and forward the idea that loss of homoeostatic astroglial function leads to an acute loss of neurons in the setting of acute insults such as ischaemia, whereas more subtle dysfunction of astrocytes over periods of months to years contributes to epilepsy and to progressive loss of neurons in neurodegenerative diseases. The majority of therapeutic drugs currently in clinical use target neuronal receptors, channels or transporters. Future therapeutic efforts may benefit by a stronger focus on the supportive homoeostatic functions of astrocytes.

  20. [Prevention of virus-related neurological diseases by vaccines].

    Takahashi, M

    1997-04-01

    Prevention of virus-related neurological diseases are surveyed. Patients of poliomyelitis has recently been drastically reduced by world-wide administrating live vaccines. In view of rare incidence of paralysis after giving live vaccine, adoption of inactivated vaccine has recently been reconsidered. A live varicella vaccine was developed and has been world-wide used for normal and high-risk children. Incidence of zoster in vaccinated acute leukemic children is several times higher in those who with rash after vaccination as compared with those without rash, and as no or few rash appears after vaccination of normal children, it is expected that vaccination of normal children would lead to reduction of zoster after their aging. Measles encephalitis has rapidly been reduced by world-wide use of live vaccines. Mouse-brain derived vaccine against Japanese encephalitis(JE) has been used in Asian countries. Development of tissue-culture derived JE vaccine is under way. PMID:9103901

  1. 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, ...

  2. 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

  3. 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

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

    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

  5. 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...... 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...... associated with most of the central neurological diseases. The prevalence and treatment of insomnia in neurological diseases still need to be studied in larger patient groups with randomized clinical trials to a) better understand their impact and causal relationship and b) to develop and improve specific...

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

    Block, Valerie A. J.; Pitsch, Erica; Tahir, Peggy; Cree, Bruce A. C.; Allen, Diane D.; Gelfand, Jeffrey M.

    2016-01-01

    Objective To perform a systematic review of studies using remote physical activity monitoring in neurological diseases, highlighting advances and determining gaps. Methods 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. Results 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. Conclusions 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. PMID:27124611

  7. 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...

  8. Ketogenic diets, mitochondria, and neurological diseases

    Gano, Lindsey B.; Patel, Manisha; Rho, Jong M

    2014-01-01

    The ketogenic diet (KD) is a broad-spectrum therapy for medically intractable epilepsy and is receiving growing attention as a potential treatment for neurological disorders arising in part from bioenergetic dysregulation. The high-fat/low-carbohydrate “classic KD”, as well as dietary variations such as the medium-chain triglyceride diet, the modified Atkins diet, the low-glycemic index treatment, and caloric restriction, enhance cellular metabolic and mitochondrial function. Hence, the broad...

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

    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.)

  10. 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.)

  11. Acute radiation disease

    Features of clinical trends in acute period of radiation disease at personnel who suffered from Chernobyl accident are considered. The main attention is paid to the results of 10 year observation of organs, systems and metabolic processes in patients. Used therapeutic, rehabilitation and preventive actions in stationary, ambulatory and sanatorium - health resort stages are described

  12. Astrocytes conspire with neurons during progression of neurological disease

    McGann, James C.; Lioy, Daniel T.; Mandel, Gail

    2012-01-01

    As astrocytes are becoming recognized as important mediators of normal brain function, studies into their roles in neurological disease have gained significance. Across mouse models for neurodevelopmental and neurodegenerative diseases, astrocytes are considered key regulators of disease progression. In Rett syndrome and Parkinson’s disease, astrocytes can even initiate certain disease phenotypes. Numerous potential mechanisms have been offered to explain these results, but research into the ...

  13. Multiple sclerosis or neurological manifestations of Celiac disease

    Vahid Shaygannejad

    2013-01-01

    Full Text Available Multiple sclerosis (MS and celiac disease (CD are considered to be T-cell-mediated autoimmune disease. We discuss about a known case of CD-showed relapsing - remitting neurological symptoms compatible with MS. In this rare co-occurrence subject, MS-CD patient, the interaction between MS - and CD-related inflammatory processes is open to discussion.

  14. Ketogenic diets, mitochondria, and neurological diseases.

    Gano, Lindsey B; Patel, Manisha; Rho, Jong M

    2014-11-01

    The ketogenic diet (KD) is a broad-spectrum therapy for medically intractable epilepsy and is receiving growing attention as a potential treatment for neurological disorders arising in part from bioenergetic dysregulation. The high-fat/low-carbohydrate "classic KD", as well as dietary variations such as the medium-chain triglyceride diet, the modified Atkins diet, the low-glycemic index treatment, and caloric restriction, enhance cellular metabolic and mitochondrial function. Hence, the broad neuroprotective properties of such therapies may stem from improved cellular metabolism. Data from clinical and preclinical studies indicate that these diets restrict glycolysis and increase fatty acid oxidation, actions which result in ketosis, replenishment of the TCA cycle (i.e., anaplerosis), restoration of neurotransmitter and ion channel function, and enhanced mitochondrial respiration. Further, there is mounting evidence that the KD and its variants can impact key signaling pathways that evolved to sense the energetic state of the cell, and that help maintain cellular homeostasis. These pathways, which include PPARs, AMP-activated kinase, mammalian target of rapamycin, and the sirtuins, have all been recently implicated in the neuroprotective effects of the KD. Further research in this area may lead to future therapeutic strategies aimed at mimicking the pleiotropic neuroprotective effects of the KD. PMID:24847102

  15. 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...

  16. 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.

  17. Association of Pesticide Exposure with Neurologic Dysfunction and Disease

    Kamel, Freya; Hoppin, Jane A

    2004-01-01

    Poisoning by acute high-level exposure to certain pesticides has well-known neurotoxic effects, but whether chronic exposure to moderate levels of pesticides is also neurotoxic is more controversial. Most studies of moderate pesticide exposure have found increased prevalence of neurologic symptoms and changes in neurobehavioral performance, reflecting cognitive and psychomotor dysfunction. There is less evidence that moderate exposure is related to deficits in sensory or motor function or per...

  18. APOPTOSIS, OXIDATIVE STRESS AND NEUROLOGICAL DISEASE

    P. Formichi

    2012-01-01

    Full Text Available Apoptosis is a selective cell deletion process which requires the triggering of a specific cell death programme. Two main pathways determining cell death have been identified: the extrinsic or receptor-mediated pathway, activated in response to extracellular pro-apoptotic signals, and the intrinsic pathway, activated by extracellular receptor-independent stimuli or by intracellular insults, such as DNA damage and oxidative stress. All these stress signals are integrated by mitochondria which participate by releasing the main effectors of this process: a family of aspartic-specific proteases known as caspase. Today there is much evidence to suggest that deregulation of apoptosis is a key feature of many neurodegenerative disease. Our group sought cell models for the study of apoptotic pathways and for the evaluation of the role of apoptosis in specific neurodegenerative diseases. We focused on oxidative stress-induced apoptosis and activation of the intrinsic mitochondrial pathway. In our in-vitro model, lymphocytes from patients and control subjects were cultured both in basal conditions and with 2-deoxy-D-ribose (dRib, a reducing sugar which induces apoptosis through oxidative stress. In the last ten years, we evaluated the role of apoptosis in the pathogenesis of several neurodegenerative diseases: Ataxiatelangiectasia,Rett syndrome, Mitochondrial disease, Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL. Here we report some of our ongoing and recently published articles.

  19. Serious unexpected sinus infection discovered by CT scanning for presumed neurological disease.

    Swift, A C; Gill, G. V.

    1994-01-01

    Serious infection in the paranasal sinuses may present with symptoms suggestive of neurological disease and thus lead to delay in the diagnosis and subsequent treatment. We present three such cases in whom the initial diagnoses had been acute optic neuritis, a posterior communicating aneurysm and an intracranial space occupying lesion. The fourth patient had meningitis but the paranasal sinuses had not initially been considered as a possible source of infection. The current methods of diagnos...

  20. 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.

  1. Management of acute neurologic syndromes in infants and children.

    Shaywitz, B A

    1984-01-01

    Neurological problems in the pediatric intensive care unit all too frequently seem to be among the most mysterious of disorders. This review provides a framework to diagnose and treat four frequently observed neurological syndromes: coma, status epilepticus, central nervous system infections, and post-infectious polyneuropathy (Guillain-Barré syndrome). An emphasis is placed on the diagnosis of coma due to metabolic disorders, the most common cause of coma, and coma as a result of supratentor...

  2. Patterns of histone acetylation as targets for novel therapeutic approaches in neurological diseases

    Ebrahimi, Azadeh

    2013-01-01

    Neurological diseases, in particular brain tumors and neurodegenerative disorders, cause significant socio-economic burdens on societies. Exploring epigenetic mechanisms in neurological disorders in recent decades has been an emerging tool for describing the pathogenesis of neurological diseases as well as developing new therapeutics. Global histone acetylation is an epigenetic entity whose alternating patterns in various neurological diseases have recently raised special attention concer...

  3. Neuroinflammation and neurological alterations in chronic liver diseases

    Carmina Montoliu; Marta Llansola; Vicente Felipo

    2015-01-01

    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 ...

  4. 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

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

    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.)

  6. Synaptic pathology: A shared mechanism in neurological disease.

    Henstridge, Christopher M; Pickett, Eleanor; Spires-Jones, Tara L

    2016-07-01

    Synaptic proteomes have evolved a rich and complex diversity to allow the exquisite control of neuronal communication and information transfer. It is therefore not surprising that many neurological disorders are associated with alterations in synaptic function. As technology has advanced, our ability to study the anatomical and physiological function of synapses in greater detail has revealed a critical role for both central and peripheral synapses in neurodegenerative disease. Synapse loss has a devastating effect on cellular communication, leading to wide ranging effects such as network disruption within central neural systems and muscle wastage in the periphery. These devastating effects link synaptic pathology to a diverse range of neurological disorders, spanning Alzheimer's disease to multiple sclerosis. This review will highlight some of the current literature on synaptic integrity in animal models of disease and human post-mortem studies. Synaptic changes in normal brain ageing will also be discussed and finally the current and prospective treatments for neurodegenerative disorders will be summarised. PMID:27108053

  7. 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.

  8. Node of Ranvier disruption as a cause of neurological diseases

    Keiichiro Susuki

    2013-08-01

    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.

  9. Neurological manifestations of ear disease in dogs and cats.

    Garosi, Laurent S; Lowrie, Mark L; Swinbourne, Natalie F

    2012-11-01

    There are four major neuroanatomical structures associated with the ear that, when damaged, result in different neurologic clinical signs. These structures are the facial nerve, the ocular sympathetic tract, the vestibular receptors, and the cochlea. The clinical signs associated with disorders of each structure are discussed, followed by a summary of the diseases that should be considered in each case. The article begins with a description of the neuroanatomy of each of these structures. PMID:23122174

  10. Nutritional Alterations Associated with Neurological and Neurosurgical Diseases

    Dionyssiotis, Yannis; Papachristos, Aris; Petropoulou, Konstantina; Papathanasiou, Jannis; Papagelopoulos, Panayiotis

    2016-01-01

    Neurological and neurosurgical diseases lead to complications producing malnutrition increasing pathology and mortality. In order to avoid complications because of malnutrition or overcome deficiencies in nutrients supplements are often used for these subjects. The physiopathological mechanisms of malnutrition, methods of nutritional assessment and the supplemental support are reviewed in this paper based on the assumption that patients need to receive adequate nutrition to promote optimal recovery, placing nutrition as a first line treatment and not an afterthought in the rehabilitation.

  11. Nutritional Alterations Associated with Neurological and Neurosurgical Diseases.

    Dionyssiotis, Yannis; Papachristos, Aris; Petropoulou, Konstantina; Papathanasiou, Jannis; Papagelopoulos, Panayiotis

    2016-01-01

    Neurological and neurosurgical diseases lead to complications producing malnutrition increasing pathology and mortality. In order to avoid complications because of malnutrition or overcome deficiencies in nutrients supplements are often used for these subjects. The physiopathological mechanisms of malnutrition, methods of nutritional assessment and the supplemental support are reviewed in this paper based on the assumption that patients need to receive adequate nutrition to promote optimal recovery, placing nutrition as a first line treatment and not an afterthought in the rehabilitation. PMID:27563361

  12. 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.

  13. Therapeutic Effects of Bee Venom on Immunological and Neurological Diseases

    Hwang, Deok-Sang; Kim, Sun Kwang; Bae, Hyunsu

    2015-01-01

    Bee Venom (BV) has long been used in Korea to relieve pain symptoms and to treat inflammatory diseases, such as rheumatoid arthritis. The underlying mechanisms of the anti-inflammatory and analgesic actions of BV have been proved to some extent. Additionally, recent clinical and experimental studies have demonstrated that BV and BV-derived active components are applicable to a wide range of immunological and neurodegenerative diseases, including autoimmune diseases and Parkinson’s disease. These effects of BV are known to be mediated by modulating immune cells in the periphery, and glial cells and neurons in the central nervous system. This review will introduce the scientific evidence of the therapeutic effects of BV and its components on several immunological and neurological diseases, and describe their detailed mechanisms involved in regulating various immune responses and pathological changes in glia and neurons. PMID:26131770

  14. 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.

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

    Mayer, Geert; Jennum, Poul; Riemann, Dieter;

    2011-01-01

    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...... antidepressants may be an effective treatment for insomnia in stroke and Parkinson's disease (PD) patients. Melatonin and light treatment can stabilize the sleep-wake circadian rhythm and shorten sleep latency in dementias and PD. Cognitive behavioral therapy (CBT) can be effective in treating insomnia symptoms......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...

