Full Text Available Dengue counts among the most commonly encountered arboviral diseases, representing the fastest spreading tropical illness in the world. It is prevalent in 128 countries, and each year >2.5 billion people are at risk of dengue virus infection worldwide. Neurological signs of dengue infection are increasingly reported. In this review, the main neurological complications of dengue virus infection, such as central nervous system (CNS, peripheral nervous system, and ophthalmic complications were discussed according to clinical features, treatment and possible pathogenesis. In addition, neurological complications in children were assessed due to their atypical clinical features. Finally, dengue infection and Japanese encephalitis were compared for pathogenesis and main clinical manifestations.
Hecht, Jacqueline T; Bodensteiner, John B; Butler, Ian J
Achondroplasia is the best described and most common form of the congenital short-limbed dwarfing conditions. Achondroplasia is apparent at birth and has a birth prevalence of 1 in 20000-30000 live-born infants. Achondroplasia is inherited as an autosomal dominant condition, although 80% of cases occur sporadically as new events in their families. Achondroplasia is caused, in virtually all of the cases, by a G380R mutation in fibroblast growth factor receptor 3 (FGFR3). Patients with achondroplasia should be evaluated by a multidisciplinary team of clinicians including geneticists, neurologists, and orthopedists, since there are numerous bony and neurological complications. The most severe complication results from craniocervical stenosis and medullary and upper spinal cord compression, which can have devastating and even lethal sequelae during early childhood. In subsequent decades, including adolescence, spinal cord and nerve compression are more prominent. The neurological complications of achondroplasia have been recognized in adults for more than a century and are attributed to bony defects, connective tissue structures, or both. Similar neurological complications are now appreciated in infants, young children, and teenagers with achondroplasia. Defective connective tissue elements in achondroplasia frequently lead to ligamentous laxity, which can aggravate the complications associated with bony stenosis. Bony abnormalities are known to cause neurological morbidity and lead to a shortened lifespan. Neurological complications associated with achondroplasia are reviewed, including recommendations for the evaluation and management of these clinical problems. © 2014 Elsevier B.V. All rights reserved.
Full Text Available Paraneoplastic neurologic disorders are extremely rare in cancer patients and are most commonly associated with certain tumors, such as ovarian cancer, small cell lung cancer, and breast cancer. We report here a paraneoplastic neurological syndrome in a 53-year-old man with colonic adenocarcinoma with a solitary liver metastasis. His paraneoplastic syndrome was successfully treated by methylprednisolone and primary oncologic therapies including neoadjuvant chemotherapy and definitive surgery. This is also the first documented case of simultaneous manifestation of a sensory neuropathy and limbic encephalitis with colon cancer.
pal P K
Full Text Available A part from the well-established syndrome of motor paralysis, hypokalemia may present with atypical neurological manifestations, which are not well documented in literature. Methods: We treated 30 patients of hypokalemia whose neurological manifestations improved after corrections of hypokalemia. A retrospective chart review of the clinical profile was done with emphasis on the evolution of symptoms and occurrence of unusual manifestations. Results: Twenty-eight patients had subacute quadriparesis with duration of symptoms varying from 10hrs to 7 days and two had slowly progressive quadriparesis. Fifty percent of patients had more than one attack of paralysis. Early asymmetric weakness (11, stiffness and abnormal posture of hands (7, predominant bibrachial weakness (4, distal paresthesias (4, hemiparesthesia (1, hyperreflexia(4, early severe weakness of neck muscles (3, chorea (1, trismus (1,and, retention of urine (1 were the unusual features observed. The means level of serum potassium on admission was 2.1+0.6mEq/L.and the serum creatine kinase was elevated in 14 out of 17 patients. All patients except two had complete recovery.
Tracy, Jennifer A; Dyck, P James B
Porphyrias are rare disorders resulting from a defect in the heme biosynthetic pathway. They can produce significant disease of both the peripheral and central nervous systems, in addition to other organ systems, with acute intermittent porphyria, hereditary coproporphyria, and variegate porphyria as the subtypes associated with neurologic manifestations. The presence of a motor-predominant peripheral neuropathy (axonal predominant), accompanied by gastrointestinal distress and neuropsychiatric manifestations, should be a strong clue to the diagnosis of porphyria. Clinical confirmation can be made through evaluation of urine porphyrins during an exacerbation of disease. While hematin is helpful for acute treatment, long-term effective management requires avoidance of overstimulation of the cytochrome P450 pathway, as well as other risk factor control. © 2014 Elsevier B.V. All rights reserved.
Bertalan, Abigail; Kent, Marc; Glass, Eric
Hypothyroidism is a common endocrine disease in dogs. A variety of clinicopathologic abnormalities may be present; however, neurologic deficits are rare. In some instances, neurologic deficits may be the sole manifestation of hypothyroidism. Consequent ly, the diagnosis and management of the neurologic disorders associated with hypothyroidism can be challenging. This article describes several neurologic manifestations of primary hypothyroidism in dogs; discusses the pathophysiology of hypothyroidism-induced neurologic disorders affecting the peripheral and central nervous systems; and reviews the evidence for the neurologic effects of hypothyroidism.
Full Text Available Hypernatremia is a relatively common entity and is more prevalent among the elderly and critically ill. A number of medical conditions are commonly associated with hypernatremia, and these differ substantially among children and adults. Severe hypernatremia is usually associated with central nervous system manifestations and carries a high mortality rate. We report a case of a female patient who presented to the emergency department of the King Khalid University Hospital, Riyadh, Saudi Arabia with severe hypernatremia and without any associated co-morbid conditions or neurological manifestations. We did not find any etiological background despite extensive eva-luation other than under hydration due to decreased fluid intake, which was secondary to beha-vioural causes.
Møller, Anette Torvin; Jensen, Troels Staehelin
. 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...
Samudio-Domínguez, G; Dávila, G; Martínez-Aguilar, G; Santos-Preciado, J I
Since the first cases of childhood AIDS were reported, the neurological involvement has been more frequently recognized. Several motor, intellectual and conductual changes as well as unexplained abnormalities have been described due to CNS infections. Findings have shown HIV to affect the CNS although it is unknown as to when the viral invasion actually occurs. This report describes the neurological manifestations found in pediatric patients with HIV infection at the Hospital Infantil de Mexico and their correlations with CT scans, EEGs, auditory evoked potentials, I.Q.s and postmortem findings. The medical records of 60 symptomatic HIV infected children, stages P0 to P2, are reviewed. Neurological abnormalities were found in 51 patients, 20 of which (39.2%) were due to perinatal infection with symptoms starting, on the average at 11 months 7 days (from the initial contact) taking into consideration in utero exposure. Nine cases (17.6%) were patients infected through transfusions with symptoms appearing on the average at 24 months 8 days; 2 cases (3.9%) were of unknown origin. The CT scans, EEGs and psychometric evaluations of the HIV infected patients correlated well with the clinical findings.
adults in Abidjan, Cote d'Ivoire and in Kinshasa and among inpatients in Ugandan hospitals. Ninety per cent of deaths ... various parts of the continent. Neurological manifestations. The spectrum of neurological diseases reported in ... Primary effects of HIV. HEADACHE. Case report. A Malawian 46-year-old male senior ...
Full Text Available The authors report on six cases of gluten-sensitivity, also defined non-celiac gluten sensitivity, characterized by abdominal features (diarrhea, bloating, pain, genetic positivity for predisposition to celiac disease (DQB1* 02 in all cases; DQA1*05 in three; DQA1*02 in two, DQB1*03 in two, negative anti-t-Transglutaminase antibodies, normal mucosa on biopsy in four cases, type 1 of Marsh in one case. The subjects presented frequent central nervous system (CNS symptoms: headache in three patients, somnolence in one, electroencephalogram aspecific alterations in three (in two of them with previous seizures, leptomeningeal cyst in one, intracranial calcification in one, cerebral gliosis in two. After a gluten-free diet, all intestinal and clinical CNS features remitted, but re-appeared after gluten reintroduction. On the basis of the neurological signs, the authors stress the relevance of immune innate system in the pathogenesis of these cases with possible subsequent evolution on immune adaptive system involvement.
Thibert, Ronald L; Larson, Anna M; Hsieh, David T; Raby, Annabel R; Thiele, Elizabeth A
Angelman syndrome is a neurogenetic disorder characterized by the loss or reduction of the ubiquitin-protein ligase E3A enzyme. Angelman syndrome results from a deletion or mutation of the maternally inherited 15q11.2-13.1 region, paternal uniparental disomy of chromosome 15, or an imprinting error. Epilepsy is common and may present with multiple seizure types, including nonconvulsive status epilepticus. Seizures are often intractable and typically require broad-spectrum antiepileptic medications. Dietary therapy has also proved successful in Angelman syndrome. Electroencephalographic patterns include notched δ and rhythmic θ activity and epileptiform discharges. Sleep disorders are also common, often characterized by abnormal sleep-wake cycles. Movement disorders are nearly universal in Angelman syndrome, most frequently presenting with ataxia and tremor. Neurocognitive impairment is always present to varying degrees, and expressive speech is typically severely affected. Individuals with Angelman syndrome often manifest psychiatric comorbidities including hyperactivity, anxiety, and challenging behaviors such as aggression and self-injury. We focus on a comprehensive whole-child approach to the diagnosis and long-term clinical care of individuals with Angelman syndrome. Copyright © 2013 Elsevier Inc. All rights reserved.
Full Text Available IntroductionThe purpose of this retrospective study was to report neurological manifestations noted in patients who were monitored for inflammatory bowel disease, in order to document the pathophysiological, clinical, progressive, and therapeutic characteristics of this entity.Material and methodsWe conducted a retrospective study on patients monitored -in the gastroenterology service in Ibn Sina Hospital in Rabat, Morocco- for inflammatory bowel disease from 1992 till 2013 and who developed neurological manifestations during its course. Patients with iatrogenic complications were excluded, as well as patients with cerebrovascular risk factors.ResultsThere were 6 patients, 4 of whom have developed peripheral manifestations. Electromyography enabled the diagnosis to be made and the outcome was favorable with disappearance of clinical manifestations and normalization of the electromyography.The other 2 patients, monitored for Crohn’s disease, developed ischemic stroke. Cerebral computed tomography angiography provided positive and topographic diagnosis. Two patients were admitted to specialized facilities.ConclusionNeurological manifestations in inflammatory bowel disease are rarely reported. Peripheral neuropathies and stroke remain the most common manifestations. The mechanisms of these manifestations are not clearly defined yet. Currently, we hypothesize the interaction of immune mediators.
Doughty, Katharine; Rood, Corey; Patel, Anup; Thackeray, Jonathan D; Brink, Farah W
Medical child abuse occurs when a child receives unnecessary and harmful, or potentially harmful, medical care at the instigation of a caretaker through exaggeration, falsification, or induction of symptoms of illness in a child. Neurological manifestations are common with this type of maltreatment. We sought to review common reported neurological manifestations that may alert the clinician to consider medical child abuse. In addition, the possible sequelae of this form of child maltreatment is discussed, as well as practice recommendations for establishing the diagnosis and stopping the abuse once it is identified. A review of the medical literature was conducted regarding the reported neurological presentations of this entity. Neurological manifestations of medical child abuse include false reports of apparent life-threatening events and seizures and reports of induction of symptoms from poisoning. Failure to correlate objective findings with subjective complaints may lead to unnecessary and potentially harmful testing or treatment. This form of child maltreatment puts a child at significant risk of long-term morbidity and mortality. A wide variety of neurological manifestations have been reported in cases of medical child abuse. It is important for the practicing neurologist to include medical child abuse on the differential diagnosis. Copyright © 2016 Elsevier Inc. All rights reserved.
Planas-Ballvé, Anna; Grau-López, Laia; Morillas, Rosa María; Planas, Ramón
This article reviews the different acute and chronic neurological manifestations of excessive alcohol consumption that affect the central or peripheral nervous system. Several mechanisms can be implicated depending on the disorder, ranging from nutritional factors, alcohol-related toxicity, metabolic changes and immune-mediated mechanisms. Recognition and early treatment of these manifestations is essential given their association with high morbidity and significantly increased mortality. Copyright © 2017 Elsevier España, S.L.U., AEEH y AEG. All rights reserved.
Martínez-Guzmán, Edgar; Gámez-González, Luisa Berenise; Rivas-Larrauri, Francisco; Sorcia-Ramírez, Giovanni; Yamazaki-Nakashimada, Marco
Kawasaki disease (KD) is a type of systemic vasculitis of unknown etiology. Atypical Kawasaki disease is defined as that where there are signs and symptoms not corresponding to the classical criteria for this nosological entity. Children with atypical Kawasaki disease may present with acute abdominal symptoms, meningeal irritation, pneumonia or renal failure. We describe 4 children with ages ranging from 2 to 12 years who had atypical Kawasaki disease, with neurological and gastrointestinal symptoms as part of the systemic presentation of the disease. Treatment consisted of immunoglobulin and corticosteroids with good evolution. KD is a systemic vasculitis that can involve many territories. Atypical manifestations can mislead the clinician and delay diagnosis. Pediatricians and sub-specialists should be aware of these neurological manifestations in order to provide adequate and opportune treatment.
Full Text Available Francisco Javier Carod-Artal1,21Neurology Department, Raigmore hospital, Inverness, UK; 2Universitat Internacional de Catalunya (UIC, Barcelona, Spain Abstract: Dengue is the most common mosquito-borne viral infection worldwide. There is increased evidence for dengue virus neurotropism, and neurological manifestations could make part of the clinical picture of dengue virus infection in at least 0.5%–7.4% of symptomatic cases. Neurological complications have been classified into dengue virus encephalopathy, dengue virus encephalitis, immune-mediated syndromes (acute disseminated encephalomyelitis, myelitis, Guillain–Barré syndrome, neuritis brachialis, acute cerebellitis, and others, neuromuscular complications (hypokalemic paralysis, transient benign muscle dysfunction and myositis, and dengue-associated stroke. Common neuro-ophthalmic complications are maculopathy and retinal vasculopathy. Pathogenic mechanisms include systemic complications and metabolic disturbances resulting in encephalopathy, direct effect of the virus provoking encephalitis, and postinfectious immune mechanisms causing immune-mediated syndromes. Dengue viruses should be considered as a cause of neurological disorders in endemic regions. Standardized case definitions for specific neurological complications are still needed. Keywords: encephalitis, encephalopathy, dengue fever, neurological complications
Full Text Available Celiac disease (CD is an immune-mediated disease characterized by permanent gastrointestinal tract sensitivity to gluten in genetically predisposed individuals. It has varied clinical manifestations, ranging from gastrointestinal to extraintestinal, including neurological, skin, reproductive and psychiatric symptoms, which makes its diagnosis difficult and challenging. Known neurological manifestations of CD include epilepsy with or without occipital calcification, attention deficit hyperactivity disorder and ataxia, headache, neuropathies and behavior disorders. We present the case of a 14-year-old female with headaches and blurred vision for 1 year; she was noted to have papilledema on ophthalmic examination with increased cerebrospinal fluid opening pressure on lumber puncture and was diagnosed as a case of pseudotumor cerebri (PTC. Meanwhile her workup for chronic constipation revealed elevated tissue transglutaminase IgA and antiendomysial IgA antibodies. Upper gastrointestinal endoscopy with duodenal biopsy confirmed the diagnosis of CD. The patient was started on a gluten-free diet, leading to resolution of not only gastrointestinal symptoms but also to almost complete resolution of symptoms of PTC. This report describes the correlation of CD and PTC as its neurological manifestation.
The brain operates in an extraordinarily intricate environment which demands precise regulation of electrolytes. Tight control over their concentrations and gradients across cellular compartments is essential and when these relationships are disturbed neurologic manifestations may develop. Perturbations of sodium are the electrolyte disturbances that most often lead to neurologic manifestations. Alterations in extracellular fluid sodium concentrations produce water shifts that lead to brain swelling or shrinkage. If marked or rapid they can result in profound changes in brain function which are proportional to the degree of cerebral edema or contraction. Adaptive mechanisms quickly respond to changes in cell size by either increasing or decreasing intracellular osmoles in order to restore size to normal. Unless cerebral edema has been severe or prolonged, correction of sodium disturbances usually restores function to normal. If the rate of correction is too rapid or overcorrection occurs, however, new neurologic manifestations may appear as a result of osmotic demyelination syndrome. Disturbances of magnesium, phosphate and calcium all may contribute to alterations in sensorium. Hypomagnesemia and hypocalcemia can lead to weakness, muscle spasms, and tetany; the weakness from hypophosphatemia and hypomagnesemia can impair respiratory function. Seizures can be seen in cases with very low concentrations of sodium, magnesium, calcium, and phosphate. © 2017 Elsevier B.V. All rights reserved.
Talys J. Pinheiro
Full Text Available ABSTRACT The epidemics of Chikungunya virus (CHIKV and Zika virus (ZIKV infections have been considered the most important epidemiological occurrences in the Americas. The clinical picture of CHIKV infection is characterized by high fever, exanthema, myalgia, headaches, and arthralgia. Besides the typical clinical picture of CHIKV, atypical manifestations of neurological complications have been reported: meningo-encephalitis, meningoencephalo-myeloradiculitis, myeloradiculitis, myelitis, myeloneuropathy, Guillain-Barré syndrome and others. The diagnosis is based on clinical, epidemiological, and laboratory criteria. The most common symptoms of ZIKV infection are skin rash (mostly maculopapular, fever, arthralgia, myalgia, headache, and conjunctivitis. Some epidemics that have recently occurred in French Polynesia and Brazil, reported the most severe conditions, with involvement of the nervous system (Guillain-Barré syndrome, transverse myelitis, microcephaly and meningitis. The treatment for ZIKV and CHIKV infections are symptomatic and the management for neurological complications depends on the type of affliction. Intravenous immunoglobulin, plasmapheresis, and corticosteroid pulse therapy are options.
I. V. Egorov
Full Text Available Despite being thoroughly studied, senile aortic stenosis (AS remains a disease that is frequently underestimated by Russian clinicians. Meanwhile, its manifestations can not only deteriorate quality of life in patients, but can also be poor prognostic signs. The most common sequels of this disease include heart failure and severe arrhythmias. However, there may be also rare, but no less dangerous complications: enteric bleeding associated with common dysembriogenetic backgrounds, infarctions of various organs, the basis for which is spontaneous calcium embolism, and consciousness loss episodes. The latter are manifestations of cardiocerebral syndrome. Apart from syncope, embolic stroke may develop within this syndrome. There is evidence that after syncope occurs, life expectancy averages 3 years. Global practice is elaborating approaches to the intracardiac calcification prevention based on the rapid development of new pathogenetic ideas on this disease. In particular, it is clear that valvular calcification is extraskeletal leaflet ossification rather than commonplace impregnation with calcium salts, i.e. the case in point is the reverse of osteoporosis. This is the basis for a new concept of drug prevention of both calcification and the latter-induced heart disease. But the view of senile AS remains more than conservative in Russia. The paper describes a clinical case of a rare complication as cerebral calcium embolism and discusses the nature of neurological symptoms of the disease, such as vertigo and syncope.
Lee, Kyung Yeon; Lee, Myoung Sook; Kim, Dong Bin
Enterovirus 71 frequently involves the central nervous system and may present with a variety of neurologic manifestations. Here, we aimed to describe the clinical features, magnetic resonance imaging (MRI) findings, and cerebrospinal fluid (CSF) profiles of patients presenting with neurologic complications of enterovirus 71 infection. We retrospectively reviewed the records of 31 pediatric patients hospitalized with acute neurologic manifestations accompanied by confirmed enterovirus 71 infection at Ulsan University Hospital between 2010 and 2014. The patients' mean age was 2.9 ± 5.5 years (range, 18 days to 12 years), and 80.6% of patients were less than 4 years old. Based on their clinical features, the patients were classified into 4 clinical groups: brainstem encephalitis (n = 21), meningitis (n = 7), encephalitis (n = 2), and acute flaccid paralysis (n = 1). The common neurologic symptoms included myoclonus (58.1%), lethargy (54.8%), irritability (54.8%), vomiting (48.4%), ataxia (38.7%), and tremor (35.5%). Twenty-five patients underwent an MRI scan; of these, 14 (56.0%) revealed the characteristic increased T2 signal intensity in the posterior region of the brainstem and bilateral cerebellar dentate nuclei. Twenty-six of 30 patients (86.7%) showed CSF pleocytosis. Thirty patients (96.8%) recovered completely without any neurologic deficits; one patient (3.2%) died due to pulmonary hemorrhage and shock. In the present study, brainstem encephalitis was the most common neurologic manifestation of enterovirus 71 infection. The characteristic clinical symptoms such as myoclonus, ataxia, and tremor in conjunction with CSF pleocytosis and brainstem lesions on MR images are pathognomonic for diagnosis of neurologic involvement by enterovirus 71 infection.
Seneviratne U; Dissanayake S
BACKGROUND AND AIMS: Snake bite is an important cause of mortality and morbidity in certain parts of Sri Lanka. This study was designed to determine the offending snakes, neurological manifestations, disease course, and outcome in neurotoxic envenomation. METHODS AND MATERIAL: Fifty six consecutive patients admitted with neurological manifestations following snake bite were studied prospectively. Data were obtained regarding the offending snakes, neurological symptoms, time taken for onset of...
Henderson, F.C.; Austin, C.; Benzel, E.; Bolognese, P.; Ellenbogen, R.; Francomano, C.A.; Ireton, C.; Klinge, P.; Koby, M.; Long, D.; Patel, S.; Singman, E.L.; Voermans, N.C.
The Ehlers-Danlos syndromes (EDS) are a heterogeneous group of heritable connective tissue disorders characterized by joint hypermobility, skin extensibility, and tissue fragility. This communication briefly reports upon the neurological manifestations that arise including the weakness of the
Sel, Çiğdem Genç; Aksoy, Erhan; Aksoy, Ayşe; Yüksel, Deniz; Özbay, Ferda
Various typical and atypical neurological manifestations can be seen as the initial symptoms of celiac disease (CD). We suggest that gluten toxicity is the most suspicious triggering risk factor for probable pathophysiological pathways of neurological involvement in atypical CD. The medical charts of 117 patients diagnosed with atypical CD were retrieved from a tertiary center in Ankara, Turkey. Eight patients reported as having neurologic manifestations as initiating symptoms were evaluated in detail. The initial neurological manifestations of CD in our study included atypical absence, which was reported first in this study, generalized tonic-clonic seizures, complex partial seizures, severe axial hypotonia and down phenotype, multifocal leukoencephalopathy, mild optic neuritis, attention deficit hyperactivity disorder, and short duration headaches. Seizures mostly emphasizing atypical absence could be the initial presentation manifestation of CD, first described in this literature. Gluten toxicity could be one of the most powerful triggering factors for developing epilepsy in CD. Learning disorders such as attention deficit hyperactivity disorder, short duration headaches, mild optic neuritis, encephalopathy, and DS could also be the initial neurological manifestations of atypical CD. A gluten-restricted diet may improve neurological complaints, epileptic discharges, and neuropsychiatric symptoms. All we found may be a small part of the full range of neurological disorders of unknown origin related to CD. Clinical suspicion should be the rule for accurate diagnosis of the disease.
Golstein, M; Meyer, O; Bourgeois, P; Palazzo, E; Nicaise, P; Labarre, C; Kahn, M F
Antiphospholipid antibodies (APL) are associated with venous and arterial thrombosis in SLE patients. Various thrombotic and non-thrombotic neurological manifestations have been reported in SLE but whether or not they are related to the presence of APL antibodies remains uncertain. To assess the possible association between neurological involvement in SLE and APL antibodies, IgG anticardiolipin antibodies (IgG ACL) were looked for using an ELISA technique in 92 consecutive SLE patients seen over a one-year period. Other APL determinations included VDRL and lupus anticoagulant (LAC) testing using APTT and the diluted thromboplastin time. Twenty-four SLE patients presented with neurological manifestations (40 episodes): 15/24 (62.5%) were found positive for APL antibodies (11 VDRL, 8 LAC, 7 ACL antibodies) versus 22/68 patients (32%) without neurological symptoms (p < 0.01). APL antibodies antedated neurological symptoms in 13/16 cases. Neurological manifestations were subsequently divided into 3 groups: thrombotic (n = 14), psychosis and convulsions (n = 15), miscellaneous (n = 10). No correlation was found between APL antibodies and any of the 3 subgroups. Among patients with neurological SLE, APL antibodies were present in two with valvular heart disease, as well as in seven with a history of either deep vein thrombosis, livedo reticularis or miscarriage. Among 7 patients with thrombocytopenia and neurological symptoms, 6 had APL antibodies. These data suggest that APL syndrome is associated with neuro-ophthalmological manifestations of SLE regardless of whether or not the mechanism of neurological involvement is thrombotic. SLE patients with APL antibodies may be at risk for future neurological manifestations. However, it is still questionable that APL positivity has definite therapeutic consequences.
Hellmuth, Joanna; Fletcher, James L K; Valcour, Victor; Kroon, Eugène; Ananworanich, Jintanat; Intasan, Jintana; Lerdlum, Sukalaya; Narvid, Jared; Pothisri, Mantana; Allen, Isabel; Krebs, Shelly J; Slike, Bonnie; Prueksakaew, Peeriya; Jagodzinski, Linda L; Puttamaswin, Suwanna; Phanuphak, Nittaya; Spudich, Serena
To determine the incidence, timing, and severity of neurologic findings in acute HIV infection (pre-antibody seroconversion), as well as persistence with combination antiretroviral therapy (cART). Participants identified with acute HIV were enrolled, underwent structured neurologic evaluations, immediately initiated cART, and were followed with neurologic evaluations at 4 and 12 weeks. Concurrent brain MRIs and both viral and inflammatory markers in plasma and CSF were obtained. Median estimated HIV infection duration was 19 days (range 3-56) at study entry for the 139 participants evaluated. Seventy-three participants (53%) experienced one or more neurologic findings in the 12 weeks after diagnosis, with one developing a fulminant neurologic manifestation (Guillain-Barré syndrome). A total of 245 neurologic findings were noted, reflecting cognitive symptoms (33%), motor findings (34%), and neuropathy (11%). Nearly half of the neurologic findings (n = 121, 49%) occurred at diagnosis, prior to cART initiation, and most of these (n = 110, 90%) remitted concurrent with 1 month on treatment. Only 9% of neurologic findings (n = 22) persisted at 24 weeks on cART. Nearly all neurologic findings (n = 236, 96%) were categorized as mild in severity. No structural neuroimaging abnormalities were observed. Participants with neurologic findings had a higher mean plasma log10 HIV RNA at diagnosis compared to those without neurologic findings (5.9 vs 5.4; p = 0.006). Acute HIV infection is commonly associated with mild neurologic findings that largely remit while on treatment, and may be mediated by direct viral factors. Severe neurologic manifestations are infrequent in treated acute HIV. © 2016 American Academy of Neurology.
Pérez Andreu, J; Parrilla, G; Arribas, J M; García-Villalba, B; Lucas, J J; Garcia Navarro, M; Marín, F; Gutierrez, F; Moreno, A
Cardiac myxoma is an important but uncommon cause of stroke in younger patients. Few published case series analyse the frequency and clinical presentation of neurological complications in patients with myxoma. To list all neurological complications from cardiac myxoma recorded in our hospital in the past 28 years. We retrospectively reviewed the neurological manifestations of cardiac myxoma in patients treated in our hospital between December 1983 and March 2012. Of the 36 patients with cardiac myxoma, 8 (22%) presented neurological manifestations. Half were women and mean age of patients was 52.4 ± 11.6 years. Sudden-onset hemiparesis was the most frequent neurological symptom (63%). Established ischaemic stroke was the most common clinical manifestation (75%), followed by transient ischemic attack. The most commonly affected territory corresponded to the middle cerebral artery. Myxoma was diagnosed by echocardiography in all cases. Mean myxoma size was 4.1cm and most of the tumours (63%) had a polypoid surface. All tumours were successfully removed by surgery. There were no in-hospital deaths. Cardiac myxomas frequently present with neurological symptoms, especially ischaemic events (established stroke or transient ischaemic attack), in younger patients with no cardiovascular risk factors. The anterior circulation is more frequently affected, especially the middle cerebral artery. Echocardiography can facilitate prompt diagnosis and early treatment of the lesion. Copyright © 2012 Sociedad Española de Neurología. Published by Elsevier Espana. All rights reserved.
Full Text Available Objective: To evaluate the neurological complications among Iranian HIV-positive patients. Methods: This cross-sectional study was conducted among 428 patients diagnosed with HIV infection between 2006 and 2009 at Imam Khomeini hospital, Tehran, Iran. Demographic and clinical variables as well as laboratory tests were extracted and analyzed. Also, another 100 patients refereed to Voluntary Counseling and Testing center of the hospital were visited and evaluated for neurological complications. Results: Among the patients, neurologic manifestations were observed in 34 (7.94% patients. Twenty three percent of the patients received antiretroviral therapy. Identified causes included brain toxoplasmosis (14.7%, progressive multi-focal leuko encephalopathy (5.9%, HIV encephalopathy (5.9%, TB meningitis (5% and unknown etiologies (11.8%. Also, among 100 patients who were admitted and visited at the Voluntary Counseling and Testing center, no one was diagnosed for any neurological manifestations. Conclusions: According to our results, toxoplasmosis is the most frequent cause of neurological conditions among Iranian HIV infected patients and should be considered in any HIV/AIDS patient with neurological manifestations.
Full Text Available BACKGROUND AND AIMS: Snake bite is an important cause of mortality and morbidity in certain parts of Sri Lanka. This study was designed to determine the offending snakes, neurological manifestations, disease course, and outcome in neurotoxic envenomation. METHODS AND MATERIAL: Fifty six consecutive patients admitted with neurological manifestations following snake bite were studied prospectively. Data were obtained regarding the offending snakes, neurological symptoms, time taken for onset of symptoms, neurological signs, and time taken for recovery. RESULTS: The offending snake was Russell′s viper in 27(48.2%, common and Sri Lankan krait in 19(33.9%, cobra in 3(5.4%, and unidentified in 7(12.5%. Ptosis was the commonest neurological manifestation seen in 48(85.7% followed by ophthalmoplegia (75%, limb weakness (26.8%, respiratory failure (17.9%, palatal weakness (10.7%, neck muscle weakness (7.1%, and delayed sensory neuropathy (1.8%. Neurological symptoms were experienced usually within 6 hours after the bite. Following administration of antivenom, the signs of recovery became evident within a few hours to several days. The duration for complete recovery ranged from four hours to two weeks. CONCLUSIONS: Complete recovery of neuromuscular weakness was observed in all patients except for one who died with intracerebral haemorrhage shortly after admission.
Maynor, M L
This case report illustrates neurological deficits as an unusual presentation of acute myelogenous leukemia. Neurological deficits are rare early in this disease. Our patient presented with anorexia, malaise, headache, and multiple cranial nerve palsies. A high WBC count and abnormal peripheral smear led to the diagnosis of leukemia. This report demonstrates that, although rare, CNS symptoms may be the initial manifestation of leukemia. Blood dyscrasias should not be overlooked in patients with the acute onset of neurological symptoms. A complete blood count and differential should be obtained under those circumstances.
Punchai, Suriya; Hanipah, Zubaidah Nor; Meister, Katherine M; Schauer, Philip R; Brethauer, Stacy A; Aminian, Ali
The aim of this study was to assess the incidence, clinical presentation, and outcomes of neurologic disorders secondary to vitamin B deficiencies following bariatric surgery. Patients at a single academic institution who underwent bariatric surgery and developed neurologic complications secondary to low levels of vitamins B1, B2, B6, and B12 between the years 2004 and 2015 were studied. In total, 47 (0.7%) bariatric surgical patients (Roux-en-Y gastric bypass n = 36, sleeve gastrectomy n = 9, and duodenal switch n = 2) developed neurologic manifestations secondary to vitamin B deficiencies. Eleven (23%) patients developed postoperative anatomical complications contributed to poor oral intake. Median duration to onset of neurologic manifestation following surgery was 12 months (IQR, 5-32). Vitamin deficiencies reported in the cohort included B1 (n = 30), B2 (n = 1), B6 (n = 12), and B12 (n = 12) deficiency. The most common manifestations were paresthesia (n = 31), muscle weakness (n = 15), abnormal gait (n = 11), and polyneuropathy (n = 7). Four patients were diagnosed with Wernicke-Korsakoff syndrome (WKS) which was developed after gastric bypass (n = 3) and sleeve gastrectomy (n = 1). Seven patients required readmission for management of severe vitamin B deficiencies. Overall, resolution of neurologic symptoms with nutritional interventions and pharmacotherapy was noted in 40 patients (85%). The WKS was not reversible, and all four patients had residual mild ataxia and nystagmus at the last follow-up time. Nutritional neurologic disorders secondary to vitamin B deficiency are relatively uncommon after bariatric surgery. While neurologic disorders are reversible in most patients (85%) with vitamin replacements, persistent residual neurologic symptoms are common in patients with WKS.
Neurological manifestations in speech after snake bite: A rare case. D Vir, D Gupta, M Modi, N Panda. Abstract. No Abstract. Full Text: EMAIL FREE FULL TEXT EMAIL FREE FULL TEXT · DOWNLOAD FULL TEXT DOWNLOAD FULL TEXT · http://dx.doi.org/10.4314/pamj.v4i1.53597 · AJOL African Journals Online. HOW TO ...
The objective: is to study the prevalence of neurological manifestations among adults Sudanese patients with lung cancer seen in the Radiation and Isotope Centre in Khartoum. Methods: This is a prospective cross sectional hospital based study, conducted at Radiation and Isotope Centre in Khartoum. Sample of 52 adults ...
Objective: To study the pattern of neurological manifestations of tuberculosis among adult Sudanese tuberculous patients seen at El-Shaab Teaching Hospital (Sudan). Methods: This study was performed on 179 Sudanese patients with tuberculosis admitted at El-Shaab Teaching Hospital during the period from May 2005 ...
Loh, W F; Hussain, I M; Soffiah, A; Lim, Y N
In a cross-sectional study of 21 children with Systemic Lupus Erythematosus, 15 (71%) were found to have neuropsychiatric manifestations. The most common finding was generalised seizures (42.8%) followed by encephalopathy (19%) and hallucinations (19%). One child (4.76%) had hemichorea. In 3 children neurological manifestations were the first symptom of SLE. Computerised Axial Tomograms (CAT scans) showed cerebral atrophy in 7 of 12 scans available for review. Ten children had abnormal EEGs. Although none of the children had clinical evidence of a peripheral neuropathy, 8 had neurophysiological evidence of a neuropathy. One child died of intracranial haemorrhage. Six children had residual neuropsychiatric sequalae.
Warrenburg, B.P.C. van de; Snijders, A.H.; Munneke, M.; Bloem, B.R.
Gait disorders are seen frequently and often have a neurological cause. The clinical management of patients presenting with a gait disorder is often complicated due to the large number of diseases that can cause a gait disorder and to the difficulties in interpreting a specific gait disorder
Henderson, Fraser C; Austin, Claudiu; Benzel, Edward; Bolognese, Paolo; Ellenbogen, Richard; Francomano, Clair A; Ireton, Candace; Klinge, Petra; Koby, Myles; Long, Donlin; Patel, Sunil; Singman, Eric L; Voermans, Nicol C
The Ehlers-Danlos syndromes (EDS) are a heterogeneous group of heritable connective tissue disorders characterized by joint hypermobility, skin extensibility, and tissue fragility. This communication briefly reports upon the neurological manifestations that arise including the weakness of the ligaments of the craniocervical junction and spine, early disc degeneration, and the weakness of the epineurium and perineurium surrounding peripheral nerves. Entrapment, deformation, and biophysical deformative stresses exerted upon the nervous system may alter gene expression, neuronal function and phenotypic expression. This report also discusses increased prevalence of migraine, idiopathic intracranial hypertension, Tarlov cysts, tethered cord syndrome, and dystonia, where associations with EDS have been anecdotally reported, but where epidemiological evidence is not yet available. Chiari Malformation Type I (CMI) has been reported to be a comorbid condition to EDS, and may be complicated by craniocervical instability or basilar invagination. Motor delay, headache, and quadriparesis have been attributed to ligamentous laxity and instability at the atlanto-occipital and atlantoaxial joints, which may complicate all forms of EDS. Discopathy and early degenerative spondylotic disease manifest by spinal segmental instability and kyphosis, rendering EDS patients prone to mechanical pain, and myelopathy. Musculoskeletal pain starts early, is chronic and debilitating, and the neuromuscular disease of EDS manifests symptomatically with weakness, myalgia, easy fatigability, limited walking, reduction of vibration sense, and mild impairment of mobility and daily activities. Consensus criteria and clinical practice guidelines, based upon stronger epidemiological and pathophysiological evidence, are needed to refine diagnosis and treatment of the various neurological and spinal manifestations of EDS. © 2017 Wiley Periodicals, Inc. © 2017 Wiley Periodicals, Inc.
Dandashi, Jad A; Nizamutdinov, Damir; Dayawansa, Samantha; Fonkem, Ekokobe; Huang, Jason H
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.
Background: The human immunodeficiency virus (HIV) is primarily neurotrophic and lymphotrophic. Diverse neurologic sequealae have been documented with variations based on disease severity, but geographic variation may determine the distribution of these neurological complications. Objective: This study was ...
Background The HIV is neurotropic and clinicians need to be aware of its myriad neurologic manifestations, as these may be the only clinical presentation. Objective To evaluate the clinical spectrum and outcome of the neurologic manifestations in patients with HIV / AIDS over a ten year period. Design case - note based ...
Sahin, Cansu B; Chaudhary, Neeraj; Trobe, Jonathan D
A 70-year-old woman suffered an anterior dorsal hypothalamic hemorrhage that caused an ipsilateral Horner syndrome (HS) as the only focal neurologic manifestation. This is only the second reported case of hypothalamic hemorrhage producing HS. Because HS was the sole focal neurologic manifestation, its confirmation with topical apraclonidine drops was a valuable clue toward prompt localization of the patient's confusional state.
... head ache, neck pain and stiffness were the frequent neurological symptoms. Papilledema was the commonest neurological finding. Saggital sinus thrombosis was found to be the major radiological finding and great majority of patients showed remarkable improvement with treatment. Conclusion: Dural sinus thrombosis ...
Ahmed Tanjimul Islam
Full Text Available Background: Leprosy is a chronic granulomatous infectious disease having major burden on humans over thousands of years. If untreated, it results in permanent damage to various systems and organs. So we designed this study to evaluate the neurological complications in early stage in adult leprosy patients. Objective: The aim of this study was to find out the pattern of neurological manifestations among adult leprosy patients. Materials and Methods: This cross-sectional hospital-based study on 85 adult tribal leprosy patients was conducted in a district level health care facility from January to December 2014 using simple, direct, standardized questionnaire including history and neurological examinations. Results: The commonest age group affected was 18–30 years (62.4%. Male group was predominant (68.2%. Majority cases (66% had multibacillary leprosy. At first visit 72.7% cases with neurological findings could not be diagnosed correctly by primary health care personnel. More than six months were required for correct diagnosis in 61.2% cases. Numbness was the commonest (74.5% neurological symptom. In upper limb, motor findings were predominant with wasting in 50.9% cases. In lower limb, sensory findings were predominant with stock pattern sensory impairment being the commonest (56.4%. Ulnar nerve was the commonest peripheral nerve to enlarge with tenderness. Facial nerve was the commonest cranial nerve involved. All cases with multiple cranial nerves involvement were of multibacillary type. Due to physical disability 92.7% cases lost their jobs. Conclusion: In this study neurological involvement was found associated with severe disability.
Phenytoin toxicity masquerading as deterioration of neurological symptoms caused by interaction with chloramphenicol is a very rare but real risk. To the authors' knowledge only one such case occurring in humans has been reported in the English literature. No case of clinical phenytoin toxicity occurring at less than ...
Full Text Available Neurological melioidosis is a very rare and very few cases have been reported from India. Presentation is an extremely varied and as this disease is associated with high mortality, high index of suspicion is needed to diagnose and treat. In this context, we report a patient presenting as Guillain Barre syndrome evaluated as melioidosis.
Opportunistic infections were the leading cause of neurological disorders in our study population. Apart from Central nervous ... There were equal responses to Amphoterecin B Cholesterol Dispersion (ABCD) and conventional Amphoterecin B therapies, and no significant differences in their side effect profiles. Keywords: ...
Nokes, Brandon T; Dumitrascu, Oana M; Shamoun, Fadi E; OʼCarroll, Cumara B
Antiphospholipid syndrome (APS) encompasses a hypercoagulable state with a markedly increased risk for cerebrovascular complications. In addition to the classic stroke features of APS, however, there are numerous recently described "non-criteria" neurological conditions such as headaches, seizures, and cognitive impairment. We present a case of APS with uncommon neurological manifestations.
Full Text Available Magnesium is the second most common intracellular cation and serves as an important metabolic cofactor to over 300 enzymatic reactions throughout the human body. Among its various roles, magnesium modulates calcium entry and release from sarcoplasmic reticulum and regulates ATP pumps in myocytes and neurons, thereby regulating cardiac and neuronal excitability. Therefore, deficiency of this essential mineral may result in serious cardiovascular and neurologic derangements. In this case, we present the clinical course of a 76-year-old woman who presented with marked cardiac and neurological signs and symptoms which developed as a result of severe hypomagnesemia. The patient promptly responded to magnesium replacement once the diagnosis was established. We herein discuss the clinical presentation, pathophysiology, diagnosis, and management of severe hypomagnesemia and emphasize the implications of magnesium deficiency in the cardiovascular and central nervous systems. Furthermore, this case highlights the importance of having high vigilance for hypomagnesemia in the appropriate clinical setting.
Garosi, Laurent S; Lowrie, Mark L; Swinbourne, Natalie F
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. Copyright © 2012 Elsevier Inc. All rights reserved.
Miyauchi-Hashimoto, H; Akaeda, T; Maihara, T; Ikenaga, M; Horio, T
Although patients with mild symptoms of atypical Cockayne syndrome (CS) have been described, there has not been a report of a patient with CS whose only clinical manifestation was cutaneous photosensitivity. Cells from patients with CS show UV sensitivity, reduced recovery of RNA synthesis, but normal UV-induced unscheduled DNA synthesis. On the other hand, the patients with UV-sensitive syndrome have only cutaneous photosensitivity and skin freckles, whereas those cells respond to UV radiation in a similar fashion to the CS cells. We describe a patient with CS who showed only photosensitivity without typical clinical manifestations of CS, but his cells showed UV sensitivity, reduced recovery of RNA synthesis, and normal unscheduled DNA synthesis after UV radiation similar to CS cells. Furthermore, the patient was assigned to complementation group B of CS on the basis of the results of complementation analysis. The present report suggests that CS has a wider spectrum than that considered previously.
Nasser, J A; Confort, C I; Ferraz, A; Esperança, J C; Duarte, F
Prospective series showing the importance of computerized stereotactic brain biopsy in the management of AIDS patients neurologically symptomatic and confirmed by images. Patients undergone an algorithm step by step done by their own doctors and referred to us for stereotactic biopsy. Our protocol was opened in August 1995 and closed in December 1996. Twenty patients were biopsied. This protocol is similar to the Levy's one (Chicago IL, USA). We have got diagnosis in all cases. Lymphoma was predominant and followed by toxoplasmosis, progressive multifocal leukoencephalopathy and HIV encephalopathy. We included one patient with diploic giant cells lymphoma. Our mortality and morbidity was zero. By these results we conclude that stereotactic biopsy in AIDS patients is safe and effective.
CONCULSION: The study revealed high incidence of Potts paraplegia and peripheral neuropathy, this is most probably due to late presentation. Keywords: Pott\\'s paraplegia, peripheral neuropathy, tubercloma, tuberculous meningitis, quadriplegia, hemiplegia, proximal myopathy. Sudan Journal of Medical Sciences Vol.
Rama-Maceiras, P; Fàbregas Julià, N; Ingelmo Ingelmo, I; Hernández-Palazón, J
The high rates of morbidity and mortality after subarachnoid hemorrhage due to spontaneous rupture of an intracranial aneurysm are mainly the result of neurologic complications. Sixty years after cerebral vasospasm was first described, this problem remains unsolved in spite of its highly adverse effect on prognosis after aneurysmatic rupture. Treatment is somewhat empirical, given that uncertainties remain in our understanding of the pathophysiology of this vascular complication, which involves structural and biochemical changes in the endothelium and smooth muscle of vessels. Vasospasm that is refractory to treatment leads to cerebral infarction. Prophylaxis, early diagnosis, and adequate treatment of neurologic complications are key elements in the management of vasospasm if neurologic damage, lengthy hospital stays, and increased use of health care resources are to be avoided. New approaches to early treatment of cerebral lesions and cortical ischemia in cases of subarachnoid hemorrhage due to aneurysm rupture should lead to more effective, specific management.
Trujillo, J R; Garcìa-Ramos, G; Novak, I S; Rivera, V M; Huerta, E; Essex, M
Neurologic complications associated with human immunodeficiency virus type 1 (HIV-1) infection vary geographically. To understand the pattern of HIV-associated neurologic complications in Mexico, 120 AIDS patients from Mexico City, Mexico, and 500 AIDS patients from Houston, Texas, were studied cross-sectionally and retrospectively. Neurologic, laboratory, imaging, and pathologic examinations identified 40 Mexican patients and 130 U.S. patients with neurologic complications. Whereas AIDS dementia complex was the most common neurologic manifestation in both groups, intracranial tuberculoma was present only in the Mexican population (10%). Primary brain lymphoma was more prevalent in the U.S. population (8.4%). The different findings in the Mexican population likely reflect afflictions common to developing countries--a high prevalence of tuberculosis and a high mortality rate. These conditions preclude complications such as lymphoma, which develop later in the natural course of HIV infection.
Almeida Leila Azevedo de
Full Text Available Cerebral infarctions of cardiac etiology are observed in around 20% of patients with ischemic stroke. Cerebral ischemia is the first clinical manifestation in 1/3 of cases of atrial myxomas. Although almost half of patients with atrial myxoma show changes at neurological exam, non-hemorrhagic cerebral infarction is seen in computed tomography in practically all cases. We present the case of a 40 year-old woman whose first clinical manifestation of atrial myxoma was an ischemic stroke. We point out to the possibility of silent cerebral infarction in atrial myxoma patients.
Khan, Maria Usman; Devlin, James Anthony Joseph; Fraser, Alexander
The overall incidence of neurological manifestations is relatively low among patients with mixed connective tissue disease (MCTD). We recently encountered a case of autoimmune adhesive arachnoiditis in a young woman with 7 years history of MCTD who presented with severe back pain and myeloradiculopathic symptoms of lower limbs. To the best of our knowledge, adhesive arachnoiditis in an MCTD patient has never been previously reported. We report here this rare case, with the clinical picture and supportive ancillary data, including serology, cerebral spinal fluid analysis, electrophysiological evaluation and spinal neuroimaging, that is, MRI and CT (CT scan) of thoracic and lumbar spine. Her neurological deficit improved after augmenting her immunosuppressant therapy. Our case suggests that adhesive arachnoiditis can contribute to significant neurological deficits in MCTD and therefore requires ongoing surveillance. 2016 BMJ Publishing Group Ltd.