  16. Texas Occurrence of Lyme Disease and Its Neurological Manifestations

    Dandashi, Jad A; Nizamutdinov, Damir; Dayawansa, Samantha; Fonkem, Ekokobe; Huang, Jason H

    2016-01-01

    Today, Lyme disease is the most commonly reported tick-borne disease in the United States and Europe. The culprits behind Lyme disease are the Borrelia species of bacteria. In the USA, Borrelia burgdorferi causes the majority of cases, while in Europe and Asia Borrelia afzelii and Borrelia garinii carry the greatest burden of disease. The clinical manifestations of Lyme disease have been identified as early localized, early disseminated, and late chronic. The neurological effects of Lyme disease include both peripheral and central nervous systems involvement, including focal nerve abnormalities, cranial neuropathies, painful radiculoneuritis, meningitis, and/or toxic metabolic encephalopathy, known as Lyme encephalopathy. Given the geographic predominance of Lyme disease in the Northeast and Midwest of the USA, no major studies have been conducted regarding Southern states. Between 2005 and 2014, the Center for Disease Control has reported 582 confirmed cases of Lyme disease in Texas. Because of the potential for increased incidence and prevalence in Texas, it has become essential for research and clinical efforts to be diverted to the region. The Texas A&M College of Veterinary Medicine and Biomedical Sciences Lyme Lab has been investigating the ecology of Lyme disease in Texas and developing a pan-specific serological test for Lyme diagnosis. This report aimed to exposure materials and raise awareness of Lyme disease to healthcare providers. PMID:27478852

  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. 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. PMID:24688139

  19. 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.

    2014-01-01

    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.

  20. Vascular endothelial growth factor: a neurovascular target in neurological diseases.

    Lange, Christian; Storkebaum, Erik; de Almodóvar, Carmen Ruiz; Dewerchin, Mieke; Carmeliet, Peter

    2016-08-01

    Brain function critically relies on blood vessels to supply oxygen and nutrients, to establish a barrier for neurotoxic substances, and to clear waste products. The archetypal vascular endothelial growth factor, VEGF, arose in evolution as a signal affecting neural cells, but was later co-opted by blood vessels to regulate vascular function. Consequently, VEGF represents an attractive target to modulate brain function at the neurovascular interface. On the one hand, VEGF is neuroprotective, through direct effects on neural cells and their progenitors and indirect effects on brain perfusion. In accordance, preclinical studies show beneficial effects of VEGF administration in neurodegenerative diseases, peripheral neuropathies and epilepsy. On the other hand, pathologically elevated VEGF levels enhance vessel permeability and leakage, and disrupt blood-brain barrier integrity, as in demyelinating diseases, for which blockade of VEGF may be beneficial. Here, we summarize current knowledge on the role and therapeutic potential of VEGF in neurological diseases. PMID:27364743

  1. Human gene therapy and imaging in neurological diseases

    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 promoted through the use of

  2. 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.

  3. [Neurological and psychiatric aspects of some gastrointestinal diseases].

    Aszalós, Zsuzsa

    2008-11-01

    The gastrointestinal tract is controlled by the independent enteric nervous system. It is also closely connected to the central nervous system, and bi-directional communication exists between them. The communication involves neural pathways as well as immune and endocrine mechanisms. The brain-gut axis plays a prominent role in the modulation of gut functions. Signals from different sources (e.g. sound, sight, smell, somatic and visceral sensations, pain) reach the brain. These inputs are modified by memory, cognition and affective mechanisms and integrated within the neural circuits of the central nervous system, spinal cord, autonomic and enteral nervous systems. These inputs can have physiologic effects, such as changes in motility, secretion, immune function, and blood flow to the gastrointestinal tract. One of the most important neurotransmitters is serotonin that plays a key role in the pathogenesis of the most common chronic functional gastrointestinal disorder: the irritable bowel syndrome. It is a biopsychosocial disease, resulting from the dysregulation of the brain-gut axis. Endogenous pain facilitation rather than inhibition, pathologic gradation of visceral perception and reduced threshold for pain are all evident in these patients. Abuse history is common in their anamnesis. Exaggerated conscientiousness, perfectionism, oversensitivity, feeling of deficiency in effectiveness, and higher demand for social parity, neuroticism and alexithymia have been detected among their constant personality features. Females are also characterized by gender role conflict and low assertiveness. Antidepressants and psychotherapy have important roles in their treatment. Also patients with inflammatory bowel disease are characterized by neuroticism and alexithymia and altered mother-child attachment is often described in their anamnesis. Autonomic neuropathy is a frequent and early neurological complication. Reflux disease and obstructive sleep apnea mutually generate

  4. MRI findings of neurologic complications in the enterovirus 71-infected hand-foot-mouth disease

    Objective: To explore the imaging characteristics of neurologic complications associated with the enterovirus 71 (EV71) epidemic by analyzing 25 cases and reviewing the literature. Methods: Twenty-five cases of hand-foot-mouth disease with neurologic complications during the recent EV71 outbreaks of Hainan province were studied for the clinical features and imaging findings, and literature were reviewed. Results: In 5 cases, acute flaccid paralysis associated with EV71-infected hand-foot-mouth disease was related to the linear high signal in the spinal cord on sagittal images. Two cases showed symmetrical, well- defined hyperintense lesions in the spinal cord on T2WI transverse. Strong enhancement of the ventral horns and root was seen on the contrast-enhanced axial T1WI. In brainstem encephalitis, all lesions presented with significant hyperintensity on T2WI and hypointense on T1WI in the posterior portions of the medulla oblongata, midbrain, and pons. The manifestations of aseptic meningitis (AM) on MRI have no characteristics, but subdural effusion, meningeal enhancement and hydrocephalus can be the indirect signs of AM. Conclusions: MRI is an effective method to investigate neurologic complications associated with the EV71 epidemic. Posterior portions of the medulla oblongata and pons, bilateral ventral horns of spinal involvement are characteristic findings of enteroviral encephalomyelitis. (authors)

  5. 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.

  6. Pertussis immunisation and serious acute neurological illness in children.

    Ebrahim, Shah

    1981-01-01

    The first 1000 cases notified to the National Childhood Encephalopathy Study were analysed. The diagnoses included encephalitis/encephalopathy, prolonged convulsions, infantile spasms, and Reye's syndrome. Eighty-eight of the children had had a recent infectious disease, including 19 with pertussis. Only 35 of the notified children (3.5%) had received pertussis antigen within seven days before becoming ill. Of 1955 control children matched for age, sex, and area of residence, 34 (1.7%) had be...

  7. A study on subsequent neurologic complications in children with acute leukemia

    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)

  8. [Palliative Care for Neurological Intractable Diseases and Home Medical Support].

    Yokoyama, Kazumasa; Ogino, Mieko; Ishigaki, Yasunori; Hattori, Nobutaka

    2015-08-01

    Many medical doctors regard the end stage and palliative care of neurological intractable diseases as the point at which aggressive treatment should be interrupted and death is imminent. However, the definition of health by the World Health Organization as the physical, psychological, and social goal to achieve a fully favorable health condition should be revisited. In the real clinical setting, the health condition, as the ability to adapt and self-manage in the face of social, physical, and emotional challenges with the aim to overcome stress (resilience), is dynamic and involves a healthy condition and satisfaction with one's own living. The most important step in palliative therapy that is shared by neurologists is the maintenance of the health status with the help of multi-disciplinary team with the view to improving the quality of life. PMID:26241362

  9. Neurological Complications Following Endoluminal Repair of Thoracic Aortic Disease

    Open surgery for thoracic aortic disease is associated with significant morbidity and the reported rates for paraplegia and stroke are 3%-19% and 6%-11%, respectively. Spinal cord ischemia and stroke have also been reported following endoluminal repair. This study reviews the incidence of paraplegia and stroke in a series of 186 patients treated with thoracic stent grafts. From July 1997 to September 2006, 186 patients (125 men) underwent endoluminal repair of thoracic aortic pathology. Mean age was 71 years (range, 17-90 years). One hundred twenty-eight patients were treated electively and 58 patients had urgent procedures. Anesthesia was epidural in 131, general in 50, and local in 5 patients. Seven patients developed paraplegia (3.8%; two urgent and five elective). All occurred in-hospital apart from one associated with severe hypotension after a myocardial infarction at 3 weeks. Four of these recovered with cerebrospinal fluid (CSF) drainage. One patient with paraplegia died and two had permanent neurological deficit. The rate of permanent paraplegia and death was 1.6%. There were seven strokes (3.8%; four urgent and three elective). Three patients made a complete recovery, one had permanent expressive dysphasia, and three died. The rate of permanent stroke and death was 2.1%. Endoluminal treatment of thoracic aortic disease is an attractive alternative to open surgery; however, there is still a risk of paraplegia and stroke. Permanent neurological deficits and death occurred in 3.7% of the patients in this series. We conclude that prompt recognition of paraplegia and immediate insertion of a CSF drain can be an effective way of recovering spinal cord function and improving the prognosis

  10. Chagas disease in a Texan horse with neurologic deficits.

    Bryan, Laura K; Hamer, Sarah A; Shaw, Sarah; Curtis-Robles, Rachel; Auckland, Lisa D; Hodo, Carolyn L; Chaffin, Keith; Rech, Raquel R

    2016-01-30

    A 10-year-old Quarter Horse gelding presented to the Texas A&M University Veterinary Teaching Hospital with a six month-history of ataxia and lameness in the hind limbs. The horse was treated presumptively for equine protozoal myeloencephalitis (EPM) based on clinical signs but was ultimately euthanized after its condition worsened. Gross lesions were limited to a small area of reddening in the gray matter of the thoracic spinal cord. Histologically, trypanosome amastigotes morphologically similar to Trypanosoma cruzi, the agent of Chagas disease in humans and dogs, were sporadically detected within segments of the thoracic spinal cord surrounded by mild lymphoplasmacytic inflammation. Ancillary testing for Sarcocystis neurona, Neospora spp., Toxoplasma gondii and Leishmania spp. was negative. Conventional and real time polymerase chain reaction (PCR) of affected paraffin embedded spinal cord were positive for T. cruzi, and sequencing of the amplified T. cruzi satellite DNA PCR fragment from the horse was homologous with various clones of T. cruzi in GenBank. While canine Chagas disease cases have been widely reported in southern Texas, this is the first report of clinical T. cruzi infection in an equid with demonstrable amastigotes in the spinal cord. In contrast to previous instances of Chagas disease in the central nervous system (CNS) of dogs and humans, no inflammation or T. cruzi amastigotes were detected in the heart of the horse. Based on clinical signs, there is a potential for misdiagnosis of Chagas disease with other infectious diseases that affect the equine CNS. T. cruzi should be considered as a differential diagnosis in horses with neurologic clinical signs and histologic evidence of meningomyelitis that originate in areas where Chagas disease is present. The prevalence of T. cruzi in horses and the role of equids in the parasite life cycle require further study. PMID:26801589

  11. 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.

  12. 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

  13. Cannabinoids: new promising agents in the treatment of neurological diseases.

    Giacoppo, Sabrina; Mandolino, Giuseppe; Galuppo, Maria; Bramanti, Placido; Mazzon, Emanuela

    2014-01-01

    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. PMID:25407719

  14. 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.

  15. Unstable mutations: cause of some neurological hereditary diseases

    Unstable mutations or amplification of triplets constitute a kind of genetic alteration discovered during the last decade. They had been found inside or near genes important for the normal neurological function of the human being. In some cases, the presence of the amplification causes the inactivation of the gene or the synthesis of a new product which functions different from the original protein. Some common characteristics of diseases caused by the amplification of triplets are that it affects the nervous system and are degenerative in nature. The expression of the manifestations varies according to age. Most of them show genetic anticipation in which the severity of the manifestations increases with each generation and appear at an earlier age. In most cases, the severity of the symptoms is correlated positively to the size of the amplification. The diagnosis of an affected individual in a family may indicate the presence of an altered gene in other relatives. These relatives may not present evident signs of the illness either because it is of late onset or because they carry premutations. The molecular diagnosis of these mutations is important to estimate the risk of developing the disease and/or of transmitting the illness to the descendants and to eliminate the fears of healthy relatives who have inherited normal copies of the gene. (Author)

  16. 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.

  17. The predictive value of thyroid hormone levels on the neurological outcomes of patients with acute ischemic stroke

    Guo-dong CHEN

    2015-03-01

    Full Text Available Objective To explore the correlation between thyroid hormone levels in patients with acute ischemic stroke and the severity of disease and short-term prognosis. Methods According to the level of serum total triiodothyronine (TT3, 98 patients who presented first acute ischemic stroke and without history of thyroid abnormality were divided into low TT3 group and normal TT3 group. Thyroid hormone levels and neurological function defect of those patients were tested, and their neural functional recovery after 3 months was evaluated.  Results Low TT3 group had more severe neural function defect compared to normal TT3 group (χ2 = 58.134, P = 0.000. There were no significant differences on total thyroxine (TT4; t = 1.636, P = 0.105 and thyroid stimulating hormone (TSH; t = 1.059, P = 0.292 between 2 groups. There was a significantly negative correlation between TT3 levels and National Insititute of Health Stroke Scale (NIHSS score on admission (r = -0.672, P = 0.000. Patients with low TT3 showed a significantly smaller percentage of neurological function improvement on both NIHSS ( χ2 = 8.993, P = 0.003 and modified Rankin Scale (mRS; χ2 = 6.247, P = 0.012 scores compared to those with normal TT3 at 90 d after onset.  Conclusions Low T3 level is associated with the severity of acute ischemic stroke and neural functional recovery, suggesting serum T3 level may be a predictor of neural function improvement in patients with acute ischemic stroke. DOI: 10.3969/j.issn.1672-6731.2015.02.009

  18. R778L, H1069Q, and I1102T mutation study in neurologic Wilson disease

    Kalita Jayantee; Somarajan Bindu; Misra Usha; Mittal Balraj

    2010-01-01

    There is paucity of the studies on mutations in neurologic Wilson disease (WD) in India. We studied H1069Q, R778L, I1102T mutations in 26 patients with neurologic WD from 25 families in north India. The basis of diagnosis of neurologic WD was clinical, Kayser-Fleischer (KF) ring, and ceruloplasmin. Data collected included: family history, clinical characteristics, laboratory data, ultrasound findings, magnetic resonance imaging (MRI) findings, and severity of the disease. DNA was isolated fro...

  19. Neurological Manifestations of an Old Disease: A Case Report

    Zia ISLAMI

    2013-08-01

    Full Text Available How to Cite This Article: Islami Z, Ataee Nakhaei MH. Neurological Manifestations of An Old Disease: A Case Report. Iran J Child Neurol. 2013 Summer; 7(3: 55-57. ObjectiveNeurological manifestations of neonatal disorders have various causes, among them neonatal tetanus, albeit rare, is a potentially fatal and preventable disease, which is seen in underdeveloped and developing countries. Although thedisease has been eradicated from I.R. Iran, pregnant women immigrating to Iran from neighboring countries, especially from eastern border, may carry a risk of neonatal tetanus to the child due to inadequate tetanus immunization and inappropriate post-delivery care. It is then important to maintain a high index of suspicion for early diagnosis and prompt treatment, when infants present with poor feeding and abnormal behavior.Case presentation Here, we report the clinical course of a newborn with neonatal tetanus, who was admitted with complaints of poor feeding and muscle rigidity, more than a decade after eradication of the disorder. References1. WHO. Immunization, Vaccines and Biologicals. Availablefrom: http://www.who.int/immunization/topics/tetanus/en/; 2008 [Access 25.10.2011].2. Lum SH, Chew MF. Neonatal Tetanus: A Study of Five Cases in Sandakan, Sabah. Med J Malaysia 2009;64(1:80-2.3. Ilic M, Pejcic L, Tiodorovic B, Hasani B, Stankovic S, Milojevic D, et al. Neonatal tetanus – report of a case. Turk J pediatr 2010;52:404-8.4. Chang SC, Wang CL. Neonatal tetanus after home delivery:report of one case. Pediatr Neonatol 2010;51(3:182-5.5. Cook TM, Protheroe RT, Handel JM. Tetanus: a review ofthe literature. Br J Anaesth 2001;87(3:477-87.6. Richard F. Edlich, MD, PhD, Lisa G. Hill, Chandra A.Mahler, et al. Management and Prevention of Tetanus.Long Term Eff Med Implants 2003;13(3139–54.7. Ministry of Health and Medical Education. Pediatricimmunization. Available from: http://behdasht.gov.ir/index.aspx?siteid=1&pageid=24979&newsvie=11776&pro

  20. Awareness Of The Neurological Diseases: Comparisons Of The Attendies At Secondary and Tertiary Neurological Oupatient Centers In The Sample Of Ankara City

    Emrah Aytaç

    2014-12-01

    Full Text Available OBJECTIVE: Both neurological diseases and related disabilities are growing all over the world and in our country. Awareness of the neurological diseases is thought to be less than the expected. METHODS: The aim of this study is; to investigate the awareness of the neurological diseases in the secondary and tertiary neurological outpatient service attendies and to assess its correlations between the patient’s demographics. RESULTS: A total of 540 patients were included in the study. Alzheimer disease was found to be most known neurological disease and Multiple sclerosis was the least one. The awareness of the diseases correlated with the level of education. Younger patients were seem to be more aware about the neurological diseases than the older patients. Although currently widespread usage of internet/media, we found that patients prefer mostly to take advice from their family practitioners for being to attend to the neurological outpatient services. We comment that this result is a positive finding in terms of usage of health care system and patient-physician relationships. CONCLUSION: There is need for increasing the awareness of the neurological diseases for both early diagnosis of neurological diseases and to decrease the related disability.

  1. Ultrasonic investigations of brain in infants with some neurological diseases

    Ulezko, E. A.; Shan'ko, G. G.

    1996-05-01

    The authors have studied 197 infants (1-12 months old) with normal psychomotor development and with various neurological disturbances. Neurosonography and dopplerometry were used to investigate the blood flow pattern and structural changes in the brain.

  2. EFNS guidelines for the use of intravenous immunoglobulin in treatment of neurological diseases: EFNS task force on the use of intravenous immunoglobulin in treatment of neurological diseases

    Elovaara, I.; Apostolski, S.; Doorn, P. van;

    2008-01-01

    Despite high-dose intravenous immunoglobulin (IVIG) is widely used in treatment of a number of immune-mediated neurological diseases, the consensus on its optimal use is insufficient. To define the evidence-based optimal use of IVIG in neurology, the recent papers of high relevance were reviewed...... second or third-line therapy in relapsing-remitting multiple sclerosis, if conventional immunomodulatory therapies are not tolerated (level B), and in relapses during pregnancy or post-partum period (good clinical practice point). IVIG seems to have a favourable effect also in paraneoplastic neurological...

  3. Transverse Myelitis in Acute Hepatitis A Infection: The Rare Co-Occurrence of Hepatology and Neurology

    Piyanant Chonmaitree

    2016-05-01

    Full Text Available Transverse myelitis refers to the inflammatory process involving the spinal cord. Clinical features can be either acute or subacute onset that results in neurological deficits such as weakness and/or numbness of extremities as well as autonomic dysfunctions. While there are some etiologies related, a viral infection is common. However, the hepatitis A virus rarely causes myelitis. This report provides details of a hepatitis A infectious patient who developed myelitis as comorbidity. Although, the disability was initially severe, the patient successfully recovered with corticosteroid treatment.

  4. 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...... of transgenic mice. MHC I immunostaining and iNOS mRNA were upregulated in their CNS, but such transgenic mice showed no spontaneous CNS inflammation or demyelination, and the incidence, severity, and histopathology of experimental autoimmune encephalomyelitis (EAE) were similar to nontransgenic...

  5. Transverse Myelitis in Acute Hepatitis A Infection: The Rare Co-Occurrence of Hepatology and Neurology.

    Chonmaitree, Piyanant; Methawasin, Kulthida

    2016-01-01

    Transverse myelitis refers to the inflammatory process involving the spinal cord. Clinical features can be either acute or subacute onset that results in neurological deficits such as weakness and/or numbness of extremities as well as autonomic dysfunctions. While there are some etiologies related, a viral infection is common. However, the hepatitis A virus rarely causes myelitis. This report provides details of a hepatitis A infectious patient who developed myelitis as comorbidity. Although, the disability was initially severe, the patient successfully recovered with corticosteroid treatment. PMID:27403101

  6. [Cannabinoid drugs for neurological diseases: what is behind?].

    Fernández-Ruiz, Javier

    2012-05-16

    In recent years progress has been made in the development of pharmaceuticals based on the plant Cannabis sativa or on synthetic molecules with a similar action. Some of these pharmaceuticals, such as the mouth spray Sativex, have recently been approved for the treatment of spasticity in multiple sclerosis, but they are not the first and others, such as Marinol or Cesamet for the treatment of vomiting and nausea, and anorexia-cachexia syndrome, had already been approved. This incipient clinical use of cannabinoid drugs confirms something that was already known from fairly ancient times up to practically the last century, which is the potential use of this plant for medicinal applications - something which was brought to a standstill by the abusive use of preparations of the plant for recreational purposes. In any case, this incipient clinical use of cannabinoid drugs is not backed just by the anecdote of the medicinal use of cannabis since ancient times, but instead the boost it has been given by scientific research, which has made it possible to identify the target molecules that are activated or inhibited by these substances. These targets are part of a new system of intercellular communication that is especially active in the central nervous system, which is called the 'endogenous cannabinoid system' and, like many other systems, can be manipulated pharmacologically. The aim of this review is to probe further into the scientific knowledge about this system generated in the last few years, as a necessary step to justify the development of pharmaceuticals based on its activation or inhibition and which can be useful in different neurological diseases. PMID:22573509

  7. 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.

  8. 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, pdisabilities attributable to neurological disorders by eliciting an acute autonomic response during the therapy and during the recovery period. PMID:26988283

  9. 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.

  10. 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.

  11. Bilirubin/Albumin Ratio for Predicting Acute Bilirubin-induced Neurologic Dysfunction

    Gholamreza Esmaeeli Djavid

    2011-03-01

    Full Text Available Objective:The aim of this study was to evaluate the bilirubin albumin (B/A ratio in comparison with total serum bilirubin (TSB for predicting acute bilirubin-induced neurologic dysfunction (BIND. Methods:Fifty two term and near term neonates requiring phototherapy and exchange transfusion for severe hyperbilirubinemia in Childrens Medical Center, Tehran, Iran, during September 2007 to September 2008, were evaluated. Serum albumin and bilirubin were measured at admission. All neonates were evaluated for acute BIND based on clinical findings. Findings:Acute BIND developed in 5 (3.8% neonates. B/A ratio in patients with BIND was significantly higher than in patients without BIND (P<0.001. Receiver operation characteristics (ROC analysis identified a TSB cut off value of 25 mg/dL [area under the curve (AUC 0.945] with a sensitivity of 100% and specificity of 85%. Also, according to the ROC curve, B/A ratio cut off value for predicting acute BIND was 8 (bil mg/al g (AUC 0.957 with sensitivity of 100% and specificity of 94%. Conclusion:Based on our results, we suggest using B/A ratio in conjunction with TSB. This can improve the specificity and prevent unnecessary invasive therapy such as exchange transfusion in icteric neonates.

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

    Amiri-Nikpour, Mohammad Reza; Nazarbaghi, Surena; Ahmadi-Salmasi, Babak; Mokari, Tayebeh; Tahamtan, Urya; Rezaei, Yousef

    2014-01-01

    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. PMID:25516711

  13. 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

  14. Ultrasound in Acute Kidney Disease.

    Meola, Mario; Nalesso, Federico; Petrucci, Ilaria; Samoni, Sara; Ronco, Claudio

    2016-01-01

    Kidneys' imaging provides useful information in acute kidney injury (AKI) diagnosis and management. Today, several imaging techniques give information on kidneys anatomy, urinary obstruction, differential diagnosis between AKI and chronic kidney disease (CKD), renal blood flow and glomerular filtration rate. Ultrasound is a safe, non-invasive and repeatable imaging technique so it is widely used in the first level work-up of AKI. The utility of contrast-enhanced computed tomography and magnetic resonance imaging in AKI or in AKI during CKD is limited because of renal toxicity associated with contrast agents used. PMID:27169556

  15. 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.

  16. 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

  17. Acute Chagas Disease in a Returning Traveler

    Carter, Yvonne L.; Juliano, Jonathan J; Montgomery, Susan P.; Qvarnstrom, Yvonne

    2012-01-01

    Acute Chagas disease is rarely recognized, and the risk for acquiring the disease is undefined in travelers to Central America. We describe a case of acute Chagas disease in a traveler to Costa Rica and highlight the need for increased awareness of this infection in travelers to Chagas-endemic areas.

  18. Bile Acid Signaling Is Involved in the Neurological Decline in a Murine Model of Acute Liver Failure.

    McMillin, Matthew; Frampton, Gabriel; Quinn, Matthew; Ashfaq, Samir; de los Santos, Mario; Grant, Stephanie; DeMorrow, Sharon

    2016-02-01

    Hepatic encephalopathy is a serious neurological complication of liver failure. Serum bile acids are elevated after liver damage and may disrupt the blood-brain barrier and enter the brain. Our aim was to assess the role of serum bile acids in the neurological complications after acute liver failure. C57Bl/6 or cytochrome p450 7A1 knockout (Cyp7A1(-/-)) mice were fed a control, cholestyramine-containing, or bile acid-containing diet before azoxymethane (AOM)-induced acute liver failure. In parallel, mice were given an intracerebroventricular infusion of farnesoid X receptor (FXR) Vivo-morpholino before AOM injection. Liver damage, neurological decline, and molecular analyses of bile acid signaling were performed. Total bile acid levels were increased in the cortex of AOM-treated mice. Reducing serum bile acids via cholestyramine feeding or using Cyp7A1(-/-) mice reduced bile acid levels and delayed AOM-induced neurological decline, whereas cholic acid or deoxycholic acid feeding worsened AOM-induced neurological decline. The expression of bile acid signaling machinery apical sodium-dependent bile acid transporter, FXR, and small heterodimer partner increased in the frontal cortex, and blocking FXR signaling delayed AOM-induced neurological decline. In conclusion, circulating bile acids may play a pathological role during hepatic encephalopathy, although precisely how they dysregulate normal brain function is unknown. Strategies to minimize serum bile acid concentrations may reduce the severity of neurological complications associated with liver failure. PMID:26683664

  19. 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.

  20. Neurological diseases as primary gliopathies: a reassessment of neurocentrism

    Verkhratsky, A.; Sofroniew, M. V.; Messing, A.; deLanerolle, N. C.; Rempe, D.; Rodríguez Arellano, Jose Julio; Nedergaard, M.

    2012-01-01

    Roč. 4, č. 3 (2012), e00082. ISSN 1759-0914 R&D Projects: GA ČR GA309/09/1696; GA ČR(CZ) GAP304/11/0184; GA ČR GA305/08/1384; GA ČR GA309/08/1381 Institutional research plan: CEZ:AV0Z50390703 Institutional support: RVO:68378041 Keywords : aging * astrocyte * brain Subject RIV: FH - Neurology Impact factor: 3.638, year: 2012

  1. Psychiatric side effects of acute high-dose corticosteroid therapy in neurological conditions.

    Lotan, Itay; Fireman, Liora; Benninger, Felix; Weizman, Abraham; Steiner, Israel

    2016-07-01

    It has been implied that high-dose corticosteroids (CSs) commonly cause psychiatric side effects. Here, we examined the rate and risk factors of psychiatric side effects during high-dose CS treatment in patients with neurological disorders. Patients treated with high-dose intravenous CSs for neurological disorders were evaluated for depression, mania, and psychosis using the Beck Depression Inventory, the Geriatric Depression Scale, the Young Mania Rating Scale, and the Brief Psychiatric Rating Scale before CS treatment, immediately after, and 1 month following treatment. Forty-nine consecutive patients were monitored. There was a reduction in the Beck Depression Inventory and Geriatric Depression Scale scores as well as in the Brief Psychiatric Rating Scale scores throughout the study period and a transitory increase in the Young Mania Rating Scale score immediately after CS administration. Thus, a tendency to develop transient mild euphoria during high-dose CS treatment exists, but is reversible at 1 month, whereas a reduction in depressive symptoms tended to persist. Overall, our data indicate that high-dose CS treatment for neurological diseases is relatively safe with respect to psychiatric complications. PMID:26938038

  2. Unusual presentations of acute kidney injury and neurologic complications due to snake bite

    Hamid Noshad

    2015-06-01

    Full Text Available Introduction: Vascularity of kidneys is very high, so these organs are potentially susceptible to be affected with toxins including snake venom. Hypersensitivity to snake venous could cause some neurological problem. Case Report: We present a 14-year-old boy with acute kidney injury (AKI due to snake bite. After a few days, kidney failure with hematuria was developed. His serum creatinine level rose to 3 mg/dl and following 2 weeks gradually and decreased to normal level without any special treatment except for anti-venom, which was not prescribed inappropriate time (this type of AKI is not reported previously. He had seizure attacks, which were according to magnetic resonance imaging due to posterior reversible encephalopathy syndrome (PRES (This neurologic complication has been seen in other kidney injuries but up to now it was not reported in snake bite victims. Conclusion: Sanke venom could cause PRES due to AKI and seizure could be one of the most important complications in snake bite.