Ali Akbar Asadi-Pooya
Full Text Available Background: World Health Organization declared pandemic phase of human infection with novel influenza A (H1N1 in April 2009. There are very few reports about the neurological complications of H1N1 virus infection in the literature. Occasionally, these complications are severe and even fatal in some individuals. The aims of this study were to report neurological complaints and/or complications associated with H1N1 virus infection. Methods: The medical files of all patients with H1N1 influenza infection admitted to a specified hospital in the city of Shiraz, Iran from October through November 2009 were reviewed. More information about the patients were obtained by phone calls to the patients or their care givers. All patients had confirmed H1N1 virus infection with real-time PCR assay. Results: Fifty-five patients with H1N1 infection were studied. Twenty-three patients had neurological signs and/or symptoms. Mild neurological complaints may be reported in up to 42% of patients infected by H1N1 virus. Severe neurological complications occurred in 9% of the patients. The most common neurological manifestations were headache, numbness and paresthesia, drowsiness and coma. One patient had a Guillain-Barre syndrome-like illness, and died in a few days. Another patient had focal status epilepticus and encephalopathy. Conclusions: The H1N1 infection seems to have been quite mild with a self-limited course in much of the world, yet there appears to be a subset, which is severely affected. We recommend performing diagnostic tests for H1N1influenza virus in all patients with respiratory illness and neurological signs/symptoms. We also recommend initiating treatment with appropriate antiviral drugs as soon as possible in those with any significant neurological presentation accompanied with respiratory illness and flu-like symptoms
Wilmshurst, P; Davidson, C; O'Connell, G; Byrne, C
1. Blind analysis of contrast echocardiograms to detect intracardiac shunts, blind analysis of lung function tests for evidence of small airways disease, smoking history and dive characteristics were examined in an attempt to explain neurological symptoms that occurred within 5 min of surfacing from unprovocative dives. 2. Pulmonary abnormalities were significantly more frequent in those divers without intracardiac shunts (50%) than in those with shunts (0%). Smoking was more common in those divers without shunts (55% versus 15%), although this just failed to reach conventional significance levels. Divers without shunts experienced cerebral rather than spinal symptoms after significantly shallower dives with lower tissue nitrogen loads. Depths of dives, tissue nitrogen loads and clinical manifestations in those divers without shunts were similar to the findings in divers who had symptoms after rapid ascents. Despite conservative dive profiles, clinical manifestations in divers with shunts resembled those observed after missed decompression stops. 3. The findings suggest that occult lung disease, and probably smoking, increase the risk of neurological symptoms, even after unprovocative dives, and the similarity of the dive profiles and clinical manifestations to cases with rapid ascents suggest that pulmonary barotrauma and arterial gas embolism are responsible. In divers with intracardiac shunts the different dive profiles and clinical manifestations imply that there is another mechanism, involving different tissue and bubble nitrogen kinetics resulting in venous gas liberation and peripheral amplification in embolized tissues, rather than paradoxical embolism per se.
Castori, Marco; C. Voermans, Nicol
The term “Ehlers-Danlos syndrome” (EDS) groups together an increasing number of heritable connective tissue disorders mainly featuring joint hypermobility and related complications, dermal dysplasia with abnormal skin texture and repair, and variable range of the hollow organ and vascular dysfunctions. Although the nervous system is not considered a primary target of the underlying molecular defect, recently, increasing attention has been posed on neurological manifestations of EDSs, such as musculoskeletal pain, fatigue, headache, muscle weakness and paresthesias. Here, a comprehensive overview of neurological findings of these conditions is presented primarily intended for the clinical neurologist. Features are organized under various subheadings, including pain, fatigue, headache, stroke and cerebrovascular disease, brain and spine structural anomalies, epilepsy, muscular findings, neuropathy and developmental features. The emerging picture defines a wide spectrum of neurological manifestations that are unexpectedly common and potentially disabling. Their evaluation and correct interpretation by the clinical neurologist is crucial for avoiding superfluous investigations, wrong therapies, and inappropriate referral. A set of basic tools for patient’s recognition is offered for raising awareness among neurologists on this underdiagnosed group of hereditary disorders. PMID:25632331
This article offered an extensive description of the clinical and pathological features and time-course of the skin manifestations of toxic syndrome caused by denatured rapeseed oil, also known as toxic oil syndrome. This new condition occurred in Spain in 1981 and was due to the ingestion of rapeseed oil intended for industrial use that had been denatured with anilines and subsequently refined and sold fraudulently as olive oil. In total, 20,000 cases and 400 deaths were reported. The disease affected mainly women, particularly in the late stages. In the acute phase, the predominant skin manifestations were toxic-allergic rashes reminiscent of allergic urticaria in the dermatopathologic study. In approximately 25% of cases, the patients' skin subsequently took on an edematous appearance, with pigmentary abnormalities shown to be related to cutaneous mucinosis. Finally, a characteristic sclerodermatous condition would develop that tended to improve spontaneously. The constant presence of mast cells in all biopsies and the development of mastocytosis in several patients pointed to an important role for these cells in the pathogenesis of the condition. This was subsequently confirmed in other sclerodermatous processes. In 1989, eosinophilia-myalgia syndrome caused by toxins present in tryptophan food supplements was reported in the United States. This syndrome resembled toxic oil syndrome in many ways and demonstrated that mucinosis and toxic sclerodermatous processes do exist.
Marijke eDe Bock
Full Text Available The coordination of tissue function is mediated by gap junctions (GJs that enable direct cell-cell transfer of metabolic and electric signals. GJs are formed by connexins of which Cx43 is most widespread in the human body. In the brain, Cx43 GJs are mostly found in astroglia where they coordinate the propagation of Ca2+ waves, spatial K+ buffering and distribution of glucose. Beyond its role in direct intercellular communication, Cx43 also forms unapposed, non-junctional hemichannels in the plasma membrane of glial cells. These allow the passage of several neuro- and gliotransmitters that may, combined with downstream paracrine signaling, complement direct GJ communication among glial cells and sustain glial-neuronal signaling. Mutations in the GJA1 gene encoding Cx43 have been identified in a rare, mostly autosomal dominant syndrome called oculodentodigital dysplasia (ODDD. ODDD patients display a pleiotropic phenotype reflected by eye, hand, teeth and foot abnormalities, as well as craniofacial and bone malformations. Remarkably, neurological symptoms such as dysarthria, neurogenic bladder (manifested as urinary incontinence, spasticity or muscle weakness, ataxia, and epilepsy are other prominent features observed in ODDD patients. Over 10 mutations detected in patients diagnosed with neurological disorders are associated with altered functionality of Cx43 GJs/hemichannels, but the link between ODDD-related abnormal channel activities and neurologic phenotype is still elusive. Here, we present an overview on the nature of the mutants conveying structural and functional changes of Cx43 channels and discuss available evidence for aberrant Cx43 GJ and hemichannel function. In a final step, we examine the possibilities of how channel dysfunction may lead to some of the neurological manifestations of ODDD.
William S. Baek
Full Text Available A 5-year-old boy presented with mild autism and attention-deficit hyperactivity disorder (ADHD. Chromosomal microarray demonstrated a 1.7 Mb deletion at Xp22.31, which was consistent with X-linked ichthyosis (XLI. Further exam revealed dry, scaly skin on his abdomen and pretibial areas. Patients with mutations involving solely the STS gene or the recurrent ~2 Mb deletion may present with ADHD, whereas those with larger deletions including the NLGN4 gene can present with both ADHD and autism. However, our patient presented with mild autism in addition to ADHD despite having only the recurrent deletion without loss of NLGN4. Such neurological manifestations of XLI warrant attention as practical targets of clinical management.
Baek, William S; Aypar, Umut
A 5-year-old boy presented with mild autism and attention-deficit hyperactivity disorder (ADHD). Chromosomal microarray demonstrated a 1.7 Mb deletion at Xp22.31, which was consistent with X-linked ichthyosis (XLI). Further exam revealed dry, scaly skin on his abdomen and pretibial areas. Patients with mutations involving solely the STS gene or the recurrent ~2 Mb deletion may present with ADHD, whereas those with larger deletions including the NLGN4 gene can present with both ADHD and autism. However, our patient presented with mild autism in addition to ADHD despite having only the recurrent deletion without loss of NLGN4. Such neurological manifestations of XLI warrant attention as practical targets of clinical management.
Full Text Available Lyme disease (LD is a tick-borne illness caused by Borrelia burgdorferi sensu stricto. An 80-year-old female from Pennsylvania, USA, presented to an outside hospital with fever, confusion, lower extremity weakness, and stool incontinence. CT head and MRI spine were unremarkable. An infectious work-up including lumbar puncture was negative. She was transferred to our tertiary care hospital. Patient was noted to have mild unilateral right-sided facial droop and a diffuse macular rash throughout the body. She denied any outdoor activities, tick bites, or previous rash. Intravenous ceftriaxone was started for suspected LD. The patient’s symptoms including facial droop resolved within 24 hours of antibiotic therapy. Polymerase chain reaction of the blood, IgM ELISA, and IgM Western blot testing for LD came back positive a few days after initiation of therapy. She was treated for a total of 21 days for neurological LD with complete symptom resolution. Not all patients have the classic “targetoid” EM rash on initial presentation, rash could develop after neurological manifestations, and prompt initiation of antibiotics without awaiting serology is paramount to making a quick and a full recovery. There should be a high index of suspicion for early disseminated LD, as presentations can be atypical.
Full Text Available Context Zika virus (ZIKV is an arbovirus of the family Flaviviridae. This virus, which is transmitted by mosquitoes, usually affects children and causes self-limited diseases, associated with fever, maculopapular rash, conjunctivitis, arthralgia, and myalgia. There is a significant relationship between ZIKV infection and central nervous system disorders in infants. In this study, we aimed to review neurologic manifestations of ZIKV infection in infants. Evidence Acquisition We searched the following Mesh terms in scientific databases: “Zika virus”, “infants”, “children”, “neurologic manifestations”, and “congenital infection”. Electronic databases including Google Scholar, Science Direct, PubMed, Web of Science, Scientific Information Database, and Scopus were searched from 2000 to 2016. Results The literature review showed a probable relationship between ZIKV infection and prevalence of microcephaly in newborns. Besides microcephaly, other central nervous system abnormalities included abnormal gyral arrangement, decreased brain parenchymal volume, cortical atrophy and malformation, cerebellar hypoplasia, and delayed myelination of the brain stem. Conclusions Vertical transmission of ZIKV in pregnant women is associated with intrauterine infection and brain malformations in the developing fetus, including microcephaly, calcification, cortical displacement, ventriculomegaly, and white-matter abnormalities.
Simon, Marleen; Campos-Xavier, Ana Belinda; Mittaz-Crettol, Lauréane; Valadares, Eugenia Ribeiro; Carvalho, Daniel; Speck-Martins, Carlos Eduardo; Nampoothiri, Sheela; Alanay, Yasemin; Mihci, Ercan; van Bever, Yolande; Garcia-Segarra, Nuria; Cavalcanti, Denise; Mortier, Geert; Bonafé, Luisa; Superti-Furga, Andrea
Spondylo-megaepiphyseal-metaphyseal dysplasia (SMMD; OMIM 613330) is a dysostosis/dysplasia caused by recessive mutations in the homeobox-containing gene, NKX3-2 (formerly known as BAPX1). Because of the rarity of the condition, its diagnostic features and natural course are not well known. We describe clinical and radiographic findings in six patients (five of which with homozygous mutations in the NKX3-2 gene) and highlight the unusual and severe changes in the cervical spine and the neurologic complications. In individuals with SMMD, the trunk and the neck are short, while the limbs, fingers and toes are disproportionately long. Radiographs show a severe ossification delay of the vertebral bodies with sagittal and coronal clefts, missing ossification of the pubic bones, large round "balloon-like" epiphyses of the long bones, and presence of multiple pseudoepiphyses at all metacarpals and phalanges. Reduced or absent ossification of the cervical vertebrae leads to cervical instability with anterior or posterior kinking of the cervical spine (swan neck-like deformity, kyknodysostosis). As a result of the cervical spine instability or deformation, five of six patients in our series suffered cervical cord injury that manifested clinically as limb spasticity. Although the number of individuals observed is small, the high incidence of cervical spine deformation in SMMD is unique among skeletal dysplasias. Early diagnosis of SMMD by recognition of the radiographic pattern might prevent of the neurologic complications via prophylactic cervical spine stabilization. Copyright © 2012 Wiley Periodicals, Inc.
Kiessling, Si-Young; Soyka, Michael B; Huber, Gerhard F; Holzmann, David; Laske, Roman D
The objective of this study is to analyze the presenting symptoms, the time to correct diagnosis and outcome of a European patient cohort with sinonasal lymphoma focusing on unilateral vs. bilateral sinonasal involvement. In a retrospective setting in a European tertiary referral center, eleven patients (9 males, 2 females) with sinonasal lymphoma diagnosed between 2002 and 2015 were identified and divided into a unilateral and bilateral group according to their CT finding. Clinical findings on first presentation, the presence of B symptoms, the radiologic findings, overall survival and disease-specific survival were assessed. 55 % of the patients suffered from bilateral manifestation, which was associated with a delayed diagnosis (10 vs. 1.5 months, p manifestation with B symptoms was shown to be common within the rare entity of sinonasal lymphomas and patients with bilateral sinonasal manifestation are at risk for a delayed diagnosis with worse outcome.
López Bravo, Alba; Parra Soto, Carlos; Bellosta Diago, Elena; Cecilio Irazola, Álvaro; Santos-Lasaosa, Sonia
Neurological involvement in Behçet's disease is rare, especially at the onset. It can present in the form of parenchymal changes or as damage to the vascular structures in its nonparenchymal form. The coexistence of both kinds of manifestations in the same patient is exceptional. We report the case of a 32-year-old patient with a history of deep venous thrombosis, who was being treated for holocranial headache, apathy, and oral and genital ulcers. Brain magnetic resonance imaging showed hyperintense lesions in the basal ganglia and white matter, and the vascular study evidenced venous thrombosis of the left transverse sinus. After confirming the diagnosis of Behçet's disease with parenchymal and nonparenchymal cerebral involvement, immunosuppressive and corticosteroid therapy was started, resulting in the remission of the symptoms. Copyright © 2017 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved.
Sechi, GianPietro; Sechi, Elia; Fois, Chiara; Kumar, Neeraj
B vitamin deficiency is a leading cause of neurological impairment and disability throughout the world. Multiple B vitamin deficiencies often coexist, and thus an understanding of the complex relationships between the different biochemical pathways regulated in the brain by these vitamins may facilitate prompter diagnosis and improved treatment. Particular populations at risk for multiple B vitamin deficiencies include the elderly, people with alcoholism, patients with heart failure, patients with recent obesity surgery, and vegetarians/vegans. Recently, new clinical settings that predispose individuals to B vitamin deficiency have been highlighted. Moreover, other data indicate a possible pathogenetic role of subclinical chronic B vitamin deficiency in neurodegenerative diseases such as Alzheimer's disease, Parkinson's disease, and amyotrophic lateral sclerosis. In light of these findings, this review examines the clinical manifestations of B vitamin deficiency and the effect of B vitamin deficiency on the adult nervous system. The interrelationships of multiple B vitamin deficiencies are emphasized, along with the clinical phenotypes related to B vitamin deficiencies. Recent advances in the clinical determinants and diagnostic clues of B vitamin deficiency, as well as the suggested therapies for B vitamin disorders, are described. © The Author(s) 2016. Published by Oxford University Press on behalf of the International Life Sciences Institute. All rights reserved. For Permissions, please e-mail: firstname.lastname@example.org.
Cristina L. Lema
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
Full Text Available Paola Caruso, Paolo Manganotti, Rita Moretti Department of Clinical Neurology, University of Trieste, Trieste, Italy Abstract: The artery of Percheron is a rare anatomical variant where a single thalamic perforating artery arises from the proximal posterior cerebral artery (P1 segment between the basilar artery and the posterior communicating artery and supplies the rostral mesencephalon and both paramedian territories of the thalami. Almost one-third of human brains present this variant. Occlusion of the artery of Percheron mostly results in a bilateral medial thalamic infarction, which usually manifests with altered consciousness (including coma, vertical gaze paresis, and cognitive disturbance. The presentation is similar to the “top of the basilar syndrome”, and early recognition should be prompted. We describe the case of a young female with this vessel variant who experienced a bilateral thalamic stroke. Magnetic resonance angiography demonstrated bilateral thalamic infarcts and a truncated artery of Percheron. Occlusion of the vessel was presumably due to embolism from a patent foramen ovale. Thrombolysis was performed, with incomplete symptom remission, cognitive impairment, and persistence of speech disorders. Early recognition and treatment of posterior circulation strokes is mandatory, and further investigation for underlying stroke etiologies is needed. Keywords: thalamus vascularization, cognitive impairment, paramedian thalamus territory, speech disorder, vertical gaze palsy
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.
Khoury, Eliana Dirce Torres; Souza, Givago da Silva; Silveira, Luiz Carlos de Lima; Costa, Carlos Araújo da; Araújo, Amélia A de; Pinheiro, Maria da Conceição Nascimento
This study evaluated current levels of mercury exposure and sensory symptoms in adults from three riverine communities in Pará State, Brazil, two of which located in the Tapajós River basin and one in the Tocantins basin. Participants in this study included 78 residents in Barreiras (Tapajós), 30 in São Luiz do Tapajós (Tapajós), and 49 in Furo do Maracujá (Tocantins). Total hair mercury concentrations were quantified by atomic absorption spectrophotometry, and neurological evaluation was conducted by routine examination. Mercury concentrations in the Tapajós communities were higher than those in the Tocantins (p neurological changes showed no significant difference between the communities in exposed areas and control areas for the changes observed by conventional neurological examination, except for gait deviation (p mercury exposure levels, there was a low frequency of sensory alterations according to conventional neurological testing.
Full Text Available BACKGROUND: The bacterium Salmonella enterica serovar Typhi causes typhoid fever, which is typically associated with fever and abdominal pain. An outbreak of typhoid fever in Malawi-Mozambique in 2009 was notable for a high proportion of neurologic illness. OBJECTIVE: Describe neurologic features complicating typhoid fever during an outbreak in Malawi-Mozambique METHODS: Persons meeting a clinical case definition were identified through surveillance, with laboratory confirmation of typhoid by antibody testing or blood/stool culture. We gathered demographic and clinical information, examined patients, and evaluated a subset of patients 11 months after onset. A sample of persons with and without neurologic signs was tested for vitamin B6 and B12 levels and urinary thiocyanate. RESULTS: Between March - November 2009, 303 cases of typhoid fever were identified. Forty (13% persons had objective neurologic findings, including 14 confirmed by culture/serology; 27 (68% were hospitalized, and 5 (13% died. Seventeen (43% had a constellation of upper motor neuron findings, including hyperreflexia, spasticity, or sustained ankle clonus. Other neurologic features included ataxia (22, 55%, parkinsonism (8, 20%, and tremors (4, 10%. Brain MRI of 3 (ages 5, 7, and 18 years demonstrated cerebral atrophy but no other abnormalities. Of 13 patients re-evaluated 11 months later, 11 recovered completely, and 2 had persistent hyperreflexia and ataxia. Vitamin B6 levels were markedly low in typhoid fever patients both with and without neurologic signs. CONCLUSIONS: Neurologic signs may complicate typhoid fever, and the diagnosis should be considered in persons with acute febrile neurologic illness in endemic areas.
Jugpal, Tejeshwar Singh; Dixit, Rashmi; Garg, Anju; Gupta, Swati; Jain, Virendra; Patel, Ronak; Agarwal, Shobhit
To describe the spectrum of magnetic resonance imaging (MRI) findings in patients with neurological manifestations of dengue. We included nine patients with dengue fever (three females and six males; age range, 9-30 years), all of whom presented with neurological manifestations. The MRI examinations, performed in 1.5 T or 3 T scanners, included T1-weighted, T2-weighted, and fluid-attenuated inversion recovery (FLAIR) sequences. Diffusion-weighted imaging with apparent diffusion coefficient mapping was also employed. Fast low-angle shot and susceptibility-weighted gradient-recalled echo sequences, as well as contrast-enhanced T1-weighted scans, were also obtained in order to assess parenchymal enhancement. MRI scans were analyzed for lesion distribution and imaging features. All patients showed areas of altered signal intensity that appeared as hyperintensity on T2-weighted and FLAIR sequences. The most commonly affected site was the basal ganglia-thalamus complex. Other affected sites were the cerebellum, cerebral cortex, white matter, and brainstem. In all cases, we observed patchy areas of restricted diffusion and focal areas of hemorrhage. Dengue encephalitis commonly affects the basal ganglia, thalamus, cerebellum, cerebral cortex, and white matter. Therefore, MRI should be an indispensable part of the evaluation of patients with neurological complications of dengue fever.
Allan Chiaratti de Oliveira
Full Text Available CONTEXT: Inborn Errors of Metabolism are hereditary affections resulting from incompetence in enzymatic reactions of intermediary metabolism. At present, several hundred hereditary metabolic disturbances are known, many of which correspond to severe life-threatening disorders. OBJECTIVE: The early detection of carriers has motivated the screening for these disturbances among newborns at the Neonatal Unit of Hospital São Paulo, in an attempt to initiate support treatment, when available, before clinical manifestations become evident. DESIGN: Prospective study of risk patients. SETTING: Laboratory for Inborn Errors of Metabolism at the Center for Medical Genetics of the Departments of Pediatrics and Morphology of Universidade Federal de São Paulo/Escola Paulista de Medicina.Newborn care unit at a tertiary care hospital. PARTICIPANTS: 101 children admitted into the Neonatal Unit were included in this study by presenting hypoglycemia, metabolic acidosis, jaundice, difficulty in gaining weight, diarrhea, vomiting, hepato- and/or splenomegaly, cataracts, apnea, convulsions, hypo- or hypertonia. DIAGNOSTIC TESTS: Tests routinely utilized, performed for qualitative research of abnormal substances excreted in the urine in situations of metabolic disorder. RESULTS: Children were included in the study mainly because of presenting hypoglycemia, jaundice and metabolic acidosis. Sixty-four newborns presented at least one positive test result. Most of the positivity was due to transitory metabolic alterations of the newborn, such as the case of Transitory Neonatal Tyrosinemia, presented by 29 patients. Nine infants were referred to the Center for Medical Genetics of Universidade Federal de São Paulo for continuation of the diagnostic investigation. For three of them, the tests applied permitted us to formulate a diagnostic hypothesis of mucopolysaccharidosis, tyrosinemia type I and non-ketotic hyperglycinemia, respectively. CONCLUSIONS: The high positivity
Simon Marleen; Campos-Xavier Ana Belinda; Mittaz-Crettol Lauréane; Valadares Eugenia Ribeiro; Carvalho Daniel; Speck-Martins Carlos Eduardo; Nampoothiri Sheela; Alanay Yasemin; Mihci Ercan; van Bever Yolande; Garcia-Segarra Nuria; Cavalcanti Denise; Mortier Geert; Bonafé Luisa; Superti-Furga Andrea
Spondylo megaepiphyseal metaphyseal dysplasia (SMMD; OMIM 613330) is a dysostosis/dysplasia caused by recessive mutations in the homeobox containing gene NKX3 2 (formerly known as BAPX1). Because of the rarity of the condition its diagnostic features and natural course are not well known. We describe clinical and radiographic findings in six patients (five of which with homozygous mutations in the NKX3 2 gene) and highlight the unusual and severe changes in the cervical spine and the neurolog...
Pors Susanne E
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.
Gredal, Hanne; Willesen, Jakob L; Jensen, Henrik E; Nielsen, Ole L; Kristensen, Annemarie T; Koch, Jørgen; Kirk, Rikke K; Pors, Susanne E; Skerritt, Geoff C; Berendt, Mette
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.
Smolka, V; Bekárek, V; Hlídková, E; Bucil, J; Mayerová, D; Skopková, Z; Adam, T; Hrubá, E; Kozich, V; Buriánková, L; Saligová, J; Buncová, M; Zeman, J
Serious hematological, metabolic and neurological complications owing to the nutritional deficiency of vitamin B12 may occur in infants of mothers on a strict vegetarian diet. The mother of the first child was a strict vegetarian. She had an elevated urinary methylmalonic acid level and a low concentration of serum vitamin B12. Her 13-month-old daughter was exclusively breast-fed until the age of 9 month and then she was fed only vegetables. Physical examination revealed psychomotoric retardation, apathy, muscular hypotonia, abnormal movements and failure to thrive. Laboratory analysis showed a megaloblastic anaemia, a low level of vitamin B12 and methylmalonic aciduria. MRI of the brain revealed diffuse frontotemporoparietal atrophy and retardation of myelination. After treatment with vitamin B12 supplements, abnormal movements disappeared and development improved, but a mild generalised hypotonia continued. A cranial MRI 9 months after treatment still showed signs of retardation of myelination. The second patient, an 8 month-old male, son of a strict vegetarian mother too, was referred for investigation of psychomotoric retardation, hypotonia, dyskinesia, failure to thrive and microcephaly. He was breast-fed and from 6 month of age he had also received fruit juices. Laboratory analysis revealed megaloblastic anaemia, high methylmalonic aciduria and homocystinuria. The patient's and his mother's serum level of vitamin B12 were low. After treatment with vitamin B12 supplements, biochemical and metabolic markers of disease were normal but there continued a generalised hypotonia, microcephaly and language delay. Our observations emphasize the health complications of nutritional cobalamine deficiency and a requirement of clinical, biochemical and metabolic monitoring in infants within strict vegetarian families.
Orlin, Anton; Sondhi, Dolan; Witmer, Matthew T; Wessel, Matthew M; Mezey, Jason G; Kaminsky, Stephen M; Hackett, Neil R; Yohay, Kaleb; Kosofsky, Barry; Souweidane, Mark M; Kaplitt, Michael G; D'Amico, Donald J; Crystal, Ronald G; Kiss, Szilárd
Late infantile neuronal ceroid lipofuscinosis (LINCL), one form of Batten's disease is a progressive neurodegenerative disorder resulting from a CLN2 gene mutation. The spectrum of ophthalmic manifestations of LINCL and the relationship with neurological function has not been previously described. Patients underwent ophthalmic evaluations, including anterior segment and dilated exams, optical coherence tomography, fluorescein and indocyanine green angiography. Patients were also assessed with the LINCL Neurological Severity Scale. Ophthalmic findings were categorized into one of five severity scores, and the association of the extent of ocular disease with neurological function was assessed. Fifty eyes of 25 patients were included. The mean age at the time of exam was 4.9 years (range 2.5 to 8.1). The mean ophthalmic severity score was 2.6 (range 1 to 5). The mean neurological severity score was 6.1 (range 2 to 11). Significantly more severe ophthalmic manifestations were observed among older patients (p<0.005) and patients with more severe neurological findings (p<0.03). A direct correlation was found between the Ophthalmic Severity Scale and the Weill Cornell Neurological Scale (p<0.002). A direct association was also found between age and the ophthalmic manifestations (p<0.0002), with older children having more severe ophthalmic manifestations. Ophthalmic manifestations of LINCL correlate closely with the degree of neurological function and the age of the patient. The newly established LINCL Ophthalmic Scale may serve as an objective marker of LINCL severity and disease progression, and may be valuable in the evaluation of novel therapeutic strategies for LINCL, including gene therapy.
Full Text Available BACKGROUND: Late infantile neuronal ceroid lipofuscinosis (LINCL, one form of Batten's disease is a progressive neurodegenerative disorder resulting from a CLN2 gene mutation. The spectrum of ophthalmic manifestations of LINCL and the relationship with neurological function has not been previously described. METHODS: Patients underwent ophthalmic evaluations, including anterior segment and dilated exams, optical coherence tomography, fluorescein and indocyanine green angiography. Patients were also assessed with the LINCL Neurological Severity Scale. Ophthalmic findings were categorized into one of five severity scores, and the association of the extent of ocular disease with neurological function was assessed. RESULTS: Fifty eyes of 25 patients were included. The mean age at the time of exam was 4.9 years (range 2.5 to 8.1. The mean ophthalmic severity score was 2.6 (range 1 to 5. The mean neurological severity score was 6.1 (range 2 to 11. Significantly more severe ophthalmic manifestations were observed among older patients (p<0.005 and patients with more severe neurological findings (p<0.03. A direct correlation was found between the Ophthalmic Severity Scale and the Weill Cornell Neurological Scale (p<0.002. A direct association was also found between age and the ophthalmic manifestations (p<0.0002, with older children having more severe ophthalmic manifestations. CONCLUSIONS: Ophthalmic manifestations of LINCL correlate closely with the degree of neurological function and the age of the patient. The newly established LINCL Ophthalmic Scale may serve as an objective marker of LINCL severity and disease progression, and may be valuable in the evaluation of novel therapeutic strategies for LINCL, including gene therapy.
Orlin, Anton; Sondhi, Dolan; Witmer, Matthew T.; Wessel, Matthew M.; Mezey, Jason G.; Kaminsky, Stephen M.; Hackett, Neil R.; Yohay, Kaleb; Kosofsky, Barry; Souweidane, Mark M.; Kaplitt, Michael G.; D’Amico, Donald J.; Crystal, Ronald G.; Kiss, Szilárd
Background Late infantile neuronal ceroid lipofuscinosis (LINCL), one form of Batten’s disease is a progressive neurodegenerative disorder resulting from a CLN2 gene mutation. The spectrum of ophthalmic manifestations of LINCL and the relationship with neurological function has not been previously described. Methods Patients underwent ophthalmic evaluations, including anterior segment and dilated exams, optical coherence tomography, fluorescein and indocyanine green angiography. Patients were also assessed with the LINCL Neurological Severity Scale. Ophthalmic findings were categorized into one of five severity scores, and the association of the extent of ocular disease with neurological function was assessed. Results Fifty eyes of 25 patients were included. The mean age at the time of exam was 4.9 years (range 2.5 to 8.1). The mean ophthalmic severity score was 2.6 (range 1 to 5). The mean neurological severity score was 6.1 (range 2 to 11). Significantly more severe ophthalmic manifestations were observed among older patients (p<0.005) and patients with more severe neurological findings (p<0.03). A direct correlation was found between the Ophthalmic Severity Scale and the Weill Cornell Neurological Scale (p<0.002). A direct association was also found between age and the ophthalmic manifestations (p<0.0002), with older children having more severe ophthalmic manifestations. Conclusions Ophthalmic manifestations of LINCL correlate closely with the degree of neurological function and the age of the patient. The newly established LINCL Ophthalmic Scale may serve as an objective marker of LINCL severity and disease progression, and may be valuable in the evaluation of novel therapeutic strategies for LINCL, including gene therapy. PMID:24015292
Full Text Available Several genetic disorders caused by defective nucleotide excision repair that affect the skin and the nervous system have been described, including Xeroderma Pigmentosum (XP, De Sanctis–Cacchione syndrome (DSC, Cockayne syndrome, and Trichothiodystrophy. Cutaneous photosensitivity with an increased risk of skin malignancy is a common feature of these disorders, but clinical manifestations commonly overlap these syndromes. Several genes have been found to be altered in these pathologies, but we lack more genotype-phenotype correlations in order to make an accurate diagnosis. Very few cases of DSC syndrome have been reported in the literature. We present a case of a 12-year-old Colombian male, with multiple skin lesions in sun-exposed areas from the age of 3 months and a history of 15 skin cancers. He also displayed severe neurologic abnormalities (intellectual disability, ataxia, altered speech, and hyperreflexia, short stature, and microcephaly, which are features associated with DSC. Genetic testing revealed a novel germline mutation in the XP-C gene (c.547A>T. This is the first case of an XP-C mutation causing De Sanctis–Cacchione syndrome. Multigene panel testing is becoming more widely available and accessible in the clinical setting and will help rapidly unveil the molecular etiology of these rare genetic disorders.
Uribe-Bojanini, Esteban; Hernandez-Quiceno, Sara; Cock-Rada, Alicia María
Several genetic disorders caused by defective nucleotide excision repair that affect the skin and the nervous system have been described, including Xeroderma Pigmentosum (XP), De Sanctis-Cacchione syndrome (DSC), Cockayne syndrome, and Trichothiodystrophy. Cutaneous photosensitivity with an increased risk of skin malignancy is a common feature of these disorders, but clinical manifestations commonly overlap these syndromes. Several genes have been found to be altered in these pathologies, but we lack more genotype-phenotype correlations in order to make an accurate diagnosis. Very few cases of DSC syndrome have been reported in the literature. We present a case of a 12-year-old Colombian male, with multiple skin lesions in sun-exposed areas from the age of 3 months and a history of 15 skin cancers. He also displayed severe neurologic abnormalities (intellectual disability, ataxia, altered speech, and hyperreflexia), short stature, and microcephaly, which are features associated with DSC. Genetic testing revealed a novel germline mutation in the XP-C gene (c.547A>T). This is the first case of an XP-C mutation causing De Sanctis-Cacchione syndrome. Multigene panel testing is becoming more widely available and accessible in the clinical setting and will help rapidly unveil the molecular etiology of these rare genetic disorders.
Several genetic disorders caused by defective nucleotide excision repair that affect the skin and the nervous system have been described, including Xeroderma Pigmentosum (XP), De Sanctis–Cacchione syndrome (DSC), Cockayne syndrome, and Trichothiodystrophy. Cutaneous photosensitivity with an increased risk of skin malignancy is a common feature of these disorders, but clinical manifestations commonly overlap these syndromes. Several genes have been found to be altered in these pathologies, but we lack more genotype-phenotype correlations in order to make an accurate diagnosis. Very few cases of DSC syndrome have been reported in the literature. We present a case of a 12-year-old Colombian male, with multiple skin lesions in sun-exposed areas from the age of 3 months and a history of 15 skin cancers. He also displayed severe neurologic abnormalities (intellectual disability, ataxia, altered speech, and hyperreflexia), short stature, and microcephaly, which are features associated with DSC. Genetic testing revealed a novel germline mutation in the XP-C gene (c.547A>T). This is the first case of an XP-C mutation causing De Sanctis–Cacchione syndrome. Multigene panel testing is becoming more widely available and accessible in the clinical setting and will help rapidly unveil the molecular etiology of these rare genetic disorders. PMID:28255305
Finsterer, Josef; Stöllberger, Claudia; Wahbi, Karim
According to the American Heart Association, cardiomyopathies are classified as primary (solely or predominantly confined to heart muscle), secondary (those showing pathological myocardial involvement as part of a neuromuscular disorder) and those in which cardiomyopathy is the first/predominant manifestation of a neuromuscular disorder. Cardiomyopathies may be further classified as hypertrophic cardiomyopathy, dilated cardiomyopathy, restrictive cardiomyopathy, arrhythmogenic right ventricular cardiomyopathy, or unclassified cardiomyopathy (noncompaction, Takotsubo-cardiomyopathy). This review focuses on secondary cardiomyopathies and those in which cardiomyopathy is the predominant manifestation of a myopathy. Any of them may cause neurological disease, and any of them may be a manifestation of a neurological disorder. Neurological disease most frequently caused by cardiomyopathies is ischemic stroke, followed by transitory ischemic attack, syncope, or vertigo. Neurological disease, which most frequently manifests with cardiomyopathies are the neuromuscular disorders. Most commonly associated with cardiomyopathies are muscular dystrophies, myofibrillar myopathies, congenital myopathies and metabolic myopathies. Management of neurological disease caused by cardiomyopathies is not at variance from the same neurological disorders due to other causes. Management of secondary cardiomyopathies is not different from that of cardiomyopathies due to other causes either. Patients with neuromuscular disorders require early cardiologic investigations and close follow-ups, patients with cardiomyopathies require neurological investigation and avoidance of muscle toxic medication if a neuromuscular disorder is diagnosed. Which patients with cardiomyopathy profit most from primary stroke prevention is unsolved and requires further investigations. Copyright © 2013 Elsevier Inc. All rights reserved.
Zimmermann, Nicolle; Goulart Corrêa, Diogo; Tukamoto, Gustavo; Netto, Tania; Batista Pereira, Denis; Paz Fonseca, Rochele; Gasparetto, Emerson Leandro
To determine whether systemic lupus erythematosus (SLE) affecting subcortical white matter volumes, deep gray matter volumes, and cortical thickness differ between groups of SLE patients with psychiatric (P-SLE), neurological (N-SLE), or nonneuropsychiatric (non-NPSLE) presentations. Sixty-seven participants were divided into three groups (P-SLE [n = 19], N-SLE [n = 12], and non-NPSLE [n = 36]) and examined with a 1.5T MRI scanner. The images were segmented in FreeSurfer software into volumetric and cortical thickness measures using T1 3D magnetization prepared rapid gradient echo-weighted imaging. For comparative analyses of volume, multivariate analyses of covariance (MANCOVA) were applied followed by Bonferroni post-hoc tests, with age as a covariate. For cortical thickness analyses, the groups were compared with the Query Design Estimate Contrast tool adjusted for age. Globus pallidus volumes in both left (P ≤ 0.01) and right (P ≤ 0.05) hemispheres were larger in the N-SLE group than in the non-NPSLE group, and the left GP volume was greater in the N-SLE group than in the P-SLE group (P ≤ 0.05) (MANCOVA, post-hoc Bonferroni). The P-SLE group presented with thinning of cortical areas relative to the N-SLE (predominantly in the left parietal and right frontal and parietal regions) (P ≤ 0.05) and non-NPSLE (predominantly in parietal and occipital regions) (P ≤ 0.05) groups, whereas the N-SLE group presented with thickening of cortical areas (mostly right frontal and left parietal regions) relative to the non-NPSLE (P ≤ 0.05) and P-SLE groups. N-SLE patients had greater local volumes and cortical thicknesses than the other two groups, whereas P-SLE patients presented with decreased volumes and cortical thinning. These findings provide evidence of distinct neuroanatomical abnormalities in neurological versus psychiatric manifestations of SLE. 2 Technical Efficacy: Stage 3 J. MAGN. RESON. IMAGING 2017;46:150-158. © 2016 International Society
Bravo J, Paulina; Ibarra C, Judith; Paredes M, Marcela
Vitamin B12 deficiency is extremely common in strict vegetarians and their variants. Infants of vegetarian mothers have a higher risk of deficiency and are more prone to its effects. To report a case in order to warn people about the importance of suspected vitamin B12 deficiency in children of vegetarian mothers. A 12-month old infant, daughter of a longtime vegetarian woman, who presented neurological and hematological compromise due to vitamin B12 deficiency, is discussed. After a short period of parenteral administration of cyanocobalamin and enteral nutrition, the patient evolved with clinical and laboratory improvement, although she still had residual development delay. Vitamin B12 deficiency is often not suspected by the pediatrician in healthy infants. Clinical manifestations can be nonspecific, such as apathy, food refusal and progressive impairment of psychomotor development. A nutritional anamnesis performed on the mother (with great emphasis on those strict vegetarians) to estimate her reserves in the period prior to, during and after delivery can be critical to detect the risk of this vitamin deficiency in young children.
Application of whole exome sequencing to a rare inherited metabolic disease with neurological and gastrointestinal manifestations: a congenital disorder of glycosylation mimicking glycogen storage disease.
Choi, Rihwa; Woo, Hye In; Choe, Byung-Ho; Park, Seungman; Yoon, Yeomin; Ki, Chang-Seok; Lee, Soo-Youn; Kim, Jong-Won; Song, Junghan; Kim, Dong Sub; Kwon, Soonhak; Park, Hyung-Doo
Rare inherited metabolic diseases with neurological and gastrointestinal manifestations can be misdiagnosed as other diseases or remain as disorders with indeterminate etiologies. This study aims to provide evidence to recommend the utility of whole exome sequencing in clinical diagnosis of a rare inherited metabolic disease. A 4-month-old female baby visited an outpatient clinic due to poor weight gain, repeated seizure-like episodes, developmental delay, and unexplained hepatomegaly with abnormal liver function test results. Although liver biopsy revealed moderate fibrosis with a suggested diagnosis of glycogen storage disease (GSD), no mutations were identified either by single gene approach for GSD (G6PC and GAA) or by next generation sequencing panels for GSD (including 21 genes). Whole exome sequencing of the patient revealed compound heterozygous mutations of PMM2: c.580C>T (p.Arg194*) and c.713G>C (p.Arg238Pro) which mutations were associated with congenital disorder of glycosylation Ia (CDG-Ia: PMM2-CDG). We successfully applied exome sequencing to diagnose the first reported Korean patient with CDG-Ia, which was misdiagnosed as GSD. Whole exome sequencing may prove to be the preferred strategy for analysis of clinical features that do not readily suggest a specific diagnosis, such as those observed in inherited metabolic diseases, including CDG. Copyright © 2015 Elsevier B.V. All rights reserved.
Sen, P; Gil, C; Estrellas, B; Middleton, J R
Inadequate therapeutic response to parenteral corticosteroids in patients with acute bronchial asthma is infrequent. We report four patients whose bronchial asthma symptoms worsened after treatment with parenteral corticosteroids. All had larvae of Strongyloides stercoralis in the stool. The new attack or the exacerbation of asthma appeared to be precipitated by systemic corticosteroid administration. The paradoxic therapeutic response of asthma to glucocorticoides was the major pulmonary manifestation of Strongyloides superinfection; there was no evidence of other organ involvement. Individuals with new onset of bronchial asthma or worsening of asthmatic episodes concurrent with the use of systemic corticosteroids should have thorough investigation for possible superinfection due to Strongyloides stercoralis. This is particularly important for patients who have resided in areas where intestinal helminthic infections are endemic. Discontinuance of steroid therapy or reduction in dosage of parenteral steroids appears necessary. Treatment with thiabendazole appears to be effective in patients with limited hyperinfection syndrome.
Canhão, P; Ferro, J M; Freitas, J P
Neurological involvement in a series of patients with Behçet's disease, evaluated at the Departments of Neurology and Dermatology, St. Maria Hospital is reported. Meningoencephalytic or encephalytic were the most common clinical forms, while headache, cerebellar and pyramidal signs were the most prevalent symptoms/signs. On follow-up (range 2-13 years) the majority of the patients had either a progressive or a remitting-progressive course. Magnetic ressonance imaging was the most valuable method of detecting central nervous system lesions.
Bøttger, Pernille; Doganli, Canan; Lykke-Hartmann, Karin
The two autosomal dominantly inherited neurological diseases: familial hemiplegic migraine type 2 (FHM2) and familial rapid-onset of dystonia-parkinsonism (Familial RDP) are caused by in vivo mutations of specific alpha subunits of the sodium–potassium pump (Na+/K+-ATPase). Intriguingly, patients...... patient symptoms and manifestations. Thus, it is interesting that mouse models targeting a specific -isoform cause different, although still comparable, phenotypes consistent with classical symptoms and other manifestations observed in FHM2 and RDP patients. This review highlights that use of mouse models...... with classical FHM2 and RDP symptoms additionally suffer from other manifestations, such as epilepsy/seizures and developmental disabilities. Recent studies of FHM2 and RDP mouse models provide valuable tools for dissecting the vital roles of the Na+/K+-ATPases, and we discuss their relevance to the complex...
Yu, Jianhai; Liu, Xuling; Ke, Changwen; Wu, Qinghua; Lu, Weizhi; Qin, Zhiran; He, Xiaoen; Liu, Yujing; Deng, Jieli; Xu, Suiqi; Li, Ying; Zhu, Li; Wan, Chengsong; Zhang, Qiwei; Xiao, Weiwei; Xie, Qian; Zhang, Bao; Zhao, Wei
Since 2015, 84 countries and territories reported evidence of vector-borne Zika Virus (ZIKV) transmission. The World Health Organization (WHO) declared that ZIKV and associated consequences especially the neurological autoimmune disorder Guillain-Barré syndrome (GBS) and microcephaly will remain a significant enduring public health challenge requiring intense action. We apply a standardization of the multi-subcutaneous dorsal inoculation method to systematically summarize clinical neurological manifestation, viral distribution, and tissue damage during the progress of viremia and systemic spread in suckling mouse models. We found that C57BL/6 and Kunming mice (KM) both showed remarkable and uniform neurologic manifestations. C57BL/6 owned the highest susceptibility and pathogenicity to the nervous system, referred to as movement disorders, with 100% incidence, while KM was an economic model for a Chinese study characterized by lower limb weakness with 62% morbidity. Slight yellow extraocular exudates were observed in BALB/c, suggesting the association with similar ocular findings to those of clinical cases. The virus distribution and pathological changes in the sera, brains, livers, kidneys, spleens, and testes during disease progression had strong regularity and uniformity, demonstrating the effectiveness and plasticity of the animal models. The successful establishment of these animal models will be conducive to expound the pathogenic mechanism of GBS.
Giannuzzi, A P; Ricciardi, M; De Simone, A; Gernone, F
In order to evaluate possible nervous system involvement in canine leishmaniasis, retrospective evaluation of all medical records of leishmaniotic dogs exhibiting neurological signs referred to our hospital over a 5-year period was performed. The records of 10 dogs were reviewed. Depending on the neuroanatomical localisation, the dogs underwent advanced diagnostic imaging, cerebrospinal fluid analysis, electrodiagnostic testing and histopathologic evaluations. The final neurological diagnosis was: meningoencephalitis (n=2), brain haemorrhagic stroke (n=1), haemorrhagic choroiditis (n=1), meningomyelitis (n=2), ischaemic myelopathy (n=1), polymyositis (n=2) and peripheral neuropathy (n=1). This study confirms that both central and peripheral nervous systems can be affected by leishmaniasis and provides an overview on the possible etiopathogenetic mechanisms. In addition, clinical and diagnostic findings, therapy and follow-up of affected dogs are described. © 2017 British Small Animal Veterinary Association.
Yokudhon N. Madjidova
Full Text Available The aim of our work was to study the neurological status and cognitive function in patients with stage I and II CCI, depending on its nature. Material and Methods: The study included 302 patients (mean age - 61.3±0.3 years; 165/54.6% men and 137/45.4% women with stage I and II CCI. Clinical and neurological examination, study of cognitive function, and MRI were performed in all patients. The degree of cognitive defect was determined by the MMSE (Mini-Mental State Exam test and the Bourdon test (visual perception and vigilance. All the subjects were categorized into two groups. Group 1 consisted of 133 patients with the amnestic type of MCI (AT-MCI; Group 2 consisted of 169 patients with the multifunction type of MCI (MT-MCI. Results: The highest frequency of complaints of a cerebral nature was observed in Group 2 patients with MT-MCI compared to Group 1 patients with AT-MCI. It should be noted that memory impairment occurred in all patients in Group 1. The clinical-neurological examination revealed that the subjective complaints of a cerebral nature occurred significantly more frequently in Group 2 patients compared with Group 1 patients, except for the memory disorders, which prevailed in Group 1 patients with AT-MCI. With regard to the objective symptoms, the focal neurological symptoms occurred with equal frequency in both groups. It should be noted that the symptoms of the carotid region were more frequent in Group 2 patients and the symptoms of the vertebrobasilar region in Group 1 patients. The parameters of the cognitive function related to the concentration and stability of attention were less disturbed in patients with AT-MCI compared with the patients having the MT-MCI, and those parameters correlated with the parameters of the neurological focal symptoms.
Uribe-Bojanini, Esteban; Hernandez-Quiceno, Sara; Cock-Rada, Alicia Mar?a
Several genetic disorders caused by defective nucleotide excision repair that affect the skin and the nervous system have been described, including Xeroderma Pigmentosum (XP), De Sanctis–Cacchione syndrome (DSC), Cockayne syndrome, and Trichothiodystrophy. Cutaneous photosensitivity with an increased risk of skin malignancy is a common feature of these disorders, but clinical manifestations commonly overlap these syndromes. Several genes have been found to be altered in these pathologies, but...
Seyed Reza Najafizadeh; Hazhir Saberi; Mohammad Hossein Harirchian; Seyed Ahad Hashemi
Background: Central nervous system (CNS) involvement has been observed in 14-80% of patients with systemic lupus erythematosus (SLE). Magnetic resonance imaging (MRI) is an appropriate method for evaluating CNS involvement in these patients. Clinical manifestations and MRI findings of CNS lupus should be differentiated from other mimicking diseases such as multiple sclerosis (MS).Objectives: The aim of this study was to evaluate the prevalence and extent of brain and cervical cord MRI lesions...