  3. 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.

  4. Enterovirus 71-induced neurological disorders in young gerbils, Meriones unguiculatus: development and application of a neurological disease model.

    Ping-Ping Yao

    Full Text Available A reliable disease model mimicking Enterovirus 71 (EV71 infection in humans is essential for understanding pathogenesis and for developing a safe and effective vaccine. Commonly used rodent models including mouse or rat models are not suitable for vaccine evaluation because the rodents are resistant to EV71 infection after they reach the age of 6 days. In this study, 21-day-old gerbils inoculated intraperitoneally (IP with a non mouse-adapted EV71 strain developed neurological lesion-related signs including hind limb paralysis, slowness, ataxia and lethargy similar to those of central nervous system (CNS infection of EV71 in humans. The infected gerbils eventually died of the neurological lesions and EV71 could be isolated from lung, liver, spleen, kidney, heart, spinal cord, brain cortex, brainstem and skeletal muscle. Significantly high virus replication was detected in spinal cord, brainstem and skeletal muscle by cellular analysis, real-time quantitative PCR (RT-PCR and immunohistochemical staining. Histopathologic changes such as neuronal degeneration, neuronal loss and neuronophagia were observed in spinal cord, brain cortex, brainstem, and skeletal muscle along with necrotizing myositis and splenic atrophy. Gerbils that received two doses of inactive whole-virus vaccine showed no EV71-specific symptoms after challenged with EV71. In contrast, gerbils that received mock vaccination died of EV71-induced neuropathology after challenged with EV71. The result indicates that gerbils can serve as a reliable disease model for evaluating safety and efficacy of EV71 vaccine.

  5. 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.

  6. 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...

  7. Evaluation of neurological complications using who warning signs for dengue disease severity

    In 2009 a new classification of dengue was proposed by WHO Tropical Disease Research, which classifies dengue into dengue (D), dengue with warning signs (DW) and severe dengue (SD). This classification highlights the warning signs of dengue disease severity. Neurological complications are one of the most serious complications of dengue disease. This study was carried out to see association of neurological complications of dengue patients with WHO warning signs for dengue disease severity, and their outcome. Methods: It was a cross-sectional analytical study and included 180 diagnosed and registered cases of dengue hemorrhagic fever. The participants were subjected to a detailed clinical evaluation, laboratory assessment including blood counts, hematocrit, serology for dengue fever and sonography at 24 hours and 48 hours of their admission. Results: Twenty-six percent patients were suffering from neurological complications due to dengue. The warning signs for dengue disease severity like altered sensorium (85.5%, p=0.001), raised hematocrit (n=47, p=0.029), gall bladder wall thickening, pleural effusion and ascites on sonographic report (n=47, p=0.024), were strongly associated with the neurological complications. Conclusion: Our study reveals significant association of WHO warning signs for dengue disease severity with neurological complications of dengue disease. (author)

  8. 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.

  9. 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

  10. Therapeutic Effects of Bee Venom on Immunological and Neurological Diseases

    Deok-Sang Hwang; Sun Kwang Kim; Hyunsu Bae

    2015-01-01

    Bee Venom (BV) has long been used in Korea to relieve pain symptoms and to treat inflammatory diseases, such as rheumatoid arthritis. The underlying mechanisms of the anti-inflammatory and analgesic actions of BV have been proved to some extent. Additionally, recent clinical and experimental studies have demonstrated that BV and BV-derived active components are applicable to a wide range of immunological and neurodegenerative diseases, including autoimmune diseases and Parkinson’s disease. T...

  11. Herpes simplex serious neurological disease in young children: incidence and long-term outcome

    Ward, Katherine N.; Ohrling, Anu; Bryant, Naomi J; Bowley, Jennifer S; Ross, Euan M; Verity, Christopher M

    2011-01-01

    Objective To determine the contribution of herpes simplex virus (HSV) to serious neurological disease. Setting and patients A 3-year prospective survey of children aged 2–23 months in Britain and Ireland. Results 19 children had HSV central nervous system (CNS) infection; 13 aged 2–11 months had focal neuroimaging abnormalities and 11 long-term neurological sequelae. Of six aged 12–35 months, one had abnormal neuroimaging and three long-term neurological sequelae. 17 of the 19 had serious neu...

  12. Lipid imbalance in the progressive neurological metabolic disorder, Farber disease

    Ribeiro, Maria Gil Roseira; Ferreira, Natália; Alves, Mariana; Ribeiro, Isaura

    2010-01-01

    Farber disease is a neurodegenerative metabolic inherited disease caused by the deficient activity of acid ceramidase which leads to ceramide accumulation within lysosomes. Besides the structural role in biomembranes ceramide also acts as signalling molecule. The present study investigated whether intracellular trafficking of lipid molecules is blocked in diseased fibroblasts. The observation of secondary lysosomal glycosphingolipids and cholesterol storage in Farber cells rein...

  13. 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

  14. Current neurology

    The topics covered in this book include: Duchenne muscular dystrophy: DNA diagnosis in practice; Central nervous system magnetic resonance imaging; and Magnetic resonance spectroscopy of neurologic diseases

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

    KARIMZADEH, Parvaneh

    2015-01-01

    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.Th...

  16. 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. PMID

  17. Obstructive lung disease in acute medical patients.

    Seemungal, T.; Harrinarine, R.; Rios, M.; Abiraj, V.; Ali, A.; Lacki, N.; Mahabir, N.; Ramoutar, V.; King, C. P.; Bhowmik, A.; Wedzicha, J A

    2008-01-01

    OBJECTIVES: To determine the proportion of adult medical patients who have chronic obstructive pulmonary disease (COPD), using the Global initiative for Chronic Obstructive Lung Disease guidelines (GOLD), and its relation to vascular disease. METHODS: This is a prospective cross-sectional study of adult patients admitted to acute medical wards. Interviewer administered questionnaire, anthropometric and spirometric measurements were done. RESULTS: Spirometry was performed in 720 acute admissio...

  18. Recent achievements in restorative neurology: Progressive neuromuscular diseases

    This book contains 27 chapters. Some of the chapter titles are: Computed Tomography of Muscles in Neuromuscular Disease; Mapping the Genes for Muscular Dystrophy; Trophic Factors and Motor Neuron Development; Size of Motor Units and Firing Rate in Muscular Dystrophy; Restorative Possibilities in Relation to the Pathology of Progressive Neuromuscular Disease; and An Approach to the Pathogenesis of some Congenital Myopathies

  19. Diarrheal Diseases - Acute and Chronic

    ... and drinking contaminated or raw foods and beverages. Screening/Diagnosis Most episodes of acute diarrhea resolve quickly without antibiotic therapy and with simple dietary modifications. See a ...

  20. Dock protein family in brain development and neurological disease

    Shi, Lei

    2013-01-01

    The family of dedicator of cytokinesis (Dock), a protein family that belongs to the atypical Rho guanine nucleotide exchange factors (GEFs) for Rac and/or Cdc42 GTPases, plays pivotal roles in various processes of brain development. To date, 11 members of Docks have been identified in the mammalian system. Emerging evidence has suggested that members of the Dock family are associated with several neurodegenerative and neuropsychiatric diseases, including Alzheimer disease and autism spectrum ...

  1. Human gene therapy and imaging in neurological diseases

    Jacobs, Andreas H.; Winkler, Alexandra; Maria G Castro; Lowenstein, Pedro

    2005-01-01

    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...

  2. 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

  3. Omega-3 fatty acids and acute neurological trauma: a perspective on clinical translation*

    Gladman Stacy

    2011-11-01

    Full Text Available Acute neurological trauma remains one of the clinical areas with the most significant unmet needs worldwide. In the central nervous system, acute trauma has two stages: the primary injury and the secondary injury. The former is irreversible, and is a direct consequence of the impact. In the aftermath of the injury, a complex series of processes exacerbate the injury and amplify tissue damage. Some of these processes are local, others involve a systemic response. It is these processes which ultimately determine the clinical outcome. The aim of the treatments is a to confer neuroprotection and b to promote neuroregeneration. The results reported so far with omega-3 fatty acids in animal models of neurotrauma suggest that these compounds have the potential to offer a novel therapeutic approach and target both protection and regeneration. They lead to increased neuronal and glial survival, they can limit the damaging neuroinflammation and they can also protect neurites. Long chain omega-3 fatty acids such as eicosapentaenoic acid and docosahexaenoic acid have a complex pharmacodynamics, which leads potentially to the activation of a multitude of targets, including voltage and ligand-gated ion channels, transcription factors and G-protein coupled receptors. They can produce tissue-specific metabolites which have intrinsic activity, either on the same or on different cellular targets. The apparent large therapeutic window of omega-3 fatty acids is an advantage in the context of trauma, with patients in an unstable state, with multiple injuries. The specific use of omega-3 fatty acids in spinal cord injury and peripheral nerve injury will be discussed, focusing on issues which need to be addressed in order to translate successfully to the clinic the efficacy reported in the initial proof of concept animal studies.

  4. 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.

  5. Acute necrotizing encephalopathy of childhood: typical findings in an atypical disease

    Skelton, Brandon W.; Phillips, C.D. [University of Virginia Health System, Department of Neuroradiology, Charlottesville, VA (United States); Hollingshead, Michael C.; Castillo, Mauricio [University of North Carolina School of Medicine, Neuroradiology Section, Chapel Hill, NC (United States); Sledd, Andrew T. [University of Virginia Health System, Department of Pediatrics, Charlottesville, VA (United States)

    2008-07-15

    Acute necrotizing encephalopathy of childhood (ANEC) is a disease entity seen nearly exclusively in East Asian children that is characterized by multifocal, symmetric lesions involving the thalami, brainstem, cerebellum, and white matter. We present a child who developed dramatic neurologic symptoms following a viral prodrome. Serial MRI examinations demonstrated characteristic lesions of ANEC, while laboratory analyses revealed evidence of acute infection with human herpesvirus-6 (HHV-6). We highlight the MRI findings in both the acute and convalescent phases of ANEC, discuss the implications of neuroimaging on the child's clinical course, and emphasize the integral role of the radiologist in correctly diagnosing this rare disease. (orig.)

  6. 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; Clear Daniella; Perera David; Mohan Anand; Podin Yuwana; Wong See; Ooi Mong; Chieng Chae; Tio Phaik; Cardosa Mary; Solomon Tom

    2009-01-01

    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 neu...

  7. Resting-state networks link invasive and noninvasive brain stimulation across diverse psychiatric and neurological diseases

    Fox, Michael D.; Buckner, Randy L.; Liu, Hesheng; Chakravarty, M. Mallar; Lozano, Andres M.; Pascual-Leone, Alvaro

    2014-01-01

    Brain stimulation is a powerful treatment for an increasing number of psychiatric and neurological diseases, but it is unclear why certain stimulation sites work or where in the brain is the best place to stimulate to treat a given patient or disease. We found that although different types of brain stimulation are applied in different locations, targets used to treat the same disease most often are nodes in the same brain network. These results suggest that brain networks might be used to und...

  8. Lymphatics in Neurological Disorders: A Neuro-Lympho-Vascular Component of Multiple Sclerosis and Alzheimer's Disease?

    Louveau, Antoine; Da Mesquita, Sandro; Kipnis, Jonathan

    2016-09-01

    Lymphatic vasculature drains interstitial fluids, which contain the tissue's waste products, and ensures immune surveillance of the tissues, allowing immune cell recirculation. Until recently, the CNS was considered to be devoid of a conventional lymphatic vasculature. The recent discovery in the meninges of a lymphatic network that drains the CNS calls into question classic models for the drainage of macromolecules and immune cells from the CNS. In the context of neurological disorders, the presence of a lymphatic system draining the CNS potentially offers a new player and a new avenue for therapy. In this review, we will attempt to integrate the known primary functions of the tissue lymphatic vasculature that exists in peripheral organs with the proposed function of meningeal lymphatic vessels in neurological disorders, specifically multiple sclerosis and Alzheimer's disease. We propose that these (and potentially other) neurological afflictions can be viewed as diseases with a neuro-lympho-vascular component and should be therapeutically targeted as such. PMID:27608759

  9. 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.

  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. 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.

  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

    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 to...

  13. 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.

  14. 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.

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

    Woods Christopher W; Hegarty Barbara C; Lantos Paul M; Maggi Ricardo G; Breitschwerdt Edward B; Bradley Julie M

    2010-01-01

    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 ...