Full Text Available We report a case of isolated homonymous hemianopsia due to presumptive cerebral tubercular abscess as the initial manifestation of human immunodeficiency virus (HIV infection. A 30-year-old man presented to our outpatient department with sudden loss of visibility in his left visual field. He had no other systemic symptoms. Perimetry showed left-sided incongruous homonymous hemianopsia denser above the horizontal meridian. Magnetic resonance imaging revealed irregular well-marginated lobulated lesions right temporo-occipital cerebral hemisphere and left high fronto-parietal cerebral hemisphere suggestive of brain tubercular abscess. Serological tests for HIV were reactive, and the patient was started only on anti-tubercular drugs with the presumptive diagnosis of cerebral tubercular abscess. Therapeutic response confirmed the diagnosis. Atypical ophthalmic manifestations may be the initial presenting feature in patients with HIV infection. This highlights the need for increased index of suspicion for HIV infection in young patients with atypical ophthalmic manifestations.
Full Text Available We analysed sera and cerebrospinal fluid (CSF of 20 cases of human T-cell lymphotropic virus type (HTLV-I associated myelopathy/tropical spastic paraparesis (HAM/TSP from Brazil and Iran and as controls, 16 Brazilian HTLVI seronegative individuals afflicted with other neurological diseases. It was observed in the HAM/TSP patients that: 1 all had an inflammatory reaction within the central nervous system (CNS; 2 95% (19/20 showed oligoclonal bands reflecting intrathecal IgG synthesis; 3 85% (17/20 presented a local synthesis of HTLVI antibodies; 4 35% (7/20 had a measurable immunoglobulin (Ig synthesis within the CNS. The CSF parameters of the HAM/TSP were compared with the clinical data (age at onset, duration of disease and disability level. Our data prove that CSF analysis is important for the diagnosis of HAM/TSP. There is no association between the severity of the disease and CSF findings.
Neurological manifestations of autosomal dominant familial Alzheimer's disease: a comparison of the published literature with the Dominantly Inherited Alzheimer Network observational study (DIAN-OBS).
Tang, Mengxuan; Ryman, Davis C; McDade, Eric; Jasielec, Mateusz S; Buckles, Virginia D; Cairns, Nigel J; Fagan, Anne M; Goate, Alison; Marcus, Daniel S; Xiong, Chengjie; Allegri, Ricardo F; Chhatwal, Jasmeer P; Danek, Adrian; Farlow, Martin R; Fox, Nick C; Ghetti, Bernardino; Graff-Radford, Neill R; Laske, Christopher; Martins, Ralph N; Masters, Colin L; Mayeux, Richard P; Ringman, John M; Rossor, Martin N; Salloway, Stephen P; Schofield, Peter R; Morris, John C; Bateman, Randall J
Autosomal dominant familial Alzheimer's disease (ADAD) is a rare disorder with non-amnestic neurological symptoms in some clinical presentations. We aimed to compile and compare data from symptomatic participants in the Dominantly Inherited Alzheimer Network observational study (DIAN-OBS) with those reported in the literature to estimate the prevalences of non-amnestic neurological symptoms in participants with ADAD. We prospectively collected data from the DIAN-OBS database, which recruited participants from study centres in the USA, Europe, and Australia, between Feb 29, 2008, and July 1, 2014. We also did a systematic review of publications to extract individual-level clinical data for symptomatic participants with ADAD. We used data for age of onset (from first report of cognitive decline), disease course from onset to death, and the presence of 13 neurological findings that have been reported in association with ADAD. Using multivariable linear regression, we investigated the prevalences of various non-amnestic neurological symptoms and the contributions of age of onset and specific mutation type on symptoms. The DIAN-OBS dataset included 107 individuals with detailed clinical data (forming the DIAN-OBS cohort). Our systematic review yielded 188 publications reporting on 1228 symptomatic individuals, with detailed neurological examination descriptions available for 753 individuals (forming the published data cohort). The most prevalent non-amnestic cognitive manifestations in participants in the DIAN-OBS cohort were those typical of mild to moderate Alzheimer's disease, including visual agnosia (55·1%, 95% CI 45·7-64·6), aphasia (57·9%, 48·6-67·3), and behavioural changes (61·7%, 51·5-70·0). Non-amnestic cognitive manifestations were less prevalent in the published data cohort (eg, visual agnosia [5·6%, 3·9-7·2], aphasia [23·0%, 20·0-26·0], and behavioural changes [31·7%, 28·4-35·1]). Prevalence of non-cognitive neurological manifestations in
Puschner, Birgit; Pratt, Chap; Tor, Elizabeth R
To describe the clinical features, treatment, diagnostic work-up, and outcome of a dog with acute neurologic signs subsequent to algal toxin exposure. A Golden Retriever was presented for evaluation of acute onset of paraparesis after swimming in a man-made pond in early June and ingesting algae from a nearby bucket. The dog was anxious, had mild ptyalism, and when excited, developed generalized self-limiting tremors that progressed to generalized fasciculations and lateral recumbency. The dog was treated with activated charcoal and crystalloid fluids. Two hours after the presentation, the dog acutely decompensated and was ultimately euthanized. Gastric contents, bucket contents, pond water, bile, and urine were positive for anatoxin-a. Anatoxin-a intoxication is rarely confirmed in dogs but should be considered as a differential diagnosis in any dog with acute neurologic signs. We report the first successful detection of anatoxin-a in urine and bile of a dog exposed to blue green algae. This new test provides an enhanced diagnostic tool in suspect cases and has possible therapeutic implications in dogs. © Veterinary Emergency and Critical Care Society 2010.
Shimoyama, Harunari; Nakashima, Chikako; Hase, Midori; Sei, Yoshihiro
A 41-year-old man visited our dermatology clinic because an eruption, which was resistant to steroid ointment treatment, had appeared on his right forearm. An oval, soybean-sized erythematous infiltrated lesion with scales and crusts was located in the central part of the extensor surface of the right forearm and showed partial erosion with attached yellow crusts. The lesion had an impetigo-like appearance. Fungal elements were confirmed from the scales by KOH examination and the fungus was identified as Trichophyton tonsurans by fungal culture and molecular method. Clinical features of T. tonsurans infection vary, wherein some patients have strong inflammatory manifestations, while others remain as asymptomatic carriers. Especially at the early stage of the infection, diagnosis is difficult because it is often misdiagnosed as eczema. We report a case of T. tonsurans infection that had impetigo-like appearance. We also studied the mechanism of the disease.
Maria Lúcia Brito Ferreira
Full Text Available Pelo aumento de casos de encefalite e meningoencefalite em pacientes com dengue, diagnosticados em hospital público referência estadual em Neurologia, adotou-se levantamento sistemático. OBJETIVO: Apresentar 41 casos com manifestações neurológicas do dengue comparando-os à literatura. MÉTODO: Realizou-se estudo descritivo, retrospectivo, entre março e julho de 1997, e prospectivo, de fevereiro a maio de 2002. Foram analisados regiões acometidas e diagnósticos em 41 pacientes. RESULTADOS: As regiões acometidas foram: encefálica (5/7 casos de 1997 - 71,4% e 20/34 casos de 2002 - 58,8%, medular (2/34 de 2002 - 5,9%, de nervos periféricos (2/7 casos de 1997 - 28,6% e 12/34 de 2002 - 35,3%. Não houve acometimento meníngeo. Quanto à topografia, foram diagnosticados acometimentos encefálicos e de nervos periféricos em ambos os períodos, e, apenas em 2002, acometimento medular. Dos 14 diagnósticos, acidente vascular cerebral hemorrágico e encefalomielite aguda disseminada não constam da literatura consultada, além da acentuação de espasmo hemifacial como possível manifestação do dengue. CONCLUSÃO: Esta é a terceira casuística em dengue e sistema nervoso. Foram firmados três diagnósticos, ainda não relatados internacionalmente.The increase of encephalitis and meningoencephalitis in patients with dengue, diagnosed at a public hospital, Neurology State reference, we adopted systematic data collection. OBJECTIVE: The objective was to present 41 cases of neurological manifestations of dengue and to compare data with literature. METHOD: This is a descriptive study, retrospective from March to July 1997 and, prospective, from February to May 2002, analyzing damaged neurological regions and diagnostic of 41 patients. RESULTS: Involved regions were brain (5/7 cases - 71.4%, in 1997, and 20/34 cases - 58.8%, in 2002, spinal cord (2/34 cases - 5.9% in 2002 and peripheral nerves (2/7 cases - 28.6% in 1997 and 12/34 cases - 35
Saito, Yoshiaki; Komaki, Hirofumi; Hattori, Ayako; Takeuchi, Fumi; Sasaki, Masayuki; Kawabata, Ken; Mitsuhashi, Satomi; Tominaga, Kayo; Hayashi, Yukiko K; Nowak, Kristen J; Laing, Nigel G; Nonaka, Ikuya; Nishino, Ichizo
We examined three patients with a severe infantile type of congenital myopathy due to dominant, missense ACTA1 mutations. In addition to muscle weakness, all three patients showed developmental delay in word comprehension during early childhood. All also showed frontal lobe hypoplasia and lateral ventricular dilatation. One patient in addition exhibited features of multiple congenital malformations including skeletal dysplasia, hepatomegaly and urinary tract stenosis. These findings may suggest a link between extramuscular expression of α-skeletal muscle actin and clinical symptoms in non-skeletal muscle tissues of patients with ACTA1 mutations, and probably a functional role of α-skeletal muscle actin during fetal development. Copyright © 2011 Elsevier B.V. All rights reserved.
Ruffenach, L; Gouzou, S; Liverneaux, P; Bruant Rodier, C; Bodin, F
Autologous fat grafting allows the correction of many volume defects whether natural or post-traumatic. In hand surgery, the most common indication is the rejuvenation of the dorsal aspect of the hands. We present, here, an original case of amyotrophic hands lipofilling due to Charcot-Marie-Tooth disease. The patient had a bilateral and asymmetric amyotrophy of the intermetacarpal spaces responsible of a social handicap. Autologous fat grafting, according to Coleman's procedure, was done at the dorsal aspect of the two hands, three years apart. The adipocyte cells were taken on the medial side of the thighs, knees and on the abdomen. Five and eight years after the procedure, the results were evaluated with satisfactory results for the patient and the surgeon. Autologous fat grafting allowed the filling of the intermetacarpal spaces which last over time. The satisfaction rate was high in the patient and the surgeon. Autologous fat cells give an aesthetic correction of neurological amyotrophic hands. Copyright © 2017 Elsevier Masson SAS. All rights reserved.
Dağdeviren Çakır, Aydilek; Turan, Hande; Aykut, Ayça; Durmaz, Asude; Ercan, Oya; Evliyaoğlu, Olcay
(VHL) disease is an autosomal dominantly inherited disorder characterized by hemangioblastomas of retina and central nervous system (CNS); renal cysts, clear cell carcinoma; PCC; endolymphatic sac tumors; cystadenomas of the epididymis in males, broad ligament of uterus in females; pancreatic cysts, cystadenomas and neuroendocrine tumors. We here report two cases of VHL disease presented with PCC as the first manifestation. Hemangioblastoma of CNS in the first case and PNET in the second case developed during follow- up and led to the diagnosis of VHL disease. Genetic analyses of cases revealed p.Arg161Gln (c.482G>A) and p.Leu129Pro(c.386T>G) heterozygous missense mutation in VHL gene, respectively. In children, PCC may be the only and/or initial manifestation of the disease with delayed manifestations of the syndrome in other organs. PNET is a very rare manifestation of VHL disease. To best of our knowledge, this is the second case in literature, presenting with combination of PNET and bilateral PCC as components of childhood VHL disease. Pediatric patients diagnosed with PCC should be investigated for the genetic causes especially for VHL.
NMDARs (eNMDARs) and protect synaptic integrity. Furthermore, our phosphoproteomic analysis of eNMDAR-mediated signaling has identified upregulation of...manifestations include intellectual disabilities, neurobehavioral abnormalities such as autism, and epilepsy . Currently, there is no cure for TSC. The...aspartate-type glutamate receptor (NMDAR). Interestingly, Memantine is also in clinical trials for children with epilepsy , intellectual disabilities
Tapia, Crisanta I; Khalaf, Kristin; Berenson, Karina; Globe, Denise; Chancellor, Michael; Carr, Lesley K
Patients with neurologic diseases often have neurogenic detrusor overactivity (NDO), which can result in a loss of voluntary bladder control and uncontrollable urinary incontinence (UI).The impact of UI due to NDO on patients' lives has not been well studied. The objective of this review was to assess the health-related quality of life (HRQoL) and economic burden in patients with urgency UI due to NDO in select countries in North America, the European Union, Asia, and Australia. Systematic literature searches and reviews of articles published in English (January 2000 to February 2011) were conducted using MEDLINE®, EMBASE®, and the Cochrane Library. Studies assessing the impact of UI on HRQoL of patients with an underlying neurologic condition of interest (i.e., multiple sclerosis, spinal cord injury, Parkinson's disease, stroke, or spina bifida) were included. Economic studies in urgency UI also were included. Of 876 citations generated in the initial search, a total of 27 articles were deemed relevant: 16 articles presented HRQoL data and 11 articles presented information on the economic burden of UI. Humanistic studies used a range of HRQoL instruments to measure HRQoL burden, and the economic studies included different cost components to quantify the economic burden, making meaningful comparisons challenging. Despite this heterogeneity, the literature suggests that HRQoL in patients with UI due to NDO is worse than patients with UI in general or those with the same underlying neurologic condition without UI. In addition, urgency UI also results in substantial economic costs. Incontinent patients with underlying neurologic conditions have impaired HRQoL as well as substantial economic burden attributable to UI due to NDO. There is a need for urgency UI treatments that improve HRQoL of these patients and alleviate the economic burden of this condition.
Background Patients with neurologic diseases often have neurogenic detrusor overactivity (NDO), which can result in a loss of voluntary bladder control and uncontrollable urinary incontinence (UI).The impact of UI due to NDO on patients’ lives has not been well studied. The objective of this review was to assess the health-related quality of life (HRQoL) and economic burden in patients with urgency UI due to NDO in select countries in North America, the European Union, Asia, and Australia. Methods Systematic literature searches and reviews of articles published in English (January 2000 to February 2011) were conducted using MEDLINE®, EMBASE®, and the Cochrane Library. Studies assessing the impact of UI on HRQoL of patients with an underlying neurologic condition of interest (i.e., multiple sclerosis, spinal cord injury, Parkinson’s disease, stroke, or spina bifida) were included. Economic studies in urgency UI also were included. Results Of 876 citations generated in the initial search, a total of 27 articles were deemed relevant: 16 articles presented HRQoL data and 11 articles presented information on the economic burden of UI. Humanistic studies used a range of HRQoL instruments to measure HRQoL burden, and the economic studies included different cost components to quantify the economic burden, making meaningful comparisons challenging. Despite this heterogeneity, the literature suggests that HRQoL in patients with UI due to NDO is worse than patients with UI in general or those with the same underlying neurologic condition without UI. In addition, urgency UI also results in substantial economic costs. Conclusions Incontinent patients with underlying neurologic conditions have impaired HRQoL as well as substantial economic burden attributable to UI due to NDO. There is a need for urgency UI treatments that improve HRQoL of these patients and alleviate the economic burden of this condition. PMID:23369111
Liwocha, Małgorzata; Galus, Krzysztof; Kozak-Szkopek, Elzbieta; Kowal, Roman
THE AIM OF THE STUDY was evaluation effects of rehabilitation in patients with consequences of neurological diseases. The study was conducted in the hospital department of rehabilitation. The study involved group of 30 patients consisting of 12 men aged from 48 to 76 years (mean age 64.3 +/- 7.9), and 18 women aged from 45 to 82 years (mean age 65.4 +/- 13,2). These were patients mostly after stroke, multiple sclerosis or Parkinson's disease enrolled in rehabilitation. TESTS were performed before and after 21 days rehabilitation, using the following scales and ratings: Activities of Daily Living (ADL), Instrumental Activities of Daily Living Scale (IADL), Scale Barthel, test Tinetti, Expanded "Get-Up-and-Go" Test (ETGUG) and Geriatric Depression Scale (GDS). RESULTS. In men, the average number of points in the scale of ADL, IADL, Barthel increased statistically significant, the results obtained in the test Tinetti, ETGUG, GSD were changed not significant statistically. In women, the average number of points in the IADL scale, Barthel scale and GDS increased, and the test ETGUG were reduced, all statistically significant. The results of the scale ADL and test Tinetti, improved statistically not significant. CONCLUSIONS. The physical rehabilitation in patients with neurological consequences had a positive impact on the ability to perform simple and complex activities of daily living, physical and mental condition. ADL, IADL, Barthel scale, GDS, test Tinetti and ETGUG can be used for evaluation of rehabilitation results.
Full Text Available Hypoparathyroidism is most commonly isolated and idiopathic. Hypoparathyroidism in autoimmune polyendocrine syndrome type-2 (APS-2 is extremely rare with few isolated reports and usually presents late. We present perhaps for the first time, hypoparathyroidism being the initial manifestation of APS-2 which was diagnosed in a 32 year lady with tingling of the extremities along with hypocalcemic tetany for 15 years. She also had generalized acral and mucosal hyperpigmentation of 2 years duration. Investigations were significant for low calcium (7.1mg/dl, elevated phosphorus (4.8mg/dl, vitamin-D insufficiency (27.4ng/ml, low intact parathyroid hormone (2.3pg/ml, low basal (4.7mcg/dl and stimulated serum cortisol (9.2mcg/dl at half hour, 11.4mcg/dl at 1 hour post synacthen, elevated triiodothyronine (2.1ng/ml and free tetraiodothyronine (2.4ng/dl along with suppressed TSH (<0.005 U/L. Imaging relevaled mild cerebellar calcification, normal adrenals with diffuse uptake of Tc 99 - pertechnate. A diagnosis of hypoparathyroidism with Addison′s and Graves′ disease as a part of APS-2 was made. This report intends to highlight the clinical heterogeneity and varied presentation of APS-2. Routine screening of other hormonal function (especially adrenal function, thyroid function and blood glucose in a patient with diagnosed hypoparathyroidism is advisable to decrease the chances of missing APS-2.
Full Text Available Background: The essential features of Posterior Reversible Encephalopathy Syndrome (PRES are headache, mental changes, seizures, visual symptoms and often arterial hypertension. Brain RMN typically shows cortico-sottocortical parieto-occipital edema, with a bilateral and symmetric distribution. PRES develops in clinical conditions as hypertensive encephalopathy, preeclampsia/ eclampsia, autoimmune diseases, after transplantation, infections and as an adverse effect of immunosuppressive drugs or chemotherapy. It usually completely reverses with treatment, although permanent sequelae are possible in case of delayed or missed diagnosis. Case report: We describe the case of a transsexual (M!F and tetraplegic patient, admitted for neck and low back pain. She suddenly developed headache, confusion, seizures and severe hypertension with normal blood tests. RMN showed multiple cortico-sottocortical areas of vasogenic and citotoxic edema in temporo-occipital, parietal, frontal, and cerebellar regions. Soon after the beginning of the antihypertensive therapy, clinical recovery was observed, as well as the disappearance of edema at RMN. Discussion and conclusions: Although PRES is usually associated with definite pathological conditions, it is not always the case, as was for the patient here described, who had no predisposing factors in her past clinical history, and presented hypertension only in the acute phase of the syndrome. Since, moreover, PRES usually presents with acute non specific features and it can be misdiagnosed with other serious diseases, the clinician will be helped by the knowledge of this syndrome to promptly start diagnostic workup and treatments, and avoid permanent neurological deficits.
Hernández-Cortés, P; Caba, M; Gómez-Sánchez, R; Gómez-Morales, M
The authors report an unusual case of flexor tenosynovitis, severe carpal tunnel syndrome, and triggering at the carpal tunnel as the first manifestation of gout. A 69-year-old man presented with digital flexion contracture and severe carpal tunnel syndrome of his right hand and was treated surgically. A flexor tenosynovectomy and a median nerve neurolysis were performed through an extended carpal tunnel approach. The sublimis and the profundus tendons were involved. Partial ruptures and multiple whitish lesions suggestive of tophacceous infiltration of the flexor tendons were seen. Macroscopically, the removed synovial tissue was involved by multiple whitish nodules that were milimetric in size and was suggestive of monosodium urate crystals deposits. By light microscopy examination, numerous nonnecrotizing granulomas of different sizes were observed that were compounded by large aggregations of acellular nonpolarized material, surrounded by epithelioid histiocytes, mononuclear cells, and foreign body multinucleated giant cells. Postoperatively, the patient recovered with resolution of the median nerve symptoms and a near-to-full range of motion of the affected digits.To the authors' knowledge, this patient is the first case report with flexor tendons tophacceous infiltration as the first clinical sign of gout. Gouty flexor tenosynovitis can occur in the absence of a long history of gout. A high index of suspicion is paramount to the initiation of proper management. Operative treatment of gouty flexor tenosynovitis is mandatory to debulk tophaceous deposits, improve tendon gliding, and decompress nerves. Routine uric acid determination could be helpful in the preoperative evaluation of patients with flexor tenosynovitis. Copyright 2011, SLACK Incorporated.
Kanukollu Venkata Madusudana Rao
Full Text Available BACKGROUND This prospective study aimed to evaluate the incidence of ocular manifestations in head injury and their correlation with the intracranial lesions. MATERIALS AND METHODS A total of 108 consecutive cases of closed head injury admitted in the neurosurgical ward of a tertiary teaching hospital underwent a thorough ophthalmic assessment. Clinical examination, radiological imaging and Glasgow Coma Scale (GCS were applied to grade the severity of injury. RESULTS Total number of 108 patients of head injury were examined of which 38 patients had ocular manifestations (35.18%. Of these, 85.18% were males, 84% of injuries were due to road traffic accidents and 16% were due to fall from a height. The ocular manifestations were as follows- Orbital complications were seen in 6 patients (15.8%. Anterior segment manifestations included black eyes seen in 10 patients (26.3%, subconjunctival haemorrhage in 10.5% of patients (4 patients, corneal involvement in 21% of patients (8 patients and pupillary involvement in 50% of patients (19 patients. Posterior segment manifestations were seen in 26.3% of patients (10 patients and were as follows- Purtscher’s retinopathy in 2 patients and optic atrophy in 5 patients. Cranial nerve palsies were seen in 15 patients (39.47% and supranuclear movement disorders were seen in 3 patients (8%. CONCLUSION Even though, neurosurgeons perform comprehensive clinical examination including eye examination, the main purpose is limited to aid topical diagnosis of neurological lesions. This study emphasises the importance of a detailed eye examination by an ophthalmologist to prevent irreversible visual loss in addition to aiding in the neurological diagnosis. Pupillary involvement, papilloedema and ocular motor paresis pointed to a more severe head injury. This observational prospective study helped us to correlate the severity of head injuries in association with ocular findings in patients admitted in neurosurgical ward
Full Text Available Abstract Introduction Bronchial carcinomas in childhood and adolescence are extremely rare; only individual cases have been reported previously. Case presentation We report on a 16-year-old Caucasian German boy with non-small cell lung carcinoma (squamous cell non-small cell lung carcinoma stage IV, T4N2M1, without epidermal growth factor receptor overexpression and/or mutation or k-ras mutation. He presented with paraplegia due to spinal metastases of the bronchial carcinoma. No familial predisposition or toxin exposure was identified. Treatment following adult protocols consisted of surgical intervention for spinal metastases, first-line cisplatinum and gemcitabine, irradiation and second-line docetaxel. After a transient response our patient experienced disease progression and died about 10 months later. Conclusion Response and survival in our 16-year-old patient were similar to adult patients with stage IV non-small cell lung carcinoma.
Massey, E Wayne; Moon, Richard E
Diving exposes a person to the combined effects of increased ambient pressure and immersion. The reduction in pressure when surfacing can precipitate decompression sickness (DCS), caused by bubble formation within tissues due to inert gas supersaturation. Arterial gas embolism (AGE) can also occur due to pulmonary barotrauma as a result of breath holding during ascent or gas trapping due to disease, causing lung hyperexpansion, rupture and direct entry of alveolar gas into the blood. Bubble disease due to either DCS or AGE is collectively known as decompression illness. Tissue and intravascular bubbles can induce a cascade of events resulting in CNS injury. Manifestations of decompression illness can vary in severity, from mild (paresthesias, joint pains, fatigue) to severe (vertigo, hearing loss, paraplegia, quadriplegia). Particularly as these conditions are uncommon, early recognition is essential to provide appropriate management, consisting of first aid oxygen, targeted fluid resuscitation and hyperbaric oxygen, which is the definitive treatment. Less common neurologic conditions that do not require hyperbaric oxygen include rupture of a labyrinthine window due to inadequate equalization of middle ear pressure during descent, which can precipitate vertigo and hearing loss. Sinus and middle ear overpressurization during ascent can compress the trigeminal and facial nerves respectively, causing temporary facial hypesthesia and lower motor neuron facial weakness. Some conditions preclude safe diving, such as seizure disorders, since a convulsion underwater is likely to be fatal. Preventive measures to reduce neurologic complications of diving include exclusion of individuals with specific medical conditions and safe diving procedures, particularly related to descent and ascent. © 2014 Elsevier B.V. All rights reserved.
Silva, Adriana C; de Oliveira Ribeiro, Natalia P; de Mello Schier, Alexandre R; Arias-Carrión, Oscar; Paes, Flavia; Nardi, Antonio E; Machado, Sergio; Pessoa, Tamires M
Despite grief being a universal experience and the increased scientific attention paid to grief and bereavement in recent years, studies that seek to better understand the role of the neurological aspects of grief are still scarce. We found 5 studies that discussed the relationship between the neurological aspects of grief due to the death of a loved one. All studies showed an activation of common areas, i.e., the anterior cingulate cortex (ACC), posterior cingulate cortex (PCC), prefrontal cortex (PFC), insula and amygdala. These findings could indicate that there is a group of areas working together and responding to generate the symptomatology of grief. Because grief is a universal experience, it is essential that the necessary and effective support can be provided to those who experience the loss of someone considered important in their lives, and this requires understanding grief's manifestation, its differential diagnosis in reference to other clinical conditions, mainly psychiatric ones, and adequate forms of intervention and treatment when necessary. Proper understanding and support can help prevent the emergence of more serious health problems.
Friedreich’s ataxia (FRDA is the most common hereditary ataxia in the caucasian population and is characterized by a mixed spinocerebellar and sensory ataxia, hypertrophic cardiomyopathy and increased incidence of diabetes. FRDA is caused by impaired expression of the FXN gene coding for the mitochondrial protein frataxin. During the past ten years, the development of mouse models of FRDA has allowed better understanding of the pathophysiology of the disease. Among the mouse models of FRDA, the liver conditional mouse model pointed to a tumor suppressor activity of frataxin leading to the hypothesis that individuals with FRDA might be predisposed to cancer. In the present work, we investigated the presence and the incidence of neoplasia in the largest FRDA patient cohorts from the USA, Australia and Europe. As no predisposition to cancer could be observed in both cohorts, we revisited the phenotype of the liver conditional mouse model. Our results show that frataxin-deficient livers developed early mitochondriopathy, iron-sulfur cluster deficits and intramitochondrial dense deposits, classical hallmarks observed in frataxin-deficient tissues and cells. With age, a minority of mice developed structures similar to the ones previously associated with tumor formation. However, these peripheral structures contained dying, frataxin-deficient hepatocytes, whereas the inner liver structure was composed of a pool of frataxin-positive cells, due to inefficient Cre-mediated recombination of the Fxn gene, that contributed to regeneration of a functional liver. Together, our data demonstrate that frataxin deficiency and tumorigenesis are not associated.
Sanjay K Mahajan
Full Text Available Scrub typhus is caused by Orientia tsutsugamushi characterized by focal or disseminated vasculitis and perivasculitis which may involve the lungs, heart, liver, spleen and central nervous system. It was thought to have been eradicated from India. Recently it is being reported from many areas of India. The clinical picture and severity of the symptoms varies widely. The neurological manifestations of scrub typhus are not uncommon but are diverse. Meningoencephalitis is classical manifestation of scrub typhus but cerebellitis, cranial nerve palsies, plexopathy, transverse myelitis, neuroleptic malignant syndrome and Guillan-Barré syndrome are other manifestations reported in literature. The availability of literature on the neurological manifestations of scrub typhus is limited to case reports mainly. This article reviews various neurological manifestations of scrub typhus reported in literature.
N. V. Vakhnina
Full Text Available Hypertension is one of the most common vascular diseases. The brain as target organs in hypertension is damaged more often and earlier. Neurological complications due to hypertension are frequently hyperdiagnosed in Russian neurological practice. Thus, headache, dizziness, impaired recall of recent events, nocturnal sleep disorders, and many other complaints in a hypertensive patient are usually regarded as a manifestation of dyscirculatory encephalopathy. At the same time headaches (tension headache and migraine in hypertensive patients are predominantly primary; headache associated with dramatic marked elevations in blood pressure is encountered in only a small number of patients. The role of cerebrovascular diseases in the development of dizziness in hypertensive patients is also overestimated. The vast majority of cases, patients with this complaint are in fact identified to have benign paroxysmal postural vertigo, Mеniеre’s disease, vestibular neuronitis, or vestibular migraine. Psychogenic disorders or multisensory insufficiency are generally responsible for non-systemic vertigo in hypertensive patients. Chronic cerebral circulatory insufficiency may cause non-systemic vertigo as a subjective equivalent of postural instability.Cognitive impairments (CIs are the most common and earliest manifestation of cerebrovascular lesion in hypertension. In most cases, CIs in hypertension were vascular and associated with cerebrovascular lesion due to lacunar infarcts and leukoaraiosis. However, mixed CIs frequently occur when hypertensive patients are also found to have signs of a degenerative disease, most commonly in Alzheimer’s disease.
Maayken Elizabeth Louise van den Berg
Full Text Available Purpose. Understanding the presentation of spinal cord injury (SCI due to tumours considering population distribution and temporal trends is key to managing SCI health services. This study quantified incidence rates, function scores, and trends of SCI due to tumour or metastasis over an 18-year time period in a defined region in Spain. Methods. A retrospective cohort study included in-and outpatients with nontraumatic SCI due to tumour or metastasis admitted to a metropolitan hospital in Spain between 1991 and 2008. Main outcome measures were crude and age- and sex-adjusted incidence rates, tumour location and type, distribution by spinal level, neurological level of injury, and impairment ASIA scores. Results. Primary tumour or metastasis accounted for 32.5% of nontraumatic SCI with an incidence rate of 4.1 per million population. Increasing rates with age and over time were observed. Major pathology groups were intradural-extramedullary masses from which meningiomas and neurinomas accounted for 40%. Lesions were mostly incomplete with predominant ASIA Grade D. Conclusions. Increasing incidence rates of tumour-related SCI over time in the middle-aged and the elderly suggest a growing need for neurooncology health resources in the future.
The human immunodeficiency virus type 2 (HIV-2) produces a similar spectrum of illness as HIV-1, including. AIDS, and is clinically indistinguishable. There is evidence that it is less pathogenic, with a longer natural history. HIV-. 2 infection is endemic in West Africa, especially in the former Portuguese and French colonies.
Historically, diphtheria was a major cause of morbidity and mortality in the prevaccine era. However, in recent times there has been a resurgence of diphtheria, especially in the newly independent states of the former USSR. Diphtheritic polyneuropathy can be a serious complication in patients who have a severe infection. In patients with pertussis, seizures and encephalopathy can occur as a complication of asphyxia. Vaccination against diphtheria and pertussis in children and booster vaccination in adults is recommended. DTP (diphtheria, tetanus, pertussis) vaccination has been shown to increase the risk of febrile seizures in children. Currently, it appears that the risk of vaccine-induced encephalopathy and/or epilepsy following DTP vaccination, if any, is extremely low. © 2014 Elsevier B.V. All rights reserved.
This review describes the clinical features of hepatic encephalopathy (HE) associated with acute liver failure or liver cirrhosis, diagnostic approaches, and current therapeutic concepts. A striking feature of HE is the reversibility of symptoms. In addition, it gives a short overview of current knowledge about hepatitis C virus ...
Full Text Available Aim: To assess the neurological complications of chickenpox with prognosis. Background: The neurological complications occur in 0.03% of persons who get chickenpox. There is no universal vaccination against chicken pox in India. Most patients prefer alternate modalities of treatment. Hence these complications of chickenpox are likely to continue to occur. Study Design: A prospective study was conducted for 2 years (from March 2002 on the admitted cases with neurological complications after chickenpox (with rash or scar. Patients were investigated with CT/MRI, CSF study, EEG and nerve conduction studies and hematological workup. They were followed-up for 1 year and outcome assessed using modified Rankin scale. Results: The latency for the neurological complications was 4-32 days (mean: 16.32 days. There were 18 cases: 10 adults (64% and 8 children (36%. Cerebellar ataxia (normal CT/MRI was observed in 7 cases (32% (mean age: 6.85 years. One patient (6 years had acute right hemiparesis in the fifth week due to left capsular infarct. All these cases spontaneously recovered by 4 weeks. The age range of the adult patients was 13-47 years (mean: 27 years. The manifestations included cerebellar and pyramidal signs (n-4 with features of demyelination in MRI who recovered spontaneously or with methylprednisolone by 8 weeks. Patient with encephalitis recovered in 2 weeks with acyclovir. Guillain Barre syndrome of the demyelinating type (n-2 was treated with Intravenous immunoglobulin (IVIG and they had a slow recovery by a modified Rankin scale (mRs score of 3 and 2 at 6 months and 1 year, respectively. One case died after hemorrhage into the occipital infarct. There were two cases of asymmetrical neuropathy, one each of the seventh cranial and brachial neuritis. Conclusion: Spontaneous recovery occurs in post-chickenpox cerebellar ataxia. Rarely, serious complications can occur in adults. The demyelinating disorders, either of the central or peripheral
... Us Website References Search Patients / What is VHL? / Manifestations People who have VHL disease may experience tumors ... very important to check regularly for possible VHL manifestations throughout a person’s lifetime. Most of these VHL ...
Faghihi, Mohammad Ali; Mottagui-Tabar, Salim; Wahlestedt, Claes
Neurological diseases are defined as an inappropriate function of the peripheral or central nervous system due to impaired electrical impulses throughout the brain and/or nervous system that may present with heterogeneous symptoms according to the parts of the system involved in these pathologic processes. Growing evidence on genetic components of neurological disease have been collected during recent years. Genetic studies have opened the way for understanding the underlying pathology of many neurological disorders. The outcome of current intense research into the genetics of neurological disorders will hopefully be the introduction of new diagnostic tools and the discovery of potential targets for new and more effective medications and preventive measures.
The Neurological Compromised Spine Due to Ewing Sarcoma. What First: Surgery or Chemotherapy? Therapy, Survival, and Neurological Outcome of 15 Cases With Primary Ewing Sarcoma of the Vertebral Column.
Mirzaei, Lida; Kaal, Suzanne E J; Schreuder, Hendrik W B; Bartels, Ronald H M A
The vertebral column is an infrequent site of primary involvement in Ewing sarcoma. Yet when Ewing sarcoma is found in the spine, the urge for decompression is high because of the often symptomatic compression of neural structures. It is unclear in alleviating a neurological deficit whether chemotherapy is preferred over decompressive laminectomy. To underline, in this case series, the efficiency of initial chemotherapy before upfront surgery in the setting of high-grade spinal cord or cauda equina compression of primary Ewing sarcoma. Fifteen patients with Ewing sarcoma primarily located in the spine were treated at our institution between 1983 and 2015. Localization, neurological deficit expressed as Frankel grade, and outcome expressed as Rankin scale before and after initial chemotherapy, the recurrence rate, and overall survival were evaluated. The multidisciplinary approach of 1 case will be discussed in detail. Nine patients (60%) were female. The age at presentation was 15.0 ± 5.5 years (range: 0.9-22.8 years). Ten patients (67%) were initially treated with chemotherapy, and 1 patient (7%) was treated primarily with radiotherapy followed by chemotherapy. The remaining 4 patients (27%) were initially treated with decompressive surgery. All patients treated primarily nonsurgically improved neurologically at follow-up, showing the importance of chemotherapy as an effective initial treatment option. Adequate and quick decompression of neural structures with similar results can be achieved by chemotherapy and radiotherapy, avoiding the local spill of malignant cells.
Neurological sleep disorders are common in the general population and may have a strong impact on quality of life. General practitioners play a key role in recognizing and managing sleep disorders in the general population. They should therefore be familiar with the most important neurological sleep disorders. This review provides a comprehensive overview of the most prevalent and important neurological sleep disorders, including Restless legs syndrome (with and without periodic limb movements in sleep), narcolepsy, NREM- and REM-sleep parasomnias and the complex relationship between sleep and epilepsies. Although narcolepsy is considered as a rare disease, recent discoveries in narcolepsy research provided insight in the function of brain circuitries involved in sleep wake regulation. REM sleep behavioral parasomnia (RBD) is increasingly recognized to represent an early manifestation of neurodegenerative disorders, in particular evolving synucleinopathies. Early diagnosis may thus open new perspectives for developing novel treatment options by targeting neuroprotective substances.
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.
Manifestação neurológica na síndrome de Baggio-Yoshinari (síndrome brasileira semelhante à doença de Lyme Neurological manifestations in Baggio-Yoshinari Syndrome (Brazilian Lyme disease-like syndrome
Samuel Katsuyuki Shinjo
multiple systemic clinical manifestations. In Brazil, a different syndrome is described that mimics LD symptoms, but that also manifests high frequencies of recurrent episodes and immune-allergic manifestations. It is transmitted by the Amblyomma cajennense tick and the etiological agent is an uncultivable spirochete with atypical morphology. Due to its particularities, this emerging zoonosis has been called Brazilian LD-like syndrome or Baggio-Yoshinari Syndrome (BYS. OBJECTIVE: To describe the neurological spectrum of BYS. PATIENTS: Thirty patients with neurological symptoms of BYS were analyzed. RESULTS: Mean age of patients was 34.2 ± 13.3 years old (6 to 63 years; 20 were females and 10 males. A high number of recurrent episodes (73.6% and severe psychiatric or psycho-social disturbances (20% were distinguishing features. Erythema migrans similar to those seen in the Northern hemisphere was identified in 43.3% of patients at disease onset. The recurrence of skin lesions diminished as the disease progressed. Articular symptoms (arthritis happened in nearly half of patients at BYS onset and during relapsing episodes. CONCLUSIONS: The BYS is considered a new tick borne disease in Brazil that differs from classical LD observed in the Northern hemisphere. BYS replicates most of the neurological symptoms observed in LD, except for the additional presence of relapsing episodes and the tendency to cause chronic neurological and articular manifestations.
Perlmutter, Joel S.
Parkinson disease (PD) is a progressive neurologic condition that causes motor and non-motor manifestations. Treatment provides symptomatic benefit but no current treatment has been proven to slow disease progression. Research studies of PD require a means of rating the severity of disease by measurement of motor manifestations, assessment of ability to perform daily functional activities, and symptomatic response to medication. The most common rating scales are the Unified Parkinson Disease ...
Conclusions: We identified NPSLE manifestations in 21.7% of patients; headache and CVD were the most frequent neurological manifestations. Continued studies into the pathogenesis of neurological involvement in patients with SLE are warranted.
Hermes, Gretchen; Ajioka, James W; Kelly, Krystyna A; Mui, Ernest; Roberts, Fiona; Kasza, Kristen; Mayr, Thomas; Kirisits, Michael J; Wollmann, Robert; Ferguson, David J P; Roberts, Craig W; Hwang, Jong-Hee; Trendler, Toria; Kennan, Richard P; Suzuki, Yasuhiro; Reardon, Catherine; Hickey, William F; Chen, Lieping; McLeod, Rima
Worldwide, approximately two billion people are chronically infected with Toxoplasma gondii with largely unknown consequences. To better understand long-term effects and pathogenesis of this common, persistent brain infection, mice were infected at a time in human years equivalent to early to mid adulthood and studied 5-12 months later. Appearance, behavior, neurologic function and brain MRIs were studied. Additional analyses of pathogenesis included: correlation of brain weight and neurologic findings; histopathology focusing on brain regions; full genome microarrays; immunohistochemistry characterizing inflammatory cells; determination of presence of tachyzoites and bradyzoites; electron microscopy; and study of markers of inflammation in serum. Histopathology in genetically resistant mice and cytokine and NRAMP knockout mice, effects of inoculation of isolated parasites, and treatment with sulfadiazine or alphaPD1 ligand were studied. Twelve months after infection, a time equivalent to middle to early elderly ages, mice had behavioral and neurological deficits, and brain MRIs showed mild to moderate ventricular dilatation. Lower brain weight correlated with greater magnitude of neurologic abnormalities and inflammation. Full genome microarrays of brains reflected inflammation causing neuronal damage (Gfap), effects on host cell protein processing (ubiquitin ligase), synapse remodeling (Complement 1q), and also increased expression of PD-1L (a ligand that allows persistent LCMV brain infection) and CD 36 (a fatty acid translocase and oxidized LDL receptor that mediates innate immune response to beta amyloid which is associated with pro-inflammation in Alzheimer's disease). Immunostaining detected no inflammation around intra-neuronal cysts, practically no free tachyzoites, and only rare bradyzoites. Nonetheless, there were perivascular, leptomeningeal inflammatory cells, particularly contiguous to the aqueduct of Sylvius and hippocampus, CD4+ and CD8+ T cells
Full Text Available Abstract Background Worldwide, approximately two billion people are chronically infected with Toxoplasma gondii with largely unknown consequences. Methods To better understand long-term effects and pathogenesis of this common, persistent brain infection, mice were infected at a time in human years equivalent to early to mid adulthood and studied 5–12 months later. Appearance, behavior, neurologic function and brain MRIs were studied. Additional analyses of pathogenesis included: correlation of brain weight and neurologic findings; histopathology focusing on brain regions; full genome microarrays; immunohistochemistry characterizing inflammatory cells; determination of presence of tachyzoites and bradyzoites; electron microscopy; and study of markers of inflammation in serum. Histopathology in genetically resistant mice and cytokine and NRAMP knockout mice, effects of inoculation of isolated parasites, and treatment with sulfadiazine or αPD1 ligand were studied. Results Twelve months after infection, a time equivalent to middle to early elderly ages, mice had behavioral and neurological deficits, and brain MRIs showed mild to moderate ventricular dilatation. Lower brain weight correlated with greater magnitude of neurologic abnormalities and inflammation. Full genome microarrays of brains reflected inflammation causing neuronal damage (Gfap, effects on host cell protein processing (ubiquitin ligase, synapse remodeling (Complement 1q, and also increased expression of PD-1L (a ligand that allows persistent LCMV brain infection and CD 36 (a fatty acid translocase and oxidized LDL receptor that mediates innate immune response to beta amyloid which is associated with pro-inflammation in Alzheimer's disease. Immunostaining detected no inflammation around intra-neuronal cysts, practically no free tachyzoites, and only rare bradyzoites. Nonetheless, there were perivascular, leptomeningeal inflammatory cells, particularly contiguous to the aqueduct of
N. N. Zavadenko
Full Text Available Treatment of developmental disorders, correction of learning disabilities and behavioral problems in children should be prompt, complex and include pharmacotherapy with nootropic agents. The results of recent studies shown in this review proved effectiveness of pharmacotherapy with pyritinol in children with perinatal injury of central nervous system and its consequences, psychomotor and speech development delay, dyslexia, attention deficit/hyperactivity disorder, cognitive disorders and learning disabilities (including manifestations of epilepsy, chronic tic disorders and Tourette syndrome. Due to its ability to optimize metabolic processes in central nervous system, pyritinol is used in treatment of vegetative dysfunction in children and adolescents, especially associated with asthenical manifestations, as well as in complex therapy of exertion headache and migraine. The drug is effective in treatment of cognitive disorders in children and adolescents with epilepsy, pyritinol was administered without changing of the basic anticonvulsive therapy and no deterioration (increase of severity of seizures or intensity of epileptiform activity on electroencephalogramms was observed. Significant nootropic effect of pyritinol, including neurometabolic, neuroprotective, neurodynamic and other mechanisms, in association with safety and rare side effects of this drug determines its wide usage in pediatric neurology.
Elnekiedy, Abdelaziz; Naguib, Nagy; Hamed, Waseem; Mekky, Jaidaa; Mamdouh Hassan, Hebatallah Hassan
.... Our purpose was to describe those MRI features linking them to their corresponding neurological manifestations and possible prior location of brain insults based on our own experience compared...
African Journal of Neurological Sciences. 2009 - Vol. 28, No 1. CLINICAL STUDIES / ETUDES CLINIQUES. OPHTHALMIC MANIFESTATIONS IN PATIENTS WITH INTRACRANIAL TUMOURS. MANIFESTATIONS OPHTALMIQUES LORS DE TUMEURS INTRACRÂNIENNES. ONAKPOYA Oluwatoyin Helen 1. KOMOLAFE ...
Deeptara Pathak Thapa
Full Text Available Introduction: Dermatology is a specialty, which not only deals with dermatological problems with outpatient but also inpatients referrals. The importances of Dermatologist in hospital setting are rising due to changing condition of medical care. Since no peer-reviewed articles are available for dermatological problems in a neurological set up, we conducted this study to know about pattern of skin disorders in neurological patients. Material and Methods: The present study was a prospective study in a neurological setup, which included data from hospital dermatology consultation request forms over a period of one year. The data included demographic profile of the patient investigation where needed, neurological diagnosis and final dermatological diagnosis. The data was analyzed using SPSS. Results: A total of 285 patients who were requested for consultation were included in the study. Face was the commonest site of involvement (19.6%. Laboratory examination of referred patients revealed abnormal blood counts in 2% cases, renal function tests in 0.7% and urine in 0.4% cases. CT scan showed abnormal findings in 65.6% patients. The most common drug used in these patients was phenytoin (29.1%. The most common dermatological diagnosis was Infection and Infestation (34.7% followed by eczema (46.6%. Drug rash was seen in 3.9% cases. Out of which one had phenytoin induced Steven Johnson syndrome. Skin biopsy was done in 5 patients. Topicals was advised in 80%. Upon discharge 10% of inpatients didn’t require any follow-up. The patients who were followed up after 4 weeks, about 48% had their symptoms resolved with topicals and oral treatment as required. About 38% required more than two follow ups due to chronic course of the diseases. Conclusions: This present study discussed about various manifestations of skin disorders in a neurological set up and emphasizes the role of dermatologist in treating skin problems both in outpatient as well as inpatient
Full Text Available The difficult types of preeclampsia and eclampsia are presented with the neurological symptoms. The break of cerebral autoregulation mechanism plays the most important role in pathogenesis of cerebral vasospasm. Nevertheless eclampsia isn’t just an ordinary hypertensive encephalopathy because other pathogenic mechanisms are involved in its appearance. The main neuropathologic changes are multifocal vasogenic edema, perivascular multiple microinfarctions and petechial hemorrhages. Neurological clinical manifestations are convulsions, headache, visual disturbances and rarely other discrete focal neurological symptoms. Eclampsia is a high-risk factor for onset of hemorrhagic or ischemic stroke. This is a reason why neurological diagnostic tests are sometimes needed. The method of choice for evaluation of complicated eclampsia is computerized brain topography that shows multiple areas of hypodensity in occipitoparietal regions. These changes are focal vasogenic cerebral edema. For differential diagnosis of eclampsia and stroke other diagnostic methods can be used - fundoscopic exam, magnetic resonance brain imaging, cerebral angiography and cerebrospinal fluid exam. The therapy of eclampsia considers using of magnesium sulfate, antihypertensive, anticonvulsive and antiedematous drugs.
Schor, Nina F
As it is currently configured, completion of child neurology residency requires performance of 12 months of training in adult neurology. Exploration of whether or not this duration of training in adult neurology is appropriate for what child neurology is today must take into account the initial reasons for this requirement and the goals of adult neurology training during child neurology residency.