  16. Leukocytosis in Patients with Neurologic Deterioration after Acute Ischemic Stroke is Associated with Poor Outcomes

    Kumar, Andre D.; Boehme, Amelia K.; Siegler, James E.; Gillette, Michael; Albright, Karen C.; Martin-Schild, Sheryl

    2016-01-01

    Background Neurologic deterioration (ND) after acute ischemic stroke (AIS) has been shown to result in poor outcomes. ND is thought to arise from penumbral excitotoxic cell death caused in part by leukocytic infiltration. Elevated admission peripheral leukocyte levels are associated with poor outcomes in stroke patients who suffer ND, but little is known about the dynamic changes that occur in leukocyte counts around the time of ND. We sought to determine if peripheral leukocyte levels in the days surrounding ND are correlated with poor outcomes. Methods Patients with AIS who presented to our center within 48 hours of symptom onset between July 2008 and June 2010 were retrospectively identified by chart review and screened for ND (defined as an increase in National Institutes of Health Stroke Scale score ≥2 within a 24-hour period). Patients were excluded for steroid use during hospitalization or in the month before admission and infection within the 48 hours before or after ND. Demographics, daily leukocyte counts, and poor functional outcome (modified Rankin Scale score 3–6) were investigated. Results Ninety-six of the 292 (33%) patients screened had ND. The mean age was 69.5 years; 62.5% were male and 65.6% were black. Patients with a poor functional outcome had significantly higher leukocyte and neutrophil levels 1 day before ND (P =.048 and P =.026, respectively), and on the day of ND (P =.013 and P =.007, respectively), compared to patients with good functional outcome. Conclusions Leukocytosis at the time of ND correlates with poor functional outcomes and may represent a marker of greater cerebral damage through increased parenchymal inflammation. PMID:23031742

  17. Neurological and Psychiatric Diseases and Their Unique Cognitive Profiles: Implications for Nursing Practice and Research

    Vance, David E; Dodson, Joan E.; Watkins, Jason; Kennedy, Bridgett H.; Keltner, Norman L.

    2013-01-01

    To successfully negotiate and interact with one’s environment, optimal cognitive functioning is needed. Unfortunately, many neurological and psychiatric diseases impede certain cognitive abilities such as executive functioning or speed of processing; this can produce a poor fit between the patient and the cognitive demands of his or her environment. Such non-dementia diseases include bipolar disorder, schizophrenia, post-traumatic stress syndrome, depression, and anxiety disorders, just to na...

  18. Maternal Stress Induces Epigenetic Signatures of Psychiatric and Neurological Diseases in the Offspring

    Zucchi, Fabiola C. R.; Youli Yao; Ward, Isaac D.; Yaroslav Ilnytskyy; Olson, David M; Karen Benzies; Igor Kovalchuk; Olga Kovalchuk; Metz, Gerlinde A. S.

    2013-01-01

    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 gesta...

  19. Diffusion-based tractography by neurological diseases: concepts, clinical application and perspectives

    Diffusion-based tractography enables the graphical reconstruction of the white matter pathways in the brain and spinal cord of living humans. This technique has many potential clinical applications, including the investigation of stroke, multiple sclerosis, epilepsy, neurodegenerative diseases, and spinal cord disorder and it enables hypotheses to be tested that could not previously be considered in living humans. This review will outline the limitations of tractography, describe its current clinical applications in the most common neurological diseases, and highlight future opportunities. (authors)

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

    Bourassa, Megan W.; Ratan, Rajiv R.

    2014-01-01

    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 ...

  1. 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.

  2. 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.

  3. 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

  4. 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 National Hospital Register (DNHR). We inquired about epilepsy and other neurological diseases during an observation period of 27 years. A similar proportion of case- and control mothers had a diagnosis of any neurological disease, 9.9% vs 10.6%. For case...... 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....

  5. 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

    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 National Hospital Register (DNHR). We inquired about epilepsy and other neurological diseases during an observation period of 27 years. A similar proportion of case- and control mothers had a diagnosis of any neurological disease, 9.9% vs 10.6%. For case...... 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...

  6. 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 musical hallucinations involved both hemispheres with a preference towards the left, and all but two included the temporal lobe. Hearing impairment was common, particularly in the not otherwise classifiable category where 67.2% had documented hearing impairment, more than in any other group (P music, which was often religious and patriotic compared to those with a structural lesion, where more modern music was heard, and those with psychiatric disorders where music was

  7. 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.)

  8. Phenotype variability of infantile-onset multisystem neurologic, endocrine, and pancreatic disease IMNEPD.

    Picker-Minh, Sylvie; Mignot, Cyril; Doummar, Diane; Hashem, Mais; Faqeih, Eissa; Josset, Patrice; Dubern, Béatrice; Alkuraya, Fowzan S; Kraemer, Nadine; Kaindl, Angela M

    2016-01-01

    Infantile-onset multisystem neurologic, endocrine, and pancreatic disease (IMNEPD) has been recently linked to biallelic mutation of the peptidyl-tRNA hydrolase 2 gene PTRH2. Two index patients with IMNEPD in the original report had multiple neurological symptoms such as postnatal microcephaly, intellectual disability, developmental delay, sensorineural deafness, cerebellar atrophy, ataxia, and peripheral neuropathy. In addition, distal muscle weakness and abnormalities of thyroid, pancreas, and liver were found. Here, we report five further IMNEPD patients with a different homozygous PTRH2 mutation, broaden the phenotypic spectrum of the disease and differentiate common symptoms and interindividual variability in IMNEPD associated with a unique mutation. We thereby hope to better define IMNEPD and promote recognition and diagnosis of this novel disease entity. PMID:27129381

  9. RNA Structures as Mediators of Neurological Diseases and as Drug Targets.

    Bernat, Viachaslau; Disney, Matthew D

    2015-07-01

    RNAs adopt diverse folded structures that are essential for function and thus play critical roles in cellular biology. A striking example of this is the ribosome, a complex, three-dimensionally folded macromolecular machine that orchestrates protein synthesis. Advances in RNA biochemistry, structural and molecular biology, and bioinformatics have revealed other non-coding RNAs whose functions are dictated by their structure. It is not surprising that aberrantly folded RNA structures contribute to disease. In this Review, we provide a brief introduction into RNA structural biology and then describe how RNA structures function in cells and cause or contribute to neurological disease. Finally, we highlight successful applications of rational design principles to provide chemical probes and lead compounds targeting structured RNAs. Based on several examples of well-characterized RNA-driven neurological disorders, we demonstrate how designed small molecules can facilitate the study of RNA dysfunction, elucidating previously unknown roles for RNA in disease, and provide lead therapeutics. PMID:26139368

  10. Association of traumatic brain injury with subsequent neurological and psychiatric disease: a meta-analysis.

    Perry, David C; Sturm, Virginia E; Peterson, Matthew J; Pieper, Carl F; Bullock, Thomas; Boeve, Bradley F; Miller, Bruce L; Guskiewicz, Kevin M; Berger, Mitchel S; Kramer, Joel H; Welsh-Bohmer, Kathleen A

    2016-02-01

    OBJECT Mild traumatic brain injury (TBI) has been proposed as a risk factor for the development of Alzheimer's disease, Parkinson's disease, depression, and other illnesses. This study's objective was to determine the association of prior mild TBI with the subsequent diagnosis (that is, at least 1 year postinjury) of neurological or psychiatric disease. METHODS All studies from January 1995 to February 2012 reporting TBI as a risk factor for diagnoses of interest were identified by searching PubMed, study references, and review articles. Reviewers abstracted the data and assessed study designs and characteristics. RESULTS Fifty-seven studies met the inclusion criteria. A random effects meta-analysis revealed a significant association of prior TBI with subsequent neurological and psychiatric diagnoses. The pooled odds ratio (OR) for the development of any illness subsequent to prior TBI was 1.67 (95% CI 1.44-1.93, p depression, mixed affective disorders, and bipolar disorder in individuals with previous TBI as compared to those without TBI. This association was present when examining only studies of mild TBI and when considering the influence of study design and characteristics. Analysis of a subset of studies demonstrated no evidence that multiple TBIs were associated with higher odds of disease than a single TBI. CONCLUSIONS History of TBI, including mild TBI, is associated with the development of neurological and psychiatric illness. This finding indicates that either TBI is a risk factor for heterogeneous pathological processes or that TBI may contribute to a common pathological mechanism. PMID:26315003

  11. 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

  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. Activation analysis in a multitechnique study of trace element imbalances in age-related neurological diseases

    It has been suggested that several age-related neurological diseases such as Alzheimer's disease and amyotrophic lateral sclerosis may be related to environmental toxins. Bulk sample multielemental analyses by INAA alone are not adequate to define the role of trace elements in these diseases. A multitechnique approach has been developed that incorporates 14 MeV, instrumental reactor, radiochemical, and pre-irradiation chemical neutron activation analysis, together with laser microprobe mass spectrometry. The analytical scheme is able to provide bulk or protein normalized elemental concentrations, as well as microstructural, cellular, and subcellular localization information. (author) 21 refs.; 3 figs.; 3 tabs

  14. Error awareness and the insula: links to neurological and psychiatric diseases

    Tilmann A Klein

    2013-02-01

    We first discuss the main findings on error awareness in healthy subjects. A region, that appears consistently involved in error awareness processes, is the insula, which also provides a link to the clinical conditions reviewed here. Then we focus on a neurological condition whose core element is an impaired awareness for neurological consequences of a disease: anosognosia for hemiplegia (AHP. The insular cortex has been implicated in both error awareness and AHP, with anterior insular regions being involved in conscious error processing and more posterior areas being related to AHP. In addition to cytoarchitectonic and connectivity data, this reflects a functional and structural gradient within the insula from anterior to posterior. Furthermore, studies dealing with error awareness and lack of insight in a number of psychiatric diseases are reported. Especially in schizophrenia, attention-deficit hyperactivity disorder (ADHD and autism spectrum disorders the performance monitoring system seems impaired, thus conscious error perception might be altered.

  15. Clinical and neuroimaging features as diagnostic guides in neonatal neurology diseases with cerebellar involvement.

    Klein, Jessica L; Lemmon, Monica E; Northington, Frances J; Boltshauser, Eugen; Huisman, Thierry A G M; Poretti, Andrea

    2016-01-01

    Cerebellar abnormalities are encountered in a high number of neurological diseases that present in the neonatal period. These disorders can be categorized broadly as inherited (e.g. malformations, inborn errors of metabolism) or acquired (e.g. hemorrhages, infections, stroke). In some disorders such as Dandy-Walker malformation or Joubert syndrome, the main abnormalities are located within the cerebellum and brainstem. In other disorders such as Krabbe disease or sulfite oxidase deficiency, the main abnormalities are found within the supratentorial brain, but the cerebellar involvement may be helpful for diagnostic purposes. In In this article, we review neurological disorders with onset in the neonatal period and cerebellar involvement with a focus on how characterization of cerebellar involvement can facilitate accurate diagnosis and improved accuracy of neuro-functional prognosis. PMID:26770813

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

    Ridder, Hanne Mette Ochsner

    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...... it is possible to adjust the communication in order to enter dialogue. The dialogue is the potential for sharing emotions and meeting psychosocial needs. The core of the music therapy is seen as the moments where interaction and dialogue occurs. But these moments are only “highlights” of the sessions, and before....... Conclusion 1. In order to compensate for missing short-term memory it is important to work deliberately with cuing. A specific song in the beginning of the music therapy session will e.g. signalise that the music therapy is to start. Using songs as cues activates cortical memory function, and work as memory...

  17. Neurological diseases and health care utilization among first-generation immigrants.

    Rinaldi, Fabrizio; Nembrini, Stefano; Concoreggi, Carlo; Magoni, Mauro; Padovani, Alessandro

    2016-04-01

    Migrants may constitute a risk group and should have specific targets for health policy. To identify their health needs, it is important to investigate their epidemiological profile and their access to health services. The aim of this study was to identify the pattern of hospital and neurological services use among immigrants living in Brescia (Italy). The analysis took into account the records of 45,645 immigrants admitted to the ER (Emergency Room) as well as the discharge data of 6419 patients hospitalized in the Department of Neurology of the Azienda Ospedaliera Spedali Civili di Brescia, over a 3.5 years period. To take confounding factors into account, immigrant patients admitted to the department of Neurology were compared to a selection of Italian patients matched by age and sex. The main objectives were to explore causes of admission of the immigrant population-along with socio-demographic characteristics-to the Emergency Room and to the Neurology Units. Immigrants showed a similar pattern of hospital use to the Italian patients, although with a higher frequency of infective diseases and traumatic injuries. They also showed a higher mean Diagnosis-Related Group (DRG) weight than the Italians. Average length of hospitalization was longer in immigrant population. However, the use of neurological services by migrants is less than their demographic share. Poorer economic and social conditions, as well as a worse labor market experienced by immigrants may expose them to risk factors for injuries and infective diseases. Reducing the language and bureaucratic barriers, as well as enhancing cross-cultural skills of physicians, might be crucial in decreasing the length and the cost of hospitalization. PMID:26872665

  18. Error awareness and the insula: links to neurological and psychiatric diseases

    Klein, Tilmann A.; Markus Ullsperger

    2013-01-01

    Becoming aware of errors that one has committed might be crucial for strategic behavioral and neuronal adjustments to avoid similar errors in the future. This review addresses conscious error perception (“error awareness”) in healthy subjects as well as the relationship between error awareness and neurological and psychiatric diseases. We first discuss the main findings on error awareness in healthy subjects. A region, that appears consistently involved in error awareness processes, is the...

  19. Error awareness and the insula: links to neurological and psychiatric diseases

    Klein, T.A.; Ullsperger, M.; Danielmeier, C.

    2013-01-01

    Becoming aware of errors that one has committed might be crucial for strategic behavioral and neuronal adjustments to avoid similar errors in the future. This review addresses conscious error perception ("error awareness") in healthy subjects as well as the relationship between error awareness and neurological and psychiatric diseases. We first discuss the main findings on error awareness in healthy subjects. A brain region, that appears consistently involved in error awareness processes, is ...

  20. Magnetic resonance imaging in neurologic diseases--comparison with computed tomography.

    Chang, K. H.; Han, M. C.; Kim, C. W.

    1987-01-01

    Magnetic resonance (MR) and computed tomography (CT) imagings were compared in 121 patients with various neurologic diseases. MR was performed with either 0.15 Tesla resistive or 2.0 Tesla superconducting systems developed by Korea Advanced Institute of Science and Technology (KAIST), using multi-slice spin echo technique with a variety of pulse sequences reflecting proton density, T1 and T2 relaxation times. MR was more advantageous in the detection of the lesions, accurate depiction of the ...