Mikhail Guryevich Poluektov
Full Text Available Sleep disorders are closely associated with both nervous system diseases and mental disorders; however, such patients prefer to seek just neurological advice. Insomnia is the most common complaint in routine clinical practice. It is characterized by different impairments in sleep and daytime awakening. Obstructive sleep apnea syndrome is less common, but more clinically important because of its negative impact on the cardiovascular and nervous systems. The common neurological disorders are restless legs syndrome and REM sleep behavior disorder, as well as narcolepsy, the major manifestations of which are impaired nocturnal sleep and daytime awakening.
Ronca, Shannon E; Dineley, Kelly T; Paessler, Slobodan
The recent surge in viral clinical cases and associated neurological deficits have reminded us that viral infections can lead to detrimental, long-term effects, termed sequelae, in survivors. Alphaviruses are enveloped, single-stranded positive-sense RNA viruses in the Togaviridae family. Transmission of alphaviruses between and within species occurs mainly via the bite of an infected mosquito bite, giving alphaviruses a place among arboviruses, or arthropod-borne viruses. Alphaviruses are found throughout the world and typically cause arthralgic or encephalitic disease in infected humans. Originally detected in the 1930s, today the major encephalitic viruses include Venezuelan, Western, and Eastern equine encephalitis viruses (VEEV, WEEV, and EEEV, respectively). VEEV, WEEV, and EEEV are endemic to the Americas and are important human pathogens, leading to thousands of human infections each year. Despite awareness of these viruses for nearly 100 years, we possess little mechanistic understanding regarding the complications (sequelae) that emerge after resolution of acute infection. Neurological sequelae are those complications involving damage to the central nervous system that results in cognitive, sensory, or motor deficits that may also manifest as emotional instability and seizures in the most severe cases. This article serves to provide an overview of clinical cases documented in the past century as well as a summary of the reported neurological sequelae due to VEEV, WEEV, and EEEV infection. We conclude with a treatise on the utility of, and practical considerations for animal models applied to the problem of neurological sequelae of viral encephalopathies in order to decipher mechanisms and interventional strategies.
Lucas, Marjolein J; Brouwer, Matthijs C; van de Beek, Diederik
We reported on occurrence and impact of neurological sequelae after bacterial meningitis. We reviewed occurrence of neurological sequelae in children and adults after pneumococcal and meningococcal meningitis. Most frequently reported sequelae are focal neurological deficits, hearing loss, cognitive impairment and epilepsy. Adults with pneumococcal meningitis have the highest risk of developing focal neurological deficits, which are most commonly caused by cerebral infarction, but can also be due to cerebritis, subdural empyema, cerebral abscess or intracerebral bleeding. Focal deficits may improve during clinical course and even after discharge, but a proportion of patients will have persisting focal neurological deficits that often interfere in patient's daily life. Hearing loss occurs in a high proportion of patients with pneumococcal meningitis and has been associated with co-existing otitis. Children and adults recovering from bacterial meningitis without apparent neurological deficits are at risk for long-term cognitive deficits. Early identification of neurological sequelae is important for children to prevent additional developmental delay, and for adults to achieve successful return in society after the disease. Neurological sequelae occur in a substantial amount of patients following bacterial meningitis. Most frequently reported sequelae are focal neurological deficits, hearing loss, cognitive impairment and epilepsy. Copyright © 2016 The British Infection Association. Published by Elsevier Ltd. All rights reserved.
Hovette, P; Camara, P; Burgel, P R; Mbaye, P S; Sane, M; Klotz, F
Pulmonary manifestations are frequently observed in children, pregnant women and travellers with malaria. The pathophysiology of these pulmonary manifestations is poorly understood but would appear to be secondary to an interaction between the parasitized red cells and the pulmonary capillary endothelium. Bronchitis and pneumonia do not directly compromise outcome but, left unrecognized, the delay in diagnosis and treatment may be fatal. Acute respiratory distress in children is the first cause of overmortality, coming before neurological involvement. The acute respiratory distress caused by severe malaria has no specific characteristics. Iatrogenic complications and pulmonary superinfections must be differentiated. The prevention of pulmonary manifestations associated with malaria can easily be accomplished by limiting water intake and carefully monitoring urinary output and weight. Treatment is the same as for acute flare-ups in combination with symptomatic respiratory treatment when required.
Martucci, Gennaro; Lo Re, Vincenzina; Arcadipane, Antonio
Extracorporeal membrane oxygenation (ECMO) is a life-saving mechanical respiratory and/or circulatory support for potentially reversible severe heart or respiratory injury untreatable with conventional therapies. Thanks to the technical and management improvements the use of ECMO has increased dramatically in the last few years. Data in the literature show a progressive increase in the overall outcome. Considering the improving survival rate of patients on ECMO, and the catastrophic effect of neurological injuries in such patients, the topic of neurological damage during the ICU stay in ECMO is gaining importance. We present a case series of six neurological injuries that occurred in 1 year during the ECMO run or after the ECMO weaning. In each case the neurological complication had a dramatic effect: ranging from brain death to prolonged ICU stay and long term disability. This case series has an informative impact for the multidisciplinary teams treating ECMO patients because of its heterogeneity in pathogenesis and clinical manifestation: cerebral hemorrhage, ischemic stroke due to cerebral fat embolism, acute disseminated encephalomyelitis due to H1N1 Influenza. In our ECMO hub we started strict neurological monitoring involving intensivists, a neurologist and our radiology service, but neurological complications are still an insidious diagnosis and treatment. Considering several possible neurological injuries may help reduce delay in diagnosis and speed rehabilitation.
mejorar la precocidad diagnóstica y terapéutica, especialmente con cofactores para reducir la morbimortalidad.Congenital metabolic diseases are considered as rare diseases because of their low incidence and their clinical symptoms at onset. Sometimes they can just begin in the neonatal period. Their progressive knowledge and the availability of specific and sensitive biochemical procedures allow us to diagnose many congenital metabolic diseases, which were not recognized some years ago. We reviewed the 52 patients with congenital metabolic diseases diagnosed during the last 25 years in our centre, evaluating the clinical presentation, neurological symptoms, complementary exams and clinical evolution. The mean age at onset of symptoms was 5 days and the mean age at diagnosis was 88 days of age. We considered a first group of 36 patients with inborn errors of intermediary metabolism, in whom hypotonia, weight loss and seizures are the main symptoms. The second group was composed of 8 patients with defective energy metabolism, who showed abnormal respiratory rhythm and hypotonia. Finally, we considered 8 patients with diseases of the complex molecules, who presented with hypotonia and cataracts as common symptoms at onset. The more common neurological symptoms in this period were hypotonia (60%, sensorial deficit (35% and refractory seizures (23%. The complementary laboratory tests in the first phases of the diseases allowed us to suspect a congenital metabolic disease especially among intermediary and energy defects. EEG and CSF samples were important to diagnose some inborn errors of intermediary metabolism. In the first steps, the neuroimaging was less orientative, even if it allow the exclusion of other diseases. More than half of the patients with inborn errors of metabolism with onset in the neonatal period died within the first two years of life. It is really important to suspect these diseases in the neonatal period so as to achieve an early diagnosis and prompt
Kienzl-Palma, D; Prosch, H
Tuberculosis (TB) is a granulomatous disease caused by Mycobacterium tuberculosis and transmission is via an airborne route by droplet infection. In the majority of cases patients have thoracic TB, which most frequently presents with hilar lymphadenopathy and pulmonary manifestation. Due to the rise in incidence of TB in central Europe to be expected over the coming years, it is essential to be acquainted with the radiological manifestations of pulmonary TB, particularly to be able to discriminate active from inactive TB. Due to the use of molecular techniques entailing DNA fingerprinting, the traditional classification of TB in primary and postprimary TB is being challenged. These genetic studies have revealed that variations in the clinical and radiographic appearance of TB are mainly affected by the immune status of the patients. Due to the low prevalence of TB in central Europe and the wide variation of radiological presentations, the diagnosis and therapy of TB is often delayed. In this article, the radiographic manifestations of thoracic TB are summarized and discussed. Together with the medical history and bacteriological tests, chest X‑ray imaging and computed tomography (CT) play a major role not only in the detection of TB but also in the follow-up during and after therapy. Chest X‑radiographs should be the primary diagnostic method in patients with suspected TB in screening as well as for diagnosis and therapy monitoring. The use of CT is more sensitive than chest radiographs and is frequently performed after chest radiographs to obtain detailed information about subtle parenchymal changes or lymph node manifestation. When active TB is suspected CT should be performed. Tree in bud, lobular consolidations, centrilobular nodules, cavities and ground-glass opacification are typical changes in active TB.
Bushara, Khalafalla O
Celiac disease (CD) long has been associated with neurologic and psychiatric disorders including cerebellar ataxia, peripheral neuropathy, epilepsy, dementia, and depression. Earlier reports mainly have documented the involvement of the nervous system as a complication of prediagnosed CD. However, more recent studies have emphasized that a wider spectrum of neurologic syndromes may be the presenting extraintestinal manifestation of gluten sensitivity with or without intestinal pathology. These include migraine, encephalopathy, chorea, brain stem dysfunction, myelopathy, mononeuritis multiplex, Guillain-Barre-like syndrome, and neuropathy with positive antiganglioside antibodies. The association between most neurologic syndromes described and gluten sensitivity remains to be confirmed by larger epidemiologic studies. It further has been suggested that gluten sensitivity (as evidenced by high antigliadin antibodies) is a common cause of neurologic syndromes (notably cerebellar ataxia) of otherwise unknown cause. Additional studies showed high prevalence of gluten sensitivity in genetic neurodegenerative disorders such as hereditary spinocerebellar ataxia and Huntington's disease. It remains unclear whether gluten sensitivity contributes to the pathogenesis of these disorders or whether it represents an epiphenomenon. Studies of gluten-free diet in patients with gluten sensitivity and neurologic syndromes have shown variable results. Diet trials also have been inconclusive in autism and schizophrenia, 2 diseases in which sensitivity to dietary gluten has been implicated. Further studies clearly are needed to assess the efficacy of gluten-free diet and to address the underlying mechanisms of nervous system pathology in gluten sensitivity.
Full Text Available Leptospirosis, a disease of great significance in tropical countries, presents commonly as a biphasic illness with acute febrile episode in the first phase followed by a brief afebrile period and then by the second phase of fever with or without jaundice and renal failure. However, it has varied manifestations and unusual clinical features ascribed to immunological phenomena can occur due to the additional involvement of pulmonary, cardiovascular, and neurological systems. Among the various neurological features, aseptic meningitis is the most common myeloradiculopathy, myelopathy, cerebellar dysfunction, transverse myelitis, Guillain-Barre syndrome, optic neuritis, peripheral neuropathy are also described. Cranial neuropathy involving facial nerve is a rare, but known neurological manifestation. Sixth nerve palsy in neuroleptospirosis has so far not been reported. We hereby present the occurrence of bilateral abducent nerve palsy in a patient with leptospirosis.
Reetta L. Hänninen
Full Text Available Mitochondrial DNA depletion syndromes (MDS are often serious autosomal recessively inherited disorders characterized by tissue-specific mtDNA copy number reduction. Many genes, including MPV17, are associated with the hepatocerebral form of MDS. MPV17 encodes for a mitochondrial inner membrane protein with a poorly characterized function. Several MPV17 mutations have been reported in association with a heterogeneous group of early-onset manifestations, including liver disease and neurological problems. Mpv17-deficient mice present renal and hearing defects. We describe here a MPV17 truncation mutation in dogs. We found a 1-bp insertion in exon 4 of the MPV17 gene, resulting in a frameshift and early truncation of the encoded protein. The mutation halves MPV17 expression in the lymphocytes of the homozygous dogs and the truncated protein is not translated in transfected cells. The insertion mutation is recurrent and exists in many unrelated breeds, although is highly enriched in the Boxer breed. Unexpectedly, despite the truncation of MPV17, we could not find any common phenotypes in the genetically affected dogs. The lack of observable phenotype could be due to a late onset, mild symptoms or potential tissue-specific compensatory mechanisms. This study suggests species-specific differences in the manifestation of the MPV17 defects and establishes a novel large animal model to further study MPV17 function and role in mitochondrial biology.
Bandmann, Oliver; Weiss, Karl Heinz; Kaler, Stephen G
The copper metabolism disorder Wilson's disease was first defined in 1912. Wilson's disease can present with hepatic and neurological deficits, including dystonia and parkinsonism. Early-onset presentations in infancy and late-onset manifestations in adults older than 70 years of age are now well recognised. Direct genetic testing for ATP7B mutations are increasingly available to confirm the clinical diagnosis of Wilson's disease, and results from biochemical and genetic prevalence studies suggest that Wilson's disease might be much more common than previously estimated. Early diagnosis of Wilson's disease is crucial to ensure that patients can be started on adequate treatment, but uncertainty remains about the best possible choice of medication. Furthermore, Wilson's disease needs to be differentiated from other conditions that also present clinically with hepatolenticular degeneration or share biochemical abnormalities with Wilson's disease, such as reduced serum ceruloplasmin concentrations. Disordered copper metabolism is also associated with other neurological conditions, including a subtype of axonal neuropathy due to ATP7A mutations and the late-onset neurodegenerative disorders Alzheimer's disease and Parkinson's disease. Copyright © 2015 Elsevier Ltd. All rights reserved.
...), launched the Neurological Alliance of Ireland, a nationwide coalition of patient advocacy groups and physicians and authored Standards of Care, the "blueprint" for the development of neurological...
Boersma, Isabel; Miyasaki, Janis; Kutner, Jean
Palliative care is an approach to the care of patients and families facing progressive and chronic illnesses that focuses on the relief of suffering due to physical symptoms, psychosocial issues, and spiritual distress. As neurologists care for patients with chronic, progressive, life-limiting, and disabling conditions, it is important that they understand and learn to apply the principles of palliative medicine. In this article, we aim to provide a practical starting point in palliative medicine for neurologists by answering the following questions: (1) What is palliative care and what is hospice care? (2) What are the palliative care needs of neurology patients? (3) Do neurology patients have unique palliative care needs? and (4) How can palliative care be integrated into neurology practice? We cover several fundamental palliative care skills relevant to neurologists, including communication of bad news, symptom assessment and management, advance care planning, caregiver assessment, and appropriate referral to hospice and other palliative care services. We conclude by suggesting areas for future educational efforts and research. PMID:24991027
Mushtaq, Raheel; Shoib, Sheikh; Raju, M. S. V. K.; Naphade, Nilesh; Shah, Tabindah; Pawar, Alka
Fahr's disease (FD) is a rare neuropsychiatric disease consisting of bilateral basal ganglia calcification with neurological, cognitive, and psychiatric manifestations. We report here a sporadic case of FDs with its neuropsychology. PMID:25013318
Full Text Available Herpes zoster (HZ is a neurocutaneous disorder due to endogenous reactivation of the varicella-zoster virus (VZV. The typical clinical manifestation is an acute segmental eruption of herpetiform umbilicated vesicles associated with malaise, pain, dysaesthesia, allodynia and probably fever. This review focuses on other possible clinical manifestations of the disease to sensitize physicians not to overlook HZ since only an early antiviral treatment can reduce the risk of post-zosteric neuralgia.
Perlmutter, Joel S.
Parkinson disease (PD) is a progressive neurologic condition that causes motor and non-motor manifestations. Treatment provides symptomatic benefit but no current treatment has been proven to slow disease progression. Research studies of PD require a means of rating the severity of disease by measurement of motor manifestations, assessment of ability to perform daily functional activities, and symptomatic response to medication. The most common rating scales are the Unified Parkinson Disease Rating Scale (UPDRS), Hoehn and Yahr staging, and the Schwab and England rating of activities of daily living. Each of these rating scales are described, including detailed instructions on how to implement these ratings. Although these are the most widely applied rating scales of PD, there are still substantial limitations to these scales that must be considered when using them for research. Finally, some common applications of these scales are described. PMID:19802812
Full Text Available West Nile virus infection in humans is mostly asymptomatic. Less than 1% of neuro-invasive cases show a fatality rate of around 10%. Acute flaccid paralysis of respiratory muscles leading to respiratory failure is the most common cause of death. Although the peripheral nervous system can be involved, isolated phrenic nerve palsy leading to respiratory failure is rare and described in only two cases in the English literature. We present another case of neurological respiratory failure due to West Nile virus-induced phrenic nerve palsy. Our case reiterates the rare, but lethal, consequences of West Nile virus infection, and the increase of its awareness among physicians.
Conidi, Francis X.; Drogan, Oksana; Giza, Christopher C.; Kutcher, Jeffery S.; Alessi, Anthony G.; Crutchfield, Kevin E.
Summary We sought to assess neurologists' interest in sports neurology and learn about their experience in treating sports-related neurologic conditions. A survey was sent to a random sample of American Academy of Neurology members. A majority of members (77%) see at least some patients with sports-related neurologic issues. Concussion is the most common sports-related condition neurologists treat. More than half of survey participants (63%) did not receive any formal or informal training in sports neurology. At least two-thirds of respondents think it is very important to address the following issues: developing evidence-based return-to-play guidelines, identifying risk factors for long-term cognitive-behavioral sequelae, and developing objective diagnostic criteria for concussion. Our findings provide an up-to-date view of the subspecialty of sports neurology and identify areas for future research. PMID:24790800
Tasnim F. Imran
Full Text Available Sarcoidosis is a granulomatous disease of unknown etiology that affects multiple organ systems. Neurological manifestations of sarcoidosis are less common and can include cranial neuropathies and intracranial lesions. We report the case of a 21-year-old man who presented with vertigo and uveitis. Extensive workup including brain imaging revealed enhancing focal lesions. A lacrimal gland biopsy confirmed the diagnosis of sarcoidosis. The patient was initially treated with prednisone, which did not adequately control his symptoms, and then was switched to methotrexate with moderate symptomatic improvement. Our patient had an atypical presentation with vertigo as the predominant manifestation of sarcoidosis. Patients with neurosarcoidosis typically present with systemic involvement of sarcoidosis followed by neurologic involvement. Vertigo is rarely reported as an initial manifestation. This case highlights the importance of consideration of neurosarcoidosis as an entity even in patients that may not have a typical presentation or systemic involvement of disease.
Carbajal-Rodríguez, L; Perea-Martínez, A; Loredo-Abdalá, A; Rodríguez-Herrera, R; del Angel-Aguilar, A; Reynes-Manzur, J N
The neurologic complication seen in children with juvenile rheumatoid arthritis (JRA) has hardly been studied for which therefore its prevalence is unknown. Some of the clinical manifestations surrounding this event have been studied and have been divided into the following two groups: cervical articular spinal disease and extra-articular manifestations, more commonly seen in adults, the atlas-axoidal subluxation and the neuropathies. A group of 213 children diagnosed as having JRA according to the criteria setforth by the American Association of Rheumatology and followed by the Department of Internal Medicine of the National Institute of Pediatrics, 10 patients were found to have neurologic symptomatology (4.6%). Their arthritis was studied as well as their association with activity data and seropositivity. We found 6 female and 4 male patients with neurologic manifestations; their ages ranged from 7 to 14 years. Six of them were diagnosed with sero-positive polyarticular JRA and the other four with polyarticular sero-negative. All patients showed some activity and the appearance of the neurologic complications ranged between two months and seven years. No correlation was found between the beginning of the arthritis and the neurologic symptomatology, their sex or the type of arthritis. Seven of the cases showed peripheral neuropathy. Two cases had atlas-atloid subluxation and another child showed having cervical column inflammation with a rheumatoid pannus.(ABSTRACT TRUNCATED AT 250 WORDS)
The human immunodeficiency virus type 2 (HIV-2) produces a similar spectrum of illness as HIV-1, including AIDS, and is clinically indistinguishable. There is evidence that it is less pathogenic, with a longer natural history. HIV2 infection is endemic in West Africa, especially in the former Portuguese and French colonies.
... personality changes, and cerebral venous thrombosis. We present the case of A.G is a 26-year old male, was a student in a Nigerian tertiary institution and resided in Lagos and Ibadan. He presented with a 2-year history of progressive and recurrent headaches, genital and mouth ulcers and weakness of the left limbs.
Herpes zoster and cognitive impairment were the most frequent, each present in 12.3% of the study group. Peripheral neuropathy and facial palsy were also present, in 10.4% and 3.9% of subjects, respectively. Brain mass lesions, seizure disorder, cryptococcal meningitis, cord compression syndrome, and trigeminal ...
Shi, Fu-Dong; Jia, Jian-Ping
In the wake of dramatic economic success during the past 2 decades, the specialized field of neurology has undergone a significant transformation in China. With an increase in life expectancy, the problems of aging and cognition have grown. Lifestyle alterations have been associated with an epidemiologic transition both in the incidence and etiology of stroke. These changes, together with an array of social issues and institution of health care reform, are creating challenges for practicing neurologists throughout China. Notable problems include overcrowded, decrepit facilities, overloaded physician schedules, deteriorating physician-patient relationships, and an insufficient infrastructure to accommodate patients who need specialized neurologic care. Conversely, with the creation of large and sophisticated neurology centers in many cities across the country, tremendous opportunities exist. Developments in neurologic subspecialties enable delivery of high-quality care. Clinical and translational research based on large patient populations as well as highly sophisticated technologies are emerging in many neurologic centers and pharmaceutical companies. Child neurology and neurorehabilitation will be fast-developing subdisciplines. Given China's extensive population, the growth and progress of its neurology complex, and its ever-improving quality control, it is reasonable to anticipate that Chinese neurologists will contribute notably to unraveling the pathogenic factors causing neurologic diseases and to providing new therapeutic solutions.
Lim, K.S., E-mail: email@example.com [Department of Radiology, Raja Isteri Pengiran Anak Saleha (RIPAS) Hospital, Bandar Seri Begawan BA 1710 (Brunei Darussalam); Chong, V.H. [Department of Medicine, Raja Isteri Pengiran Anak Saleha (RIPAS) Hospital, Bandar Seri Begawan BA 1710 (Brunei Darussalam)
Melioidosis is a serious infection that is associated with high mortality. It is due to a Gram-negative bacterium, Burkholderia pseudomallei which is an environmental saprophyte found in wet soils. Melioidosis is endemic to northern Australia and the Southeast Asia. However, there is now increasing number of reports of imported cases to regions where this infection has not been previously encountered. Almost any organ can be affected. Like many other conditions, radiological imaging is an integral part of the diagnostic workup of melioidosis. Awareness of the various radiological manifestations can help direct appropriate investigations to achieve early diagnosis and the initiation of appropriate treatment. Generally, there are no known characteristic features on imaging that can specifically differentiate melioidosis from other infections. However, the 'honeycomb' appearance has been described to be characteristic for large melioidosis liver abscesses. Simultaneous involvement of various organs is also characteristics. To date, there are few data available on the radiological manifestations of melioidosis. The present pictorial essay describes melioidosis affecting the various organs.
Full Text Available Background: Hydatid disease or echinococcosis is a common parasitic disease of human and bovine, caused by infection with larva of the cestode echinococcus. Liver is the most common organ that is involved in this disease. Pelvic involvement and neurological symptoms, due to mass effect of pelvic involvement, in lower extremities are very uncommon manifestations of the disease.Case presentation: A forty six year old man was referred to clinic of surgery at Ghaem Hospital, Medical University of Mashhad, Iran. The patient complained about weakness and motor impairment in right lower extremity accompanied by numbness and radicular pain over past two months. Physical examination demonstrated muscular atrophy and reduced muscular strength in right lower extremity. Computed tomography and ultrasonographic studies showed a cystic mass in right side of the pelvic cavity with extention to the sciatic notch and another cystic mass in right gluteal region. Surgical operation revealed a cystic mass deep in pelvic cavity with the extention to the right sciatic notch with compression of nerve roots. The cystic mass was contained of daughter cysts which confirmed the diagnosis of hydatid cyst disease. This diagnosis was confirmed by pathologic assessment.Conclusion: Although uncommon, but hydatid disease can involve the pelvic cavity and make a pelvic, usually cystic, mass; that can make compression on nerve roots and so making neurologic symptoms in lower extremities. So in endemic areas for hydatid disease, such as Iran, pelvic hydatid cysts should be considered as a possible differential diagnosis in patients presenting with the sciatic pain and neurological manifestations in whom a pelvic mass has been found too.
Prejbisz, A.; Lenders, J.W.M.; Eisenhofer, G.; Januszewicz, A.
Clinical expression of phaeochromocytoma may involve numerous cardiovascular manifestations, but usually presents as sustained or paroxysmal hypertension associated with other signs and symptoms of catecholamine excess. Most of the life-threatening cardiovascular manifestations of phaeochromocytoma,
Full Text Available Dengue infection is a leading cause of illness and death in tropical and subtropical regions of the world. Forty percent of the world’s population currently lives in these areas. The clinical picture resulting from dengue infection can range from relatively minor to catastrophic hemorrhagic fever. Recently, reports have increased of neurological manifestations. Neuropathogenesis seems to be related to direct nervous system viral invasion, autoimmune reaction, metabolic and hemorrhagic disturbance. Neurological manifestations include encephalitis, encephalopathy, meningitis, Guillain-Barré syndrome, myelitis, acute disseminated encephalomyelitis, polyneuropathy, mononeuropathy, and cerebromeningeal hemorrhage. The development of neurological symptoms in patients with positive Immunoglobulin M (IgM dengue serology suggests a means of diagnosing the neurological complications associated with dengue. Viral antigens, specific IgM antibodies, and the intrathecal synthesis of dengue antibodies have been successfully detected in cerebrospinal fluid. However, despite diagnostic advancements, the treatment of neurological dengue is problematic. The launch of a dengue vaccine is expected to be beneficial.
Urbán, Edina; Szél, István; Fáy, Veronika; Dénes, Zoltán; Lippai, Zoltán; Fazekas, Gábor
We have read several publications of great authority on the neurological profession in the last two years in which were expressed assessments of the current situation combined with opinions about neurology and the necessity to reorganize neurological patient care. These articles took up the question of neurorehabilitation too. The authors, who on a daily basis, deal with the rehabilitation of people with disabilities as a consequence of neurological conditions, summarize some important definitions of rehabilitation medicine and the present system of neurological rehabilitation, as it is defined by the rehabilitation profession.
Kondziella, Daniel; Waldemar, Gunhild
This updated and expanded new edition takes neurology trainees by the hand and guides them through the whole patient encounter - from an efficient neurological history and bedside examination through to differential diagnosis, diagnostic procedures and treatment. At each step the expert authors......, as have new chapters including neurogenetics, neurorehabilitation, neurocritical care and heuristic neurological reasoning. In addition, this second edition now includes more than 100 unique case histories. Neurology at the Bedside, Second Edition is written for neurologists in all stages of training....... Medical students, general practitioners and others with an interest in neurology will also find invaluable information here....
The burden of neurological illness is much higher in developing countries. Neurological disorders in these countries are mainly due to poverty and malnutrition. Spectrums of diseases are also different in comparison with developed countries. Lack of resources, ignorance, and overpopulation make it very difficult and challenging to tackle this problem. Majority of the patients are seen by general practitioners who have little knowledge about neurological illnesses. Most of the countries have very few or no neurologist. There is a greater need of taking neurological care at primary care level where majority of the patients struggle with epilepsy, stroke and neuroinfections.
Josef Finsterer; Michael Panny
Bilateral peripheral facial palsy (facial diplegia) has been repeatedly reported as a neurologic manifestation of acute myeloid leukemia but has not been reported as the initial clinical manifestation of myelomonocytic leukemia...
Preiss, Yudith; Santos, José L; Smalley, Susan V; Maiz, Alberto
Cerebrotendinous xanthomatosis (CTX) is a rare autosomal recessive disease, caused by genetic deficiency of the 27-hydroxylase enzyme (encoded by CYP27A1). It plays a key role in cholesterol metabolism, especially in bile acid synthesis and in the 25-hydroxylation of vitamin D3 in the liver. Its deficiency causes reduced bile acid synthesis and tissue accumulation of cholestanol. Clinical manifestations are related to the presence of cholestanol deposits and include tendon xanthomas, premature cataracts, chronic diarrhea, progressive neurologic impairment and less frequently coronary heart disease, early onset osteoporosis and abnormalities in the optic disk and retina. An early diagnosis and treatment with quenodeoxycholic acid may prevent further complications, mainly neurological manifestations. This review summarizes cholesterol metabolism related to bile acid synthesis, physiopathology, biochemistry and treatment of cerebrotendinous xanthomatosis.
G. А. Karkashadze
Full Text Available Deficiency of micronutrients in a diet of the modern children increases risk of the formation of chronic neurologic and somatic pathology. Magnesium deficiency, which initiates a various neurologic symptomatology, has a particular importance in the progress of nervous system diseases at children. Diagnosis of neurologic manifestations of a micronutrient deficiency requires comprehension of the main mechanisms of their development, and also peculiarities of laboratory diagnostics. In treatment of consequences of a magnesium deficiency the special role belongs to the micronutrient stock replacement (medication, alimentary combined with traditional methods of treatment of neurologic disorders.
Freemon, Frank R
The great formative event in the history of North America, the Civil War of 1861 to 1865, was the stimulus for the development of clinical neurology and the neurosciences. The first neurological research center on the continent was the US Army hospital at Turner's Lane, Philadelphia, PA. Silas Weir Mitchell and his colleagues described causalgia (reflex sympathetic dystrophy), phantom limb sensation, and Horner's syndrome (before Horner). The medical leader of the Northern army was William Hammond. After the conclusion of hostilities, he began a huge clinical practice in New York City. In the United States, clinical neurology began in private practice, unlike Europe, where neurology began in institutions. Hammond's textbook, which first used the term athetosis, was used by a generation of physicians who encountered patients with neurological signs and symptoms. Early in the 20th century, neurological institutions were formed around universities; probably the most famous was the Montreal Neurological Institute founded by Wilder Penfield. The US federal government sponsored extensive research into the function and dysfunction of the nervous system through the Neurological Institute of Neurological Diseases and Blindness, later called the National Institute of Neurological Diseases and Stroke. The government officially classified the final 10 years of the 20th century as the Decade of the Brain and provided an even greater level of research funding.
Sotelo-Cruz, N; Gómez-Rivera, N
Envenomation by black widow spiders manifests clinically with signs of neurotoxicity in paediatric patients. Identify typical neurological signs and symptoms in paediatric patients of different ages, and describe treatment and outcomes in a paediatric hospital in northwest Mexico. We reviewed 70 clinical records of patients hospitalised due to black widow spider bite between 1978 and 2014. We divided the total into 2 groups: Group 1, infants and preschool children; and Group 2, school-age children and adolescents. The demographic variables were age, sex, birthplace, place where envenomation occurred, body part(s) affected, degree of envenomation according to signs and symptoms, treatment, clinical outcome, and statistical differences. Boys accounted for 61.4% of all cases, and infants younger than one year old made up 14.2%. Most patients (70%) were bitten by the spider at home; the anatomical areas most frequently affected were the legs, neck, thorax, and abdomen. The neurological signs and symptoms displayed by Group 1 were irritability, constant crying, sialorrhoea, nausea, tachycardia, arrhythmias, fatigue when walking, agitation, muscle spasms paraesthesia, tetany, seizures, and nystagmus. Signs in Group 2 included localized pain, headache, sialorrhoea, paraesthesia, profuse sweating, anxiety, muscle weakness, muscle spasms, and fine tremor. The predominant autonomic sign in Group 1 was sialorrhoea (P<.0001) and in Group 2, paraesthesia (P<.0001). Patients who received Fab antivenom treatment displayed better outcomes and shorter hospital stays than those who did not. No deaths were reported. The neurological signs and symptoms caused by black widow spider bite are predominantly autonomic, and identifying them permits early diagnosis and more effective treatment. Copyright © 2015 Sociedad Española de Neurología. Published by Elsevier España, S.L.U. All rights reserved.
S. D. Mathebula
Full Text Available Mitochondrial disease caused by mutations in mitochondrial DNA is recognized as one of the most common causes of inherited neurological disease. Neuro-ophthalmic manifestations are a common feature of mitochondrial disease. Optic atrophy causing central visual loss is the dominant feature of mitochondrial DNA diseases. Nystagmus is also encountered in mitochondrial disease.Although optometrists are not involved with the management of mitochondrial disease, they are likely to see more patients with this disease. Oph-thalmic examination forms part of the clinical assessment of mitochondrial disease. Mitochondrial disease should be suspected in any patient with unexplained optic neuropathy, ophthalmoplegia, pigmentary retinopathy or retrochiasmal visual loss. Despite considerable advances in the under-standing of mitochondrial genetics and the patho-genesis of mtDNA diseases, no effective treatment options are currently available for patients withmitochondrial dysfunction. (S Afr Optom 201271(1 46-50
Bandmann, Oliver; Weiss, Karl Heinz; Kaler, Stephen G.
Summary The classic copper metabolism disorder, Wilson disease (WD), was first defined in 1912. Both early onset presentations in infancy and late onset manifestations in adults > 70 years are now well recognized. Modern biochemical and genetic prevalence studies suggest that WD may be considerably more common than previously appreciated. Early diagnosis of WD is crucial to ensure that patients can be started on adequate treatment but uncertainty remains about the best possible choice of medication. Direct genetic testing for ATP7B mutations is increasingly available to confirm the clinical diagnosis of WD. WD needs to be differentiated from other conditions that present clinically with hepatolenticular degeneration or share biochemical abnormalities with WD, such as reduced serum cerulo plasmin levels. Disordered copper metabolism is also implied in an increasing number of other neurological conditions, including a subtype of axonal neuropathy due to ATP7A mutations, and the common late-onset neurodegenerative disorders Alzheimer’s disease and Parkinson’s disease. PMID:25496901
Full Text Available Ocular manifestation of granulomatosis with polyangitis. Proptosis is the most common ocular manifestation of granulomatosis with polyangitis. It is due to inflammation of the extraocular muscles or vasculitis of the vas nervosum. It is a poor prognostic sign.
Ebert, Ellen C; Hagspiel, Klaus D
Gastrointestinal (GI) manifestations of leukemia occur in up to 25% of patients at autopsy, generally during relapse. Its presence varies with the type of leukemia and has been decreasing over time due to improved chemotherapy. Gross leukemic lesions are most common in the stomach, ileum, and proximal colon. Leukemia in the esophagus and stomach includes hemorrhagic lesions from petechiae to ulcers, leukemic infiltrates, pseudomembranous esophagitis, and fungal esophagitis. Lesions in the small and large bowel are usually hemorrhagic or infiltrative. Infiltration of lymphoreticular organs, mainly spleen, liver, and lymph nodes, is more prominent in chronic than acute leukemia. Neutropenic enterocolitis, a necrotizing process involving the cecum, ascending colon, and terminal ileum, is increasing in incidence due to greater intensity of chemotherapy. Distension of bowel leads to mucosal breaches, permitting entry of organisms that grow profusely in the absence of neutrophils. Ischemic necrosis follows, leading to perforation and/or peritonitis. Patients present with fever, abdominal pain, diarrhea, nausea, vomiting, abdominal distension and tenderness. Ultrasound and computed tomography scans show thickening of the bowel wall. Treatment is supportive with surgery for necrosis and perforation. The main GI causes of death in leukemia are hemorrhage, infection, and necrotizing enterocolitis. © 2011 Journal of Gastroenterology and Hepatology Foundation and Blackwell Publishing Asia Pty Ltd.
Hugh J Freeman
Full Text Available Celiac disease may initially present as a neurological disorder. Alternatively, celiac disease may be complicated by neurological changes. With impaired nutrient absorption, different deficiency syndromes may occur and these may be manifested clinically with neurological changes. However, in patients with deficiency syndromes, extensive involvement of the small intestine with celiac disease is often evident. There are a number of reports of celiac disease associated with neuropathy, ataxia, dementia and seizure disorder. In these reports, there is no clear relationship with nutrient deficiency and a precise mechanism for the neurological changes has not been defined. A small number of patients have been reported to have responded to vitamin E administration, but most do not. In some, gluten antibodies have also been described, especially in those with ataxia, but a consistent response to a gluten-free diet has not been defined. Screening for celiac disease should be considered in patients with unexplained neurological disorders, including ataxia and dementia. Further studies are needed, however, to determine if a gluten-free diet will lead to improvement in the associated neurological disorder.
Talcott, Katherine E; Garg, Ravin J; Garg, Sunir J
This article aims to describe the ocular manifestations of leukemia, resulting both from direct infiltration of neoplastic cells and from the more common secondary effects of leukemia and its treatment. The prevalence of these findings is also discussed, along with their clinical significance, association with hematologic markers and the ophthalmologist's role caring for these patients. Recent studies have included a large case series examining the prevalence of ocular manifestations in newly diagnosed leukemic patients as well as case reports of ocular manifestations of leukemia. Patients with leukemia often have ocular manifestations. These occur either from direct infiltration of neoplastic cells or from indirect or secondary causes, including hematologic abnormalities, central nervous system involvement, opportunistic infections, or from treatment. Although nearly all ocular structures can be affected, leukemic retinopathy is often the most clinically apparent manifestation. Awareness of the ophthalmic manifestations of leukemia is important as they may precede systemic diagnosis or may be a sign of leukemia recurrence.
... or head Electromyogram (EMG), nerve conduction velocities (NCV) MRI of the back, neck, or head Spinal tap Alternative Names Neurological deficits - focal Images Brain References Daroff RB, Jankovic ...
N. N. Zavadenko
Full Text Available During a clinical examination of children with autistic spectrum disorders, attention should be drawn to both their major clinical manifestations and neurological comorbidities. The paper considers the mechanisms of autism-induced neurological disorders, the spectrum of which may include manifestations, such as retarded and disharmonic early psychomotor development; the specific features of sensory perception/processing; rigidity and monotony of motor and psychic reactions; motor disinhibition and hyperexcitability; motor stereotypies; uncoordinated movements; developmental coordination disorders (dyspraxia; impaired expressive motor skills; speech and articulation disorders; tics; epilepsy. It describes the specific features of neurological symptoms in Asperger’s syndrome, particularly in semantic-pragmatic language disorders, higher incidence rates of hyperlexia, motor and vocal tics. The incidence rate of epilepsy in autistic spectrum disorders is emphasized to be greater than the average population one. At the same time, the risk of epilepsy is higher in mentally retarded patients with autism. Identification of neurological disorders is of great importance in determining the tactics of complex care for patients with autistic spectrum disorders.
Lyons, Jennifer L.; Coleman, Mary E.; Engstrom, John W.
Objective: To ascertain the current status of global health training and humanitarian relief opportunities in US and Canadian postgraduate neurology programs. Background: There is a growing interest among North American trainees to pursue medical electives in low- and middle-income countries. Such training opportunities provide many educational and humanitarian benefits but also pose several challenges related to organization, human resources, funding, and trainee and patient safety. The current support and engagement of neurology postgraduate training programs for trainees to pursue international rotations is unknown. Methods: A survey was distributed to all program directors in the United States and Canada (December 2012–February 2013) through the American Academy of Neurology to assess the training opportunities, institutional partnerships, and support available for international neurology electives. Results: Approximately half of responding programs (53%) allow residents to pursue global health–related electives, and 11% reported that at least 1 trainee participated in humanitarian relief during training (survey response rate 61%, 143/234 program directors). Canadian programs were more likely to allow residents to pursue international electives than US programs (10/11, 91% vs 65/129, 50%, p = 0.023). The number of trainees participating in international electives was low: 0%–9% of residents (55% of programs) and 10%–19% of residents (21% of programs). Lack of funding was the most commonly cited reason for residents not participating in global health electives. If funding was available, 93% of program directors stated there would be time for residents to participate. Most program directors (75%) were interested in further information on global health electives. Conclusions: In spite of high perceived interest, only half of US neurology training programs include international electives, mostly due to a reported lack of funding. By contrast, the majority
Functional neurological disorders, also known as conversion disorder, are unexplained neurological symptoms. These symptoms are common and can be associated with significant consequences. This review covers the neuroimaging literature focusing on functional motor symptoms including motor functioning and upstream influences including self-monitoring and internal representations, voluntariness and arousal and trauma. Copyright © 2014. Published by Elsevier SAS.
... the National Library of Medicine’s MedlinePlus Living with HIV/AIDS × What research is being done? The National Institute of Neurological ... the National Library of Medicine’s MedlinePlus Living with HIV/AIDS See More About Research The National Institute of Neurological Disorders and Stroke ( ...
DEGRAAF, JH; TAMMINGA, RYJ; KAMPS, WA
Paraneoplastic manifestations are signs and symptoms observed in patients with cancer, distant from the tumour or its metastases and not caused by invasion, obstruction or bulk mass. In children with cancer, paraneoplastic manifestations are rare and distinct from those observed in adults. Knowledge
This is the primary hub for those seeking information about the e-Manifest system, its advisory board, and its development. Once the system is complete this area will serve as the portal into the e-Manifest system from EPA webpages.
S. van Hattem (Simone); A.H. Bootsma (Aart); H.B. Thio (Bing)
textabstractDiabetes mellitus can be complicated by a variety of cutaneous manifestations. Good metabolic control may prevent some of these manifestations and may support cure. Unfortunately, most glucose-lowering drugs also have cutaneous side effects. It is important to be able to recognize these
Im, Jung Gi; Yeon, Kyung Mo; Han, Man Chung; Kim, Chu Wan; Lee, Jung Sang; Kim, Suhng Gwon; Han, Yong Chol; Chang, Woo Hyun; Chi, Je Geun [Seoul National University College of Medicine, Seoul (Korea, Republic of)
Authors analysed and present chest X-ray findings of serologically proven leptospirosis from Seoul National University Hospital, either admitted or referred for serological verification, during recent 2 years. Radiological findings were correlated with the lung specimen findings of experimentally induced leptospirosis in guinea pig. The results are as follows: 1. 24 cases (56%) showed positive X-ray findings. 2. Predominant radiological patterns of involved lung were tiny dot, small nodule, rosette density in 11 cases, massive confluent consolidation in 4 cases, and diffuse ill-defined velly increased density in 9 cases. 3. Distribution of pulmonary lesions were bilateral (100%), non-lobar, non-segmental (95%), and there were conspicuous tendency of peripheral lung predominance. 4. Extrapulmonary manifestation, such as pleural effusion or cardiomegaly was rate. 5. Pulmonary lesions resolved completely usually 5 to 10 days after their appearance. 6. From the gross and microscopic findings of serially sacrificed guinea pig's lung and a case of autopsy, authors concluded that fine dot-like density in chest X-ray was due to paleolithic hemorrhage in intraalveolar space at initial stage, growing up to ressette density or confluent consolidation as the pathetic extends to surrounding lung forming purpura and coalescent hemorrhage.
Madi, Deepak; Achappa, Basavaprabhu; Ramapuram, John T; Chowta, Nityananda; Laxman, Mridula; Mahalingam, Soundarya
The clinical spectrum of dengue fever ranges from asymptomatic infection to dengue shock syndrome. Dengue is classically considered a non-neurotropic virus. Neurological complications are not commonly seen in dengue. The neurological manifestations seen in dengue are encephalitis, meningitis, encephalopathy, stroke and Guillain-Barré syndrome. Dengue encephalitis is a rare disease. We report an interesting case of dengue encephalitis from Southern India. A 49-year-old gentleman presented with...
Miravalle, Augusto A; Schreiner, Teri
This chapter reviews the most common neurologic disorders associated with common vaccines, evaluates the data linking the disorder with the vaccine, and discusses the potential mechanism of disease. A literature search was conducted in PubMed using a combination of the following terms: vaccines, vaccination, immunization, and neurologic complications. Data were also gathered from publications of the American Academy of Pediatrics Committee on Infectious Diseases, the World Health Organization, the US Centers for Disease Control and Prevention, and the Vaccine Adverse Event Reporting System. Neurologic complications of vaccination are rare. Many associations have been asserted without objective data to support a causal relationship. Rarely, patients with a neurologic complication will have a poor outcome. However, most patients recover fully from the neurologic complication. Vaccinations have altered the landscape of infectious disease. However, perception of risk associated with vaccinations has limited the success of disease eradication measures. Neurologic complications can be severe, and can provoke fear in potential vaccines. Evaluating whether there is causal link between neurologic disorders and vaccinations, not just temporal association, is critical to addressing public misperception of risk of vaccination. Among the vaccines available today, the cost-benefit analysis of vaccinations and complications strongly argues in favor of vaccination. © 2014 Elsevier B.V. All rights reserved.
Asia is important as it accounts for more than half of the world population. The majority of Asian countries fall into the middle income category. As for cultural traditions, Asia is highly varied, with many languages spoken. The pattern of neurologic diseases in Asia is largely similar to the West, with some disease features being specific to Asia. Whereas Asia constitutes 60% of the world's population, it contains only 20% of the world's neurologists. This disparity is particularly evident in South and South East Asia. As for neurologic care, it is highly variable depending on whether it is an urban or rural setting, the level of economic development, and the system of health care financing. To help remedy the shortage of neurologists, most counties with larger populations have established training programs in neurology. These programs are diverse, with many areas of concern. There are regional organizations serving as a vehicle for networking in neurology and various subspecialties, as well as an official journal (Neurology Asia). The Asian Epilepsy Academy, with its emphasis on workshops in various locations, EEG certification examination, and fellowships, may provide a template of effective regional networking for improving neurology care in the region. © 2015 American Academy of Neurology.
Srivastava, Smita; Chadda, Rakesh Kumar; Bala, Kiran; Majumdar, Pradipta
Background: Neurocysticercosis (NCC) is an endemic parasitic infection of Asia, Africa, Latin America, and central Europe. Neuropsychiatric manifestations of the illness include epilepsy and behavioral disturbances. There is a dearth of systematic studies on psychiatric manifestations of NCC from various Asian counties. The present study assessed the prevalence of various psychiatric disorders in a cohort of patients with NCC attending a neurological service. Materials and Methods: Detailed p...
Mateen, Farrah J
A growing number of international stakeholders are engaged with neurologic diseases. This article provides a brief overview of important international stakeholders in the practice of neurology, including global disease-specific programs, United Nations agencies, governmental agencies with international influence, nongovernmental organizations, international professional organizations, large private donors, private-public partnerships, commercial interests, armed forces, and universities and colleges. The continued engagement of neurologists is essential for the growing number of international organizations that can and should incorporate neurologic disease into their global agendas.
Vázquez-López, María Esther; Díez-Morrondo, Carolina; Sánchez-Andrade, Amalia; Pego-Reigosa, Robustiano; Díaz, Pablo; Castro-Gago, Manuel
To determine the percentage of Lyme patients with articular manifestations in NW Spain and to know their evolution and response to treatment. A retrospective study (2006-2013) was performed using medical histories of confirmed cases of Lyme disease showing articular manifestations. Clinical and laboratory characteristics, together with the treatment and evolution of the patients, were analysed. Seventeen out of 108 LD confirmed patients (15.7%) showed articular manifestations. Regarding those 17 patients, 64.7%, 29.4% and 5.9% presented arthritis, arthralgia and bursitis, respectively. The knee was the most affected joint. Articular manifestations were often associated to neurological, dermatological and cardiac pathologies. Otherwise, most patients were in Stage III. The 11.8% of the cases progressed to a recurrent chronic arthritis despite the administration of an appropriate treatment. Lyme disease patients showing articular manifestations should be included in the diagnosis of articular affections in areas of high risk of hard tick bite, in order to establish a suitable and early treatment and to avoid sequels. Copyright © 2015 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved.
Kjeldsen, A D; Oxhøj, H; Andersen, P E
Hereditary haemorrhagic telangiectasia (HHT) is a dominantly inherited disease. HHT is characterized by a wide variety of clinical manifestations, including pulmonary arteriovenous malformations (PAVMs) and neurological symptoms.......Hereditary haemorrhagic telangiectasia (HHT) is a dominantly inherited disease. HHT is characterized by a wide variety of clinical manifestations, including pulmonary arteriovenous malformations (PAVMs) and neurological symptoms....
Marielza Fernández Veiga
Full Text Available Objetivo: discutir os aspectos clínicos, eletroencefalográficos e os mecanismos genéticos de três síndromes neurogenéticas, que se identificam como entidades nosológicas no grupo heterogêneo de patologias que cursam com retardo mental e autismo. Fontes dos dados: os autores realizaram revisão literária sobre cada síndrome do estudo, atualizando as informações, correlacionando e caracterizando as manifestações neurológicas, assim como a descrição dos mecanismos genéticos e a identificação dos marcadores biológicos. Síntese dos dados: houve a confirmação de que a síndrome de Rett é uma doença genética, conseqüente à mutação no gene MECP2, com variações clínicas que podem ser explicadas por diferentes mutações nesse gene. A síndrome de Angelman tem quatro mecanismos genéticos responsáveis pela variação fenotípica e pelos diferentes riscos de recorrência. Na síndrome do X-Frágil, o grau de comprometimento cognitivo está relacionado com o número de repetições dos trinucleotídeos. Conclusões: os diferentes mecanismos genéticos das três síndromes são responsáveis pela variabilidade clínica. Com a identificação de marcadores biológicos, o diagnóstico será mais precoce, ademais, poderão ser identificadas novas e mais sutis formas de expressão.Objective: to discuss clinical and electroencephalographic aspects and the genetic mechanisms of three neurogenic syndromes that can be related to nosologic entities in the heterogenic pathological group presenting symptoms of mental retardation and autism. Sources: the authors carried out a bibliographic review on each syndrome involved, correlating and characterizing the neurological manifestations, as well as describing genetic mechanisms and identifying biological markers. Summary of the findings: the authors were able to confirm that Rett Sydrome is a genetic disease resulting from the mutation of the MECP2 gene and clinical variations can be
Poggesi, Anna; Gouw, Alida; van der Flier, Wiesje
To investigate the role of neurological abnormalities and magnetic resonance imaging (MRI) lesions in predicting global functional decline in a cohort of initially independent-living elderly subjects. The Leukoaraiosis And DISability (LADIS) Study, involving 11 European centres, was primarily aimed...... at evaluating age-related white matter changes (ARWMC) as an independent predictor of the transition to disability (according to Instrumental Activities of Daily Living scale) or death in independent elderly subjects that were followed up for 3 years. At baseline, a standardized neurological examination.......0 years, 45 % males), 327 (51.7 %) presented at the initial visit with ≥1 neurological abnormality and 242 (38 %) reached the main study outcome. Cox regression analyses, adjusting for MRI features and other determinants of functional decline, showed that the baseline presence of any neurological...
Deeptara Pathak Thapa; Amit Thapa
Introduction: Dermatology is a specialty, which not only deals with dermatological problems with outpatient but also inpatients referrals. The importances of Dermatologist in hospital setting are rising due to changing condition of medical care. Since no peer-reviewed articles are available for dermatological problems in a neurological set up, we conducted this study to know about pattern of skin disorders in neurological patients. Material and Methods: The present study was a prospectiv...
Chronic pain is a frequent component of many neurological disorders, affecting 20–40% of patients for many primary neurological diseases. These diseases result from a wide range of pathophysiologies including traumatic injury to the central nervous system, neurodegeneration and neuroinflammation, and exploring the aetiology of pain in these disorders is an opportunity to achieve new insight into pain processing. Whether pain originates in the central or peripheral nervous system, it frequentl...
Brigo, Francesco; Igwe, Stanley C; Nardone, Raffaele; Lochner, Piergiorgio; Tezzon, Frediano; Otte, Willem M
Our aim was to evaluate Wikipedia page visits in relation to the most common neurological disorders by determining which factors are related to peaks in Wikipedia searches for these conditions. Millions of people worldwide use the internet daily as a source of health information. Wikipedia is a popular free online encyclopedia used by patients and physicians to search for health-related information. The following Wikipedia articles were considered: Alzheimer's disease; Amyotrophic lateral sclerosis; Dementia; Epilepsy; Epileptic seizure; Migraine; Multiple sclerosis; Parkinson's disease; Stroke; Traumatic brain injury. We analyzed information regarding the total article views for 90 days and the rank of these articles among all those available in Wikipedia. We determined the highest search volume peaks to identify possible relation with online news headlines. No relation between incidence or prevalence of neurological disorders and the search volume for the related articles was found. Seven out of 10 neurological conditions showed relations in search volume peaks and news headlines. Six out of these seven peaks were related to news about famous people suffering from neurological disorders, especially those from showbusiness. Identification of discrepancies between disease burden and health seeking behavior on Wikipedia is useful in the planning of public health campaigns. Celebrities who publicly announce their neurological diagnosis might effectively promote awareness programs, increase public knowledge and reduce stigma related to diagnoses of neurological disorders. Copyright © 2015 Elsevier Ltd. All rights reserved.
Momeni, Mahnaz; Taylor, Nora; Tehrani, Mahsa
Ankylosing spondylitis is a chronic inflammatory condition that usually affects young men. Cardiac dysfunction and pulmonary disease are well-known and commonly reported extra-articular manifestation, associated with ankylosing spondylitis (AS). AS has also been reported to be specifically associated with aortitis, aortic valve diseases, conduction disturbances, cardiomyopathy and ischemic heart disease. The pulmonary manifestations of the disease include fibrosis of the upper lobes, interstitial lung disease, ventilatory impairment due to chest wall restriction, sleep apnea, and spontaneous pneumothorax. They are many reports detailing pathophysiology, hypothesized mechanisms leading to these derangements, and estimated prevalence of such findings in the AS populations. At this time, there are no clear guidelines regarding a stepwise approach to screen these patients for cardiovascular and pulmonary complications. PMID:21547038
Full Text Available Leptospirosis has protean clinical manifestations. The classical presentation of the disease is an acute biphasic febrile illness with or without jaundice. Unusual clinical manifestations may result from involvement of pulmonary, cardiovascular, neural, gastrointestinal, ocular and other systems. Immunological phenomena secondary to antigenic mimicry may also be an important component of many clinical features and may be responsible for reactive arthritis. Leptospirosis in early pregnancy may lead to fetal loss. There are a few reports of leptospirosis in HIV- infected individuals but no generalisation can be made due to paucity of data. It is important to bear in mind that leptospiral illness may be a significant component in cases of dual infections or in simultaneous infections with more than two pathogens.
Nathan R. Schaefer, BExSc, MBBS (Hons
Full Text Available Summary: Neurological involvement is not uncommon in patients who sustain electrical injury. The exact mechanism of nervous system damage following electrical trauma is not fully understood. The gamut of possible neurologic manifestations following electrical injury is diverse. This case report describes a young man with a unique pattern of neurological injury following an electrical burn. The combination of brachial plexopathy, partial Horner’s syndrome, and phrenic nerve palsy secondary to electrical injury has not been previously described in the literature.
Priscilla Maria Rodrigues Pereira
Full Text Available No Brasil, a paracoccidioidomicose é uma micose sistêmica endêmica frequente na zona rural e em homens adultos. É relatado caso em agricultor, usuário de drogas ilícitas, com manifestações insidiosas, atingindo rins, pulmões, gânglios, ossos e tardiamente pele, com atraso no diagnóstico e na terapêutica eficaz em mais de um ano. É importante incluir a paracoccidioidomicose como diagnóstico diferencial frente a um quadro sugestivo, mesmo na ausência de lesões cutâneas, para reconhecimento e tratamento precoce, em vista da elevada morbimortalidade desta entidade.Paracoccidioidomycosis is an endemic systemic mycosis in Brazil, frequent in the rural areas and often in adult men. It is reported the case of a farmer, who is an illicit drugs' user, with insidious manifestations affecting kidneys, lungs, lymphonodes, bones and lately, the skin, with a delay of more than one year in the diagnosis and effective therapy It is important to include paracoccidioidomycosis as differential diagnosis, even in the absence of cutaneous lesions, for early recognition and treatment, given the high mortality of this entity.
Fayyaz, Anum; Igoe, Ann; Kurien, Biji T; Danda, Debashish; James, Judith A; Stafford, Haraldine A; Scofield, R Hal
Our purpose was to compile information on the haematological manifestations of systemic lupus erythematosus (SLE), namely leucopenia, lymphopenia, thrombocytopenia, autoimmune haemolytic anaemia (AIHA), thrombotic thrombocytopenic purpura (TTP) and myelofibrosis. During our search of the English-language MEDLINE sources, we did not place a date-of-publication constraint. Hence, we have reviewed previous as well as most recent studies with the subject heading SLE in combination with each manifestation. Neutropenia can lead to morbidity and mortality from increased susceptibility to infection. Severe neutropenia can be successfully treated with granulocyte colony-stimulating factor. While related to disease activity, there is no specific therapy for lymphopenia. Severe lymphopenia may require the use of prophylactic therapy to prevent select opportunistic infections. Isolated idiopathic thrombocytopenic purpura maybe the first manifestation of SLE by months or even years. Some manifestations of lupus occur more frequently in association with low platelet count in these patients, for example, neuropsychiatric manifestation, haemolytic anaemia, the antiphospholipid syndrome and renal disease. Thrombocytopenia can be regarded as an important prognostic indicator of survival in patients with SLE. Medical, surgical and biological treatment modalities are reviewed for this manifestation. First-line therapy remains glucocorticoids. Through our review, we conclude glucocorticoids do produce a response in majority of patients initially, but sustained response to therapy is unlikely. Glucocorticoids are used as first-line therapy in patients with SLE with AIHA, but there is no conclusive evidence to guide second-line therapy. Rituximab is promising in refractory and non-responding AIHA. TTP is not recognised as a criteria for classification of SLE, but there is a considerable overlap between the presenting features of TTP and SLE, and a few patients with SLE have concurrent
Ghaffar Poor M
Full Text Available This syndrome is a very rare neurological presentation which was first reported by Susac in 1975, and usually involves the young women with triad of: (1 Visual loss due to occlusion of retinal artery branches. (2 Subacute encephalopathy with psychic manifestation, personality disorders, involvement of the cortico-spianal tracts, pseudo-bulbar, focal or generalized seizures and myoclonic jerks. (3 Bilateral sensory neural hearing loss with tinnitus. All of the symptoms and signs are pathologically due to microinfarcts. It's pathogenesis is unknown. Brain biopsy shows multiple microinfarctions. Diagnosis of this syndrome is based on the clinical tiard, retinal angiography and neuroimaging studies. In this report, a young lady of 25 years old was referred to neurology ward of Imam Khomeini Hospital with above mentioned symptoms an signes and was recognized as a case of this syndrome.
Full Text Available Myeloproliferative neoplasms (MPN arise from genetic deficiencies at the level of pluripotent stem cells. Each of these neoplasms is a clonal stem cell disorder with specific phenotypic, genetic and clinical properties. Age is one of the most important factors in the development of symptoms and complications associated with MPNs.High white blood cell counts in chronic myelocytic leukemia also known as leukocytosis may lead to central nervous system findings. Tumors developing outside the bone marrow named as extramedullary myeloid tumors (EMMT could be detected at the initial diagnosis or during the prognosis of the disease, which may cause neurological symptoms due to pressure of leukemic cell mass on various tissues along with spinal cord. Central nervous system involvement and thrombocytopenic hemorrhage may lead to diverse neurological symptoms and findings.Transient ischemic attack and thrombotic stroke are the most common symptoms in polycythemia vera. Besides thrombosis and hemorrage, transformation to acute leukemia can cause neurological symptoms and findings. Transient ischemic attack, thrombotic stroke and specifically hemorrage can give rise to neurological symptoms similar to MPN in essential thrombocytosis.Extramedullary hematopoiesis refers to hematopoietic centers arise in organ/tissues other than bone marrow in myelofibrosis. Extramedullar hematopoietic centers may cause intracranial involvement, spinal cord compression, seizures and hydrocephalia. Though rare, extramedullary hematopoiesis can be detected in cranial/spinal meninges, paraspinal tissue and intracerebral regions. Extramedullary hematopoiesis has been reported in peripheral neurons, choroid plexus, pituitary, orbits, orbital and lacrimal fossa and in sphenoidal sinuses. [Cukurova Med J 2013; 38(2.000: 157-169
Korsten, P; Chehab, G
Musculoskeletal manifestations in the context of sarcoidosis are frequently observed. The rheumatologist regularly encounters this disease in clinical practice. In the present review, we aim to give a current overview of the manifestations and treatments relevant to the practicing rheumatologist. The most frequently encountered manifestation is Lofgren's syndrome, which is characterized by bilateral ankle periarthritis, bilateral hilar lymphadenopathy, and erythema nodosum and has an excellent prognosis. Chronic arthropathy most commonly manifests as oligoarthritis, which sometimes hampers its differentiation from spondylarthropathies, especially when sacroiliitis, enthesitis or dactylitis are simultaneously present. Isolated vertebral granulomas are rare and require infectious and malignant disorders to be excluded, since there are no specific imaging findings that are exclusively found in vertebral sarcoidosis. The presence of granulomas in skeletal muscle is common in muscle biopsies, whereas clinically overt myopathy is present in only around 1-2% of patients. Therapeutic responses vary among the different clinical phenotypes. Non-steroidal anti-inflammatory drugs and low to medium dose glucocorticoids are the first-line therapy for musculoskeletal manifestations and often lead to adequate disease control in acute sarcoidosis. When these are ineffective or not tolerated, steroid-sparing agents are increasingly used in chronic sarcoidosis. Evidence for all medications used in sarcoid-related arthritis is comparatively scant. When supplementing vitamin D, the possible development of hypercalcemia, even at standard doses, needs to be considered; the optimal therapeutic levels for the prevention of medication-induced osteoporosis in sarcoidosis have not been firmly established.
Rajapakse, Senaka; Rodrigo, Chaturaka; Balaji, Krishan; Fernando, Sumadhya Deepika
Leptospirosis is an illness with a wide spectrum of clinical manifestations and severe illness affects nearly all organ systems. Serious and potentially life-threatening clinical manifestations of acute leptospirosis are caused by both direct tissue invasion by spirochaetes and by the host immune responses. In its severe form, leptospirosis can cause multi-organ dysfunction and death in a matter of days. Therefore it is critical to suspect and recognize the disease early, in order to initiate timely treatment. While the classical presentation of the disease is easily recognized by experienced clinicians practising in endemic regions, rarer manifestations can be easily missed. In this systematic review, we summarize the atypical manifestations reported in literature in patients with confirmed leptospirosis. Awareness of these unusual manifestations would hopefully guide clinicians towards early diagnosis. © The Author 2015. Published by Oxford University Press on behalf of Royal Society of Tropical Medicine and Hygiene. All rights reserved. For permissions, please e-mail: firstname.lastname@example.org.
Carabin, Hélène; Ndimubanzi, Patrick Cyaga; Budke, Christine M.; Nguyen, Hai; Qian, Yingjun; Cowan, Linda Demetry; Stoner, Julie Ann; Rainwater, Elizabeth; Dickey, Mary
Background The clinical manifestations of neurocysticercosis (NCC) are poorly understood. This systematic review aims to estimate the frequencies of different manifestations, complications and disabilities associated with NCC. Methods A systematic search of the literature published from January 1, 1990, to June 1, 2008, in 24 different electronic databases and 8 languages was conducted. Meta-analyses were conducted when appropriate. Results A total of 1569 documents were identified, and 21 included in the analysis. Among patients seen in neurology clinics, seizures/epilepsy were the most common manifestations (78.8%, 95%CI: 65.1%–89.7%) followed by headaches (37.9%, 95%CI: 23.3%–53.7%), focal deficits (16.0%, 95%CI: 9.7%–23.6%) and signs of increased intracranial pressure (11.7%, 95%CI: 6.0%–18.9%). All other manifestations occurred in less than 10% of symptomatic NCC patients. Only four studies reported on the mortality rate of NCC. Conclusions NCC is a pleomorphic disease linked to a range of manifestations. Although definitions of manifestations were very rarely provided, and varied from study to study, the proportion of NCC cases with seizures/epilepsy and the proportion of headaches were consistent across studies. These estimates are only applicable to patients who are ill enough to seek care in neurology clinics and likely over estimate the frequency of manifestations among all NCC cases. PMID:21629722
Lolli, Valentina; Soto Ares, Gustavo; Pruvo, Jean-Pierre [Roger Salengro Hospital, CHRU, Neuroradiology Department, Lille (France); Abou Chahla, Wadih [Jeanne de Flandre Hospital, Pediatric Hematology and Oncology Department, Lille (France); Jissendi-Tchofo, Patrice [University Hospital Saint-Pierre, Radiology Department - Pediatric Neuroradiology Section, Brussels (Belgium)
Chediak-Higashi syndrome is a rare inherited metabolic disorder characterized by partial oculocutaneous albinism, immunodeficiency, and neurological dysfunction. We present the brain magnetic resonance imaging (MRI) and MR spectroscopy (MRS) findings obtained during the accelerated phase of the disorder in an 8-year-old. The brain MRI manifestations at recurrences 15 months and 24 months later are reported as well. (orig.)
Oral health is an integral part of general health. Menopausal period has an important role in the reproductive life of a woman and gives rise to many physical and mental problems. The oral manifestations may vary for each patient in the form of burning mouth syndrome, xerostomia, mucosal changes, neurological disorders, ...
Literature complements medical literature in the academic and clinical development of neurologists. The present article explores the contributions of writers of fiction on neurology. Literary works of fiction with particular reference to neurology. A symbiosis between writers of fiction and doctors has been well recognised. From Shakespeare to Cervantes by way of Dickens and Cela to writer - physicians such as Anton Chekhov or António Lobo Antunes have contributed through their medically informed literature to the better understanding of neurology. Some writers like Dostoevsky, Machado de Assis and Margiad Evans have written about their own experiences with disease thus bringing new insights to medicine. Furthermore, some neurological disorders have been largely based on literary descriptions. For instance, Dostoevsky's epilepsy has been retrospectively analysed by famous neurologists including Freud, Alajouanine or Gastaut, whilst his writings and biography have prompted others like Waxman and Geschwind to describe typical behavioural changes in temporal lobe epilepsy, finding their source of inspiration in Dostoevsky. Likewise, Cirignotta et al have named an unusual type of seizure after the Russian novelist. Inspired by Lewis Carroll, Todd introduced the term Alice in Wonderland Syndrome to refer to visual distortions generally associated with migraine. Writers of fiction offer a humanised perception of disease by contributing new insights into the clinical history, informing about the subjective experience of the illness and helping to eradicate the stigma associated to neurological disorders.
Arciniegas, David B.; Anderson, C. Alan
The risk of attempted or completed suicide is increased in patients with migraine with aura, epilepsy, stroke, multiple sclerosis, traumatic brain injury, and Huntington's disease. Contrary to the general perception that the risk of suicide among patients with Alzheimer's disease and other dementing conditions is low, several reports suggest that the risk of suicide in these patients increases relative to the general population. Some patients at risk for neurologic disorders are also at increased risk for suicide; in particular, the risk of suicide is increased among persons at risk for Huntington's disease, independent of the presence or absence of the Huntington's gene mutation. The risk of attempted or completed suicide in neurologic illness is strongly associated with depression, feelings of hopelessness or helplessness, and social isolation. Additional suicide risk factors in persons with neurologic illness include cognitive impairment, relatively younger age (under 60 years), moderate physical disability, recent onset or change in illness, a lack of future plans or perceived meaning in life, recent losses (personal, occupational, or financial), and prior history of psychiatric illness or suicidal behavior. Substance dependence, psychotic disorders, anxiety disorders, and some personality disorders (eg, borderline personality disorder) may also contribute to increased risk of suicide among persons with neurologic illnesses. Identification and aggressive treatment of psychiatric problems, especially depression, as well as reduction of modifiable suicide risk factors among patients with neurologic illness is needed to reduce the risk of attempted and completed suicide in this population.
Vardhanabhuti, V. [Radiology Department, Derriford Hospital, Plymouth (United Kingdom); Venkatanarasimha, N. [St Michael' s Hospital, 30 Bond Street, Toronto, Ontario M5B 1W8 (Canada); Bhatnagar, G.; Maviki, M.; Iyengar, S.; Adams, W.M. [Radiology Department, Derriford Hospital, Plymouth (United Kingdom); Suresh, P., E-mail: email@example.com [Radiology Department, Derriford Hospital, Plymouth (United Kingdom)
Although, the diagnosis and evaluation of sarcoidosis has traditionally remained confined to the chest, its multi-system nature has been widely recognized. Radiological features of pulmonary sarcoidosis are well known but extra-pulmonary manifestations can produce a plethora of non-specific imaging findings that can affect subcutaneous tissue, and the neurological, cardiac, gastrointestinal, urological, liver, spleen, and skeletal systems. In the literature, there are various case reports and specific system reviews but there are few reviews that encompass all the extra-pulmonary manifestations. In this paper, we comprehensively review the imaging features of extra-pulmonary sarcoidosis with characteristic features as well as atypical presentations. In addition, we discuss the emerging role of nuclear medicine in sarcoidosis.
Pedroza-Seres, M; Serna-Ojeda, J C; Flores-Suárez, L F
Sclerodermatomyositis is an overlap syndrome of myositis and scleroderma, with dermatological, muscular and joint involvement, but may also present with ocular manifestations. A 57 year-old woman presented with ophthalmological manifestations, including scleral thinning 360°, and the presence of cells in the anterior and posterior chamber. Oriented physical examination and laboratory studies led to the diagnosis, with the need for systemic treatment. Sclerodermatomyositis is a rare disease. Its diagnosis needs thorough clinical and laboratory studies, and its management should be multidisciplinary when inflammatory ocular manifestations may be present. Copyright © 2016 Sociedad Española de Oftalmología. Publicado por Elsevier España, S.L.U. All rights reserved.
Rahbari Manesh A A; Ashrafi M R; Entezari A R; Bakhtiar Ghazely
...."nMaterials and Methods: In a cross sectional study, 390 patients with shigellosis that were hospitalized in Bahrami Hospital of Tehran in period of 5 years 1997-2002 were evaluated with Neurological Manifestations and Complications ."nResults...
Full Text Available Leptospirosis has a spectrum of presentation which ranges from mild disease to a severe form comprising of jaundice and renal failure. Involvement of the lung can vary from subtle clinical features to deadly pulmonary hemorrhage and acute respiratory distress syndrome. Of late, it has been identified that leptospirosis can present atypically with predominant pulmonary manifestations. This can delay diagnosis making and hence optimum treatment. The purpose of this review is to bring together all the reported pulmonary manifestations of leptospirosis and the recent trends in the management.
Jensen, Mark; Cox, Alexander P; Chaudhry, Naveed; Ng, Marcus; Sule, Donat; Duncan, William; Ray, Patrick; Weinstock-Guttman, Bianca; Smith, Barry; Ruttenberg, Alan; Szigeti, Kinga; Diehl, Alexander D
We are developing the Neurological Disease Ontology (ND) to provide a framework to enable representation of aspects of neurological diseases that are relevant to their treatment and study. ND is a representational tool that addresses the need for unambiguous annotation, storage, and retrieval of data associated with the treatment and study of neurological diseases. ND is being developed in compliance with the Open Biomedical Ontology Foundry principles and builds upon the paradigm established by the Ontology for General Medical Science (OGMS) for the representation of entities in the domain of disease and medical practice. Initial applications of ND will include the annotation and analysis of large data sets and patient records for Alzheimer's disease, multiple sclerosis, and stroke. ND is implemented in OWL 2 and currently has more than 450 terms that refer to and describe various aspects of neurological diseases. ND directly imports the development version of OGMS, which uses BFO 2. Term development in ND has primarily extended the OGMS terms 'disease', 'diagnosis', 'disease course', and 'disorder'. We have imported and utilize over 700 classes from related ontology efforts including the Foundational Model of Anatomy, Ontology for Biomedical Investigations, and Protein Ontology. ND terms are annotated with ontology metadata such as a label (term name), term editors, textual definition, definition source, curation status, and alternative terms (synonyms). Many terms have logical definitions in addition to these annotations. Current development has focused on the establishment of the upper-level structure of the ND hierarchy, as well as on the representation of Alzheimer's disease, multiple sclerosis, and stroke. The ontology is available as a version-controlled file at http://code.google.com/p/neurological-disease-ontology along with a discussion list and an issue tracker. ND seeks to provide a formal foundation for the representation of clinical and research data
Full Text Available Hashimoto encephalopathy (HE is an autoimmune disease with neurological and neuropsychiatric manifestations and elevated titers of antithyroid antibodies in serum and cerebrospinal fluid. Patients are mostly women. Age varies from 8 to 86 years. Prevalence of HE is estimated to be 2.1/100,000. Neurological and/or psychiatric symptoms and signs constitute the clinical picture. The disease responds well to corticosteroid therapy, but sometimes other immunomodulatory therapies must be applied. Autoimmune mechanisms with antibodies against antigens in the brain cortex are suspected. The course of the disease can be acute, subacute, chronic, or relapsing/remitting. Some patients improve spontaneously, but a few died in spite of adequate therapy.
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
Barone, Daniel A; Chokroverty, Sudansu
Sleep disorders and neurologic illness are common and burdensome in their own right; when combined, they can have tremendous negative impact at an individual level as well as societally. The socioeconomic burden of sleep disorders and neurologic illness can be identified, but the real cost of these conditions lies far beyond the financial realm. There is an urgent need for comprehensive care and support systems to help with the burden of disease. Further research in improving patient outcomes in those who suffer with these conditions will help patients and their families, and society in general. Copyright © 2016 Elsevier Inc. All rights reserved.
Cuero, Mauricio Ruiz; Varelas, Panayiotis N
Pregnant women are subject to the same complications as the general population, as well to specific neurologic complications associated with pregnancy, such as preeclampsia or eclampsia. The hormonal and physiologic changes during pregnancy lead to altered incidences of these complications, which usually present during the late period of pregnancy, labor, or the puerperium. In addition, the treatment of these conditions is different from that of nonpregnant women, because special attention is paid to avoid any abnormalities or death of the fetus. This article discusses the most common of these neurologic complications. Copyright © 2016 Elsevier Inc. All rights reserved.
Khoujah, Danya; Chang, Wan-Tsu W; Abraham, Michael K
Emergency neurology is a complex and rapidly changing field. Its evolution can be attributed in part to increased imaging options, debates about optimal treatment, and simply the growth of emergency medicine as a specialty. Every year, a number of articles published in emergency medicine or other specialty journals should become familiar to the emergency physician. This review summarizes neurology articles published in 2016, which the authors consider crucial to the practice of emergency medicine. The articles are categorized according to disease process, with the understanding that there can be significant overlap among articles. Copyright © 2017 Elsevier Inc. All rights reserved.
Gofton, T E; Chum, M; Schulz, V; Gofton, B T; Sarpal, A; Watling, C
This study aimed to develop a conceptual understanding of the specific characteristics of palliative care in neurology and the challenges of providing palliative care in the setting of neurological illness. The study was conducted at London Health Sciences Centre in Canada using grounded theory methodology. Qualitative thematic analysis was applied to focus group (health care providers physicians, nursing, allied health, trainees) and semi-structured interview (patient-caregiver dyads) data to explore challenges facing the delivery of palliative care in neurology. Specific characteristics of neurological disease that affect palliative care in neurology were identified: 1) timelines of disease progression, 2) barriers to communication arising from neurologic disease, 3) variability across disease progression, and 4) threat to personhood arising from functional and cognitive impairments related to neurologic disease. Moreover, three key challenges that shaped and complicated palliative care in neurology were identified: 1) uncertainty with respect to prognosis, support availability and disease trajectory, 2) inconsistency in information, attitudes and skills among care providers, care teams, caregivers and families, and 3) existential distress specific to neurological disease, including emotional, psychological and spiritual distress resulting from loss of function, autonomy and death. These challenges were experienced across groups, but manifested themselves in different ways for each group. Further research regarding prognosis, improved identification of patients with palliative care needs, developing an approach to palliative care delivery within neurology and the creation of more robust educational resources for teaching palliative neurology are expected to improve neurologists' comfort with palliative care, thereby enhancing care delivery in neurology. Copyright © 2017 Elsevier B.V. All rights reserved.
Extramedullary plasmacytoma can affect practically all the systems in the body including the eyes, nervous system, head and neck, respiratory system, breast, gastrointestinal system, urogenital system and lymph nodes. These systemic manifestations are reviewed. Key words: Plasmacytoma, ocular, outcome, neurological, ...
Tai, Mei-Ling Sharon; Nor, Hazman Mohd; Kadir, Khairul Azmi Abdul; Viswanathan, Shanthi; Rahmat, Kartini; Zain, Norzaini Rose Mohd; Ong, Kuo Ghee; Rafia, Mohd Hanip; Tan, Chong Tin
Paradoxical manifestation is worsening of pre-existing tuberculous lesion or appearance of new lesions in patients whose condition initially improved with antituberculous treatment. Our hypothesis was that paradoxical manifestation in non-HIV tuberculous meningitis (TBM) patients was underestimated and this could contribute to patients' prognosis. This was the first systemic study of paradoxical manifestation in HIV-negative TBM patients. Between 2009 and 2014, TBM patients were studied prospectively in 2 hospitals. Clinical features, cerebrospinal fluid, and radiological findings were monitored. Paradoxical manifestation was divided into definite (4 weeks or more) and probable (between 14 and 27 d) after commencement of antituberculous treatment. Forty-one non-HIV TBM patients were recruited. Definite paradoxical manifestation occurred in 23/41 (56%) of the patients. Time to onset of paradoxical manifestation was between 28 days and 9 months, and majority was between 28 and 50 days. Neuroimaging manifestation in the brain (22/41 patients, 54%) and clinical manifestation (22/41 patients, 54%) were most commonly seen, followed by cerebrospinal fluid manifestation (7/41 patients, 17%). Neuroimaging changes most commonly seen were worsening of leptomeningeal enhancement, new infarcts, new tuberculomas, and enlargement of tuberculoma. Initial Computed Tomography Angiography/magnetic resonance angiography brain showed vasculitis in 14 patients, with 2 (12.5%) showing paradoxical vasculitis during follow-up. Recurrence of the paradoxical manifestation was seen in 7/23 (30%) of the patients. More than half (14/23, 61%) of the patients improved, 6 (26%) patients died, and 3 (13%) patients had persistent neurological deficit. Paradoxical manifestation was very common in non-HIV TBM patients. Neuroimaging paradoxical manifestation of 2-4 weeks may not be paradoxical manifestation but could be delayed treatment response.
Louis, Elan D
Neurological Association was in many ways one manifestation of a larger societal struggle in a post-bellum (post-World War II) America with a changing demographic. The struggle involved the rise of democratic medical populism in the country's Midwest versus establishment medicine (mainly situated in the Northeast), and perhaps, the rise of the middle class versus the aristocrats.
Joseph Babinski (1857-1932) was an excellent clinician. André Breton, a French poet, described Babinski's way of clinical examination in his Manifeste du surréalisme (1924), which vividly revealed Babinski's meticulous character. Babinski is well known by his eponymous Babinski reflex. Although some predecessors had described this phenomenon briefly, its meaning was interpreted by Babinski. His contribution to neurological symptomatology was not restricted to his plantar skin reflex, but also to other wide area. In this article, symptoms described by Babinski, i.e. plantar skin reflex, cerebellar symptoms including cerebellar asynergy, adiadochokinesis, dysmetria, cerebellar catalepsy, and rising sign, platysma sign, anosognosia are explained and are critically discussed.
Kienzl-Palma, D.; Prosch, H. [Medizinische Universitaet Wien, Abteilung fuer Allgemeine Radiologie und Kinderradiologie, Universitaetsklinik fuer Radiologie und Nuklearmedizin, Wien (Austria)
Tuberculosis (TB) is a granulomatous disease caused by Mycobacterium tuberculosis and transmission is via an airborne route by droplet infection. In the majority of cases patients have thoracic TB, which most frequently presents with hilar lymphadenopathy and pulmonary manifestation. Due to the rise in incidence of TB in central Europe to be expected over the coming years, it is essential to be acquainted with the radiological manifestations of pulmonary TB, particularly to be able to discriminate active from inactive TB. Due to the use of molecular techniques entailing DNA fingerprinting, the traditional classification of TB in primary and postprimary TB is being challenged. These genetic studies have revealed that variations in the clinical and radiographic appearance of TB are mainly affected by the immune status of the patients. Due to the low prevalence of TB in central Europe and the wide variation of radiological presentations, the diagnosis and therapy of TB is often delayed. In this article, the radiographic manifestations of thoracic TB are summarized and discussed. Together with the medical history and bacteriological tests, chest X-ray imaging and computed tomography (CT) play a major role not only in the detection of TB but also in the follow-up during and after therapy. Chest X-radiographs should be the primary diagnostic method in patients with suspected TB in screening as well as for diagnosis and therapy monitoring. The use of CT is more sensitive than chest radiographs and is frequently performed after chest radiographs to obtain detailed information about subtle parenchymal changes or lymph node manifestation. When active TB is suspected CT should be performed. Tree in bud, lobular consolidations, centrilobular nodules, cavities and ground-glass opacification are typical changes in active TB. (orig.) [German] Tuberkulose (Tbc) ist eine durch Troepfchen uebertragene granulomatoese Infektionserkrankung, die durch das Mycobacterium tuberculosis
Brigo, Francesco; Igwe, Stanley C.; Nardone, Raffaele; Lochner, Piergiorgio; Tezzon, Frediano; Otte, WM
Our aim was to evaluate Wikipedia page visits in relation to the most common neurological disorders by determining which factors are related to peaks in Wikipedia searches for these conditions. Millions of people worldwide use the internet daily as a source of health information. Wikipedia is a
Verkhratsky, Alexei; Rodríguez Arellano, Jose Julio; Parpura, V.
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
Zara, Gabriella; Codemo, Valentina; Palmieri, Arianna; Schiff, Sami; Cagnin, Annachiara; Citton, Valentina; Manara, Renzo
Hyperemesis gravidarum can impair correct absorption of an adequate amount of thiamine and can cause electrolyte imbalance. This study investigated the neurological complications in a pregnant woman with hyperemesis gravidarum. A 29-year-old pregnant woman was admitted for hyperemesis gravidarum. Besides undernutrition, a neurological examination disclosed weakness with hyporeflexia, ophthalmoparesis, multidirectional nystagmus and optic disks swelling; the patient became rapidly comatose. Brain MRI showed symmetric signal hyperintensity and swelling of periaqueductal area, hypothalamus and mammillary bodies, medial and posterior portions of the thalamus and columns of fornix, consistent with Wernicke encephalopathy (WE). Neurophysiological studies revealed an axonal sensory-motor polyneuropathy, likely due to thiamine deficiency or critical illness polyneuropathy. Sodium and potassium supplementation and parenteral thiamine were administered with improvement of consciousness state in a few days. WE evolved in Korsakoff syndrome. A repeat MRI showed a marked improvement of WE-related alterations and a new hyperintense lesion in the pons, suggestive of central pontine myelinolysis. No sign or symptom due to involvement of the pons was present.
McCunney, R J
Trichloroethylene, a solvent used in a variety of industrial settings for more than 60 years, has caused adverse health effects on the central and peripheral nervous system, the skin, liver, kidney, and heart. Three men have shown relatively unusual manifestations secondary to exposure to trichloroethylene in degreasing operations in the jewelry industry. Toxic encephalopathy, hepatitis, and carpal spasm occurred among young, healthy workers. Clinical and laboratory data, including measurement of urinary trichloroacetic acid concentrations, are presented. PMID:3342194
Kaushik, Atul; Kumar, Munish; Rohilla, Smriti; Tanwar, Renu; Vinod, V.C.
Achondroplasia (Online Mendelian Inheritance in Man [OMIM] 100800), is considered as a form of skeletal dysplasia dwarfism that manifests with stunted stature and disproportionate limb shortening. Achondroplasia is of special interest in the field of dentistry because of its characteristic craniofacial features which include relative macrocephaly, depressed nasal bridge and maxillary hypoplasia. Presence of large head, implanted shunt, airway obstruction and difficulty in head control require...
Full Text Available Leptospiral uveitis is a common entity in tropical countries. Ocular manifestations are noted in the second phase of illness, but these remain under-diagnosed mainly because of the prolonged symptom-free period that separates the systemic manifestations from detection of ocular manifestations.Varying ophthalmic presentations and the intrinsic nature of different types of uveitis to mimic one another also challenge the accuracy of the diagnosis. Of the individual ocular signs, the combination of acute, non-granulomatous, panuveitis, hypopyon, vasculitis, optic disc edema, membranous vitreous opacities and absence of choroiditis or retinitis have high predictive value for the clinical diagnosis of leptospiral uveitis. Geographic location of the patient, occupation, socio-economic status, risk factors related to exposure, past history of fever or jaundice also aid in diagnosis.Steroids are the mainstay of treatment for leptospiral uveitis. Depending upon the severity and anatomical location of inflammatory lesion, topical, peri-ocular and/or systemic steroids are given. The prognosis is generally good, even when the inflammation is severe.
Pulido-Díaz, Nancy; Medina, Gabriela; Palomino, Nymrod; Peralta, Fidelio
To describe the type and frequency of cutaneous manifestations of leukemia. Observational, descriptive study. We included patients over 16 years of age, with confirmed diagnosis of leukemia from the Hematology and Dermatology Departments of the outpatient clinic and from in-patients. Patients with bone marrow transplantation were excluded. A complete history and physical examination of the skin and appendages was performed, with biopsy and cultures if required. The cutaneous manifestations were classified as infection or drug-related, leukemic infiltration, associated dermatosis to leukemia and non-specific lesions. Descriptive statistics was employed. We included 142 patients (62 females, 80 males) with the following diagnoses: acute myeloid leukemia (n=36), acute lymphoblastic leukemia (n=52), chronic myeloid leukemia (n=21), chronic lymphocitic leukemia (n=30) and hairy cells leukemia (n=3). 42% of patients (n=60) presented some dermatoses. There were 36 non-specific dermatoses, 21 drug-related, 20 infectious, 3 infiltrative and none associated. Cutaneous manifestations directly related to leukemia are frequent, being the non-specific ones, the most commonly observed. However, a thorough dermatologic examination is important in these patients as part of an overall evaluation.
Kostić, Tomislav; Momčilović, Stefan; Perišić, Zoran D; Apostolović, Svetlana R; Cvetković, Jovana; Jovanović, Andriana; Barać, Aleksandra; Šalinger-Martinović, Sonja; Tasić-Otašević, Suzana
The first data of Lyme carditis, a relatively rare manifestation of Lyme disease, were published in eighties of the last century. Clinical manifestations include syncope, light-headedness, fainting, shortness of breath, palpitations, and/or chest pain. Atrioventricular (AV) electrical block of varying severity presents the most common conduction disorder in Lyme carditis. Although is usually mild, AV block can fluctuates rapidly and progress from a prolonged P-R interval to a His-Purkinje block within minutes to hours and days. Rarely, Lyme disease may be the cause of endocarditis, while some studies and reports, based on serological and/or molecular investigations, have suggested possible influence of Borrelia burgdorferi on degenerative cardiac valvular disease. Myocarditis, pericarditis, pancarditis, dilated cardiomyopathy, and heart failure have also been described as possible manifestations of Lyme carditis. The clinical course of Lyme carditis is generally mild, short term, and in most cases, completely reversible after adequate antibiotic treatment. Copyright Â© 2016 Elsevier Ireland Ltd. All rights reserved.
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
Van Lancker, D
Although proverb tests are commonly used in the mental status examination surprisingly little is known about either normal comprehension or the interpretation of proverbial expressions. Current proverbs tests have conceptual and linguistic shortcomings, and few studies have been done to investigate the specific effects of neurological and psychiatric disorders on the interpretation of proverbs. Although frontal lobes have traditionally been impugned in patients who are "concrete", recent studies targeting deficient comprehension of non literal language (e.g. proverbs, idioms, speech formulas, and indirect requests) point to an important role of the right hemisphere (RH). Research describing responses of psychiatrically and neurologically classified groups to tests of proverb and idiom usage is needed to clarify details of aberrant processing of nonliteral meanings. Meanwhile, the proverb test, drawing on diverse cognitive skills, is a nonspecific but sensitive probe of mental status.
Diana Van Lancker
Full Text Available Although proverb tests are commonly used in the mental status examination surprisingly little is known about either normal comprehension or the interpretation of proverbial expressions. Current proverbs tests have conceptual and linguistic shortcomings, and few studies have been done to investigate the specific effects of neurological and psychiatric disorders on the interpretation of proverbs. Although frontal lobes have traditionally been impugned in patients who are “concrete”, recent studies targeting deficient comprehension of non literal language (e.g. proverbs, idioms, speech formulas, and indirect requests point to an important role of the right hemisphere (RH. Research describing responses of psychiatrically and neurologically classified groups to tests of proverb and idiom usage is needed to clarify details of aberrant processing of nonliteral meanings. Meanwhile, the proverb test, drawing on diverse cognitive skills, is a nonspecific but sensitive probe of mental status.
Full Text Available Active immunization of children has been proven very effective in elimination of life threatening complications of many infectious diseases in developed countries. However, as vaccination-preventable infectious diseases and their complications have become rare, the interest focuses on immunization-related adverse reactions. Unfortunately, fear of vaccination-related adverse effects can led to decreased vaccination coverage and subsequent epidemics of infectious diseases. This review includes reports about possible side effects following vaccinations in children with neurological disorders and also published recommendations about vaccinating children with neurological disorders. From all international published data anyone can conclude that vaccines are safer than ever before, but the challenge remains to convey this message to society.
Thouvenot, Éric; Camu, William
Vitamin D deficiency is associated with a higher risk of multiple sclerosis and also with a higher relapse rate as well as a higher number of MRI lesions. Elders with vitamin D deficiency have worse cognitive performance. Vitamin D deficiency is a risk factor for developing Alzheimer's disease. Ischemic stroke are more frequent and more severe in patients with low vitamin D levels. Carotid atherosclerosis is more frequent and more severe in patients with vitamin D deficiency. Vitamin D deficiency is associated with a higher risk and worse prognosis of Parkinson's disease. In the different neurological disorders discussed herein, gene polymorphisms that could alter vitamin D metabolism are also associated with a higher incidence or a worse disease prognosis. Despite the links between vitamin D deficiency and the risks of developing neurological disorders, there is, to date, no proof that supplementation could alter the course of these diseases. Copyright © 2013. Published by Elsevier Masson SAS.
Full Text Available Neurological disorders with a prolonged course, either remediable or otherwise are being seen increasingly in clinical practice and many such patients are young and are part of some organization or other wherein their services are needed if they were healthy and fit. The neurologists who are on the panel of these organizations are asked to certify whether these subjects are fit to work or how long they should be given leave. These certificates may be produced in the court of law and may be subjected to verification by another neurologist or a medical board. At present there are no standard guidelines in our country to effect such certification unlike in orthopedic specialty or in ophthalmology. The following is a beginning, based on which the neurologist can certify the neurological disability of such subjects and convey the same meaning to all neurologists across the country.
Noble, James M; Weimer, Louis H
This review serves as an overview of neurologic conditions associated with alcohol abuse or withdrawal, including epidemiology, clinical symptoms, diagnostic approach, and treatment. Frequent alcohol abuse and frank alcoholism are very common among adults in the United States. Although rates decline with each decade, as many as 10% of the elderly drink excessively. Given the ubiquitous nature of alcoholism in society, its complications have been clinically recognized for generations, with recent advances focusing on improved understanding of ethanol's biochemical targets and the pathophysiology of its complications. The chronic effects of alcohol abuse are myriad and include neurologic complications through both direct and indirect effects on the central and peripheral nervous systems. These disorders include several encephalopathic states related to alcohol intoxication, withdrawal, and related nutritional deficiencies; acute and chronic toxic and nutritional peripheral neuropathies; and myopathy. Although prevention of alcoholism and its neurologic complications is the optimal strategy, this article reviews the specific treatment algorithms for alcohol withdrawal and its related nutritional deficiency states.
Full Text Available Central pontine myelinolysis (CPM is an acute demyelinating neurological disorder affecting primarily the central pons and is frequently associated with rapid correction of hyponatremia. Common clinical manifestations of CPM include spastic quadriparesis, dysarthria, pseudobulbar palsy, and encephalopathy of various degrees; however, coma, “locked-in” syndrome, or death can occur in most severe cases. Rarely, CPM presents with neuropsychiatric manifestations, such as personality changes, acute psychosis, paranoia, hallucinations, or catatonia, typically associated with additional injury to the brain, described as extrapontine myelinolysis (EPM. We present a patient with primarily neuropsychiatric manifestations of CPM, in the absence of focal neurologic deficits or radiographic extrapontine involvement. A 51-year-old female without significant medical history presented with dizziness, frequent falls, diarrhea, generalized weakness, and weight loss. Physical examination showed no focal neurological deficits. Laboratory data showed severe hyponatremia, which was corrected rather rapidly. Subsequently, the patient developed symptoms of an acute psychotic illness. Initial brain magnetic resonance imaging (MRI was unremarkable, although a repeat MRI two weeks later revealed changes compatible with CPM. This case demonstrates that acute psychosis might represent the main manifestation of CPM, especially in early stages of the disease, which should be taken into consideration when assessing patients with acute abnormalities of sodium metabolism.
Zeeman, Gerda G.
Pre-eclampsia is mainly responsible for the world's large maternal mortality rates, mostly due to acute cerebral complications. This review provides insight into the pathogenesis of the neurologic complications of hypertensive disease in pregnancy. In addition, practical relevance for clinical care
López, Víctor; Martín, Sara; Gómez-Serranillos, Maria Pilar
Melissa officinalis has traditionally been used due to its effects on nervous system. Both methanolic and aqueous extracts were tested for protective effects on the PC12 cell line, free radical scavenging properties and neurological activities (inhibition of MAO-A and acetylcholinesterase enzymes...
Yee, Alan H; Rabinstein, Alejandro A
Accurate identification of nervous system dysfunction is vital in the assessment of any multisystem disorder. The neurologic manifestations of acid-base disturbances, abnormal electrolyte concentrations, and acute endocrinopathies are protean and typically determined by the acuity of the underlying derangement. Detailed history and physical examination may guide appropriate laboratory testing and lead to prompt and accurate diagnosis. Neurologic manifestations of primary and secondary systemic disorders are frequently encountered in all subspecialties of medicine. This article focuses on key neurologic presentations of respiratory and metabolic acid-base derangements and potentially life-threatening endocrinopathies.
J Gordon Millichap
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.
African Journal of Neurological Sciences (AJNS) is owned and controlled by the Pan African Association of Neurological Sciences (PAANS). The AJNS's aim is to publish scientific papers of any aspects of Neurological Sciences. AJNS is published quarterly. Articles submitted exclusively to the AJNS are accepted if neither ...
A variety of conditions mimicking rheumatologic syndromes may be associated with an underlying malignancy. Therefore, distinguishing these syndromes from more common, nonparaneoplastic rheumatologic conditions can be perplexing. Some autoimmune conditions and the medications used for their management can be associated with increased future risk of malignancy. Some cancers can directly involve the musculoskeletal structures, whereas others present with systemic manifestations at sites away from the tumor and its metastases. Better awareness and timely recognition of these associations may lead to earlier cancer detection and hopefully better long-term survival. Copyright Â© 2016 Elsevier Inc. All rights reserved.
García Ballesta, C; Cegarra Beltri, M; Cabrerizo Merino, M C
In this essay the authors study a problem which has been considered a "social illness" pediatric the one about child abuse, talking under the oral manifestations point of view. In this way due to the relative frequency of this appearance, the odontopediatric, must known before a traumatism orofacial, the possible existence of child abuse.