  1. Genetics of complex neurological disease: Challenges and opportunities for modeling epilepsy in mice and rats

    Frankel, Wayne N.

    2009-01-01

    Epilepsy is a complex neurological disease. Currently ~20 genetic variants are known to cause Mendelian forms of human epilepsy, leaving a vast heritability undefined with future hopes resting on candidate gene resequencing and/or large scale genome-wide association studies. Rodent models for genetically complex epilepsy have been studied for many years, but only recently have strong candidate genes emerged, including Cacna1g in the GAERS rat model of absence epilepsy and Kcnj10 in the low se...

  2. Infection of Immunodeficient Horses with Sarcocystis neurona Does Not Result in Neurologic Disease

    Sellon, Debra C.; Knowles, Donald P.; Ellis C Greiner; Long, Maureen T.; Hines, Melissa T.; Hochstatter, Tressa; Tibary, Ahmed; Dame, John B

    2004-01-01

    Equine protozoal myeloencephalitis is a progressive neurologic disease of horses most commonly caused by infection with the apicomplexan parasite Sarcocystis neurona. Factors affecting neuroinvasion and neurovirulence have not been determined. We investigated the pathogenesis of infection with S. neurona in horses with severe combined immune deficiency (SCID). Two immunocompetent (IC) Arabian horses and two Arabian horses with SCID were infected orally with 5 × 105 sporocysts of S. neurona. F...

  3. An Acute Hemorrhagic Infectious Disease: Ebola Virus Disease

    Lei JIAO

    2014-09-01

    Full Text Available 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.

  4. Iodobenzamide SPET in neurological and psychiatric diseases: technical aspects and clinical impact

    Cerebral dopamine receptor assessment using single photon emission tomography (SPET) was recently accepted as a valuable method for a relative quantification of these receptors. We have performed 45 examinations using iodobenzamide (123-IBZM), which is a ligand for post-synaptic D2 receptors, in a variety of neurological or psychiatric diseases in which the dopaminergic system could be involved. The relative quantification of these D2 receptors was performed using different ratios with striatal, cerebellar and total tracer uptake. We observed a significant decrease in 123-IBZM striatal tracer uptake in Parkinson's disease, supra-nuclear palsy, Alzheimer disease or Huntington disease, in comparison with control subjects (p<0.05). The present study indicates that 123-IBZM SPET could be easily performed in the different nuclear medicine departments and is useful in atypical Parkinson's disease to access the diagnosis and drug efficacy. This could be of prognostic value in suspected or marked Huntington's disease. (authors). 27 refs., 1 tab., 4 figs

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

    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.)

  6. HTLV-I/II seroindeterminate Western blot reactivity in a cohort of patients with neurological disease.

    Soldan, S S; Graf, M D; Waziri, A; Flerlage, A N; Robinson, S M; Kawanishi, T; Leist, T P; Lehky, T J; Levin, M C; Jacobson, S

    1999-09-01

    The human T-cell lymphotropic virus type I (HTLV-I) is associated with a chronic, progressive neurological disease known as HTLV-I-associated myelopathy/tropical spastic paraparesis. Screening for HTLV-I involves the detection of virus-specific serum antibodies by EIA and confirmation by Western blot. HTLV-I/II seroindeterminate Western blot patterns have been described worldwide. However, the significance of this blot pattern is unclear. We identified 8 patients with neurological disease and an HTLV-I/II seroindeterminate Western blot pattern, none of whom demonstrated increased spontaneous proliferation and HTLV-I-specific cytotoxic T lymphocyte activity. However, HTLV-I tax sequence was amplified from the peripheral blood lymphocytes of 4 of them. These data suggest that patients with chronic progressive neurological disease and HTLV-I/II Western blot seroindeterminate reactivity may harbor either defective HTLV-I, novel retrovirus with partial homology to HTLV-I, or HTLV-I in low copy number. PMID:10438355

  7. [Swiss scrapie surveillance. I. Clinical aspects of neurological diseases in sheep and goats].

    Maurer, E; Botteron, C; Ehrensperger, F; Fatzer, R; Jaggy, A; Kolly, C; Meylan, M; Zurbriggen, A; Doherr, M G

    2005-10-01

    Small ruminants infected with scrapie show a large range of often unspecific clinical symptoms. The most-often described signs, locomotion, sensibility and behavioural disorders and emaciation, rarely occur together, and cases have been described in which only one of those signs was detectable.Thus, formulating a well-circumscribed definition of a clinical suspect case is difficult. Most animals with CNS-effecting diseases such as listeriosis, polioencephalomacia, cerebrospinal nematidiasis and enterotoxemia will, in a thorough neurological examination, show at least some scrapie-like symptoms. Among the 22 neurological field cases examined in this study, a goat with cerebral gliomatosis and hair lice showed the closest similarity to clinical scrapie. The unilateral deficiency of the cerebral nerves has potential as an clinical exclusion criterion for scrapie. However, the laboratory confirmation--or exclusion--of scrapie remains important. It thus needs to be realized that a consistent and thorough examination of neurologically diseased small ruminants (including fallen stock) is the backbone of a good surveillance system for these diseases. This should be a motivation for submitting adult sheep and goats for neuropathological examination. PMID:16259408

  8. Neurogenesis in neurological and psychiatric diseases and brain injury: from bench to bedside.

    Ruan, Linhui; Lau, Benson Wui-Man; Wang, Jixian; Huang, Lijie; Zhuge, Qichuan; Wang, Brian; Jin, Kunlin; So, Kwok-Fai

    2014-04-01

    Researchers who have uncovered the presence of stem cells in an adult's central nervous system have not only challenged the dogma that new neurons cannot be generated during adulthood, but also shed light on the etiology and disease mechanisms underlying many neurological and psychiatric disorders. Brain trauma, neurodegenerative diseases, and psychiatric disorders pose enormous burdens at both personal and societal levels. Although medications for these disorders are widely used, the treatment mechanisms underlying the illnesses remain largely elusive. In the past decade, an increasing amount of evidence indicate that adult neurogenesis (i.e. generating new CNS neurons during adulthood) may be involved in the pathology of different CNS disorders, and thus neurogenesis may be a potential target area for treatments. Although new neurons were shown to be a major player in mediating treatment efficacy of neurological and psychotropic drugs on cognitive functions, it is still debatable if the altered production of new neurons can cause the disorders. This review hence seeks to discuss pre and current clinical studies that demonstrate the functional impact adult neurogenesis have on neurological and psychiatric illnesses while examining the related underlying disease mechanisms. PMID:24384539

  9. Microcirculation in Acute and Chronic Kidney Diseases.

    Zafrani, Lara; Ince, Can

    2015-12-01

    The renal microvasculature is emerging as a key player in acute and chronic kidney diseases. Renal microvascular disease involves alterations in endothelial barrier permeability, exaggerated inflammation, impairment of endothelium-dependent vasorelaxation involving the nitric oxide system, increased oxidative stress, and loss of angiogenic factors. Moreover, evidence suggests that there is a microvascular component to the pathogenesis of renal scarring. New technology is being developed to explore renal microcirculation in vivo in experimental models and humans. This technology will provide a better understanding of the pathogenesis of kidney diseases and will help guide specific therapeutic strategies aimed at restoring the renal microcirculation. This article reviews the cellular and molecular mechanisms of renal microvascular dysfunction in acute and chronic kidney diseases and the potential diagnostic and therapeutic implications of these findings. Recent developments in the monitoring of renal microcirculation are described with respect to their advantages and limitations, and future directions are outlined. PMID:26231789

  10. Benefit of intravenous antibiotic therapy in patients referred for treatment of neurologic Lyme disease

    Stricker RB

    2011-09-01

    Full Text Available Raphael B Stricker1,3, Allison K DeLong2, Christine L Green1,3, Virginia R Savely1,3, Stanley N Chamallas1,4, Lorraine Johnson1,31International Lyme and Associated Diseases Society, Bethesda, MD, USA; 2Center for Statistical Sciences, Brown University, Providence, RI, USA; 3California Lyme Disease Association, Marysville, CA, USA; 4QMedRx Inc, Maitland, FL, USABackground: We have shown previously that extended intravenous antibiotic therapy is associated with low morbidity and no mortality in patients referred for treatment of neurologic Lyme disease. In this study, we evaluated the benefit of extended intravenous antibiotic therapy in patients with symptoms of neurologic Lyme disease.Methods: Patients with significant neurologic symptoms and positive testing for Borrelia burgdorferi were treated with intravenous antibiotics, and biweekly evaluation of symptom severity was performed using a six-level ordinal scale. Four symptoms were selected a priori as primary outcome measures in the study, ie, fatigue, cognition, myalgias, and arthralgias. Patients were placed into five groups according to time on treatment (1–4, 5–8, 9–12, 13–24, and 25–52 weeks, and changes in the primary symptoms as a function of time on treatment were analyzed using a mixed-effects proportional odds model.Results: Among 158 patients with more than one follow-up visit who were monitored for up to 1 year, there were on average 6.7 visits per person (median 5, range 2–24. The last follow-up day was on average 96 days after enrollment (median 69, range 7–354 days, corresponding to the length of antibiotic therapy. Each primary symptom was significantly improved at one or more time points during the study. For cognition, fatigue, and myalgias, the greatest improvement occurred in patients on the longest courses of treatment (25–52 weeks with odds ratios (OR for improvement of 1.97 (P = 0.02, 2.22 (P < 0.01, and 2.08 (P = 0.01, respectively. In contrast

  11. Crohn's Disease and Acute Pancreatitis: A Review of Literature

    Sarfaraz Jasdanwala; Mark Babyatsky

    2015-01-01

    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 ...

  12. Strain Echocardiography in Acute Cardiovascular Diseases.

    Favot, Mark; Courage, Cheryl; Ehrman, Robert; Khait, Lyudmila; Levy, Phillip

    2016-01-01

    Echocardiography has become a critical tool in the evaluation of patients presenting to the emergency department (ED) with acute cardiovascular diseases and undifferentiated cardiopulmonary symptoms. New technological advances allow clinicians to accurately measure left ventricular (LV) strain, a superior marker of LV systolic function compared to traditional measures such as ejection fraction, but most emergency physicians (EPs) are unfamiliar with this method of echocardiographic assessment. This article discusses the application of LV longitudinal strain in the ED and reviews how it has been used in various disease states including acute heart failure, acute coronary syndromes (ACS) and pulmonary embolism. It is important for EPs to understand the utility of technological and software advances in ultrasound and how new methods can build on traditional two-dimensional and Doppler techniques of standard echocardiography. The next step in competency development for EP-performed focused echocardiography is to adopt novel approaches such as strain using speckle-tracking software in the management of patients with acute cardiovascular disease. With the advent of speckle tracking, strain image acquisition and interpretation has become semi-automated making it something that could be routinely added to the sonographic evaluation of patients presenting to the ED with cardiovascular disease. Once strain imaging is adopted by skilled EPs, focused echocardiography can be expanded and more direct, phenotype-driven care may be achievable for ED patients with a variety of conditions including heart failure, ACS and shock. PMID:26823931

  13. Acute dysautonomia associated with Hodgkin's disease.

    van Lieshout, J. J.; Wieling, W.; van Montfrans, G A; Settels, J J; Speelman, J D; Endert, E.; Karemaker, J. M.

    1986-01-01

    A patient is described with acute dysautonomia associated with Hodgkin's disease. Testing of cardiovascular reflex control showed that this patient had a rare manifestation of autonomic cardiovascular neuropathy, namely intact parasympathetic heart rate control in combination with a sympathetic postganglionic lesion affecting the control of the vascular tree.

  14. Minimal Residual Disease in Acute Myeloid Leukemia

    Ommen, Hans Beier; Nederby, Line; Toft-Petersen, Marie;

    2014-01-01

    This chapter discusses how minimal residual disease (MRD) is detected and managed in acute myeloid leukemia (AML) patients. The most commonly used techniques to detect residual leukemia in patients in complete remission (CR) are quantitative PCR (qPCR) and multicolor flow cytometry (MFC). While q...

  15. Association of neurological diseases with metabolic syndrome among out-patients

    Metabolic syndrome (MetS) is highly prevalent in Japan; however, most previous surveys have studied only adults able to engage fully in normal daily activities, after excluding persons with diseases or disabilities. Recently, lifestyle-related risk factors have been strongly linked to a number of major diseases. In particular, the incidence of atherosclerotic vascular diseases associated with MetS has increased markedly, and this trend is projected to continue. We focused on the prevalence of MetS among out-patients with neurological diseases. The subjects for this hospital-based study were 713 out-patients with various neurological diseases (329 men, mean age 65.2±14.5 yr, age range 40-78 yr, and 384 women, mean age 64.6±15.3 yr, age range 40-88 yr) who presented at the Department of Neurology, Nara Medical University Hospital. A total of 120 patients had cerebral infarction, 102 Parkinson's disease, 32 spinal spondylosis, 30 headache, 32 myositis, and the rest various other neurological diseases. MetS was diagnosed according to the criteria proposed by The Japanese Society of Internal Medicine in 2005. The cutoff values for waist circumference (WC) were greater than 85 cm in men and 90 cm in women. A diagnosis of MetS additionally required two or more of the following: a serum triglyceride level (TG) of at least 150 mg/dl and/or a high-density lipoprotein cholesterol level (HDL-C) of less than 40 mg/dl; a blood pressure (BP) of greater than 130/85; or a fasting plasma glucose level (FPG) of greater than 110 mg/dl. Visceral fat accumulation was measured by abdominal CT scanning (N2system, K.K., Japan). WC positively correlated with visceral fat area as determined by CT scanning. WC also positively correlated with TG in both sexes and fasting blood sugar (FBS) in women, but negatively correlated with HDL-C in both sexes. The mean prevalence of MetS among subjects 40 to 70 years of age was 25.1% in men and 12.6% in women. To assess the incidence of MetS in the

  16. Focused ultrasound as a non-invasive intervention for neurological disease: a review.

    Piper, Rory J; Hughes, Mark A; Moran, Carmel M; Kandasamy, Jothy

    2016-06-01

    Focused ultrasound (FUS) is an incision-less intervention that is a Food and Drug Association (FDA) approved surgical treatment for various pathologies including uterine fibroids and bone metastases. Recent advances in magnetic resonance imaging thermometry and ability to use FUS across the intact calvarium have re-opened interest in the use of FUS in the treatment of neurological diseases. FUS currently has a European CE mark for use in movement disorders. However, it shows potential in the treatment of other neuropathologies including tumours and as a lesional tool in epilepsy. FUS may exert its therapeutic effect through thermal or mechanical fragmentation of intracranial lesions, or by enhancing delivery of pharmaceutical agents across the blood-brain barrier. In this review, we summarise the mechanisms, clinical applications and potential future of FUS for the treatment of neurological disease. We have searched for and described the recently completed and on-going clinical trials investigating FUS for the treatment of neurological disorders. We identified phase one trials investigating utility of FUS in: movement disorders (including essential tremor and Parkinson's disease), chronic pain, obsessive-compulsive disorder and cerebral tumours. Current literature also reports pre-clinical work exploring utility in epilepsy, neurodegenerative conditions (such as Alzheimer's disease) and thrombolysis. Safety and early efficacy data are now emerging, suggesting that transcalvarial FUS is a feasible and safe intervention. Further evidence is required to determine whether FUS is an effective alternative in comparison to current neurosurgical interventions. The cost of requisite hardware is currently a barrier to widespread uptake in UK neurosurgical centres. PMID:27101792

  17. 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.