Good literary fiction has the potential to move us, extend our sense of life, transform our prospective views and help us in the face of adversity. A neurological disorder is likely to be the most challenging experience a human being may have to confront in a lifetime. As such, literary recreations of illnesses have a doubly powerful effect. Study the synergies between neurology and fictional literature with particular reference to narrative based medicine (NBM). Doctors establish boundaries between the normal and the abnormal. Taking a clinical history is an act of interpretation in which the doctor integrates the science of objective signs and measurable quantities with the art of subjective clinical judgment. The more discrepancy there is between the patient's experience with the illness and the doctor's interpretation of that disease, the less likely the doctor-patient interaction is to succeed. NBM contributes to a better discernment of the meanings, thus considering disease as a biographical event rather than just a natural fact. Drawing from their own experience with disease, writers of fiction provide universal insights through their narratives, whilst neuroscientists, like Cajal, have occasionally devoted their scientific knowledge to literary narratives. Furthermore, neurologists from Alzheimer to Oliver Sacks remind us of the essential value of NBM in the clinic. Integrating NBM (the narrative of patients) and the classic holistic approach to patients with our current paradigm of evidence based medicine represents a challenge as relevant to neurologists as keeping up with technological and scientific advances. Copyright © 2011 Sociedad Española de Neurología. Published by Elsevier Espana. All rights reserved.
Cioni, G; Bos, AF; Einspieler, C; Ferrari, F; Martijn, A; Paolicelli, PB; Rapisardi, G; Roversi, MF; Prechtl, HFR
The aim of the study was to document the early developmental course of neurological signs in a group of preterm infants at risk for hemiplegia due to unilateral intraparenchymal echodensity (UIPE). Sixteen preterm infants with UIPE and sixteen controls were given serial neurological examinations,
Владимир Ильич Карасик
Full Text Available The paper deals with approaches to discourse studies. The approaches may be regarded as discourse dimensions associated with models presented and substantiated by M.M. Bakhtin, J. Habermas, Yu.M. Lotman and M.A.K. Halliday. They comprise contents, participants, tonality, channel and communicative actions. The approaches described complement each other and make it possible to synthesize the results achieved in Text Linguistics, Pragmatics, Social Linguistics, and Discourse Studies. They are joined by and oriented towards the concept of Language Personality, i.e. persons who manifest themselves in various kinds of communicative situations. However, each discourse dimension corresponds to a certain type of discourse. A Topic Approach to discourse focuses upon its contents (its referential substance and specifies the correlation between explicit and implicit meaning expression. A Subject Approach to discourse makes it possible to single out and describe two basic types of communication as presented in Personality bound and Status bound discourse, the former diverges into two subtypes - Habitual and Existential communication, whereas the latter is manifested in various kinds of Institutional discourse. A Tonality Approach to discourse highlights different types of stylistic and emotional modes of communication and correspondingly characterizes personality types who are engaged in such situations. A Regime Approach is concentrated on communication channel and technical peculiarities of behavior determined by those restrictions. An Action Approach to discourse gives us a broader understanding of a performative act, i.e. an action incorporated in culture and a situational behavioral interaction.
Sweiss, Nadera J.; Patterson, Karen; Sawaqed, Ray; Jabbar, Umair; Korsten, Peter; Hogarth, Kyle; Wollman, Robert; Garcia, Joe G.N.; Niewold, Timothy B.; Baughman, Robert P.
Sarcoidosis is a systemic, clinically heterogeneous disease characterized by the development of granulomas. Any organ system can be involved, and patients may present with any number of rheumatologic symptoms. There are no U.S. Food and Drug Administration–approved therapies for the treatment of sarcoidosis. Diagnosing sarcoidosis becomes challenging, particularly when its complications cause patients’ symptoms to mimic other conditions, including polymyositis, Sjögren syndrome, or vasculitis. This review presents an overview of the etiology of and biomarkers associated with sarcoidosis. We then provide a detailed description of the rheumatologic manifestations of sarcoidosis and present a treatment algorithm based on current clinical evidence for patients with sarcoid arthritis. The discussion will focus on characteristic findings in patients with sarcoid arthritis, osseous involvement in sarcoidosis, and sarcoid myopathy. Arthritic conditions that sometimes coexist with sarcoidosis are described as well. We present two cases of sarcoidosis with rheumatologic manifestations. Our intent is to encourage a multidisciplinary, translational approach to meet the challenges and difficulties in understanding and treating sarcoidosis. PMID:20665396
Dryankova, M M; Popova, C L
Modern scientific achievements in the etiology, pathogenesis, diagnosis and treatment of the vesiculobullous lesions of the oral mucosa are of basic significance for the students, who study the diagnosis and the treatment of these diseases, as well as for the clinical practitioners in their everyday practice. The presented new information about the drug-induced or herpes-associated erythema multiforme, the more severe forms - the Stevens-Johnson syndrome and the toxic epidermal necrolysis, is necessary for each practising dentist especially in the diagnosis and treatment of medically compromised patients. Modern investigations confirm the susceptibility of these patients to infections due to primary or secondary immune deficiency. The clinical oral manifestations of erythema multiforme and their treatment are presented.
Roche-Martínez, A; Póo, P; Maristany-Cucurella, M; Jiménez-Llort, A; Camacho, J A; Campistol, J
Haemolytic-uraemic syndrome (HUS) is characterized by microangiopathic hemolytic anaemia, thrombopenia and multiorganic aggression, specially renal, gastrointestinal and central nervous system disturbances. Sporadic in Spain (2/1,500,000 inhabitants), its clinical onset includes acute renal failure, hypertension and central nervous system symptoms (irritability, drowsiness, convulsions, cortical blindness, hemiparesia or coma), due to metabolic distress, hypertension or central nervous system microangiopathy. Few long-term outcome studies have been published. A retrospective analysis of a series of 58 patients with HUS between 1981 and 2006, is reported. Clinical onset, laboratory, electrophysiology, neuroimaging tests, and prognosis factors are reviewed, together with long-term clinical outcome. 22 children presented neurologic symptoms, seven had some neurological test; one patient died; in five some neurological sequelae persisted (hemiparesia, cognitive deficit, visual-perception deficit), the other 16 remaining asymptomatic. Neurological morbility is high in HUS (27% of the children with neurological symptoms), with a 1.7% mortality. Seizure at onset was not a poor prognosis factor in our group. No positive correlation can be established between neuroimaging and long-term outcome.
Mayans, David; Schneider, Logan; Adams, Nellie; Khawaja, Ayaz M.; Engstrom, John
Objective: To survey US-trained graduating neurology residents who are American Academy of Neurology members, in an effort to trend perceived quality and completeness of graduate neurology education. Methods: An electronic survey was sent to all American Academy of Neurology members graduating from US neurology residency programs in the Spring of 2014. Results: Of 805 eligible respondents, 24% completed the survey. Ninety-three percent of adult neurology residents and 56% of child neurology residents reported plans to pursue fellowship training after residency. Respondents reported a desire for additional training in neurocritical care, neuro-oncology, neuromuscular diseases, botulinum toxin injection, and nerve blocks. There remains a clear deficit in business training of neurology residents, although there was notable improvement in knowledge of coding and office management compared to previous surveys. Discussion: Although there are still areas of perceived weakness in neurology training, graduating neurology residents feel generally well prepared for their chosen careers. However, most still pursue fellowship training for reasons that are little understood. In addition to certain subspecialties and procedures, practice management remains deficient in neurology training and is a point of future insecurity for most residents. Future curriculum changes should consider resident-reported gaps in knowledge, with careful consideration of improving business training. PMID:26976522
Weaver, Donald F
Based upon an analysis of 6 major historical technological advances over the last 150 years, a new syndrome, disruptive technology disorder (DTD), is introduced. DTD describes the human health ailments that accompany the implementation of disruptive technologies. Elevator sickness, railway spine, and bicycle face are representative examples. Though the underlying causative disruptive technologies may differ, many neurologic symptoms (headache, dizziness, weakness) are common to multiple DTDs. Born of technology-driven societal change, DTDs manifest as a complex interplay between biological and psychological symptoms. © 2017 American Academy of Neurology.
Sorensen, Jesper Roed; Winther, Kristian Hillert; Bonnema, Steen Joop
BACKGROUND: Hypothyroidism has been associated with increased pulmonary morbidity and overall mortality. We conducted a systematic review to identify the prevalence and underlying mechanisms of respiratory problems among patients with thyroid insufficiency. METHODS: PubMed and EMBASE databases were...... searched for relevant literature from January 1950 through January 2015 with study eligibility criteria: English-language publications; Adult subclinical or overt hypothyroid patients; Intervention, observational or retrospective studies; and respiratory manifestations. We followed the PRISMA statement...... and used the Cochrane's risk of bias tool. RESULTS: A total of 1699 papers were screened by two independent authors for relevant titles. Of 109 relevant abstracts, 28 papers underwent full text analyses, of which 22 were included in the review. We identified possible mechanisms explaining respiratory...
E. E. Grishina
Full Text Available Ophthalmic manifestations of leukemia are variable and are predominantly represented by leukemic infiltration of the eye and hemodynamic abnormalities in the retinal vasculature. Leukemic infiltration of the retina should be differentiated from an inflammatory process, such as retinitis, chorioretinitis of viral or fungal origin that are not uncommon in patients with hematological malignancies. Retinal involvement is mainly seen in adult patients with acute myeloid leukemia and is less common in patients with acute lymphoblastic leukemia. Retinopathy is more frequent during relapses of the underlying disease. Also, retinopathy can be the first sign of the disease. Leukemia should be included into the list for differential diagnosis with disorders that can be associated with optical nerve edema and retinal hemorrhages. Fundus abnormalities correlate with peripheral blood parameters. Retinopathy and leukemic optic neuropathy are predictors of unfavorable prognosis. Early diagnostics and timely and adequate treatment may fully eliminate ocular symptoms and improve quality of life in patients with hemoblastoses.
Chalifoux, Sara L; Konyn, Peter G; Choi, Gina; Saab, Sammy
Primary biliary cholangitis (PBC) is an autoimmune liver disease characterized by progressive destruction of the intrahepatic bile ducts, leading to cholestasis. PBC is known to have both hepatic and extrahepatic manifestations. Extrahepatic manifestations are seen in up to 73% of patients with PBC, with the most common being Sjogren's syndrome, thyroid dysfunction and systemic sclerosis. It is thought that patients with PBC are at increased risk of developing these extrahepatic manifestations, almost all of which are autoimmune, because patients with autoimmune disease are at higher risk of developing another autoimmune condition. Due to the high prevalence of extrahepatic diseases in patients with PBC, it is important to complete a thorough medical history at the time of diagnosis. Prompt recognition of extrahepatic disease can lead to improved patient outcomes and quality of life. The following review summarizes the most common extrahepatic conditions associated with PBC.
Loftus, Angela M; Wade, Carrie; McCarron, Mark O
Neurophobia (fear of neural sciences) and evaluation of independent sector contracts in neurology have seldom been examined among general practitioners (GPs). A questionnaire determined GPs' perceptions of neurology compared with other medical specialties. GP experiences of neurology services with independent sector companies and the local National Health Service (NHS) were compared. Areas of potential improvement in NHS neurology services were recorded from thematic analyses. Among 76 GPs neurology was perceived to be as interesting as other medical specialties. GPs reported less knowledge, more difficulty and less confidence in neurology compared with other medical specialties. There was a preference for a local NHS neurology service (pneurology services provided better patient satisfaction. GPs prefer local NHS neurology services to independent sector contracts. GPs' evaluations should inform commissioning of neurology services. Combating neurophobia should be an integral part of responsive commissioning. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/
The significance of deja vu is widely recognised in the context of temporal lobe epilepsy, and enquiry about deja vu is frequently made in the clinical assessment of patients with possible epilepsy. Deja vu has also been associated with several psychiatric disorders. The historical context of current understanding of deja vu is discussed. The literature reveals deja vu to be a common phenomenon consistent with normality. Several authors have suggested the existence of a "pathological" form of deja vu that differs, qualitatively or quantitatively, from "non-pathological" deja vu. The features of deja vu suggesting neurological or psychiatric pathology are discussed. Several neuroanatomical and psychological models of the deja vu experience are highlighted, implicating the perceptual, mnemonic and affective regions of the lateral temporal cortex, hippocampus and amygdala in the genesis of deja vu. A possible genetic basis for a neurochemical model of deja vu is discussed. Clinical approaches to the patient presenting with possible deja vu are proposed.
Andrea E. Cavanna
Full Text Available Consciousness is a state so essentially entwined with human experience, yet so difficult to conceptually define and measure. In this article, we explore how a bidimensional model of consciousness involving both level of arousal and subjective awareness of the contents of consciousness can be used to differentiate a range of healthy and altered conscious states. These include the different sleep stages of healthy individuals and the altered states of consciousness associated with neurological conditions such as epilepsy, vegetative state and coma. In particular, we discuss how arousal and awareness are positively correlated in normal physiological states with the exception of REM sleep, while a disturbance in this relationship is characteristic of vegetative state, minimally conscious state, complex partial seizures and sleepwalking.
Solis, Constanza; Martinez-Bermejo, Antonio; Naidich, Thomas P; Kaufmann, Walter E; Astrin, Kenneth H; Bishop, David F; Desnick, Robert J
Acute intermittent porphyria (AIP), due to half-normal hydroxymethylbilane synthase activity,is characterized by acute life-threatening neurologic attacks whose etiology remains unclear. To date, only 3 patients confirmed to have homozygous dominant AIP (HD-AIP) have been described (hydroxymethylbilane synthase genotypes R167Q/R167Q and R167W/R173Q). To investigate the genetic, biochemical, clinical, and neuroradiologic features of a severely affected infant with HD-AIP. Clinical, imaging, and genotype/phenotype studies were performed. The proband, homoallelic for hydroxymethylbilane synthase mutation R167W, had approximately 1% of normal hydroxymethylbilane synthase activity, elevated porphyrins and porphyrin precursors, severe psychomotor delay, and central and peripheral neurologic manifestations. When expressed in vitro, the R167W mutant enzyme had less than 2% of normal activity but was markedly unstable, consistent with the proband's severe phenotype. Mitochondrial respiratory chain enzymes were normal. Neuroradiologic studies revealed a unique pattern of deep cerebral white matter injury, with relative preservation of the corpus callosum, anterior limb of the internal capsule, cerebral gray matter, and infratentorial structures. This severely affected patient with HD-AIP expanded the phenotypic spectrum of HD-AIP. His brain magnetic resonance imaging studies suggested selective cerebral oligodendrocyte postnatal involvement in HD-AIP, whereas most structures developed prenatally were intact. These findings indicate that the neurologic manifestations result from porphyrin precursor toxicity rather than heme deficiency and suggest that porphyrin precursor toxicity is primarily responsible for the acute neurologic attacks in heterozygous AIP and other porphyrias.
Boes, Christopher J
The objective of this study was to create an annotated list of textbooks dedicated to teaching the neurologic examination. Monographs focused primarily on the complete neurologic examination published prior to 1960 were reviewed. This analysis was limited to books with the word "examination" in the title, with exceptions for the texts of Robert Wartenberg and Gordon Holmes. Ten manuals met the criteria. Works dedicated primarily to the neurologic examination without a major emphasis on disease description or treatment first appeared in the early 1900s. Georg Monrad-Krohn's "Blue Book of Neurology" ("Blue Bible") was the earliest success. These treatises served the important purpose of educating trainees on proper neurologic examination technique. They could make a reputation and be profitable for the author (Monrad-Krohn), highlight how neurology was practiced at individual institutions (McKendree, Denny-Brown, Holmes, DeJong, Mayo Clinic authors), and honor retiring mentors (Mayo Clinic authors).
Full Text Available Seizures and focal neurologic deficits may be the complications of neurosyphilis, but status epilepticus as a presenting picture of neurosyphilis is rare. We describe a 41-year-old man with an acute onset of expressive dysphasia, followed by persistent seizure state and severe complications of systemic medical problems. An extensive laboratory evaluation confirmed the diagnosis of neurosyphilis and diabetes mellitus. Brain magnetic resonance imaging showed edematous change in the left cingulate gyrus, left temporal lobe, and peri-Rolandic area, which suggested an inflammatory process. Due to varied clinical manifestations of neurosyphilis, we underscore the importance of considering neurosyphilis among the possible causes of status epilepticus and any central nervous system diseases.
Background: Diabetes mellitus is a non- communicable disease with an increasing prevalence in developing countries. Skin manifestations in this condition are due to metabolic derangements or chronic degenerative complications. Skin manifestations are commonly observed after developing clinical diabetes mellitus, but ...
Manjeet Singh Dhallu
Full Text Available Perioperative care of the patients with neurological diseases can be challenging. Most important consideration is the management and understanding of pathophysiology of these disorders and evaluation of new neurological changes that occur perioperatively. Perioperative generally refers to 3 phases of surgery: preoperative, intraoperative, and postoperative. We have tried to address few commonly encountered neurological conditions in clinical practice, such as delirium, stroke, epilepsy, myasthenia gravis, and Parkinson disease. In this article, we emphasize on early diagnosis and management strategies of neurological disorders in the perioperative period to minimize morbidity and mortality of patients.
Licatalosi, Donny D; Darnell, Robert B
.... It is becoming evident that alternative splicing plays a particularly important role in neurologic disease, which is perhaps not surprising given the important role splicing plays in generating...
Sonia M. D. Brucki
Full Text Available The use of cannabidiol in some neurological conditions was allowed by Conselho Regional de Medicina de São Paulo and by Agência Nacional de Vigilância Sanitária (ANVISA. Specialists on behalf of Academia Brasileira de Neurologia prepared a critical statement about use of cannabidiol and other cannabis derivatives in neurological diseases.
Weber, M-A; Schönknecht, P; Pilz, J; Storch-Hagenlocher, B
Postpolio syndrome is defined as a clinical syndrome of new pareses in individuals who had been affected by acute paralytic poliomyelitis years before. The objective of this study was to describe neurologic and psychiatric signs of the disease. We evaluated the clinical signs and treatment of 16 patients with postpolio syndrome. Possible symptoms of depression were evaluated by the Hamilton and Geriatric Depression Scales. Postpolio syndrome manifested at a median age of 57.5 years (range 25-73) in a median of 41 years (range 16-70 years) after acute poliomyelitis. Muscles already affected during acute poliomyelitis were affected in all patients with postpolio syndrome. Six of 16 patients (37.5%) developed paresis in muscles formerly not affected by acute poliomyelitis. In eight of 15 patients (53%), depressive episodes were recognized according to the ICD-10 criteria. Symptoms of depression should be recognized in patients with postpolio syndrome and incorporated in therapy based on physiotherapy.
Canevelli, M; Troili, F; Bruno, G
To date, the frailty syndrome has surprisingly attracted limited attention in the field of neurology and neuroscience. Nevertheless, several concepts closely related to frailty, such as vulnerability, susceptibility, and homeostatic reserves, have been increasingly investigated and documented at level of neuronal cells, brain networks, and functions. Similarly, several aspects commonly assessed in the neurological practice, including cognitive functioning and emotional/affective status, clearly appear to be major determinants of the individual's vulnerability and resiliency to stressors. Therefore, they should be carefully considered in the clinical approach to frail subjects. Moreover, dysfunctions of these domains, if timely detected, may be suitable to be targeted by interventions providing beneficial effects to the overall health status of the individual. In the present article, we discuss the neurobiological processes potentially contributing to frailty. Moreover, we reason about the clinical manifestations allowing the prompt and easy recognition of frail persons in the neurological practice.
Wong, Frank; Rayner-Hartley, Erin; Byrne, Michael F
Helicobacter pylori (H. pylori) infection has been clearly linked to peptic ulcer disease and some gastrointestinal malignancies. Increasing evidence demonstrates possible associations to disease states in other organ systems, known as the extraintestinal manifestations of H. pylori. Different conditions associated with H. pylori infection include those from hematologic, cardiopulmonary, metabolic, neurologic, and dermatologic systems. The aim of this article is to provide a concise review of the evidence that supports or refutes the associations of H. pylori and its proposed extraintestinal manifestations. Based on data from the literature, PUD, mucosal associated lymphoid tumors lymphoma, and gastric adenocarcinoma has well-established links. Current evidence most supports extraintestinal manifestations with H. pylori in immune thrombocytopenic purpura, iron deficiency anemia, urticaria, Parkinson’s, migraines and rosacea; however, there is still plausible link with other diseases that requires further research. PMID:25232230
Sironi, Vittorio A; Riva, Michele A
Neurological and neuropsychological aspects of folk music and traditional dance have been poorly investigated by historical and scientific literature. Some of these performances could be indeed the manifestation of latent pathological conditions or the expression of liberation rituals. This chapter aimed at analyzing the relationships between traditional dance, folk music, and neurological and psychiatric disorders. Since ancient times, dance has been used in the individual or collective as treatment of some diseases, including epilepsy and movement disorders (dyskinesia, chorea, etc.). Dionysia in Ancient Greece, St. Vitus dance in the Middle Age, tarantism and other traditional dances of southern Italy and of non-Western countries might be credited as curative rituals of these neurological and psychiatric conditions. During the nineteenth century, dance was also used for the treatment of psychiatric patients; the relationship between dance and insanity could also be reflected in classical ballets and music of that period. Nowadays, neuropsychiatric manifestations could also be evidenced in modern dances (mass fainting at rock concerts, flash mobs); some ballroom dances are commonly used for the rehabilitation of patients suffering from neurodegenerative and psychiatric conditions. Interdisciplinary research on these subjects (ethnomusicology and cultural anthropology, clinical neurology and dynamic psychology, neuroradiology and neurophysiology, and socioneurology and neuromusicology) should be increased. © 2015 Elsevier B.V. All rights reserved.
Full Text Available Background: Vitiligo is one of the disorder that has social impact . Both skin and mucous membrane show depigmentation in vitiligo. Depigmentation in oral cavity can be more easily observed and the patient can be given awareness regarding the condition if they are unaware of vitiligo elsewhere in their body and can be guided for treatment. Aim and objectives: The aim of this study is to determine the frequency of occurrence of oral mucosal vitiligo in vitiligo patients and to determine the most commonly involved oral mucosal site. Materials and methods: The study sample included 100 vitiligo patients. The patients of all age groups and both genders were included. Vitiligo patients associated with systemic conditions such as thyroid disorders, juvenile diabetes mellitus, pernicious anemia, Addison′s disease were excluded in this study. Results: Out of 100 vitiligo patients 44 % male and 56% were female. The oral presentation of vitiligo in this study showed depigmentation of buccal mucosa in 5% of patients, labial mucosa in 5% of patients, palate in 8% of patients, gingiva in 2% of patients and alveolar mucosa 1% . Depigmentation of lip was seen in 42% of patients. Lip involvement refers to depigmentation of both the lips or either lip. Also vermilion border involvement was noted in majority of cases. In some cases, the depigmentation of lip extended to the facial skin also. Conclusion: In this study 55 patients out of 100 patients showed depigmentation in the oral cavity. Lip involvement was most common in this study showing about 42% of patients. Intraoral mucosal involvement was found in 21% of patients. Among intraoral mucosal site palate was common followed by buccal and labial mucosa, gingiva. Two patients had lip pigmentation as the only manifestation without any depigmentation in the skin.
Nagarajan, Anitha; Masthan, Mahaboob Kader; Sankar, Leena Sankari; Narayanasamy, Aravindha Babu; Elumalai, Rajesh
Vitiligo is one of the disorder that has social impact. Both skin and mucous membrane show depigmentation in vitiligo. Depigmentation in oral cavity can be more easily observed and the patient can be given awareness regarding the condition if they are unaware of vitiligo elsewhere in their body and can be guided for treatment. The aim of this study is to determine the frequency of occurrence of oral mucosal vitiligo in vitiligo patients and to determine the most commonly involved oral mucosal site. The study sample included 100 vitiligo patients. The patients of all age groups and both genders were included. Vitiligo patients associated with systemic conditions such as thyroid disorders, juvenile diabetes mellitus, pernicious anemia, Addison's disease were excluded in this study. Out of 100 vitiligo patients 44 % male and 56% were female. The oral presentation of vitiligo in this study showed depigmentation of buccal mucosa in 5% of patients, labial mucosa in 5% of patients, palate in 8% of patients, gingiva in 2% of patients and alveolar mucosa 1%. Depigmentation of lip was seen in 42% of patients. Lip involvement refers to depigmentation of both the lips or either lip. Also vermilion border involvement was noted in majority of cases. In some cases, the depigmentation of lip extended to the facial skin also. In this study 55 patients out of 100 patients showed depigmentation in the oral cavity. Lip involvement was most common in this study showing about 42% of patients. Intraoral mucosal involvement was found in 21% of patients. Among intraoral mucosal site palate was common followed by buccal and labial mucosa, gingiva. Two patients had lip pigmentation as the only manifestation without any depigmentation in the skin.
Neurogenetics has developed enormously in recent years, and the genetic basis of human disorders is being unravelled rapidly. Many neurological disorders are Mendelian disorders, caused by mutations in genes involved in normal function of the brain, spinal cord, peripheral nerves or muscles. Due to high costs and time-consuming procedures, genetic tests have normally been performed late in the diagnostic process, when clinical examination and other tests have indicated a specific gene as the likely disease cause. Many neurological phenotypes are genetically very heterogeneous, and testing of all possible disease genes has been impossible. As a result, many patients with genetic neurological disorders have remained without a specific diagnosis, even when the disease is caused by mutations in known disease genes. Recent technological advances, in particular next-generation DNA sequencing techniques, have resulted in rapid identification of genes involved in Mendelian disorders and provided new possibilities for diagnostic genetic testing. The development of methods for coupling targeted capture and massively parallel DNA sequencing has made it possible to examine a large number of genes in a single reaction. Diagnostic genetic testing can today be performed by the use of gene panels and exome sequencing. This allows a more precise diagnosis of many neurological disorders, and genetic testing should now be considered earlier in the diagnostic procedure. © 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.
Full Text Available Hodgkin's lymphoma is a hematolymphoid neoplasm, primarily of B cell lineage, that has unique histologic, immunophenotypic, and clinical features. Neurologic complications of Hodgkin's Lymphoma can be separated into those that result directly from the disease, indirectly from the disease, or from its treatment. Direct neurologic dysfunction from Hodgkin's Lymphoma results from metastatic intracranial spinal disease, epidural metastases causing spinal cord/cauda equina compression, leptomeningeal metastases, or intradural intramedullary spinal cord metastases. Indirect neurologic dysfunction may be caused by paraneoplastic disorders (such as paraneoplastic cerebellar degeneration or limbic encephalitis and primary angiitis of the central nervous system. Hodgkin's lymphoma treatment typically includes chemotherapy or radiotherapy with potential treatment-related complications affecting the nervous system. Neurologic complications resulting from mantle-field radiotherapy include the “dropped head syndrome,” acute brachial plexopathy, and transient ischemic attacks/cerebral infarcts. Chemotherapy for Hodgkin's lymphoma may cause cerebral infarction (due to emboli from anthracycline-induced cardiomyopathy and peripheral neuropathy.
Zucconi, M; Bruni, O
Pediatric neurologic diseases are often associated with different kinds of sleep disruption (mainly insomnia, less frequently hypersomnia or parasomnias). Due to the key-role of sleep for development, the effort to ameliorate sleep patterns in these children could have important prognostic benefits. Study of sleep architecture and organization in neurologic disorders could lead to a better comprehension of the pathogenesis and a better treatment of the disorders. This article focuses on the following specific neurologic diseases: nocturnal frontal lobe epilepsy and abnormal motor behaviors of epileptic origin, evaluating differential diagnosis with parasomnias; achondroplasia, confirming the crucial role of craniofacial deformity in determining sleep-disordered breathing; neuromuscular diseases, mainly Duchenne's muscular dystrophy and myotonic dystrophy; cerebral palsy, evaluating either the features of sleep architecture and the importance of the respiratory problems associated; headaches, confirming the strict relationships with sleep in terms of neurochemical and neurobehavioral substrates; and finally a review on the effectiveness of melatonin for sleep problems in children with neurologic syndromes and mental retardation, blindness, and epilepsy.
Pareja, J A; Gil-Nagel, A
Cerebral cortical activity is constant throughout the entire human life, but substantially changes during the different phases of the sleep-wake cycle (wakefulness, non-REM sleep and REM sleep), as well as in relation to available information. In particular, perception of the environment is closely linked to the wake-state, while during sleep perception turns to the internal domain or endogenous cerebral activity. External and internal information are mutually exclusive. During wakefulness a neuronal mechanism allows attention to focus on the environment whereas endogenous cortical activity is ignored. The opposite process is provided during sleep. The function external attention-internal attention is coupled with the two modes of brain function during wakefulness and during sleep, providing two possible cortical status: thinking and dreaming. Several neurological processes may influence the declaration of the three states of being or may modify their orderly oscillation through the sleep-wake cycle. In addition, endogenous information and its perception (dreams) may be modified. Disturbances of dreaming may configurate in different general clinical scenarios: lack of dreaming, excess of dreaming (epic dreaming), paroxysmal dreaming (epileptic), nightmares, violent dreaming, daytime-dreaming (hallucinations), and lucid dreaming. Sensorial deprivation, as well as the emergence of internal perception may be the underlying mechanism of hallucinations. The probable isomorphism between hallucinations and dreaming is postulated, analyzed and discussed.
Edgell, Randall C; Alshekhlee, Amer; Yavagal, Dileep R; Vora, Nirav; Cruz-Flores, Salvador
Neurologists have a long history of involvement in cerebral angiography; however, the roots of neurologist involvement in therapeutic endovascular procedures have not been previously documented. As outlined in this article, it has taken the efforts of several early pioneers to lay the ground work for interventional neurology, a specialty that has become one of the fastest growing neurological subspecialties. The ground work, along with a great clinical need, has allowed the modern interventional neurologist to tackle some of the most intractable diseases, especially those affecting the cerebral vasculature. The institutionalization of interventional neurology as a subspecialty was first advocated in 1995 in an article entitled, "Interventional Neurology, a subspecialty whose time has come." The institutions created in the wake of this article have provided the framework that has allowed interventional neurology to transition from "a subspecialty whose time has come" to a subspecialty that is here to stay and thrive. Copyright © 2010 by the American Society of Neuroimaging.
Full Text Available Paraneoplastic neurologic syndrome (PNS is an uncommon manifestation of cancer that is not caused by the tumor or metastasis. Trigeminal neuralgia (TN is an initial symptom of this disease, but it has rarely been reported in the literature. Here, we report the case of a 76-year-old woman who presented with classic TN, followed by limbic encephalitis due to an underlying ovarian intestinal-type mucinous borderline tumor, with the presence of anti-Hu antibodies. She recovered quickly after removal of the tumor and was essentially free of symptoms 2 weeks after surgery. Because PNS precedes the tumor in approximately 60% of cases, its rapid detection and treatment are crucial. Therefore, we propose that PNS be considered during the management of TN when brain imaging is normal, as it is followed by other central and/or peripheral neurological manifestations as well as the presence of systemic symptoms such as anemia, fatigability, loss of appetite, or weight loss.
Marques, Silvio Alencar; Stolf, Hamilton Ometto; Polizel, Juliana Ocanha; Munhoz, Tânia; Brandão, Marcela Calixto; Marques, Mariangela Esther Alencar
Hyaline fibromatosis syndrome is the current name for clinical manifestations of diseases previously known as “infantile systemic hyalinosis” and “juvenile hyaline fibromatosis”. The authors report representative clinical cases of each one of the above subtypes with emphasis on cutaneous manifestations and difficulties for early diagnosis in this syndrome, essentially of multidisciplinary approach. PMID:27192526
Bertorini, Tulio E; Perez, Angel
Disorders of the adrenal glands frequently have secondary neurological manifestations, while some diseases that involve the central nervous system are accompanied by adrenal gland dysfunction. Excessive corticosteroid secretions in primary or secondary Cushing's syndrome causes muscle weakness and behavioral disturbances, such as emotional lability and sometimes depression, while adrenal insufficiency may cause fatigue, weakness, and depression. Adrenoleukodystrophy and adrenoneuromyelopathy are X-linked recessive disorders of the metabolism of very long chain fatty acids that manifest with white matter abnormalities of the brain, myelopathy and/or neuropathy, as well as adrenal insufficiency. Other disorders of the adrenal glands include hyperaldosteroidism, which may cause weakness from hypokalemia. Dysfunction of the adrenal medulla causes excessive or deficient secretion of catecholamines, primarily causing cardiovascular symptoms. This chapter reviews the clinical manifestations and diagnostic aspects and treatment of the various disorders of the adrenal glands. Some of the congenital adrenal diseases are also discussed. © 2014 Elsevier B.V. All rights reserved.
Madani, G. [Department of Radiology, Royal Free Hospital NHS Trust, London (United Kingdom)]. E-mail: firstname.lastname@example.org; Papadopoulou, A.M. [Department of Radiology, Royal Free Hospital NHS Trust, London (United Kingdom); Holloway, B. [Department of Radiology, Royal Free Hospital NHS Trust, London (United Kingdom); Robins, A. [Department of Paediatrics, Whittington Hospital NHS Trust, London (United Kingdom); Davis, J. [Department of Radiology, Whittington Hospital NHS Trust, London (United Kingdom); Murray, D. [Department of Radiology, Whittington Hospital NHS Trust, London (United Kingdom)
Sickle cell disease (SCD) is an inherited abnormality of the ss-globin chain, which causes a spectrum of haemolytic anaemias. Clinical manifestations in SCD include anaemia, jaundice, recurrent vaso-occlusive crises, and infections (particularly by encapsulated bacteria) due to functional asplenia and cerebrovascular accidents. Radiological investigations play a critical role both in the diagnosis and in the primary prevention of the complications of SCD.
Woolson, K L; Forbes, A; Vine, L; Beynon, L; McElhinney, L; Panayi, V; Hunter, J G; Madden, R G; Glasgow, T; Kotecha, A; Dalton, H C; Mihailescu, L; Warshow, U; Hussaini, H S; Palmer, J; Mclean, B N; Haywood, B; Bendall, R P; Dalton, H R
Autochthonous (locally acquired) hepatitis E is increasingly recognised in developed countries, and is thought to be a porcine zoonosis. A range of extra-hepatic manifestations of hepatitis E infection have been described, but have never been systematically studied. To report the extra-hepatic manifestations of hepatitis E virus. Retrospective review of data of 106 cases of autochthonous hepatitis E (acute n = 105, chronic n = 1). Eight (7.5%) cases presented with neurological syndromes, which included brachial neuritis, Guillain-Barré syndrome, peripheral neuropathy, neuromyopathy and vestibular neuritis. Patients with neurological syndromes were younger (median age 40 years, range 34-92 years, P = 0.048) and had a more modest transaminitis (median ALT 471 IU/L, P = 0.015) compared to cases without neurological symptoms [median age 64 years (range 18-88 years), median ALT 1135 IU/L]. One patient presented with a cardiac arrhythmia,twelve patients (11.3%) presented with thrombocytopenia, fourteen (13.2%) with lymphocytosis and eight (7.5%) with a lymphopenia, none of which had any clinical consequence. Serum electrophoresis was performed in 65 patients at presentation, of whom 17 (26%) had a monoclonal gammopathy of uncertain significance. Two cases developed haematological malignancies, acute myeloid leukaemia and duodenal plasmacytoma, 18 and 36 months after presenting with acute hepatitis E infection. A range of extra-hepatic manifestations can occur with hepatitis E. Neurological and haematological features of hepatitis E infection are relatively frequent in this UK cohort, and result in significant morbidity which warrants further study. © 2014 John Wiley & Sons Ltd.
Ushiyama, Satoru; Kinoshita, Tomomi; Shimojima, Yasuhiro; Ohashi, Nobuhiko; Kishida, Dai; Miyazaki, Daigo; Nakamura, Katsuya; Sekijima, Yoshiki; Ikeda, Shu-Ichi
Neurological involvement in relapsing polychondritis (RP) is relatively rare. We describe the case of an 80-year-old man who presented with hypertrophic pachymeningitis (HP) together with arthritis as the first manifestation of RP. Auricular chondritis, which subsequently determined the diagnosis of RP, occurred a few weeks after the detection of HP. The neurological symptoms, as well as arthritis, were promptly improved by treatment with corticosteroids. It is generally difficult to diagnose RP in the absence of typical cartilaginous involvement; however, the present case suggests that HP may occur as an early clinical manifestation of RP.
Sebastian, Swapna; Nair, Prem G; Thomas, Philip; Tyagi, Amit Kumar
To determine the type, severity and manifestation of dysphagia in patients with neurogenic etiology. Clinical documentation was done on the different etiologies, its manifestation, assessment findings and management strategies taken for patients with neurogenic oropharyngeal dysphagia who were referred for assessment and management of dysphagia over a period of three months in a tertiary care teaching hospital. Flexible endoscopic examination was done in all the patients. The severity of dysphagia in these patients were graded based on Gugging Swallowing Screen (GUSS). A total of 53 patients with neurogenic oropharyngeal dysphagia were evaluated by an otolaryngologist and a speech language pathologist over a period of three months. The grading of severity based on GUSS for these patients were done. There were 30 patients with recurrent laryngeal nerve injury due to various etiologies, one patient with Neurofibroma-vestibular schwanoma who underwent surgical excision, 16 patients with stroke, two patients with traumatic brain injury, two patients with Parkinsonism and two patients with myasthenia gravis. The manifestation of dysphagia was mainly in the form of prolonged masticatory time, oral transit time, and increased number of swallows required for each bolus, cricopharyngeal spasms and aspiration. Among the dysphagia patients with neurogenic etiology, dysphagia is manifested with a gradual onset and is found to have a progressive course in degenerative disorders. Morbidity and mortality may be reduced with early identification and management of neurogenic dysphagia.
Murad, Shatha; Eisenberg, Yuval
Primary hyperoxaluria type 1 (PH1) is a rare metabolic disorder of oxalate overproduction. It is associated with urolithiasis and nephrocalcinosis which progress to ESRD and systemic oxalosis. As oxalate deposits in tissues, non-parathyroid hormone (nonPTH) mediated hypercalcemia, oxalate osteopathy, primary hypothyroidism and primary hypogonadism develop. In this review, we will present a case of PH1 and provide an overview of this clinical entity and its endocrine manifestations. We conducted a PubMed search for articles related to PH1. The terms "primary hyperoxaluria," "nonPTH mediated hypercalcemia," "hypothyroidism," and "hypogonadism" were used to identify pertinent literature. Given the rarity of PH1, there is scant literature regarding the incidence and clinical significance of endocrine manifestations of this disorder. There are rare reports of hypercalcemia secondary to osteoclast-stimulating activity of macrophages in bone granulomas, which occur in response to oxalate deposits. We report that hypercalcemia may also be mediated by 1,25-dihydroxyvitamin D (1,25 (OH)2D) and parathyroid hormone-related protein (PTHrP). Primary hypothyroidism and primary hypogonadism are thought to be due partly to calcium oxalate deposition in thyroid and testicular tissue. The presented case is the first to report PTHrP mediated hypercalcemia and primary hypogonadism in a patient with PH1. PH1 is a metabolic disease with significant morbidity and mortality. Owing to its rarity, it is not widely recognized in the field of endocrinology, despite presenting with several endocrinopathies. Recognition of endocrine disturbances can result in early and successful treatment, limiting morbidity and improving quality of life in these challenging patients. PH = primary hyperoxaluria; ESRD = end stage renal disease; PTH = parathyroid hormone; 1,25 (OH)2D = 1,25 dihydoxyvitamin D; PTHrP = parathyroid hormone related protein; CKD = chronic kidney disease; AGT = alanine: glyoxylate
Full Text Available This clinical review about the neurological examination in small animals describes the basics about the first steps of investigation when dealing with neurological patients. The knowledge of how to perform the neurological examination is important however more important is how to correctly interpret these performed tests. A step-by-step approach is mandatory and examiners should master the order and the style of performing these tests. Neurological conditions can be sometimes very distressing for owners and for pets that might not be the most cooperating. The role of a veterinary surgeon, as a professional, is therefore to collect the most relevant history, to examine a patient in a professional manner and to give to owners an educated opinion about the further treatment and prognosis. However neurological examinations might look challenging for many. But it is only the clinical application of neuroanatomy and neurophysiology to an every-day situation for practicing veterinarians and it does not require any specific in-to-depth knowledge. This clinical review is aimed not only to provide the information on how to perform the neurological examination but it is also aimed to appeal on veterinarians to challenge their daily routine and to start practicing on neurologically normal patients. This is the best and only way to differentiate between the normal and abnormal in a real situation.
Full Text Available Introduction: Knowledge of etiopathogenesis of post-stroke depressive phenomena contributes to early diagnostics which shortens recovery to a great extent and suits the social and professional rehabilitation of patients, if followed by proper psycho/pharmacotherapy. The aim of this work is to research dependence of depressive manifestations considering the size and anatomical localization of lesion. Subjects and Methods: The research included 118 patients with stroke. Lesion localization was defined on computerized axial tomography records, whereas the area and perimeter of lesion were measured by AutoCAD 2004 software. Examinations by means of Hamilton Rating Scale for Depression were carried out by the method of random selection 11–40 days after stroke. Correlation analysis was made by simple linear/nonlinear regression and Cox’s hazard regression model. Results: Negative correlation was observed between the intensity of depressive manifestations and the size of cerebrovascular lesion (Spearman’s r= – 0.263, P= 0.004. By means of Cox’s regression model we determined 4.389 times higher risk for depression occurrence in female patients (P< 0.001, as well as higher risk due to lobus limbicus structure damages (hazard ratio eb(HR = 2.661, P= 0.019. Conclusion: Lower intensity of depressive manifestations with larger cerebrovascular lesions, we have explained by activation of reparation mechanisms with energy savings and decrease (due to neurological deficits of afferent peripheral sensations which antecedent the occurrence of emotions (James-Lange peripheral theory of emotions.
Kameda, G; Vieker, S; Duck, C; Blaes, F; Längler, A
We present a 9-year-old girl who developed acute muscular weakness of proximal muscles of the upper and lower limbs. Investigations revealed a common acute lymphoblastic leukemia. The neuromuscular symptoms are classified as a paraneoplastic neurological syndrome (PNS). Under chemotherapy according to ALL-BFM-2000 protocol symptoms resolved within 4 weeks. This case presents a rare manifestation of acute lymphoblastic manifestation. © Georg Thieme Verlag KG Stuttgart · New York.
Albert, Dara V; Hoyle, Chad; Yin, Han; McCoyd, Matthew; Lukas, Rimas V
To evaluate the factors which influence the decision to pursue a career in neurology. An anonymous survey was developed using a Likert scale to rate responses. The survey was sent to adult and child neurology faculty, residents and fellows, as well as medical students applying for neurology. Descriptive statistics were used to analyse the factors of influence. Respondents were subsequently categorized into pre-neurology trainees, neurology trainees, child neurologists and adult neurologists, and differences between the groups were analysed using Pearson's chi-square test. One hundred and thirty-three anonymous responses were received. The respondents were neurologists across all levels of training and practice. Across all respondents, the most common factor of high importance was intellectual content of specialty, challenging diagnostic problems, type of patient encountered and interest in helping people. Responses were similar across the groups; however, the earliest trainees cited interest in helping people as most important, while those in neurology training and beyond cite intellectual content of the specialty as most important. As trainees transition from their earliest levels of clinical experience into working as residents and faculty, there is a shift in the cited important factors. Lifestyle and financial factors seem to be the least motivating across all groups. Encouragement from peers, mentors, faculty and practicing physicians is considered high influences in a smaller number of neurologists. This may present an opportunity for practicing neurologists to make connections with medical students early in their education in an effort to encourage and mentor candidates.
Planas Vilà, Mercè
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
Cystic fibrosis has historically been considered a pulmonary disease, but with the increasing life expectancy of these patients, gastrointestinal manifestations are becoming more important. Furthermore, nutritional status is closely linked to pulmonary function and, thus, overall mortality. This article discusses gastrointestinal manifestations (which involve nutritional, pancreatic, hepatobiliary, and, in particular, gastrointestinal tract issues) of cystic fibrosis as well as management of the disease. In addition, the article discusses studies that have been critical to our understanding of gastrointestinal manifestations of cystic fibrosis. PMID:27330503
Hagen, Joshua W; Swoger, Jason M; Grandinetti, Lisa M
Awareness of the extraintestinal manifestations of Crohn disease is increasing in dermatology and gastroenterology, with enhanced identification of entities that range from granulomatous diseases recapitulating the underlying inflammatory bowel disease to reactive conditions and associated dermatoses. In this review, the underlying etiopathology of Crohn disease is discussed, and how this mirrors certain skin manifestations that present in a subset of patients is explored. The array of extraintestinal manifestations that do not share a similar pathology, but which are often seen in association with inflammatory bowel disease, is also discussed. Treatment and pathogenetic mechanisms, where available, are discussed. Copyright © 2015 Elsevier Inc. All rights reserved.
Full Text Available Moyamoya disease is a rare neurological condition that affects children and adults of all ages. It is characterized by chronic, progressive stenosis of the circle of Willis that ultimately leads to the development of extensive collateral vessels. Presenting symptoms are usually due to cerebral ischemia or hemorrhage. The Japanese term moyamoya (meaning puffy or obscure was coined to describe the characteristic ‘smoke in the air’ appearance of these vessels on cerebral angiography. Moyamoya has the highest recorded incidence in Japan (0.28 per 100,000. In the west it is an extremely rare condition with an overall incidence of (0.086 per 100,000 in the Western United States. Etiology for the most part is unknown; however, genetic susceptibility related to RNF213 gene on chromosome 17q25.3 has been suggested. Moyamoya is being diagnosed more frequently in all races with varying clinical manifestations. Moyamoya disease is a rare progressive neurologic condition characterized by occlusion of the cerebral circulation with extensive collaterals recruitment in children and adults. Distinguished radiological findings confirm the diagnosis. Early recognition and swift institution of therapy is vital in order to minimize neurological deficits. We present the case of a 19-year-old African American female who presented with left-sided parastheia, weakness, and headache for 2 days duration.
Sebastián, Ugarte Ubiergo; Ricardo, Arenas Villamizar Angel; Alvarez, Bruno C; Cubides, Angela; Luna, Angélica F; Arroyo-Parejo, Max; Acuña, Cayri E; Quintero, Agamenón V; Villareal, Orlando Ch; Pinillos, Oscar S; Vieda, Elías; Bello, Manuel; Peña, Susana; Dueñas-Castell, Carmelo; Rodriguez, Gloria M V; Ranero, Jorge L M; López, Rosa L M; Olaya, Sandra G; Vergara, José C; Tandazo, Ana; Ospina, Juan P S; Leyton Soto, Igor M; Fowler, R A; Marshall, John C
Zika virus (ZIKAV) is classically described as causing minor symptoms in adult patients, however neurologic complications have been recognized. The recent outbreak in Central and South America has resulted in serious illness in some adult patients. We report adult patients in Latin America diagnosed with ZIKAV infection admitted to Intensive Care Units (ICUs). Multicenter, prospective case series of adult patients with laboratory diagnosis of ZIKAV in 16 ICUs in 8 countries. Between December 1st 2015 and April 2nd 2016, 16 ICUs in 8 countries enrolled 49 critically ill patients with diagnosis of ZIKAV infection. We included 10 critically ill patients with ZIKAV infection, as diagnosed with RT-PCR, admitted to the ICU. Neurologic manifestations concordant with Guillain-Barre Syndrome (GBS) were present in all patients, although 2 evolved into an encephalitis-like picture. 2 cases died, one due to encephalitis, the other septic shock. Differing from what was usually reported, ZIKAV infection can result in life-threatening neurologic illness in adults, including GBS and encephalitis. Collaborative reporting to identify severe illness from an emerging pathogen can provide valuable insights into disease epidemiology and clinical presentation, and inform public health authorities about acute care priorities. Copyright © 2017 Elsevier Inc. All rights reserved.
... of diagnostic imaging techniques and chemical and metabolic analyses to detect, manage, and treat neurological disease. Some ... performed in a doctor’s office or at a clinic. Fluoroscopy is a type of x-ray that ...