  18. Neurological PRESentations in Sickle Cell Patients Are Not Always Stroke: A Review of Posterior Reversible Encephalopathy Syndrome in Sickle Cell Disease.

    Solh, Ziad; Taccone, Michael S; Marin, Samantha; Athale, Uma; Breakey, Vicky R

    2016-06-01

    Acute neurological changes in sickle cell disease (SCD) patients often raise the suspicion for stroke. Posterior reversible encephalopathy syndrome (PRES) can mimic stroke in its clinical presentation. We aimed to (i) review the PRES literature in SCD patients including clinical presentation, risk factors, pathophysiology, and management and (ii) elucidate the distinction between PRES and stroke in SCD. The exact pathophysiology of PRES in SCD remains elusive but is likely multifactorial and related to sickling, ischemia, and chronic anemia predisposing to vasogenic edema. PRES and stroke in SCD are distinguishable conditions. Our review may help elucidate a clinical approach to this distinction. PMID:26871763

  19. 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.

  20. Clinical studies on rising and re-rising neurological diseases in Japan--a personal contribution.

    Igata, Akihiro

    2010-01-01

    Throughout my research life, I experienced to discover the causes of some neurological diseases in Japan. 1) SMON (subacute myelo-optico-neuropathy). Since the early 1960s, a peculiar neurological disease became prevalent throughout Japan. Through the chemical analysis of the green urine, characteristic of this disease, it was found that this disease was caused by intoxication of the administered clioquinol, an anti-diarrheal drug. This discovery is a big topic in the history of Japanese medicine. 2) In early 1970s, I experienced many young patients with oedema and polyneuropathy in Kagoshima. Finally it was found that the disease was the long-forgotten beriberi, which had disappeared several decades ago. We must always be aware of beriberi even now, as far as we eat well-polished rice. 3) In 1972, we noticed a group of sporadic paraparesis in Kagoshima, which was 20 years later confirmed to be induced by human T lymphotropic virus type-I (HTLV-I). We named this disease as "HTLV-I associated myelopathy" (HAM). It gave a strong impact that the causative virus of adult T cell leukemia (ATL) can induce entirely different diseases, in terms of both the clinical course and the pathological features. It was also proven that HAM was identical with tropical spastic paraparesis, (TSP), which had been prevalent in many areas of tropical zones. These experiences are good examples of our slogan "to keep in mind to send message of scientific progress from the local area to the international stage. PMID:20431261

  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. MINIMAL RESIDUAL DISEASE IN ACUTE LYMPHOBLASTIC LEUKEMIA

    Campana, Dario

    2009-01-01

    In patients with acute lymphoblastic leukemia (ALL), monitoring of minimal residual disease (MRD) offers a way to precisely assess early treatment response and detect relapse. Established methods to study MRD are flow cytometric detection of abnormal immunophenotypes, polymerase chain reaction (PCR) amplification of antigen-receptor genes, and PCR amplification of fusion transcripts. The strong correlation between MRD levels and risk of relapse in childhood ALL is well established; studies in...

  3. 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. PMID:25909152

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

    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

  5. 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.

  6. 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.

  7. Neurological status predicts response to alpha-blockers in men with voiding dysfunction and Parkinson's disease

    Cristiano M. Gomes

    2014-01-01

    Full Text Available OBJECTIVES: To evaluate predictors of the response to doxazosin, a selective alpha-adrenoceptor antagonist, when used for the treatment of lower urinary tract symptoms in men with Parkinson's disease. METHODS: In a prospective study, 33 consecutive men (mean age 59.2±7.0 years with Parkinson's disease and lower urinary tract symptoms were evaluated. Neurological dysfunction was assessed with the Unified Parkinson's Disease Rating Scale. Urological assessment was performed at baseline and after 12 weeks of treatment with 4 mg/day of extended-release doxazosin, including symptom evaluation with the International Continence Society male short-form questionnaire, an assessment of the impact of lower urinary tract symptoms on quality of life and urodynamics. Clinical and urodynamic predictors of response were specifically evaluated. RESULTS: Compared with the score at baseline, the total International Continence Society male short-form score was reduced after doxazosin administration, from 17.4±7.5 to 11.1±6.9 (p<0.001. The impact of lower urinary tract symptoms on quality of life was also significantly reduced, from 1.8±1.1 to 1.0±1.0 (p<0.001 and the maximum urinary flow varied from 9.3±4.4 to 11.2±4.6 ml/s (p = 0.025. The severity of neurological impairment was the only predictor of the clinical response. Additionally, patients with a Unified Parkinson's Disease Rating Scale score lower than 70 had a significantly higher chance of clinical improvement with doxazosin treatment than those with higher Unified Parkinson's Disease Rating Scale scores did (RR = 3.10, 95% CI = [1.15 to 5.37], p = 0.011. CONCLUSIONS: Doxazosin resulted in the improvement of lower urinary tract symptoms and the maximum flow rate and was well tolerated in men with Parkinson's disease. The response to treatment is dependent on the severity of neurological disability.

  8. Resting-state networks link invasive and noninvasive brain stimulation across diverse psychiatric and neurological diseases.

    Fox, Michael D; Buckner, Randy L; Liu, Hesheng; Chakravarty, M Mallar; Lozano, Andres M; Pascual-Leone, Alvaro

    2014-10-14

    Brain stimulation, a therapy increasingly used for neurological and psychiatric disease, traditionally is divided into invasive approaches, such as deep brain stimulation (DBS), and noninvasive approaches, such as transcranial magnetic stimulation. The relationship between these approaches is unknown, therapeutic mechanisms remain unclear, and the ideal stimulation site for a given technique is often ambiguous, limiting optimization of the stimulation and its application in further disorders. In this article, we identify diseases treated with both types of stimulation, list the stimulation sites thought to be most effective in each disease, and test the hypothesis that these sites are different nodes within the same brain network as defined by resting-state functional-connectivity MRI. Sites where DBS was effective were functionally connected to sites where noninvasive brain stimulation was effective across diseases including depression, Parkinson's disease, obsessive-compulsive disorder, essential tremor, addiction, pain, minimally conscious states, and Alzheimer's disease. A lack of functional connectivity identified sites where stimulation was ineffective, and the sign of the correlation related to whether excitatory or inhibitory noninvasive stimulation was found clinically effective. These results suggest that resting-state functional connectivity may be useful for translating therapy between stimulation modalities, optimizing treatment, and identifying new stimulation targets. More broadly, this work supports a network perspective toward understanding and treating neuropsychiatric disease, highlighting the therapeutic potential of targeted brain network modulation. PMID:25267639

  9. Studies of generalized elemental imbalances in neurological disease patients using INAA [instrumental neutron activation analysis

    Evidence has been presented in the literature to implicate trace elements in the etiology of several age-related neurological diseases. Most of these studies are based on brain analyses. Using instrumental neutron activation analysis (INAA), we have observed trace element imbalances in brains of patients with Alzheimer's disease, amyotrophic lateral sclerosis (ALS), and Picks's disease. The most prevalent elemental imbalances found in the brain were for bromine, mercury, and the alkali metals. In this study the authors report INAA studies of trace elements in nonneural tissues from Alzheimer's disease and ALS patients. Samples from household relatives were collected for use as controls wherever possible. Hair samples were washed according to the International Atomic Energy Agency recommended procedure. Fingernail samples were scraped with a quartz knife prior to washing by the same procedure. For ALS patients, blood samples were also collected. These data indicate that elemental imbalances in Alzheimer's disease and ALS are not restricted to the brain. Many elements perturbed in the brain are also altered in the several nonneural tissues examined to date. The imbalances in different tissues, however, are not always in the same direction. The changes observed may represent causes, effects, or simply epiphenomena. Longitudinal studies of nonneural tissues and blood, as well as tissue microprobe analyses at the cellular and subcellular level, will be required in order to better assess the role of trace elements in the etiology of these diseases

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

    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. 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.

  12. 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.

  13. Crohn's Disease and Acute Pancreatitis: A Review of Literature

    Sarfaraz Jasdanwala

    2015-03-01

    Full Text Available 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.

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

    Lili Long; Lin Xu; Zhenghui Xiao; Shixiong Hu; Ruping Luo; Hua Wang; Xiulan Lu; Zhiyue Xu; Xu Yao; Luo Zhou; Hongyu Long; Jiaoe Gong; Yanmin Song; Li Zhao; Kaiwei Luo

    2016-01-01

    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 ...

  15. Burden of invasive group B Streptococcus disease and early neurological sequelae in South African infants.

    Ziyaad Dangor

    Full Text Available Group B Streptococcus (GBS is a leading cause of neonatal sepsis and meningitis. We aimed to evaluate the burden of invasive early-onset (0-6 days of life, EOD and late-onset (7-89 days, LOD GBS disease and subsequent neurological sequelae in infants from a setting with a high prevalence (29.5% of HIV among pregnant women.A case-control study was undertaken at three secondary-tertiary care public hospitals in Johannesburg. Invasive cases in infants <3 months age were identified by surveillance of laboratories from November 2012 to February 2014. Neurodevelopmental screening was done in surviving cases and controls at 3 and 6 months of age.We identified 122 cases of invasive GBS disease over a 12 month period. Although the incidence (per 1,000 live births of EOD was similar between HIV-exposed and HIV-unexposed infants (1.13 vs. 1.46; p = 0.487, there was a 4.67-fold (95%CI: 2.24-9.74 greater risk for LOD in HIV-exposed infants (2.27 vs. 0.49; p<0.001. Overall, serotypes Ia, Ib and III constituted 75.8% and 92.5% of EOD and LOD, respectively. Risk factors for EOD included offensive draining liquor (adjusted Odds Ratio: 27.37; 95%CI: 1.94-386.50 and maternal GBS bacteriuria (aOR: 8.41; 95%CI: 1.44-49.15, which was also a risk-factor for LOD (aOR: 3.49; 95%CI: 1.17-10.40. The overall case fatality rate among cases was 18.0%. The adjusted odds for neurological sequelae at 6 months age was 13.18-fold (95%CI: 1.44-120.95 greater in cases (13.2% than controls (0.4%.The high burden of invasive GBS disease in South Africa, which is also associated with high case fatality rates and significant neurological sequelae among survivors, is partly due to the heightened risk for LOD in infants born to HIV-infected women. An effective trivalent GBS conjugate vaccine targeted at pregnant women could prevent invasive GBS disease in this setting.

  16. Decrease of CBF measured by SPECT correlates to neurological features in Wilson's disease

    Objective: To investigate changes of cerebral blood flow and its association with neurological features in the patients with Wilson's disease (WD). Methods: In 13 WD patients and 12 age-matched control subjects, the authors quantitatively determined CBF and rCBF with SPECT using the non-blood-withdraw method. Five neurological signs including dysarthria, dystonia, bradykinesia/rigidity, tremor and gait ataxia were assessed semi-quantitatively. The severity of each sign was scored from 0 (absent) to 3 (severe). The individual scores collected from a subject were then summed up, resulting in a 'total clinical score' (TCS). The authors studied the correlations between the CBF or rCBF and semiquantitative clinical scores. Results: CBF in case group was (40.94 +- 2.33) mL·100 g-1·min-1 and was significantly lower than that in control group [(43.82 +- 1.58) mL·100 g-1·min-1] (P<0.01). Statistical comparison found more significant rCBF reduction in bilateral basal ganglia, thalamus, front lobe (P<0.01) and left temporal lobe (P<0.05). The reduction in CBF was correlated with the TCS (r = -0.620, P<0.05). The reduction in rCBF of bilateral basal ganglia correlated with the severity of dysarthria (r = -0.770, P<0.01 and -0.576, P<0.05). The reduction in rCBF of right parietal lobe and bilateral thalamus correlated with the severity of bradykinesia/rigidity (r = -0.757, P<0.01 and -0.635, P<0.05). Conclusions: These results suggest Wilson's disease patients have CBF or rCBF reduction to different degrees, and lower rCBF was found in bilateral basal ganglia, thalamus, front lobe and left temporal lobe. The neurological deficits may have relationship with reduction of cerebral blood flow in Wilson's disease. SPECT is useful for evaluation of cerebral function in Wilson's disease patients

  17. 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.