Rosińska, Justyna; Łukasik, Maria; Kozubski, Wojciech
The diver's nervous system is extremely sensitive to high ambient pressure, which is the sum of atmospheric and hydrostatic pressure. Neurological complications associated with diving are a difficult diagnostic and therapeutic challenge. They occur in both commercial and recreational diving and are connected with increasing interest in the sport of diving. Hence it is very important to know the possible complications associated with this kind of sport. Complications of the nervous system may result from decompression sickness, pulmonary barotrauma associated with cerebral arterial air embolism (AGE), otic and sinus barotrauma, high pressure neurological syndrome (HPNS) and undesirable effect of gases used for breathing. The purpose of this review is to discuss the range of neurological symptoms that can occur during diving accidents and also the role of patent foramen ovale (PFO) and internal carotid artery (ICA) dissection in pathogenesis of stroke in divers. Copyright © 2014 Polish Neurological Society. Published by Elsevier Urban & Partner Sp. z o.o. All rights reserved.
J Gordon Millichap
Full Text Available Smallpox and smallpox vaccination is reviewed from the Departments of Neurology, Yale University School of Medicine, New Haven, CT, and University of New Mexico School of Medicine, Albuquerque.
Goodman, Jerry Clay
Obesity has attained pandemic proportions, and bariatric surgery is increasingly being employed resulting in turn to more neurological complications which must be recognized and managed. Neurological complications may result from mechanical or inflammatory mechanisms but primarily result from micro-nutritional deficiencies. Vitamin B12, thiamine, and copper constitute the most frequent deficiencies. Neurological complications may occur at reasonably predictable times after bariatric surgery and are associated with the type of surgery used. During the early post-operative period, compressive or stretch peripheral nerve injury, rhabdomyolysis, Wernicke's encephalopathy, and inflammatory polyradiculoneuropathy may occur. Late complications ensue after months to years and include combined system degeneration (vitamin B12 deficiency) and hypocupric myelopathy. Bariatric surgery patients require careful nutritional follow-up with routine monitoring of micronutrients at 6 weeks and 3, 6, and 12 months post-operatively and then annually after surgery and multivitamin supplementation for life. Sustained vigilance for common and rare neurological complications is essential.
Sufferers from neurologic and psychiatric disorders are not uncommonly defendants in criminal trials. This chapter surveys a variety of different ways in which neurologic disorder bears on criminal responsibility. It discusses the way in which a neurologic disorder might bear on the questions of whether or not the defendant acted voluntarily; whether or not he or she was in the mental state that is required for guilt for the crime; and whether or not he or she is deserving of an insanity defense. The discussion demonstrates that a just determination of whether a sufferer from a neurologic disorder is diminished in his or her criminal responsibility for harmful conduct requires equal appreciation of the nature of the relevant disorder and its impact on behavior, on the one hand, and of the legal import of facts about the psychologic mechanisms through which behavior is generated, on the other. © 2013 Elsevier B.V. All rights reserved.
Richards, Mary R.; Plummer, Lacey; Chan, Yee-Ming; Lippincott, Margaret F.; Quinton, Richard; Kumanov, Philip; Seminara, Stephanie B.
Background A constellation of neurodegenerative disorders exist (Gordon Holmes syndrome, 4H leukodystrophy, Boucher Neuhauser syndrome) in which patients suffer from both neurologic disease (typically manifested by ataxia) and reproductive failure (hypogonadotropic hypogonadism (IHH)). POLR3B, which encodes the second largest subunit of RNA polymerase III (pol III), and POLR3A, which forms the pol III catalytic center, are associated with 4H leukodystrophy. Methods Whole exome sequencing was performed on a large cohort of subjects with IHH (n = 565). Detailed neuroendocrine studies were performed in some individuals within this cohort. Results Four individuals (2 of them siblings) were identified with two rare nucleotide variants in POLR3B. On initial evaluation, all subjects were free of neurologic disease. One patient underwent treatment with exogenous pulsatile GnRH for 8 weeks which failed to result in normalization of his sex steroid milieu due to pituitary resistance. Conclusions These findings suggest that the spectrum of phenotypes resulting from POLR3B mutations is wider than previously believed and that POLR3B can be associated exclusively with disorders characterized by abnormal gonadotropin secretion. PMID:27512013
Full Text Available Objective: To chronicle the history of medicine and neurology in India with a focus on its establishment and evolution. Background: The history of neurology in India is divided into two periods: ancient and modern. The ancient period dates back to the mid-second millennium Before Christ (B.C. during the creation of the Ayurvedic Indian system of Medicine, which detailed descriptions of neurological disorders called Vata Vyadhi. The early 20 th century witnessed the birth of modern Indian medicine with the onset of formal physician training at the nation′s first allopathic medical colleges located in Madras (1835, Calcutta (1835 and Mumbai (1848. Prior to India′s independence from Britain in 1947, only 25 medical schools existed in the entire country. Today, there are over 355. In 1951, physicians across the field of neurology and neurosurgery united to create the Neurological Society of India (NSI. Four decades later in 1991, neurologists branched out to establish a separate organization called the Indian Academy of Neurology (IAN. Design/Methods: Information was gathered through literature review using PubMed, MD Consult, OVID, primary texts and research at various academic institutions in India. Results: Neurological disorders were first described in ancient India under Ayurveda. The transition to modern medicine occurred more recently through formal training at medical schools beginning in the 1930′s. Early pioneers and founders of the NSI (1951 include Dr. Jacob Chandy, Dr. B Ramamurthi, Dr. S. T. Narasimhan and Dr. Baldev Singh. Later, Dr. J. S. Chopra, a prominent neurologist and visionary, recognized the need for primary centers of collaboration and subsequently established the IAN (1991. The future of Neurology in India is growing rapidly. Currently, there are 1100 practicing neurologists and more than 150 post-graduate trainees who join the ranks every year. As the number of neurologists rises across India, there is an increase in
Mishra, Shrikant; Trikamji, Bhavesh; Singh, Sandeep; Singh, Parampreet; Nair, Rajasekharan
Objective: To chronicle the history of medicine and neurology in India with a focus on its establishment and evolution. Background: The history of neurology in India is divided into two periods: ancient and modern. The ancient period dates back to the mid-second millennium Before Christ (B.C.) during the creation of the Ayurvedic Indian system of Medicine, which detailed descriptions of neurological disorders called Vata Vyadhi. The early 20th century witnessed the birth of modern Indian medicine with the onset of formal physician training at the nation's first allopathic medical colleges located in Madras (1835), Calcutta (1835) and Mumbai (1848). Prior to India's independence from Britain in 1947, only 25 medical schools existed in the entire country. Today, there are over 355. In 1951, physicians across the field of neurology and neurosurgery united to create the Neurological Society of India (NSI). Four decades later in 1991, neurologists branched out to establish a separate organization called the Indian Academy of Neurology (IAN). Design/Methods: Information was gathered through literature review using PubMed, MD Consult, OVID, primary texts and research at various academic institutions in India. Results: Neurological disorders were first described in ancient India under Ayurveda. The transition to modern medicine occurred more recently through formal training at medical schools beginning in the 1930's. Early pioneers and founders of the NSI (1951) include Dr. Jacob Chandy, Dr. B Ramamurthi, Dr. S. T. Narasimhan and Dr. Baldev Singh. Later, Dr. J. S. Chopra, a prominent neurologist and visionary, recognized the need for primary centers of collaboration and subsequently established the IAN (1991). The future of Neurology in India is growing rapidly. Currently, there are 1100 practicing neurologists and more than 150 post-graduate trainees who join the ranks every year. As the number of neurologists rises across India, there is an increase in the amount of
Full Text Available Aims: To evaluate the prevalence and characteristics of different neurological and psychiatric presentations in patients admitted to hospital with systemic lupus erythematosus (SLE. Materials and Methods: In this retrospective hospital-based study, we examined the medical records of patients with SLE who were referred to the hospitals affiliated to Shiraz University of Medical Sciences from 1995 to 2005. All patients of SLE who had clinical neurological or psychiatric features were included in this study. The patients′ demographic data, findings on general examination, neuropsychiatric manifestations, and the results of laboratory investigations and imaging studies were recorded. Clinical manifestations were classified according to the American College of Rheumatology (ACR case definitions. Results: Of the 407 study patients, 11.3% had neuropsychiatric complications. The most frequent findings were seizure (63%, headache (60%, and decreased level of consciousness (50%. Cerebrovascular disease (28.3%, seizure disorder (26.5%, and acute confusional state (19.6% were the most prevalent syndromes. Conclusion: The prevalence and nature of different neurological presentations of SLE in Iranian patients has some similarities to that seen in other populations, as well as some differences. Ethnic and environmental factors may contribute to these differences.
Tan, C X W; de Boer, N K H; Brand, H S
Gastrointestinal symptoms are predominant in Crohn's disease. Oral manifestations may also occur. The prevalence of oral manifestations varies between 0.5% and 37%. The manifestations may coincide with or precede gastrointestinal symptoms, and can be subdivided into specific and non-specific lesions. In most patients, lesions are asymptomatic but some patients experience serious discomfort. Oral manifestations can be classified as specific lesions, such as diffuse lip and buccal swelling and cobblestones, and non-specific lesions, such as aphthous ulcers, pyostomatitis vegetans, caries, gingivitis and periodontitis. In many patients, these oral symptoms do not cause pain or discomfort and do not require treatment. For patients who do experience discomfort, pain caused by aphthous ulcers, for example, can be relieved with a lidocaine solution or a 0.1% dexamethasone gel, and corticosteroids can be used to treat pain caused by ulceration or cobblestoning. It is advisable in complex cases to consult the patient's gastroenterologist.
Cahali Juliana Burihan; Kakuda Everton Yuji Soyama; Santi Cláudia Giuli; Maruta Celina Wakisaka
Nail involvement in pemphigus vulgaris is rare. We describe 5 patients with pemphigus vulgaris presenting nail involvement. In this disease, nail manifestations present, by order of frequency, as chronic paronychia, onychomadesis, onycholysis, Beau's lines and trachyonychia. All our 5 cases presented with paronychia, and 1 of them also had Beau's lines. Treatment with prednisone and/or cyclophosphamide controlled mucocutaneous and nail manifestations in all cases. O acometimento ungueal no...
Full Text Available Involving systemic autoimmune diseases, they primarily affect the joints, muscles and connective tissues. Cardiovascular impairment is often common in these disease manifestations ranging from asymptomatic to life-situations in danger. Otherwise impaired cardiovascular reason may be the first presentation. This may require aggressive therapy immunosuppressed, therefore the diagnosis is very important for a good choice of therapy. This article discusses the cardiovascular manifestations of systemic autoimmune diseases, mainly rheumatic diseases, focusing on diagnosis and manangement cardiovascular implications.
Alina-Costina LUCA; Constantin IORDACHE; Mariana PĂGUȚE
Involving systemic autoimmune diseases, they primarily affect the joints, muscles and connective tissues. Cardiovascular impairment is often common in these disease manifestations ranging from asymptomatic to life-situations in danger. Otherwise impaired cardiovascular reason may be the first presentation. This may require aggressive therapy immunosuppressed, therefore the diagnosis is very important for a good choice of therapy. This article discusses the cardiovascular manifestations of sys...
Ivanović Mirjana; Jovičić Olivera; Mandić Jelena; Bogetić Duško; Maddalone Marcello
Acute leukaemia is the most common form of chilhood cancer. The aim of this paper was to underline the importance of oral manifestations in children with acute leukaemia. The disease and its treatment can directly or indirectly affect oral health. Oral manifestations are gingival inflammation and enlargement. Leukaemic cells are capable of infiltrating the gingiva and the deeper periodontal tissues which leads to ulceration and infection of oral tissues. Gingival bleeding is a common si...
Pappu, Ramesh; Jabbour, Serge A; Regianto, Antonio M; Reginato, Antonio J
Primary hyperparathyroidism (PHPT) can be associated with a variety of musculoskeletal complaints, which occasionally can be the leading or presenting manifestation. In this paper, we describe the musculoskeletal manifestations observed in patients with primary hyperparathyroidism. Medical record reviews of a select population of 74 patients with primary hyperparathyroidism are seen in a rheumatology practice. Bone manifestations included back pain in 11 patients (15.2 %), generalized bone pain in 7 patients (9.7 %), rib cage/chest pain in 6 (8.3 %), pseudoclubbing in 3, and a giant cell tumor of the mandible in 2 (2.3 %) patients. Articular manifestations such as chondrocalcinosis with or without apatite deposition disease were seen in 13 (17.7 %), arthralgias in 11 (15.2 %), and non-specific synovitis in 7 (9.7 %). Muscle weakness was observed in six patients (8.3 %) and myalgias in three (4.6 %). Less common manifestations such as Achilles tendon rupture, Jaccoud-like arthropathy, sacral insufficiency fracture, arthritis associated with fever of unknown origin (FUO), meningitis, cervical cord compression, and persistent headache were observed in single patients. Musculoskeletal findings are still a frequent and important presentation in patients with primary hyperparathyroidism seen in rheumatology practice. Some of these manifestations can be quite unusual and may represent diagnostic dilemmas to the practicing rheumatologist and/or endocrinologist.
Full Text Available Background: Diabetes mellitus affects individuals of all ages and socioeconomic status. Skin is affected by the acute metabolic derangements as well as by chronic degenerative complications of diabetes. Aims: To evaluate the prevalence of skin manifestations in patients with diabetes mellitus. To analyze the prevalence and pattern of skin disorders among diabetic patients from this region of Western Himalayas. Materials and Methods: One hundred consecutive patients with the diagnosis of diabetes mellitus and having skin lesions, either attending the diabetic clinic or admitted in medical wards were included in this study. Results: The common skin disorders were: x0 erosis (44%, diabetic dermopathy (36%, skin tags (32%, cutaneous infections (31%, and seborrheic keratosis (30%. Conclusion: Skin is involved in diabetes quite often and the manifestations are numerous. High prevalence of xerosis in our diabetic population is perhaps due to cold and dry climatic conditions in the region for most of the time in the year.
Cosano-Quero, A; Velasco-Tirado, V; Sánchez Seco, M P; Manzanedo-Bueno, L; Belhassen-García, M
Zika virus infection should be suspected in travelers or immigrants with the signs or symptoms of a viral infection (rash, fever, joint pains, conjunctivitis, headache, etc.) and a compatible epidemiological history. Although cutaneous manifestations are among the most common clinical signs of Zika, they are not specific and very few images are available. We present 3 patients (2 travelers and 1 immigrant) in whom a rash was the presenting manifestation of Zika virus infection. Prompt diagnosis optimizes outcomes in these patients, improves the management of severe disease, and minimizes the risk of local transmission by Aedes albopictus, now a potential local vector for the virus due to its presence in areas along Spain's Mediterranean coast. Copyright © 2017 AEDV. Publicado por Elsevier España, S.L.U. All rights reserved.
Full Text Available Acromegaly and gigantism are due to excess GH production, usually as a result of a pituitary adenoma. The incidence of acromegaly is 5 cases per million per year and the prevalence is 60 cases per million. Clinical manifestations in each patient depend on the levels of GH and IGF-I, age, tumor size, and the delay in diagnosis. Manifestations of acromegaly are varied and include acral and soft tissue overgrowth, joint pain, diabetes mellitus, hypertension, and heart and respiratory failure. Acromegaly is a disabling disease that is associated with increased morbidity and reduced life expectancy. The diagnosis is based primarily on clinical features and confirmed by measuring GH levels after oral glucose loading and the estimation of IGF-I. It has been suggested that the rate of mortality in patients with acromegaly is correlated with the degree of control of GH. Adequately treated, the relative mortality risk can be markedly reduced towards normal.
Kurek, Nicholas S.; Chandra, Sathees B., E-mail: email@example.com [Department of Biological, Chemical and Physical Sciences, Roosevelt University, Chicago, IL (United States)
Nanotechnology involves probing and manipulating matter at the molecular level. Nanotechnology based molecular diagnostics have the potential to alleviate the suffering caused by many diseases, including neurological disorders, due to the unique properties of nanomaterials. Most neurological illnesses are multifactorial conditions and many of these are also classified as neurobehavioral disorders. Alzheimer's disease, Parkinson's disease, Huntington disease, cerebral ischemia, epilepsy, schizophrenia and autism spectrum disorders like Rett syndrome are some examples of neurological disorders that could be better treated, diagnosed, prevented and possibly cured using nanotechnology. In order to improve the quality of life for disease afflicted people, a wide range of nanomaterials that include gold and silica nanoparticles, quantum dots and DNA along with countless other forms of nanotechnology have been investigated regarding their usefulness in advancing molecular diagnostics. Other small scaled materials like viruses and proteins also have potential for use as molecular diagnostic tools. Information obtained from nanotechnology based diagnostics can be stored and manipulated using bioinformatics software. More advanced nanotechnology based diagnostic procedures for the acquisition of even greater proteomic and genomic knowledge can then be developed along with better ways to fight various diseases. Nanotechnology also has numerous applications besides those related to biotechnology and medicine. In this article, we will discuss and analyze many novel nanotechnology based diagnostic techniques at our disposal today. (author)
McGeachan, Alexander J; Mcdermott, Christopher J
Sialorrhoea is a common and problematic symptom that arises from a range of neurological conditions associated with bulbar or facial muscle dysfunction. Drooling can significantly affect quality of life due to both physical complications such as oral chapping, and psychological complications such as embarrassment and social isolation. Thicker, tenacious oral and pharyngeal secretions may result from the drying management approach to sialorrhoea. The management of sialorrhoea in neurological diseases depends on the underlying pathology and severity of symptoms. Interventions include anticholinergic drugs, salivary gland-targeted radiotherapy, salivary gland botulinum toxin and surgical approaches. The management of thick secretions involves mainly conservative measures such as pineapple juice as a lytic agent, cough assist, saline nebulisers and suctioning or mucolytic drugs like carbocisteine. Despite a current lack of evidence and variable practice, management of sialorrhoea should form a part of the multidisciplinary approach needed for long-term neurological conditions. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.
Bhattacharya, Basabdatta; Cochran, Amy
This book presents the latest research in computational methods for modeling and simulating brain disorders. In particular, it shows how mathematical models can be used to study the relationship between a given disorder and the specific brain structure associated with that disorder. It also describes the emerging field of computational psychiatry, including the study of pathological behavior due to impaired functional connectivity, pathophysiological activity, and/or aberrant decision-making. Further, it discusses the data analysis techniques that will be required to analyze the increasing amount of data being generated about the brain. Lastly, the book offers some tips on the application of computational models in the field of quantitative systems pharmacology. Mainly written for computational scientists eager to discover new application fields for their model, this book also benefits neurologists and psychiatrists wanting to learn about new methods.
Full Text Available Background: Computed tomography (CT used in pediatric pediatrics brain injury (TBI to ascertain neurological manifestations. Nevertheless, this practice is associated with adverse effects. Reports in the literature suggest incidents of morbidity and mortality in children due to exposure to radiation. Hence, it is found imperative to search for a reliable alternative. Objectives: The aim of this study is to find a reliable clinical alternative to detect an intracranial injury without resorting to the CT. Materials and Methods: Retrospective cross-sectional study was undertaken in patients (1-14 years with blunt head injury and having a Glasgow Coma Scale (GCS of 13-15 who had CT performed on them. Using statistical analysis, the correlation between clinical examination and positive CT manifestation is analyzed for different age-groups and various mechanisms of injury. Results: No statistically significant association between parameteres such as Loss of Consciousness, ′fall′ as mechanism of injury, motor vehicle accidents (MVA, more than two discrete episodes of vomiting and the CT finding of intracranial injury could be noted. Analyzed data have led to believe that GCS of 13 at presentation is the only important clinical predictor of intracranial injury. Conclusion: Retrospective data, small sample size and limited number of factors for assessing clinical manifestation might present constraints on the predictive rule that was derived from this review. Such limitations notwithstanding, the decision to determine which patients should undergo neuroimaging is encouraged to be based on clinical judgments. Further analysis with higher sample sizes may be required to authenticate and validate findings.
Al Bashapshe Ali
Full Text Available A 40 year old woman presented with a short history of acute onset of breathlessness to the ER of our hospital and after initial evaluation for acute pulmonary embolism which was ruled out after carrying out the appropriate investigations, she was diagnosed to be afflicted with syringomyelia based on her neurological symptoms and clinical findings, which was confirmed by doing an MRI scan, which was her basic diagnosis that was complicated by acute hypercapnic respiratory failure. This case is being reported to highlight syringomyelia as an unusual cause of acute respiratory failure, which manifested clinically in this patient as its first presentation and the underlying neurological diagnosis has been found to be present in very few reported cases (less than 0.01% of case reports in the available literature as the basic disease in the absence of its classical presenting features. Problems associated with acute respiratory failure in the setting of syringomyelia are discussed.
Martinho Pimenta, A J; Castelo Branco, N A
Mood and behavioral abnormalities are the most common early findings related to vibroacoustic disease (VAD). Other signs and symptoms have been observed in VAD patients. Brain MRI discloses small multifocal lesions in about 50% of subjects with more than 10 yr of occupational exposure to large pressure amplitude (> or = 90 dB SPL) and low frequency (< or = 500 Hz) (LPALF) noise. However, to date, there have been no studies globally integrating all the neurological, imaging and neurophysiological data of VAD patients. This is the main goal of this study. The 60 male Caucasians diagnosed with VAD were neurologically evaluated in extreme detail in order to systematically identify the most common and significant neurological disturbances in VAD. This population demonstrates cognitive changes (identified through psychological and neurophysiological studies (ERP P300)), vertigo and auditory changes, visual impairment, epilepsy, and cerebrovascular diseases. Neurological examination reveals pathological signs and reflexes, most commonly the palmo-mental reflex. A vascular pattern underlying the multifocal hyperintensities in T2 MR imaging, with predominant involvement of the small arteries of the white matter, is probably the visible organic substratum of the neurological picture. However, other pathophyisological mechanisms are involved in epileptic symptomatology.
Aldrete, J A
Of late, regional anesthesia has enjoyed unprecedented popularity; this increase in cases has brought a higher frequency of instances of neurological deficit and arachnoiditis that may appear as transient nerve root irritation, cauda equina, and conus medullaris syndromes, and later as radiculitis, clumped nerve roots, fibrosis, scarring dural sac deformities, pachymeningitis, pseudomeningocele, and syringomyelia, etc., all associated with arachnoiditis. Arachnoiditis may be caused by infections, myelograms (mostly from oil-based dyes), blood in the intrathecal space, neuroirritant, neurotoxic and/or neurolytic substances, surgical interventions in the spine, intrathecal corticosteroids, and trauma. Regarding regional anesthesia in the neuroaxis, arachnoiditis has resulted from epidural abscesses, traumatic punctures (blood), local anesthetics, detergents, antiseptics or other substances unintentionally injected into the spinal canal. Direct trauma to nerve roots or the spinal cord may be manifested as paraesthesia that has not been considered an injurious event; however, it usually implies dural penetration, as there are no nerve roots in the epidural space posteriorly. Sudden severe headache while or shortly after an epidural block using the loss of resistance to air approach usually suggests pneumocephalus from an intradural injection of air. Burning severe pain in the lower back and lower extremities, dysesthesia and numbness not following the usual dermatome distribution, along with bladder, bowel and/or sexual dysfunction, are the most common symptoms of direct trauma to the spinal cord. Such patients should be subjected to a neurological examination followed by an MRI of the effected area. Further spinal procedures are best avoided and the prompt administration of IV corticosteroids and NSAIDs need to be considered in the hope of preventing the inflammatory response from evolving into the proliferative phase of arachnoiditis.
The nervous system manifestations of HIV infection are protean. Initially assumed to be a late feature of HIV infection, neurologic involvement can occur quite early. Recognition and management of this condition can be very challenging. A review of the literature is presented. An extensive search of all materials related to the ...
Freeman, William D; Vatz, Kenneth A; Griggs, Robert C; Pedley, Timothy
The American Academy of Neurology Workforce Task Force (WFTF) report predicts a future shortfall of neurologists in the United States. The WFTF data also suggest that for most states, the current demand for neurologist services already exceeds the supply, and by 2025 the demand for neurologists will be even higher. This future demand is fueled by the aging of the US population, the higher health care utilization rates of neurologic services, and by a greater number of patients gaining access to the health care system due to the Patient Protection and Affordable Care Act. Uncertainties in health care delivery and patient access exist due to looming concerns about further Medicare reimbursement cuts. This uncertainty is set against a backdrop of Congressional volatility on a variety of issues, including the repeal of the sustainable growth rate for physician reimbursement. The impact of these US health care changes on the neurology workforce, future increasing demands, reimbursement, and alternative health care delivery models including accountable care organizations, nonphysician providers such as nurse practitioners and physician assistants, and teleneurology for both stroke and general neurology are discussed. The data lead to the conclusion that neurologists will need to play an even larger role in caring for the aging US population by 2025. We propose solutions to increase the availability of neurologic services in the future and provide other ways of meeting the anticipated increased demand for neurologic care.
Liao, Wei-Hua; Ramkalawan, Divya; Liu, Jian-Ling; Shi, Wei [Department of Radiology, Xiangya Hospital, Central South University, Changsha 410008, Hunan (China); Zee, Chi-Shing [Department of Radiology, Keck School of Medicine, University of Southern California, Los Angeles, CA 90033 (United States); Yang, Xiao-Su; Li, Guo-Liang; Li, Jing [Department of Neurology, Xiangya Hospital, Central South University, Changsha 410008, Hunan (China); Wang, Xiao-Yi, E-mail: firstname.lastname@example.org [Department of Radiology, Xiangya Hospital, Central South University, Changsha 410008, Hunan (China)
Background: Neurologic complications may be the first symptoms of atrial myxomas. Understanding the imaging features of neurologic complications of atrial myxomas can be helpful for the prompt diagnosis. Objective: To identify neuroimaging features for patients with neurologic complications attributed to atrial myxoma. Methods: We retrospectively reviewed the medical records of 103 patients with pathologically confirmed atrial myxoma at Xiangya Hospital from January 2009 to January 2014. The neuroimaging data for patients with neurologic complications were analyzed. Results: Eight patients with atrial myxomas (7.77%) presented with neurologic manifestations, which constituted the initial symptoms for seven patients (87.5%). Neuroimaging showed five cases of cerebral infarctions and three cases of aneurysms. The main patterns of the infarctions were multiplicity (100.0%) and involvement of the middle cerebral artery territory (80.0%). The aneurysms were fusiform in shape, multiple in number (100.0%) and located in the distal middle cerebral artery (100.0%). More specifically, high-density in the vicinity of the aneurysms was observed on CT for two patients (66.7%), and homogenous enhancement surrounding the aneurysms was detected in the enhanced imaging for two patients (66.7%). Conclusion: Neurologic complications secondary to atrial myxoma consist of cerebral infarctions and aneurysms, which show certain characteristic features in neuroimaging. Echocardiography should be performed in patients with multiple cerebral infarctions, and multiple aneurysms, especially when aneurysms are distal in location. More importantly, greater attention should be paid to the imaging changes surrounding the aneurysms when myxomatous aneurysms are suspected and these are going to be the relevant features in our article.
Card, TR; Langan, SM; Chu, TP
Extra-intestinal manifestations are well recognized in inflammatory bowel disease (IBD). To what extent the commonly recognized extra-intestinal manifestations seen in IBD patients are attributable to IBD is, however, not clear due to the limited number of controlled studies published. We have conducted a study of these manifestations using electronic primary care records. We have identified extra-intestinal manifestations in IBD and non-IBD patients and derived odds ratios (ORs) using condit...
Varol, Sefer; Ozdemir, Hasan Huseyin; Akil, Esref; Arslan, Demet; Aluclu, M Ufuk; Demir, Caner F; Yucel, Yavuz
Facial diplegia (FD) is a rare neurological manifestation with diverse causes. This article aims to systematically evaluate the etiology, diagnostic evaluation and treatment of FD. The study was performed retrospectively and included 17 patients with a diagnosis of FD. Patients were diagnosed with Guillain-Barré syndrome (GBS) (11), Bickerstaff's brainstem encephalitis (1), neurosarcoidosis (1), non-Hodgkin's Lymphoma (1), tuberculous meningitis (1) herpes simplex reactivation (1) and idiopathic (1). In addition, two patients had developed FD during pregnancy. Facial diplegia is an ominous symptom with widely varying causes that requires careful investigation.
Ramazanova, L Sh
Palinopsy or perseveration of a pre-seen image is impaired visual perception as preservation or repeated occurrence of a visual image of an object after its disappearance from the field of vision. Patients with palinopsy are rather frequently encountered in neurological practice; however, they may first visit an ophthalmologist in some cases. The author describes a few cases in which palinopsy was the first manifestation of cerebral arteriovenous malformation, ischemic stroke, and multiple sclerosis, as well as a case of visual perseverations while using narcotic drugs. The correct evaluation of a patient's status and the determination of the scope of additional studies are of great importance in this situation.
Full Text Available ABSTRACT Objective Facial diplegia (FD is a rare neurological manifestation with diverse causes. This article aims to systematically evaluate the etiology, diagnostic evaluation and treatment of FD. Method The study was performed retrospectively and included 17 patients with a diagnosis of FD. Results Patients were diagnosed with Guillain-Barré syndrome (GBS (11, Bickerstaff’s brainstem encephalitis (1, neurosarcoidosis (1, non-Hodgkin’s Lymphoma (1, tuberculous meningitis (1 herpes simplex reactivation (1 and idiopathic (1. In addition, two patients had developed FD during pregnancy. Conclusion Facial diplegia is an ominous symptom with widely varying causes that requires careful investigation.
Fallah-Nejad, M; Wallace, H W; Su, C C; Kutty, A C; Blakemore, W S
Penetrating cardiac injuries frequently first appear in an unusual and insidious manner, and their diagnosis may not be immediately obvious. In a series of 20 cases of cardiac injury, ten cases were indicative of such subtle symptoms, several of which were life-threatening. These unusual manifestations can be categorized as early, intermediate, or late. Early problems of four patients included the following: (1) sudden onset of shock during laparotomy, performed due to apparent abdominal trauma; (2) cardiac arrest on arrival in the emergency room; and (3) cerebral air embolus and mimicked symptoms of possible irreversible anoxic brain damage. The intermediate manifestations of cardiac injury are usually discovered in the early recovery period, and include myocardial infarction with cardiogenic shock and bullet embolus to a peripheral artery. Intermediate manifestations were observed in two patients. Four patients had late complications that included pseudoaneurysm, ventricular septal defect, valvular damage, and recurrent pericarditis. These late complications were observed between one month and 21 years after cardiac injury. This indicates the necessity of long-term follow-up of these patients.
Raymond J Dattwyler
Full Text Available Lyme disease, caused by the spirochete Borrelia burgdorferi, has classically been divided into three stages: erythema migrans; neurological or cardiac involvement; and arthritis. Rather than defining a set disease pattern, however, one should, more logically, conceptualize a progressive infection that may be localized or disseminated, acute or chronic. Erythema migrans, the earliest and most easily recognized manifestation of B burgdorferi infection, is an expanding annular erythematous skin lesion with a central clearing that develops soon after the bite of an infected ixodes tick. Musculoskeletal manifestations are common, with approximately one-half of untreated individuals developing arthritis. Of these, only 10% have chronic arthritis. Invasion of the central nervous system occurs as the infection disseminates hematogenously, with encephalitis, myelitis and meningopolyneuritis being the most severe results. Acute cardiac involvement is recognized in up to 8% of adult patients, and less often in children. Early antibiotic treatment of the infection is highly effective.
Schreiber, John; Chapman, Kimberly A; Summar, Marshall L; Ah Mew, Nicholas; Sutton, V Reid; MacLeod, Erin; Stagni, Kathy; Ueda, Keiko; Franks, Jill; Island, Eddie; Matern, Dietrich; Peña, Loren; Smith, Brittany; Urv, Tiina; Venditti, Charles; Chakarapani, Anupam; Gropman, Andrea L
Propionic acidemia (PA) is an organic acidemia which has a broad range of neurological complications, including developmental delay, intellectual disability, structural abnormalities, metabolic stroke-like episodes, seizures, optic neuropathy, and cranial nerve abnormalities. As the PA consensus conference hosted by Children's National Medical Center progressed from January 28 to 30, 2011, it became evident that neurological complications were common and a major component of morbidity, but the role of imaging and the basis for brain pathophysiology were unclear. This paper reviews the hypothesized pathophysiology, presentation and uses the best available evidence to suggest programs for treatment, imaging, and monitoring the neurological complications of PA. Copyright © 2011. Published by Elsevier Inc. All rights reserved.
Lee, Hyangsook; Park, Hi-Joon; Park, Jongbae; Kim, Mi-Ja; Hong, Meesuk; Yang, Jongsoo; Choi, Sunmi; Lee, Hyejung
Acupuncture has been widely used for a range of neurological disorders. Despite its popularity, the evidence to support the use of acupuncture is contradictory. This review was designed to summarize and to evaluate the available evidence of acupuncture for neurological disorders. Most of the reviewed studies suffer from lack of methodological rigor. Owing to paucity and poor quality of the primary studies, no firm conclusion could be drawn on the use of acupuncture for epilepsy, Alzheimer's disease, Parkinson's disease, ataxic disorders, multiple sclerosis, amyotrophic lateral sclerosis and spinal cord injury. For stroke rehabilitation, the evidence from recent high-quality trials and previous systematic reviews is not convincing. More rigorous trials are warranted to establish acupuncture's role in neurological disorders.
Quality of care in the context of inpatient neurology is the standard of performance by neurologists and the hospital system as measured against ideal models of care. There are growing regulatory pressures to define health care value through concrete quantifiable metrics linked to reimbursement. Theoretical models of quality acknowledge its multimodal character with quantitative and qualitative dimensions. For example, the Donabedian model distils quality as a phenomenon of three interconnected domains, structure-process-outcome, with each domain mutually influential. The actual measurement of quality may be implicit, as in peer review in morbidity and mortality rounds, or explicit, in which criteria are prespecified and systemized before assessment. As a practical contribution, in this article a set of candidate quality indicators for inpatient neurology based on an updated review of treatment guidelines is proposed. These quality indicators may serve as an initial blueprint for explicit quality metrics long overdue for inpatient neurology. Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.
Methods of computer technology (intelligent technology, IT), such as multimedia and virtual reality, are utilized more and more in all medical fields including child neurology. Advances in the digitalization of individual medical data and multi-media technology have enabled patients to be able to obtain their own medical data by small media and to receive medical treatment at any hospitals even if they are located in distance place. Changes from a doctor oriented to patients oriented medicine is anticipated. It is necessary to store medical data from birth to adulthood and to accumulate epidemiological data of rare diseases such as metabolic diseases or degenerative diseases especially in child neurology, which highly require tele medicine and telecare at home. Moreover, IT may improve in the QOL of patients with neurological diseases and of their families. Cooperation of medicine and engineering is therefore necessary. Results of our experiments on telemedicine, telecare and virtual reality are described.
Rondon-Berrios, Helbert; Argyropoulos, Christos; Ing, Todd S; Raj, Dominic S; Malhotra, Deepak; Agaba, Emmanuel I; Rohrscheib, Mark; Khitan, Zeid J; Murata, Glen H; Shapiro, Joseph I; Tzamaloukas, Antonios H
Hypertonicity causes severe clinical manifestations and is associated with mortality and severe short-term and long-term neurological sequelae. The main clinical syndromes of hypertonicity are hypernatremia and hyperglycemia. Hypernatremia results from relative excess of body sodium over body water. Loss of water in excess of intake, gain of sodium salts in excess of losses or a combination of the two are the main mechanisms of hypernatremia. Hypernatremia can be hypervolemic, euvolemic or hypovolemic. The management of hypernatremia addresses both a quantitative replacement of water and, if present, sodium deficit, and correction of the underlying pathophysiologic process that led to hypernatremia. Hypertonicity in hyperglycemia has two components, solute gain secondary to glucose accumulation in the extracellular compartment and water loss through hyperglycemic osmotic diuresis in excess of the losses of sodium and potassium. Differentiating between these two components of hypertonicity has major therapeutic implications because the first component will be reversed simply by normalization of serum glucose concentration while the second component will require hypotonic fluid replacement. An estimate of the magnitude of the relative water deficit secondary to osmotic diuresis is obtained by the corrected sodium concentration, which represents a calculated value of the serum sodium concentration that would result from reduction of the serum glucose concentration to a normal level. PMID:28101446
Fontanari, Daniele, E-mail: email@example.com [Département de physique, Université du Littoral – Côte d' Opale, 59140 Dunkerque (France); Fassò, Francesco, E-mail: firstname.lastname@example.org [Università di Padova, Dipartimento di Matematica, Via Trieste 63, Padova 35121 (Italy); Sadovskií, Dmitrií A., E-mail: email@example.com [Département de physique, Université du Littoral – Côte d' Opale, 59140 Dunkerque (France)
We uncover quantum manifestations of classical Nekhoroshev theory of resonant dynamics using a simple quantum system of two coupled angular momenta with conserved equal magnitudes which corresponds to a perturbed classical integrable anisochronous Hamiltonian system. - Highlights: • Basic quantum manifestations of classical Nekhoroshev theory are studied. • A simple anisochronous convex system with two degrees of freedom is proposed. • Zones are uncovered in the joint expectation value spectrum of quantized actions. • The width of the zones is given by the Nekhoroshev resonant normal forms.
A. A. Ziganshina
Full Text Available Objective: to highlight the current concepts of gastrointestinal manifestations of mitochondrial dysfunction. The data available in Russian and foreign literature on the gastrointestinal manifestations of mitochondrial dysfunction were analyzed. Functional digestive diseases are common in pediatric practice; however, their etiopathogenesis has not been adequately explored today. According to the literature, impaired cellular energy metabolism may underlie gastrointestinal motility disorders in cyclic vomiting syndrome, gastroesophageal reflux, gastric stasis, chronic diarrhea, constipation, intestinal pseudoobstruction, malabsorption syndrome, irritable bowel syndrome, as well as diseases of the liver and pancreas.
Dedania, Bhavtosh; Wu, George Y.
Hepatitis C virus (HCV) affects millions of people worldwide, and an estimated 3.2 million people in the United States. HCV is a hepatotropic and lymphotropic virus that causes not only liver disease, but also a significant number of extrahepatic manifestations (EHMs). Up to 74% of patients affected by HCV will have HCV-related EHMs of some severity in their lifetime. The EHMs vary from simple cutaneous palpable purpura to complex lymphoproliferative disorders, including lymphomas and immune-complex deposit diseases causing local and/or systemic complications. Mixed cryoglobulinemia (MC) is manifested by multiple systemic organ involvement, mainly skin, kidney, peripheral nerves, and salivary glands, and less frequently causes widespread vasculitis and malignant lymphoma. MC affects up to 3% of HCV-infected patients with cryoglobulinemia of clinical significance, i.e. >6%. Severe disease requires immunosuppressive or plasma exchange therapy. HCV prevalence in the United States in patients with porphyria cutanea tarda (PCT) was reported to be 66%, much higher than that in general population. Therefore, all patients with PCT should be screened for HCV. The skin rash of PCT varies from large blisters to small vesicles and/or milia on the hands. Skin manifestations due to PCT usually respond to anti‐HCV treatment together with reducing skin sun exposure, avoiding triggers, having routine phlebotomy (especially for people with chronic iron overload states), and using chloroquine. Lichen planus (LP), which typically affects both the skin and oral mucosa is a chronic inflammatory disease of squamous cell origin affecting about 1% of the worldwide population. The prevalence of HCV in patients with LP varies based on geographic location. We review here the basic pathophysiology, clinical features, and management of dermatologic manifestations of HCV. PMID:26357639
Heinzen, Erin L.; Arzimanoglou, Alexis; Brashear, Allison; Clapcote, Steven J.; Gurrieri, Fiorella; Goldstein, David B.; Jóhannesson, Sigurður H.; Mikati, Mohamad A.; Neville, Brian; Nicole, Sophie; Ozelius, Laurie J.; Poulsen, Hanne; Schyns, Tsveta; Sweadner, Kathleen J.; van den Maagdenberg, Arn; Vilsen, Bente
Genetic research has shown that mutations that modify the protein-coding sequence of ATP1A3, the gene encoding the α3 subunit of Na+/K+-ATPase, cause both rapid-onset dystonia parkinsonism and alternating hemiplegia of childhood. These discoveries link two clinically distinct neurological diseases to the same gene, however, ATP1A3 mutations are, with one exception, disease-specific. Although the exact mechanism of how these mutations lead to disease is still unknown, much knowledge has been gained about functional consequences of ATP1A3 mutations using a range of in vitro and animal model systems, and the role of Na+/K+-ATPases in the brain. Researchers and clinicians are attempting to further characterise neurological manifestations associated with mutations in ATP1A3, and to build on the existing molecular knowledge to understand how specific mutations can lead to different diseases. PMID:24739246
Ourad, Wassila; Chaïbi, Pascal; Thomas, Caroline; Lahlou, Amina; Hached, Djoher; Bussy, Caroline; Piette, François; Robain, Gilberte
PROBLEMS OF THE PROSTATE: Benign hypertrophy of the prostate (BHP), when it occurs, is manifested by an obstruction or irritation related to overactivity of the bladder. The obstructive syndrome is defined by urodynamic tests. Urge incontinence and study of the pressure-flow ratio are the tests of choice. The functional handicap and impact on quality of life are assessed using the International Prostatism Symptoms Score (I-PSS). Efficient and fairly well tolerated medical treatment has reduced the indications for surgery. It relies on alpha-blockers, 5a-reductase inhibitors and phytotherapy. When indicated, the surgical treatment of choice is endoscopic resection of the prostate. Among the non-prostatic micturition disorders, urge micturition with, in extreme cases, incontinence are due to detrusor instability. This is of multifactor origin; enhanced by the local irritation or environmental factors, it usually occurs within a context of acute or chronic pathologies. Treatment is recommended with anticholinergic agents. New molecules have recently been launched, better tolerated than oxybutinine. Electrostimulation can be a good alternative in mentally normal patients. Micturition due to excess urine may be due to overactivity of the bladder, the major risk of which is acute urine retention. It can also be observed during neurological affections such as Parkinson's disease or during administration of certain drugs. Nocturnal polyuria is a frequent problem. However, simple hygiene and dietary measures and the control of certain concomitant diseases can usually relieve the symptoms. Medical treatment relies on desmopressine.
Breuer, M.E.; Koopman, W.J.H.; Koene, S.; Nooteboom, M.; Rodenburg, R.J.T.; Willems, P.H.G.M.; Smeitink, J.A.M.
The development of neurologic disease is a complex and multi-faceted process. Several factors, such as physiology, environment and genetics may play key roles in the manifestation of the associated illnesses. During the past decades, it has become clear that, at the cellular level, mitochondria
Yang, Kee Hyuk; Choi, Yo Won; Jeon, Seok Chol; Park, Choong Ki; Joo, Kyung Bin; Hahm, Chang Kok; Lee, Seung Ro [College of Medicine, Hanyang Univ., Seoul (Korea, Republic of)
Pulmonary involvement is more common in systemic lupus erythematosus (SLE) than in any other connective tissue disease, and more than half of patients with SLE suffer from respiratory dysfunction during the course of their illness. Although sepsis and renal disease are the most common causes of death in SLE, lung disease is the predominant manifestation and is an indicator of overall prognosis. Respiratory disease may be due to direct involvement of the lung or as a secondary consequence of the effect of the disease on other organ systems.
Salvatore Calabrò, Rocco; Gervasi, Giuseppe; Naro, Antonino; De Luca, Rosaria; Marullo, Michelangelo; Bramanti, Placido
Objective: Neurogenic erectile dysfunction can be broadly defined as an inability to sustain or maintain a penile erection due to neurologic impairment. Sexual problems can occur due to any lesion affecting the central and peripheral nervous system. The aim of this study was to evaluate the prevalence and causes of erectile dysfunction in a group of hospital inpatients suffering from neurologic disorders.
Johnson, Jennifer A; Le, Kenneth L; Palacios, Enrique
We describe late-onset propionic acidemia in a 12-year-old boy who presented with vomiting, cough, and fever, and manifested a precipitous decline in mental status, accompanied by acute encephalopathy and severe neurologic damage, with bilateral basal ganglia involvement upon neuroimaging. He exhibited metabolic acidosis, hyperammonemia, hypocarnitinemia, and elevated plasma glycine. Urinary organic-acid analysis demonstrated very highly elevated 3-hydroxypropionate, propionylglycine, methylcitrate, and tiglylglycine, without an elevation of methylmalonate. Despite intensive medical care, this particular case proved fatal, highlighting the importance of metabolic testing in cases of acute mental-status changes and encephalopathy of unknown etiology.
Schmitt, W H; Yanuck, S F
Functional Neurologic Assessment and treatment methods common to the practice of applied kinesiology are presented. These methods are proposed to enhance neurological examination and treatment procedures toward more effective assessment and care of functional impairment. A neurologic model for these procedures is proposed. Manual assessment of muscular function is used to identify changes associated with facilitation and inhibition, in response to the introduction of sensory receptor-based stimuli. Muscle testing responses to sensory stimulation of known value are compared with usually predictable patterns based on known neuroanatomy and neurophysiology, guiding the clinician to an understanding of the functional status of the patient's nervous system. These assessment procedures are used in addition to other standard diagnostic measures to augment rather than replace the existing diagnostic armamentarium. The proper understanding of the neurophysiologic basis of muscle testing procedures will assist in the design of further investigations into applied kinesiology. Accordingly, the neurophysiologic basis and proposed mechanisms of these methods are reviewed.
Kundoor Vinay Kumar Reddy
Full Text Available Cowden's syndrome is an autosomal dominant genodermatosis with variable orofacial and systemic manifestations. Here we present one such classical case of Cowden's syndrome in a 45-year-old female patient with features such as multiple cutaneous papillomatosis, oral fibromas, and fibromas involving multiple organs such as gastrointestinal tract (multiple polyps, thyroid disorders, and breast cancer.
Hélio Afonso Ghizoni Teive
Full Text Available Edgar Allan Poe was one of the most celebrated writers of all time. He published several masterpieces, some of which include references to neurological diseases. Poe suffered from recurrent depression, suggesting a bipolar disorder, as well as alcohol and drug abuse, which in fact led to his death from complications related to alcoholism. Various hypotheses were put forward, including Wernicke's encephalopathy.
Teive, Hélio Afonso Ghizoni; Paola, Luciano de; Munhoz, Renato Puppi
Edgar Allan Poe was one of the most celebrated writers of all time. He published several masterpieces, some of which include references to neurological diseases. Poe suffered from recurrent depression, suggesting a bipolar disorder, as well as alcohol and drug abuse, which in fact led to his death from complications related to alcoholism. Various hypotheses were put forward, including Wernicke's encephalopathy.
Proprioceptive reflexes play an important role during the control of movement and posture. Disturbed modulation of proprioceptive reflexes is often suggested as the cause for the motoric features present in neurological disorders. In this thesis methods are developed and evaluated to quantify
de Frutos González, V; Guerrero Peral, A L
In medical medieval literature some works about dietetics stand out. Dietetics, as a separate branch of medicine, includes not only food or drinks, but other environmental factors influencing on health. They are known as regimina sanitatis or salutis, and specially developed in the Christian west. They generally consisted of a balance between the Galenic "six non-natural things"; factors regulating health and its protection: environment, exercise, food, sleep, bowel movements and emotions. After reviewing the sources and defining the different stages of this genre, we have considered three of the most out-standing medieval regimina, the anonymous Regimen sanitatis salernitanum, Arnaldo de Vilanova's Regimen sanitatis ad regem aragonum and Bernardo de Gordon's Tractatus of conservatione vite humane. In them we review references to neurological disease. Though not independently considered, there is a significant presence of neurological diseases in the regimina. Dietetics measures are proposed to preserve memory, nerves, or hearing, as well as for the treatment of migraine, epilepsy, stroke or dizziness. Regimina are quiet representative among medical medieval literature, and they show medieval physicians vision of neurological diseases. Dietetics was considered useful to preserve health, and therapeutics was based on natural remedies. 2010 Sociedad Española de Neurología. Published by Elsevier Espana. All rights reserved.