  18. Transvaginal sonography of acute pelvic inflammatory disease

    To determine the value of transvaginal sonography in evaluating women with acute pelvic inflammatory disease (PID). Transvaginal sonography was performed in 25 patients with clinically suggested PID during recent 36 months. The sonographic findings of fallopian tubes and ovaries were analyzed and correlated with pathological findings of 2 fallopian tubes and 19 ovaries in 16 patients who had operations. The correct diagnosis of acute PID was made in 20/25 (80%) by transvaginal sonography. the abnormal sonographic findings of the fallopian tube include tubal thickening or dilatation with internal echo. The sensitivity, specificity, and accuracy for tubal abnormality were 88%, 96%, and 86% , respectively. Ovarian changes were seen on TVS in 14/19 (73%), which include multiple follicular enlargement in 5, tubo-ovarian complex in 9 (tubo-ovarian adhesion in 3, tubo-ovarian abscess in 6). At surgery, the ovay was not involved in all three women who showed tubo-ovarian adhesion on TVS. Among 6 women who showed tubo-ovarian abscess on TVS, tubo-ovarian abscess was confirmed in 3 and the remaining 3 had ovarian cysts. Trandvaginal sonography, a facilitative and accurate modality, is highly sensitive in detecting the abnormality of the tube and useful in differentiating the tubo-ovarian complex in patients with acute PID.

  19. 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

    2014-01-01

    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,...

  20. 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.

  1. 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.

  2. Impact of Rhesus disease on the global problem of bilirubin-induced neurologic dysfunction.

    Zipursky, Alvin; Bhutani, Vinod K

    2015-02-01

    Clinical experience with Rhesus (Rh) disease and its post-icteric sequelae is limited among high-income countries because of nearly over four decades of effective prevention care. We hypothesized that Rh disease is prevalent in other regions of the world because it is likely that protection is limited or non-existent. Following a worldwide study, it has been concluded that Rh hemolytic disease is a significant public health problem resulting in stillbirths and neonatal deaths, and is a major cause of severe hyperbilirubinemia with its sequelae, kernicterus and bilirubin-induced neurologic dysfunction. Knowing that effective Rh-disease prophylaxis depends on maternal blood-type screening, healthcare afforded to the high-risk mothers needs to be free of bottlenecks and coupled with unfettered access to effective Rh-immunoglobulin. Future studies that match the universal identification of Rh-negative status of women and targeted use of immunoprophylaxis to prevent childhood bilirubin neurotoxicity are within reach, based on vast prior experiences. PMID:25582277

  3. 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

  4. [My way to "Keep Pioneering": integrated neuroscience and immunology research produces a paradigm shift for intractable neurological disease].

    Kira, Jun-ichi

    2014-01-01

    The motto of Prof. Yoshigoro Kuroiwa, who established the first independent neurology department in Japan at Kyushu University, is "Keep Pioneering". His students have followed this motto in all fields. I hereby present my efforts to keep pioneering in the following fields: (1) multiple sclerosis (MS); (2) central nervous system (CNS) involvement associated with peripheral atopic inflammation; and (3) care network for patients with intractable neurological disease. In MS, I propose that Th1/Th17 cell-mediated connexin astrocytopathy may play a critical role in producing huge demyelinating lesions in MS, neuromyelitis optica (NMO), and Baló's concentric sclerosis. I discovered a peculiar myelitis that occurred in patients with atopic disorders, and designated it atopic myelitis. In this condition, allodynia and neuropathic pain are cardinal features, regardless of the presence or absence of spinal cord MRI lesions. We found that peripheral atopic inflammation in mice produces allodynia as well as activation of microglia and astroglia in the spinal cord. It is important to involve a variety of medical specialists and care coordinators for collaborative work on medical and social care issues for people with intractable disease. The motto of "Keep Pioneering" in neurology covers not only advanced research for the creation of new therapies for intractable neurological disease, but also caring for actual people with intractable disease, which I believe is the corporate social responsibility of our neurological society. I think that "Keep Pioneering" is a challenging process that never ends throughout one's life. PMID:25672676

  5. Clinical impact of 18F-fluorodeoxyglucose positron emission tomography in the diagnosis of neurological diseases

    In this review it will be discussed in which neurological disorders positron emission tomography can yield important diagnostic information. Because positron emission tomography is an expensive method indications have to be cleary defined. One important question concerns the differentiation of tumor recurrence and scar due to radiation therapy or an operation. The grading of brain tumors is another application. In HIV patients fluorodeoxyglucose positron emission tomography can separate lymphoma and toxoplasmosis. In the evaluation of dementia positron emission tomography can help to clarify the differential diagnosis. Another important area is the presurgical evaluation of epilepsy patients and patients with cerebrovascular disease in whom a surgical revascularization procedure is planned. In extrapyramidal disorders, positron emission tomography can often help to establish the final diagnosis. (author)

  6. Histone turnover and chromatin accessibility: Critical mediators of neurological development, plasticity, and disease.

    Wenderski, Wendy; Maze, Ian

    2016-05-01

    In postmitotic neurons, nucleosomal turnover was long considered to be a static process that is inconsequential to transcription. However, our recent studies in human and rodent brain indicate that replication-independent (RI) nucleosomal turnover, which requires the histone variant H3.3, is dynamic throughout life and is necessary for activity-dependent gene expression, synaptic connectivity, and cognition. H3.3 turnover also facilitates cellular lineage specification and plays a role in suppressing the expression of heterochromatic repetitive elements, including mutagenic transposable sequences, in mouse embryonic stem cells. In this essay, we review mechanisms and functions for RI nucleosomal turnover in brain and present the hypothesis that defects in histone dynamics may represent a common mechanism underlying neurological aging and disease. PMID:26990528

  7. 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.

  8. Pathophysiology of acute small bowel disease with CT correlation

    The objective of this article is to review the pathophysiology of acute small bowel diseases, and to correlate the mechanisms of disease with computed tomography (CT) findings. Disease entities will be classified into the following: immune mediated and infectious causes, vascular causes, mechanical causes, trauma, and others. Having an understanding of acute small bowel pathophysiology is a useful teaching tool, and can lead to imaging clues to the most likely diagnosis of acute small bowel disorders.

  9. Pathophysiology of acute small bowel disease with CT correlation

    Sarwani, N., E-mail: nsarwani@hmc.psu.ed [Department of Radiology, Section of Abdominal Imaging, Penn State Milton Hershey Medical Center, Hershey, PA (United States); Tappouni, R.; Tice, J. [Department of Radiology, Section of Abdominal Imaging, Penn State Milton Hershey Medical Center, Hershey, PA (United States)

    2011-01-15

    The objective of this article is to review the pathophysiology of acute small bowel diseases, and to correlate the mechanisms of disease with computed tomography (CT) findings. Disease entities will be classified into the following: immune mediated and infectious causes, vascular causes, mechanical causes, trauma, and others. Having an understanding of acute small bowel pathophysiology is a useful teaching tool, and can lead to imaging clues to the most likely diagnosis of acute small bowel disorders.

  10. 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

  11. 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.

  12. Responsibilities of Health Care Professionals in Counseling and Educating Patients With Incurable Neurological Diseases Regarding "Stem Cell Tourism": Caveat Emptor.

    Bowman, Michelle; Racke, Michael; Kissel, John; Imitola, Jaime

    2015-11-01

    "Stem cell tourism" is a rising Internet-based industry that aims to offer unproven procedures to patients with incurable diseases. This unregulated activity is reaching the neurologist's office as well as across the world, as patients request information or clearance for such procedures. Herein, we posit the need for medical societies and licensing boards to bring this issue to the forefront of neurology because it has the potential to affect patient care with risk of morbidity and mortality, as well as to undermine public confidence in legitimate stem cell research for incurable neurological diseases such as multiple sclerosis and amyotrophic lateral sclerosis. PMID:26322563

  13. 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.

  14. 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

  15. 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

  16. 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

    2016-01-01

    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. PMID:27188830

  17. 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

  18. The use of herpes simplex virus vectors for gene therapy in neurological diseases.

    Kennedy, P G; Steiner, I

    1993-11-01

    Herpes simplex virus type 1 (HSV-1) vectors have now been developed and enable the efficient delivery of foreign genes under the control of appropriate promoter elements into non-dividing neurons in vitro and in vivo. Their use is based on the natural ability of HSV-1 to spread throughout the nervous system and to establish a lifelong latent infection in neurons. HSV is present in an episomal form in the neuronal nucleus, and normal neuronal functions remain unaltered. A wide variety of foreign genes can theoretically be packaged into the large HSV genome. A number of technical problems will need to be overcome to ensure the stable expression of the foreign gene products, adequate control of the levels of their expression, the safety of the vectors and the correct targeting of the vectors to the appropriate neuronal cell populations. Such vectors have the potential to replace missing gene products in neurons in patients with a variety of metabolic and neurodegenerative diseases, and also to insert growth factors or enzymes into the local vicinity of neurological lesions to promote neuronal repair. HSV-1 vectors also have the potential to define the genetic basis of various neurophysiological functions which may prove to be useful in evaluating altered neuronal function encountered in disease. PMID:8265768

  19. Modeling Neurological Disease by Rapid Conversion of Human Urine Cells into Functional Neurons

    Shu-Zhen Zhang

    2016-01-01

    Full Text Available Somatic cells can be directly converted into functional neurons by ectopic expression of defined factors and/or microRNAs. Since the first report of conversion mouse embryonic fibroblasts into functional neurons, the postnatal mouse, and human fibroblasts, astroglia, hepatocytes, and pericyte-derived cells have been converted into functional dopaminergic and motor neurons both in vitro and in vivo. However, it is invasive to get all these materials. In the current study, we provide a noninvasive approach to obtain directly reprogrammed functional neurons by overexpression of the transcription factors Ascl1, Brn2, NeuroD, c-Myc, and Myt1l in human urine cells. These induced neuronal (iN cells could express multiple neuron-specific proteins and generate action potentials. Moreover, urine cells from Wilson’s disease (WD patient could also be directly converted into neurons. In conclusion, generation of iN cells from nonneural lineages is a feasible and befitting approach for neurological disease modeling.

  20. 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…

  1. 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...... treatment with TNF inhibitors are aware of this potentially serious AE and report these events to the proper medical authorities....

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

    Abdulkadir Turgut; Ali Özler; Neval Yaman Görük; Senem Yaman Tunç; Nurullah Peker; Recep Tekin

    2013-01-01

    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 ...

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

    Kostev, Karel; Rex, Juliana; Waehlert, Lilia; Hog, Daniela; Heilmaier, Christina

    2014-01-01

    [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 mobbin...

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

    Kostev, K.; Rex, J.; Waehlert, L; Hog, D; Heilmaier, C

    2014-01-01

    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.Materia...

  5. Hans von Bülow: creativity and neurological disease in a famous pianist and conductor.

    Wöhrle, Johannes C; Haas, Frithjof

    2007-01-01

    Hans von Bülow (1830-1894) was a conductor and pianist of worldwide reputation and founder of many stylistic interpretations of classic and romantic symphonies. The close friendship with Richard Wagner, but not the enthusiastic admiration of his dramatic musical opus, ended abruptly when Hans von Bülow became aware of the betrayal of his wife Cosima and Richard Wagner. Hans von Bülow reported symptoms and signs of neurological disease in many letters that were kept and edited by his second wife Marie. For decades he suffered from chronic neuralgiforme headaches, which were caused by a tumor of the cervical radicular nerves. At the age of 45 years, he suddenly developed a motorsensory deficit in the right arm and hand and a contralateral facial deficit, suggestive of brainstem infarction. He recovered and celebrated even greater successes as a musician, although phases of major depression also interfered with his professional life. In the last, phase of his life, he experienced the consequences of generalized atherosclerosis and cerebral microangiopathy. It was a second cerebrovascular accident of the brainstem that caused his death, only 10 months after his last concert performance. Although his death occurred in Egypt, an autopsy was performed by Professor Ludwig Edinger and the results will be presented. PMID:17495513

  6. Neurological disorders and travel.

    Awada, Adnan; Kojan, Suleiman

    2003-02-01

    Travel is associated with a number of neurological disorders that can be divided into two categories: (1) Neurological infections including encephalitides, neurotuberculosis, neurobrucellosis, cysticercosis and trichinosis. Some of these disorders can be prevented by vaccinations, such as Japanese B encephalitis and rabies, some by the use of insect repellents and some by avoiding raw milk products and undercooked meat. (2) Non-infective neurological disorders, such as acute mountain sickness and high altitude cerebral oedema, problems occurring during air travel such as syncope, seizures, strokes, nerve compression, barotrauma and vertigo, motion sickness and foodborne neurotoxic disorders such as ciguatera, shellfish poisoning and intoxication by cassava. This group of diseases and disorders could be prevented if the traveller knows about them, applies simple physiological rules, takes some specific medications and knows how to avoid intoxications in certain geographical areas. Meningococcal meningitis, malaria and jet lag syndrome are extensively discussed in other articles of this issue. The discussion in this paper will be limited to the other disorders. PMID:12615385

  7. 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

    2016-01-01

    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 routin...

  8. 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. PMID:26224517

  9. 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

    "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: ...

  10. Minimal residual disease in acute lymphoblastic leukemia.

    Campana, Dario

    2009-01-01

    In patients with acute lymphoblastic leukemia (ALL), monitoring of minimal residual disease (MRD) offers a way to precisely assess early treatment response and detect relapse. Established methods to study MRD are flow cytometric detection of abnormal immunophenotypes, polymerase chain reaction (PCR) amplification of antigen-receptor genes, and PCR amplification of fusion transcripts. The strong correlation between MRD levels and risk of relapse in childhood ALL is well demonstrated; studies in adult patients also support its prognostic value. Hence, results of MRD studies can be used to select treatment intensity and duration, and to estimate the optimal timing for hematopoietic stem cell transplantation. Practical issues in the implementation of MRD assays in clinical studies include determining the most informative time point to study MRD and the levels of MRD that will trigger changes in treatment intensity, as well as the relative cost and informative power of different methodologies. The identification of new markers of leukemia and the use of increasingly refined assays should further facilitate routine monitoring of MRD and help to clarify the cellular and biologic features of leukemic cells that resist chemotherapy in vivo. PMID:19100372