Maas, Matthew B.; Coleman, Mary; Jozefowicz, Ralph; Engstrom, John
Objective: To assess the strengths and weaknesses of neurology resident education using survey methodology. Methods: A 27-question survey was sent to all neurology residents completing residency training in the United States in 2011. Results: Of eligible respondents, 49.8% of residents returned the survey. Most residents believed previously instituted duty hour restrictions had a positive impact on resident quality of life without impacting patient care. Most residents rated their faculty and clinical didactics favorably. However, many residents reported suboptimal preparation in basic neuroscience and practice management issues. Most residents (71%) noted that the Residency In-service Training Examination (RITE) assisted in self-study. A minority of residents (14%) reported that the RITE scores were used for reasons other than self-study. The vast majority (86%) of residents will enter fellowship training following residency and were satisfied with the fellowship offers they received. Conclusions: Graduating residents had largely favorable neurology training experiences. Several common deficiencies include education in basic neuroscience and clinical practice management. Importantly, prior changes to duty hours did not negatively affect the resident perception of neurology residency training. PMID:23091077
Iacovou, Emily; Vlastarakos, Petros V; Papacharalampous, George; Kampessis, George; Nikolopoulos, Thomas P
Almost 30 years after its first description, HIV still remains a global pandemic. The present paper aims to review the current knowledge on the ear, nose and throat (ENT) manifestations of HIV infection, and present the available diagnostic and treatment options. A literature review was conducted in Medline and other available database sources. Information from related books was also included in the data analysis. It is well acknowledged that up to 80% of HIV-infected patients eventually develop ENT manifestations; among which, oral disease appears to be the most common. Oro-pharyngeal manifestations include candidiasis, periodontal and gingival disease, HSV and HPV infection, oral hairy leucoplakia, Kaposi's sarcoma, and non-Hodgkin's lymphoma. ENT manifestations in the neck can present as cervical lymphadenopathy or parotid gland enlargement. Respective nasal manifestations include sinusitis (often due to atypical bacteria), and allergic rhinitis. Finally, otological manifestations include otitis (externa, or media), inner ear involvement (sensorineural hearing loss, disequilibrium), and facial nerve palsy (up to 100 times more frequently compared to the general population). Although ENT symptoms are not diagnostic of the disease, they might be suggestive of HIV infection, or related to its progression and the respective treatment failure. ENT doctors should be aware of the ENT manifestations associated with HIV disease, and the respective diagnosis and treatment. A multi-disciplinary approach may be required to provide the appropriate level of care to HIV patients.
Full Text Available Almost 30 years after its first description, HIV still remains a global pandemic. The present paper aims to review the current knowledge on the ear, nose and throat (ENT manifestations of HIV infection, and present the available diagnostic and treatment options. A literature review was conducted in Medline and other available database sources. Information from related books was also included in the data analysis. It is well acknowledged that up to 80% of HIV-infected patients eventually develop ENT manifestations; among which, oral disease appears to be the most common. Oro-pharyngeal manifestations include candidiasis, periodontal and gingival disease, HSV and HPV infection, oral hairy leucoplakia, Kaposi’s sarcoma, and non- Hodgkin’s lymphoma. ENT manifestations in the neck can present as cervical lymphadenopathy or parotid gland enlargement. Respective nasal manifestations include sinusitis (often due to atypical bacteria, and allergic rhinitis. Finally, otological manifestations include otitis (externa, or media, inner ear involvement (sensorineural hearing loss, disequilibrium, and facial nerve palsy (up to 100 times more frequently compared to the general population. Although ENT symptoms are not diagnostic of the disease, they might be suggestive of HIV infection, or related to its progression and the respective treatment failure. ENT doctors should be aware of the ENT manifestations associated with HIV disease, and the respective diagnosis and treatment. A multi-disciplinary approach may be required to provide the appropriate level of care to HIV patients.
Almeida Leila Azevedo de; Hueb João Carlos; Silva Marcos Augusto de Moraes; Bazan Rodrigo; Estrozi Bruna; Raffin Cesar N.
Infartos cerebrais de etiologia cardíaca são observados em cerca de 20% dos pacientes com acidente vascular cerebral isquêmico. Infarto cerebral ocorre como manifestação clínica inicial em um terço dos casos de mixoma atrial. Embora quase metade dos pacientes com mixoma atrial apresente alteração ao exame neurológico, infarto cerebral não hemorrágico é visto na tomografia computadorizada em praticamente todos os casos. Os autores apresentam o caso de uma paciente, cuja primeira manifestação c...
van Lingen, Christiaan P.; Ettema, Harmen B.; Van Der Straeten, Catherine; Kollen, Bouwdewijn J.; Verheyen, Cees C. P. M.
Adverse reactions to metal particle debris have been increasingly reported as a complication following large head metal-on-metal (MoM) hip arthroplasty. Elevated metal ion levels are a cause for concern. The aim of this study is to evaluate whether exposure to cobalt is associated with patient
Full Text Available HTLV-1 is the causal agent of HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP, a disease observed in up to 5% of individuals infected with HTLV-1. However, infected individuals without the disease can present neurological complaints relating to sensory, motor or urinary manifestations. The aim of this study was to investigate the incidence of neurological manifestations among patients with HTLV-1. Method HTLV-1 patients in Salvador, Bahia, Brazil, were enrolled into a cohort study. Results Among 414 subjects, 76 had definite and 87 had possible or probable HAM/TSP at the baseline, whereas 251 subjects had no neurological signs or symptoms. Definite HAM/TSP developed in 5 patients (1.74%. The asymptomatic subjects were selected for analysis. The incidence rate expressed per 1,000 persons-year was calculated. It was 206 for hand numbness, 129 for nocturia and 126 for urinary urgency. In the neurological examination, leg hyperreflexia presented an average incidence rate of 76; leg paraparesis, 52; and Babinski sign, 36. Kaplan-Meyer curves categorized according to gender and proviral load showed that females and patients with proviral load of more than 100,000 copies per 106 peripheral blood mononuclear cells (PBMCs presented higher risk. Conclusion Development of neurological symptoms or signs occurred in up to 30% of asymptomatic subjects during 8 years of follow-up. Female gender and high proviral load were risk factors for neurological disease.
Bassetti, C.L.; Ferini-Strambi, L.; Brown, S.; Adamantidis, A.; Benedetti, F.; Bruni, O.; Cajochen, C.; Dolenc-Groselj, L.; Ferri, R.; Gais, S.; Huber, R.; Khatami, R.; Lammers, G.J.; Luppi, P.H.; Manconi, M.; Nissen, C.; Nobili, L.; Peigneux, P.; Pollmacher, T.; Randerath, W.; Riemann, D.; Santamaria, J.; Schindler, K.; Tafti, M.; van Someren, E.J.W.; Wetter, T.C.
In recent years, evidence has emerged for a bidirectional relationship between sleep and neurological and psychiatric disorders. First, sleep-wake disorders (SWDs) are very common and may be the first/main manifestation of underlying neurological and psychiatric disorders. Secondly, SWDs may
Bassetti, C L; Ferini-Strambi, L; Brown, S; Adamantidis, A; Benedetti, F; Bruni, O; Cajochen, C; Dolenc-Groselj, L; Ferri, R; Gais, S; Huber, R; Khatami, R; Lammers, G J; Luppi, P H; Manconi, M; Nissen, C; Nobili, L; Peigneux, P; Pollmächer, T; Randerath, W; Riemann, D; Santamaria, J; Schindler, K; Tafti, M; Van Someren, E; Wetter, T C
In recent years, evidence has emerged for a bidirectional relationship between sleep and neurological and psychiatric disorders. First, sleep-wake disorders (SWDs) are very common and may be the first/main manifestation of underlying neurological and psychiatric disorders. Secondly, SWDs may
Wang, Hong; Siddharthan, Venkatraman; Kesler, Kyle K; Hall, Jeffery O; Motter, Neil E; Julander, Justin G; Morrey, John D
Neurological respiratory insufficiency strongly correlates with mortality among rodents infected with West Nile virus (WNV), which suggests that this is a primary mechanism of death in rodents and possibly fatal West Nile neurological disease in human patients. To explore the possibility that neurological respiratory insufficiency is a broad mechanism of death in cases of viral encephalitis, plethysmography was evaluated in mice infected with 3 flaviviruses and 2 alphaviruses. Pathology was investigated by challenging the diaphragm, using electromyography with hypercapnia and optogenetic photoactivation. Among infections due to all but 1 alphavirus, death was strongly associated with a suppressed minute volume. Virally infected mice with a very low minute volume did not neurologically respond to hypercapnia or optogenetic photoactivation of the C4 cervical cord. Neurons with the orexin 1 receptor protein in the ventral C3-5 cervical cord were statistically diminished in WNV-infected mice with a low minute volume as compared to WNV-infected or sham-infected mice without respiratory insufficiency. Also, WNV-infected cells were adjacent to neurons with respiratory functions in the medulla. Detection of a common neurological mechanism of death among viral encephalitides creates opportunities to create broad-spectrum therapies that target relevant neurological cells in patients with types of viral encephalitis that have not been treatable in the past.
N. V. Vakhnina
Full Text Available Neurological disorders in hypertensive patients can be caused by both brain injury and concomitant diseases. The elucidation of the causes of neurological disorders and their effective treatment contribute to hypertensive patients’ better adherence to long-term antihypertensive therapy, which normalizes blood pressure (BP and reduces the risk of cerebral complications Objective: to study of the causes of neurological disorders in hypertensive patients and the efficiency of their correction using a new dispersible vinpocetine formulation (Cavinton® Comforte in combined therapy.Patients and methods. A total of 80 patients (men (20% and women (80%; mean age 63±12.3 years with neurological complaints in the presence of hypertension were examined. All the patients were diagnosed with dyscirculatory encephalopathy or chronic brain ischemia, whether they had vascular cognitive impairment. The examination of patients revealed that the neurological complaints were mainly due to concomitant diseases, such as migraine (12%, tension-type headache (66%, and the latter concurrent with migraine (4%.Results and discussion. The effective treatment of concomitant diseases in combination with antihypertensive therapy contributed to normalization of BP and regression of complaints. The most pronounced effect was noted in 40 patients whose combination therapy included Vinpocetine (Cavinton® Comforte 10 mg thrice daily.Conclusion. The therapy resulted in the less severity of both the symptoms of cerebrovascular disease (vascular cognitive impairment and comorbid neurological disorders (headache, dizziness, etc..
Tsutsumi, Satoshi; Ogino, Ikuko; Miyajima, Masakazu; Arai, Hajime; Ito, Masanori; Yasumoto, Yukimasa
Cerebral cavernous malformations (CCMs) are a distinct cerebrovascular disease. A fraction of CCMs present as diffuse manifestations distributed over the cerebral hemispheres, cerebellum, and brainstem. The purpose of the present study was to explore the clinical picture of such CCMs. This study assessed the appearance of CCMs on magnetic resonance (MR) images, the presence of genetic mutations using the polymerase chain reaction method, and disease course over long-term follow-up in a total of 10 patients with diffuse CCMs. The 10 patients were Japanese and comprised 5 males and 5 females with a mean age of 48.7years. Three of them presented with seizures, two with headache and intracerebral hemorrhage, two with numbness, and one with dizziness, while the remaining two were asymptomatic. Genetic analysis revealed CCM1 mutations in four patients, CCM2 mutations in three, and a CCM3 mutation in one. In a family with 2 CCM2 patients, the appearance of sustained diffuse CCMs on MR images significantly differed between the 2 patients despite the mutation being identical. During the mean follow-up period of 13.7years, none of the 10 patients showed evidence of neurological deterioration or symptomatic hemorrhage. The appearance of their CCMs on MRI did not show significant changes. Eight patients maintained normal neurological function. CCMs with diffuse manifestation is a hereditary disease with satisfactory prognosis. Unrecognized genomic mutations may be involved in the genesis of these CCMs. Copyright © 2016 Elsevier B.V. All rights reserved.
Full Text Available Cerebral venous thrombosis (CVT has a wide spectrum of clinical manifestations that may mimic many other neurological disorders and lead to misdiagnoses. Headache is the most common symptom and may be associated with other symptoms or remain isolated. The other frequent manifestations are focal neurological deficits and diffuse encephalopathies with seizures. The key to the diagnosis is the imaging of the occluded vessel or of the intravascular thrombus, by a combination of magnetic resonance imaging (MRI and magnetic resonance venography (MRV. Causes and risk factors include medical, surgical and obstetrical causes of deep vein thrombosis, genetic and acquired prothrombotic disorders, cancer and hematological disorders, inflammatory systemic disorders, pregnancy and puerperium, infections and local causes such as tumors, arteriovenous malformations, trauma, central nervous system infections and local infections. The breakdown of causes differs in different parts of the world. A meta-analysis of the most recent prospectively collected series showed an overall 15% case-fatality or dependency rate. Heparin therapy is the standard therapy at the acute stage, followed by 3-6 months of oral anticoagulation. Patients with isolated intracranial hypertension may require a lumbar puncture to remove cerebrospinal fluid before starting heparin when they develop a papilloedema that may threaten the visual acuity or decompressive hemicraniectomy. Patients who develop seizures should receive antiepileptic drugs. Cerebral venous thrombosis - even pregnancy-related - should not contraindicate future pregnancies. The efficacy and safety of local thrombolysis and decompressive hemicraniectomy should be tested
To present the cinematographic production of Arthur Van Gehuchten (1861-1914) and to put this collection into its medical and sociocultural context. The arrival of Edison's Kinetoscope (1891) and Lumière's Cinématographe (1895) provoked the immediate interest of neurologists who foresaw the potential of motion pictures for illustration, research, and teaching. Arthur Van Gehuchten, professor of anatomy and neurology at the Catholic University of Louvain, was trained as a microscopist and a cytologist. From neuroanatomy, he progressively broadened his interest to neurology. Van Gehuchten was an avant-garde teacher, eager to adopt new visual aids. In 1895, he attended the first cinematographic screenings. Medical cinematography was soon brought into disrepute in European academic circles, when films made by the French surgeon Doyen were copied and shown on fairgrounds. Nevertheless, in 1905, Van Gehuchten began to film neurologic patients. He used this technique extensively to demonstrate clinical signs, to illustrate neurologic diseases, and to document functional evolution following surgery. For decades, these films were screened for medical students by Van Gehuchten's successors to the chair of neurology. The original nitrate films (more than 2 hours) have been recently rediscovered. They have been restored by the Royal Belgian Film Archive, where they are the oldest Belgian films. At the beginning of the 20th century, Van Gehuchten built up a collection of moving pictures for teaching purposes. This was one of the first such undertakings. This unique set of films has miraculously survived, and serves as an important archive of nervous diseases and their manifestations prior to the advent of modern therapies.
Kuan, Edward C; Peng, Kevin A; Suh, Jeffrey D; Bergsneider, Marvin; Wang, Marilene B
Cushing disease is a relatively rare cause of Cushing syndrome secondary to a hyperfunctioning pituitary adenoma. In addition to signs and symptoms of hypercortisolism, Cushing disease may present with diverse otolaryngic manifestations, which may guide diagnosis and management. We performed a retrospective chart review of patients who were found to have Cushing disease and who underwent transnasal transsphenoidal surgery for pituitary adenomas between January 1, 2007, and July 1, 2014, at a tertiary academic medical center. There were 37 consecutive patients in this series with Cushing disease caused by a pituitary adenoma. Fifteen (41%) patients complained of visual changes. Five (14%) patients suffered from obstructive sleep apnea. Four (11%) patients had thyroid disease. Other symptoms included hearing loss, vertigo, tinnitus, epistaxis, dysphagia, and salivary gland swelling. Although Cushing disease traditionally presents with classic "Cushingoid" systemic features, it also may present with various otolaryngic manifestations. A thorough workup by otolaryngologists is critical in the comprehensive management of these patients.
Delair, Emmanuelle; Latkany, Paul; Noble, A Gwendolyn; Rabiah, Peter; McLeod, Rima; Brézin, Antoine
Clinical manifestations of ocular toxoplasmosis are reviewed. Findings of congenital and acute acquired ocular toxoplasmosis include retinal scars, white-appearing lesions in the active phase often associated with vitritis. Complications can include fibrous bands, secondary serous or rhegmatogenous retinal detachments, optic neuritis and neuropathy, cataracts, increased intraocular pressure during active infection, and choroidal neovascular membranes. Recurrences in untreated congenital toxoplasmosis occur in teenage years. Manifestations at birth are less severe, and recurrences are fewer in those who were treated promptly early in the course of their disease in utero and in the first year of life. Severe retinal involvement is common at diagnosis of symptomatic congenital toxoplasmosis in the United States and Brazil. Acute acquired infections also may be complicated by toxoplasmic retinochoroiditis, with recurrences most common close to the time of acquisition. Suppressive treatment can reduce recurrent disease.
Month to years after an early local or an early disseminated infection some patients develop late manifestations of lyme borreliosis. Most frequently involved organs are the skin (acrodermatitis chronica atrophicans), joints (Lyme arthritis) and the nervous system. A history of exposure and the clinical picture may suggest Lyme borreliosis, however, confirmation by serological and other tests is needed. Antibiotic treatment during early stages normally prevents development of late manifestations. Late stages persist if not treated. By adequate antimicrobial therapy they are treatable and usually show a good prognosis. Recovery may be delayed, some patients suffer from residual difficulties. Currently there is no accepted case definition for a "post lyme syndrome". The term "chronic Lyme disease" suggests (a never proven) persistent infection by viable bacteria. Repeated and prolonged antibiotic treatments are not indicated.
Full Text Available The Japanese subscribe to ethnic nationalism, which is an ideology with the aim to develop an ethnically exclusive and homogeneous nationhood. One manifestation of ethnic nationalism is the belief that Japan is, or should be, a mono-ethnic society. Ethnic nationalism is manifested in the exclusionary attitude or opinion of the Japanese. In the context of foreigners living in Japan, the exclusionary attitude or opinion of the Japanese often translates into the insistence that foreigners should do things the Japanese way. This is unfriendly to foreigners living in Japan, to say the least. This article illustrates how unwelcoming and inconvenient Japanese exclusionism can be by using two examples that directly affect foreigners: housing and discrimination against foreigners.
Full Text Available Only few cases with sacral radiculitis due to infection with Borrelia burgdorferi leading to neurogenic urinary dysfunction have been reported. A 57-year-old male developed urethral pain and urinary retention, requiring permanent catheterization. Extensive urological investigations did not reveal a specific cause, which was why neurogenic bladder dysfunction was suspected. Neurologic exam revealed only mildly reduced tendon reflexes. Cerebral and spinal MRI were noninformative. CSF investigations, however, revealed pleocytosis, elevated protein, and antibodies against Borrelia burgdorferi. Intravenous ceftriaxone for three weeks resulted in immediate improvement of bladder dysfunction, with continuous decline of residual urine volume and continuous increase of spontaneous urine volume even after removal of the catheter and initiation of self-catheterization. Sacral radiculitis due to infection with Borrelia burgdorferi is a potential cause of detrusor areflexia and urethral, perineal, inguinal, and scrotal pain and may be misinterpreted as cystitis or urethritis. Ceftriaxone may result in progressive recovery of bladder dysfunction and pain. Neuroborreliosis may manifest exclusively as neurourological problem.
Sreedhar, Krishna Prasad
SUMMARY Using a vernacular adaptation of MAS 50 bronchial asthma patients were compared with 102 normals, 60 hospital general out-patients and 50 neurotics to determine the level of anxiety in asthma. The manifest anxiety scores of the bronchial asthma patients were found to be significantly high showing that their level of anxiety was abnormally higher in comparison with that of the normals and the hospital general out-patients. The bronchial asthmatics and the neurotics did not differ in an...
Ashraf, Said; Briasoulis, Alexandros; Afonso, Luis
Cardiac sarcoidosis (CS) is found in 2-7% of patients with systemic sarcoidosis (SS). Its diagnosis and treatment is challenging, notwithstanding the poor prognosis and treatment. Hereby, we present a case of systemic sarcoidosis with rare cardiac manifestations of severe mitral incompetence and large coronary aneurysm in a previously healthy woman. She underwent successful mitral valve replacement and coronary artery bypass surgery and was maintained on low dose glucocorticoid therapy. Copyright © 2016. Published by Elsevier Inc.
Said A. Al-Busafi
Full Text Available The portal hypertension is responsible for many of the manifestations of liver cirrhosis. Some of these complications are the direct consequences of portal hypertension, such as gastrointestinal bleeding from ruptured gastroesophageal varices and from portal hypertensive gastropathy and colopathy, ascites and hepatorenal syndrome, and hypersplenism. In other complications, portal hypertension plays a key role, although it is not the only pathophysiological factor in their development. These include spontaneous bacterial peritonitis, hepatic encephalopathy, cirrhotic cardiomyopathy, hepatopulmonary syndrome, and portopulmonary hypertension.
Although psychophysics has already been used in many neurological evaluations including the visual and hearing tests, the use of psychophysics has been limited to the evaluation of sensory disorders. In this review paper, however, the author introduced recent attempts to apply psychophysics to the evaluation of higher cognitive functions such as perception of scenes and facial expressions. Psychophysics was also used to measure visual hypersensitivity in a patient with migraine. The benefits of the use of psychophysics in neurological and neuropsychological settings would be as follows. (1) We can evaluate higher cognitive functions quantitatively. (2) We can measure performance both above and below the normal range by the same method. (3) We can use the same stimulus and task as other research areas such as neuroscience and neuroimaging, and compare results between research areas.
Piechowski-Jozwiak, Bartlomiej; Bogousslavsky, Julien
Visual art production involves multiple processes including basic motor skills, such as coordination of movements, visual-spatial processing, emotional output, sociocultural context, and creativity. Thus, the relationship between artistic output and brain diseases is particularly complex, and brain disorders may lead to impairment of artistic production in multiple domains. Neurological conditions may also occasionally modify artistic style and lead to surprisingly innovative features in people with an initial loss of creativity. This chapter focuses on anecdotal reports of various neurological disorders and their potential consequences on works produced by famous or well-established artists, including Carl Frederik Reutersward, Giorgio de Chirico, Krystyna Habura, Leo Schnug, Ignatius Brennan, and many others. © 2013 Elsevier B.V. All rights reserved.
Dierckx, Rudi A.J.O. [Groningen University Medical Center (Netherlands). Dept. of Nuclear Medicine and Molecular Imaging; Ghent Univ. (Belgium). Dept. of Radiology and Nuclear Medicine; Vries, Erik F.J. de; Waarde, Aren van [Groningen University Medical Center (Netherlands). Dept. of Nuclear Medicine and Molecular Imaging; Otte, Andreas (ed.) [Univ. of Applied Sciences Offenburg (Germany). Faculty of Electrical Engineering and Information Technology
PET and SPECT in Neurology highlights the combined expertise of renowned authors whose dedication to the investigation of neurological disorders through nuclear medicine technology has achieved international recognition. Classical neurodegenerative disorders are discussed as well as cerebrovascular disorders, brain tumors, epilepsy, head trauma, coma, sleeping disorders, and inflammatory and infectious diseases of the CNS. The latest results in nuclear brain imaging are detailed. Most chapters are written jointly by a clinical neurologist and a nuclear medicine specialist to ensure a multidisciplinary approach. This state-of-the-art compendium will be valuable to anybody in the field of neuroscience, from the neurologist and the radiologist/nuclear medicine specialist to the interested general practitioner and geriatrician. It is the second volume of a trilogy on PET and SPECT imaging in the neurosciences, the other volumes covering PET and SPECT in psychiatry and in neurobiological systems.
Teive, Hélio A G; Munhoz, Renato P; Cardoso, Francisco
Marcel Proust is one of the most important French writers of the 20th century. His relationship with medicine and with neurology is possibly linked to the fact that his asthma was considered to be a psychosomatic disease classified as neurasthenia. Stendhal's syndrome is a rare psychiatric syndrome characterized by anxiety and affective and thought disturbances when a person is exposed to a work of art. Here, the authors describe neurological aspects of Proust's work, particularly the occurrence of Stendhal's syndrome and syncope when he as well as one of the characters of In Search of Lost Time see Vermeer's View of Delft during a visit to a museum. © 2014 S. Karger AG, Basel.
Collado-Vázquez, Susana; Cano de la Cuerda, Roberto; Jiménez-Antona, Carmen
Cinema has been defined in many different ways, but most of them agree that it should be considered both a technique and an art. Although films often depict fantasy stories, in many cases they also reflect day-to-day realities. In its earliest days cinema was already attracted to the world of health and sickness, and frequently addressed topics like medical practice, how patients lived with their illnesses, bioethical issues, the relationship between physician and patient or research. To review the presence of neurological pathologies in the cinema with a view to identifying the main neurological disorders that have been portrayed in films. Likewise it also intends to describe the medical praxis that is employed, the relationship between physician and patient, how the experiences of the patient and the family are represented, the adaptation to social and occupational situations, and the intervention of other health care professionals related with neurological patients. Some of the most significant films that have addressed these topics were reviewed and it was seen that in some of them the illness is dealt with in a very true-to-life manner, whereas others tend to include a greater number of inaccuracies and a larger degree of fiction. Cinema has helped to shape certain ways of thinking about the health care professionals who work with neurological patients, the importance of support from the family and the social role, among other things. This confirms that resorting to cinematographic productions is a fruitful tool for stimulating a critical interest in the past and present of medical practice.
Cowan, W M; Kandel, E R
Neurological and psychiatric illnesses are among the most common and most serious health problems in developed societies. The most promising advances in neurological and psychiatric diseases will require advances in neuroscience for their elucidation, prevention, and treatment. Technical advances have improved methods for identifying brain regions involved during various types of cognitive activity, for tracing connections between parts of the brain, for visualizing individual neurons in living brain preparations, for recording the activities of neurons, and for studying the activity of single-ion channels and the receptors for various neurotransmitters. The most significant advances in the past 20 years have come from the application to the nervous system of molecular genetics and molecular cell biology. Discovery of the monogenic disorder responsible for Huntington disease and understanding its pathogenesis can serve as a paradigm for unraveling the much more complex, polygenic disorders responsible for such psychiatric diseases as schizophrenia, manic depressive illness, and borderline personality disorder. Thus, a new degree of cooperation between neurology and psychiatry is likely to result, especially for the treatment of patients with illnesses such as autism, mental retardation, cognitive disorders associated with Alzheimer and Parkinson disease that overlap between the 2 disciplines.
India. Key-words: Primary myxoma, intracranial tumor. ABSTRACT. Myxomas are benign primary tumors of the heart of mesenchymal origin. Neurological complications attributed to atrial myxoma occurs in 10% to 12% of patients, with ischemic presentation due to cerebral infarct in 83%-89% of cases. Few case reports are ...
Askeland, Eric J; Arlen, Angela M; Erickson, Bradley A; Mathews, Katherine D; Cooper, Christopher S
Duchenne muscular dystrophy is a dystrophinopathy affecting males that is associated with multiple organ system complications. To our knowledge urological complications of Duchenne muscular dystrophy have been described only anecdotally to date. We reviewed the medical charts of 135 patients with Duchenne or Duchenne-Becker muscular dystrophy for demographics and disease progression, urological diagnoses, intervention and followup. Of 135 patients 67 (50%) had at least 1 documented urological diagnosis and 38 (28%) had multiple manifestations. Lower urinary tract symptoms were the most common urological diagnosis (32% of patients). Survival analysis revealed a median age at onset of lower urinary tract symptoms of 23 years (95% CI 17.7-23.9). Intervention was required in 12 patients (9%), most commonly due to nephrolithiasis. Urological morbidity increased with Duchenne muscular dystrophy progression when stratified by clinical progression. Lower urinary tract symptoms were more common in nonambulatory patients (40.7% vs 19%, p = 0.007), those with a diagnosis of scoliosis (44% vs 19.7%, p = 0.003) and/or scoliosis spine surgery (60% vs 22%, p <0.001), and those on invasive respiratory support (53% vs 29%, p = 0.046). Likewise, nephrolithiasis was more common in nonambulatory patients (10% vs 0%, p = 0.017), those with scoliosis (12% vs 0%, p = 0.004) and/or scoliosis spine surgery (20% vs 1%, p <0.001), and those on invasive respiratory support (29% vs 3%, p <0.001). Only 28% of patients with a urological manifestation were referred to urology. As these patients transition into adolescence and adulthood, the increased prevalence of urological manifestations warrants increased awareness and referral to urologists. Copyright © 2013 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.
Stone, Jon; Hoeritzauer, Ingrid; Gelauff, Jeannette; Lehn, Alex; Gardiner, Paula; van Gils, Anne; Carson, Alan
Functional, often called psychogenic, disorders are common in neurological practice. We illustrate clinical issues and highlight some recent research findings using six case studies of functional neurological disorders. We discuss dizziness as a functional disorder, describing the relatively new
.... Clinical Trials in Neurology aims to improve the efficiency of clinical trials and the development of interventions in order to enhance the development of new treatments for neurologic diseases...
Iorio, Justin A; Reid, Patrick; Kim, Han Jo
The number of surgeries performed for adult spinal deformity (ASD) has been increasing due to an aging population, longer life expectancy, and studies supporting an improvement in health-related quality of life scores after operative intervention. However, medical and surgical complication rates remain high, and neurological complications such as spinal cord injury and motor deficits can be especially debilitating to patients. Several independent factors potentially influence the likelihood of neurological complications including surgical approach (anterior, lateral, or posterior), use of osteotomies, thoracic hyperkyphosis, spinal region, patient characteristics, and revision surgery status. The majority of ASD surgeries are performed by a posterior approach to the thoracic and/or lumbar spine, but anterior and lateral approaches are commonly performed and are associated with unique neural complications such as femoral nerve palsy and lumbar plexus injuries. Spinal morphology, such as that of hyperkyphosis, has been reported to be a risk factor for complications in addition to three-column osteotomies, which are often utilized to correct large deformities. Additionally, revision surgeries are common in ASD and these patients are at an increased risk of procedure-related complications and nervous system injury. Patient selection, surgical technique, and use of intraoperative neuromonitoring may reduce the incidence of complications and optimize outcomes.
Jorge Enrique Aponte
Full Text Available Systemic lupus erythematosus (SLE is an inflammatory disease with a wide range of clinical manifestations and complications related to disease activity. One of them is the increase risk to infections secondary to immunological alterations due to pharmacological therapy (especially steroids. Few reports have documented the association of SLE and meningococcal infection with subsequent development of immunological activation (continuous inflammation and intolerance of the immune system. The attempts to make an early and appropriate approach to these type of patients, generate benefits in survival rates and decrease sequelae among those who survive, especially in those in whose infection compromised central nervous system. We present the case of a patient that presented with neurological symptoms compatible with neuroeffector by Neisseria meningitis isolated in CSF cultures. Despite adequate antibiotic treatment the patient continued to deteriorate neurologically, and alternative diagnosis were evaluated after findings of vasculitis in brain CT scan. Immunological panel was performed with positivity of antibodies commonly present in SLE considering that infection by Neisseria meningitis was a trigger of immunological intolerance and development of SLE. We present the following case to understand the physiopathology and relationship between meningococcal infections, complement consumption, immunological intolerance and the development of autoimmune disease.
Full Text Available 【Abstract】Cervical spinal canal narrowing can lead to injury of the spinal cord and neurological symptoms in-cluding neck pain, headache, weakness and parasthesisas. According to previous and recent clinical researches, we investigated the geometric parameters of normal cervical spinal canal including the sagittal and transverse diameters as well as Torg ratio. The mean sagittal diameter of cervical spinal canal at C 1 to C 7 ranges from 15.33 mm to 20.46 mm, the mean transverse diameter at the same levels ranges from 24.45 mm to 27.00 mm and the mean value of Torg ratio is 0.96. With respect to narrow cervical spinal canal, the following charaterstics are found: firstly, extension of the cervical spine results in statistically significant stenosis as compared with the flexed or neutral positions; secondly, females sustain cervical spinal canal narrowing more easily than males; finally, the consistent narrowest cervical canal level is at C 4 for all ethnicity, but there is a slight variation in the sagittal diameter of cervical spinal stenosis (≤14 mm in Whites, ≤ 12 mm in Japanese, ≤13.7 mm in Chinese. Narrow sagittal cervical canal diameter brings about an increased risk of neurological injuries in traumatic, degenerative and inflam-matory conditions and is related with extension of cervical spine, gender, as well as ethnicity. It is hoped that this re-view will be helpful in diagnosing spinal cord and neuro-logical injuries with the geometric parameters of cervical spine in the future. Key words: Spinal cord injuries; Spinal stenosis; Trauma, nervous system
Comunicação e inclusão de crianças com alterações de linguagem de origem neurológica na perspectiva de pais e educadores Communication and inclusion of children with language disorders due to neurologic aspects from the perspective of parents and teachers
Érica Mayumi Takase
communication and inclusion of children with language disorders due to neurological causes require further attention, specifically in Speech-Language and Hearing Sciences. For the purpose of finding out how professionals in this field can contribute to the inclusion process, the aim of the study is to investigate the expectations, difficulties and support encountered by families and teachers of those children who undergo Speech-Language Pathology therapy. The corpus is composed of 11 children, 12 parents and 7 educators. The data was collected through the study of the children files, as well as from semi-structured interviews with families and teachers, video-recorded and transcribed orthographically. The data was analyzed according to references that support this study. The results show challenges in including some of the children in regular schools, particularly those with the most severe language disorders. There were also issues related to the preparation of the educators and the preparedness of some of the schools that received these students. Nevertheless, the study demonstrated that there have been advances, expressed by the inclusion of most of these children in regular schools and by greater willingness of the schools to receive these students. This finding was deduced from references to parents, educators and Speech-Language Pathologists sharing of information and experiences. From the perspective of the families and teachers, the findings point to the need for specialized health assistance and highlight the contribution of the Speech-Language and Hearing Sciences in communication and feeding in the process of inclusion.
The study demonstrated that the most common non- neurological symptoms was locomotor symptoms (24%) ,while the most common neurological symptoms were backache and neck pain .The most common neurological findings were cord compression (8%) followed by peripheral neuropathy (2%) and CVA (2%). 22% of ...
Full Text Available Hypoparathyroidism is a disorder of calcium and phosphorus metabolism due to decreased secretion of parathyroid hormone. Hypoparathyroidism can be hereditary and acquired. Acquired hypoparathyroidism usually occurs following neck surgery (thyroid surgery or parathyroid surgery. Along with systemic manifestations, hypoparathyroidism produces some skin manifestations. Lack of study regarding mucocutaneous manifestations of acquired hypoparathyroidism prompted us to undertake this study. To evaluate the mucocutaneous manifestations of acquired hypoparathyroidism. An observational study done in a tertiary care hospital of Kolkata by comprehensive history taking, through clinical examination and relevant laboratory investigations. Twenty-one patients were included in the study. The commonest form of acquired hypoparathyroidism was neck surgery (thyroidectomy and parathyroidectomy operation. Mucocutaneous manifestations were present in 76.19% of patients. The most frequent mucocutaneous manifestation was found in the hairs like the loss of axillary hair (61.9%, loss of pubic hair (52.38%, coarsening of body hair (47.62%, and alopecia areata (9.52%. The nail changes noted were brittle and ridged nail, followed by onycholysis, onychosezia, and onychomedesis. The most common skin features were xerotic skin in 11 patients (52.38%, followed by pellagra-like skin pigmentation, pustular psoriasis and acne form eruption, bullous impetigo, etc. Mucosa was normal in all the cases excepting the one which showed oral candidiasis.
Srivastava, Smita; Chadda, Rakesh Kumar; Bala, Kiran; Majumdar, Pradipta
Neurocysticercosis (NCC) is an endemic parasitic infection of Asia, Africa, Latin America, and central Europe. Neuropsychiatric manifestations of the illness include epilepsy and behavioral disturbances. There is a dearth of systematic studies on psychiatric manifestations of NCC from various Asian counties. The present study assessed the prevalence of various psychiatric disorders in a cohort of patients with NCC attending a neurological service. Detailed psychiatric assessment was carried out on 50 patients of NCC with epilepsy and 50 patients of epilepsy without any evidence of NCC. Comprehensive Psychopathological Rating Scale was used to elicit the symptoms. Cognitive functions were assessed using Mini Mental Status Examination. Psychiatric diagnoses were made as per International Classification of Diseases, 10(th) edition (ICD-10). Sixty eight percent of the patients with NCC suffered from a psychiatric disorder, as compared to 44% of those without NCC (P=0.02). Major depression and mixed anxiety depression were the two most common diagnoses. None of the patients was to found to suffer from a psychotic disorder. The most frequent site of brain lesion of NCC was the parietal lobe, followed by frontal lobes and disseminated lesions. Left sided lesions were associated with greater psychiatric morbidity. Focal seizures with or without secondary generalizations were present more frequently in patients with NCC whereas primary generalized seizures were more common in patients with idiopathic epilepsy (P=0.05). Psychiatric manifestations are more common in patients of epilepsy with NCC than those without NCC. The treating clinician need to be vigilant about the phenomenon.
Full Text Available Daniel P Schwartz, Matthew S RobbinsDepartment of Neurology, Montefiore Headache Center, Albert Einstein College of Medicine, Bronx, NY, USAAbstract: Headache is an extraordinarily common complaint presenting to medical practitioners in all arenas and specialties, particularly primary care physicians, neurologists, and ophthalmologists. A wide variety of headache disorders may manifest with a myriad of neuro-ophthalmologic symptoms, including orbital pain, disturbances of vision, aura, photophobia, lacrimation, conjunctival injection, ptosis, and other manifestations. The differential diagnosis in these patients is broad and includes both secondary, or symptomatic, and primary headache disorders. Awareness of the headache patterns and associated symptoms of these various disorders is essential to achieve the correct diagnosis. This paper reviews the primary headache disorders that prominently feature neuro-ophthalmologic manifestations, including migraine, the trigeminal autonomic cephalalgias, and hemicrania continua. Migraine variants with prominent neuro-ophthalmologic symptoms including aura without headache, basilar-type migraine, retinal migraine, and ophthalmoplegic migraine are also reviewed. This paper focuses particularly on the symptomatology of these primary headache disorders, but also discusses their epidemiology, clinical features, and treatment.Keywords: headache, migraine, trigeminal autonomic cephalalgias, neuro-ophthalmologic, aura, photophobia
Biase, Noemi Grigoletto De
Full Text Available Introduction: The post-polio syndrome (PPS is characterized by a new episod of atrophy or muscle weakness in individuals previously affected by poliomyelitis. The symptoms start from an extended period of clinical stability ranging from 20 to 40 years, after the initial chart of polio. Among the pathophysiological mechanisms, the most likely is that the syndrome represents a process of attrition and neuronal metabolic exhaustion due to a continuous process of denervation reinnervation which begins after the initial acute. Objective: Review the clinical characteristics and pathophysiological of PPS,as well as present the approach in cases with pharyngolaryngeal manifestations. Case Report: We present the clinical case of a male patient with 48 years old, with main complaints of dysphonia, dysphagia and previous history of polio. The patient underwent a diagnostic investigation, in which the voice psychoacoustic findings are described. Laringoscopic, electromyographic and the videoendoscopy of swallowing compatible with PPS. The treatment consisted in phonotherapy in a total of 11 weekly sessions and guidelines for swallowing, with satisfactory improvement of the symptoms. Final comments: The pharyngolaryngeal manifestations of PPS are susceptible to treatment, obtaining satisfactory results, with improvement of life quality of the patients.
Hespanhol, Fernando Luiz; Tinoco, Eduardo Muniz Barretto; Teixeira, Henrique Guilherme de Castro; Falabella, Márcio Eduardo Vieira; Assis, Neuza Maria de Souza Picorelli
Several changes in the oral cavity due to chemotherapy can be observed and can lead to important systemic complications, increasing the time of the patient in hospital and the costs of the treatment as well as affect the quality of life of the patients. The aim of this study was to assess the oral manifestation in patients treated with chemotherapy according to sex, age and tumor type. Data was collected in an oncology hospital in Juiz de Fora, Minas Gerais State, from patients' records that were submitted to oncologic treatment. It was possible to verify that mucositis, associated or not to other type of lesions, was the most common lesion in both sex of all ages (15.5%). Xerostomia and other lesions, such as Candida infection and aphthous lesions, were also present. It is possible to improve the quality of life of the patient during and after anti-neoplastic therapies through a protocol of odontological assistance that includes changes of the oral environment previous to chemotherapy such as profilaxis, caries removal, treatment of periodontal and periapical lesions, oral hygiene instructions, diet orientation and laser therapy. It is very important the insertion of the dentist in the oncologic medical team for the early diagnosis of the oral manifestation and follow-up during treatment time.
Full Text Available The Lichen planus is frequent dermatosis that only attack mucus with no skin manifestation. Its only location can be only upon oral mucus.The aim of the paper is to examine the oral localization incidence in all its key variants.From March, 1999, to December, 2002, 35 patients were examined, namely, 28 women and 7 men of 19 to 63 years of age. Of 6 variants of the Lichen planus oral manifestations 17 had an erosive-ulcerous form, 13 had a reticular one and 5 patients had an atrophic form.The erosive-ulcerous form that was dominant in the last few years in its clinic variants was diagnosed upon the byccal mucus, gingiva, tongue, hard palate, lips and mouth cavity floor.This form requires an adequate therapy regarding its chronic nature as well as frequent check-ups due to the possibility of the "lichen dysplasia". Similar treatment is required with the atrophic form while the reticular variant requires occasional controls with no specific therapy except for the one referring to everyday maintenance of good oral hygiene, caries elimination and periodontal diseases along with the hygienic-diet regime.
The Bible, a major pillar of Western Civilization consists of Hebrew Scriptures, assembled over a millennium and accepted as of divine origin. The Talmud is a compendium of Jewish laws, covering every possible aspect of life, analyzed in depth from 200 BCE to 600 CE, becoming the foundation of Jewish existence. The all-encompassing character of the books provides numerous medical problems and observations that appear in various connotations. When in need to clarify various legal dilemmas, the Talmudic sages displayed astoundingly accurate anatomical knowledge and were pioneers in clinical-pathological correlations. The descriptions of "neurological" events in the Bible are very precise but show no evidence of neurological knowledge. Those reported in the various tractates of the Talmud are evidence of a substantial medical knowledge, marked by Hellenistic influence. Subjects such as head and spinal injuries, epilepsy, handedness neuralgias aphasia tinnitus and tremor were discussed in depth. This chapter is an updated collection of the studies, extracting observations and discussions of neurological manifestations from the ancient texts.
Magner, Martin; Kolářová, Hana; Honzik, Tomáš; Švandová, Ivana; Zeman, Jiří
Mitochondrial disorders (MD) represent a clinically, biochemically and genetically heterogeneous group of diseases associated with dysfunction of the oxidative phosphorylation system and pyruvate dehydrogenase complex. Our aim was to illustrate the most common clinical presentation of MD on the example of selected diseases and syndromes. The minimal prevalence of MD is estimated as 1 to 5,000. MD may manifest at any age since birth until late-adulthood with acute manifestation or as a chronic progressive disease. Virtually any organ may be impaired, but the organs with the highest energetic demands are most frequently involved, including brain, muscle, heart and liver. Some MD may manifest as a characteristic cluster of clinical features (e.g. MELAS syndrome, Kearns-Sayre syndrome). Diagnostics includes detailed history, the comprehensive clinical examination, results of specialized examinations (especially cardiology, visual fundus examination, brain imaging, EMG), laboratory testing of body fluids (lactate, aminoacids, organic acids), and analysis of bioptic samples of muscle, skin, and liver, eventually. Normal lactate level in blood does not exclude the possibility of MD. Although the aimed molecular genetic analyses may be indicated in some of mitochondrial diseases, the methods of next generation sequencing come into focus. Examples of treatment are arginine supplementation in MELAS syndrome, ketogenic diet in pyruvate oxidation disorders or quinone analogs in patients with LHON. Conclusion: The clinical suspicion of a mitochondrial disorder is often delayed, or the disease remains undiagnosed. The correct diagnosis and adequate treatment can improve prognosis of the patient. Access to genetic counseling is also of great importance.
Căpraru, Cosette Laura; Dorobăţ, Carmen; Forna, Norina Consuela
Although the medical and biological importance of ringworm infections was acknowledged a long time ago, the putting-up-to-date of some clinical notions is justified for at least three reasons: the improvement and accessibility of molecular biology techniques, the consideration of the major pathogenic role of these viruses in circumstances of immune depression, the possibility to control these infections by means of specific chemotherapy. A prospective study was carried out in the interval 1.01.2006 - 31.12. 2008, of all cases of ringworm infections with oral eclat, hospitalised in Infectious Diseases Hospital Iaşi. The diagnosis was established according to the clinical manifestations, the hematological modifications and, if the case, serological examination. The study included 291 cases. The infection with EVB (infectious mononucleosis) was diagnosed within the age interval 8 months-60 years (median-10 years). The oral manifestations were mainly located at the level of the palatine and velopalatine vault. Chickenpox was diagnosed within the age interval 7 days-52 years (median-8 years). The chickenpox enantem was observed in 90.42% of the cases, being represented by varix wounds, or "hemorrhagic spots", mostly located at the level of the fibrous mucose membrane of the palatine vault--40.2%. The knowledge of the oral manifestations in ringworm infections with highly contagious potential and a high risk of severe complications represents a fundamental method in early diagnosis as well as an important step in establishing the prophylaxy measures meant to prevent these pathogens from being transmitted to the dentist office.
Full Text Available This paper presents a case of ocular changes in leishmaniasis in a dog brought in for examination because of visible changes in the eyes. An ocular manifestation in leishmaniasis can be bilateral or unilateral. Changes most often occur on the conjunctive and the front segment of the eye, the cornea and iris. From 70 to 80% of dogs diseased with leishmaniasis exhibit frontal uveitis, and dry eye, keratoconjunctivitis sicca, is also a frequent finding. Based on the clinical picture and the positive immunochromatographic rapid test it was established that the dog was diseased with leishmaniasis.
Full Text Available In Brazil soccer has assumed a role beyond a simple sports modality by becoming a social phenomenon. Historically soccer is perceptible from two different perspectives: as a means of transmitting ideology and as an important element of Brazilian culture. The aim of this bibliographical study is to discuss soccer as a cultural manifestation and, at some historical moments, as a mean of transmitting ideology. This discussion made it possible to notice that soccer provides subsidies for a better understanding of organizational and relational forms that permeate Brazilian society.
Andersen, Ellen Sloth; Kofoed, Kristian; Andersen, Mads Varis Nis
We describe two cases of neurosyphilis whose diagnosis was based on ocular symptoms. Both patients were treated in the Eye Department for bilateral uveitis, and they were tested serologically positive for syphilis. The diagnoses of neurosyphilis were confirmed by demonstration of CSF pleocytosis...... and specific intrathecal antibody production. Both patients were treated with 21 million units of penicillin IV daily for 15 days. Ocular syphilis is an unusual manifestation of the disease but should be considered in patients with uveitis of uncertain origin, especially if the patient has a rash and...
Grunwald, Michael R; McDonnell, Megan H; Induru, Raghava; Gerber, Jonathan M
Cutaneous complications are common in patients with leukemia. However, the cause is not always immediately clear, as there are often numerous potential etiologies. Thrombocytopenia or coagulopathy can result in ecchymoses or petechiae, whereas extramedullary (EM) involvement by leukemia can present as a rash. Leukemia can also result in skin manifestations via indirect means, including several types of paraneoplastic phenomena. Moreover, various agents routinely used to treat leukemia-most notably cytarabine (cytosine arabinoside)-can precipitate quite profound skin eruptions. Finally, infections, including fungal invasion of the skin, can be responsible for rashes, as can the vast array of antimicrobials that are administered to leukemia patients. Copyright © 2016. Published by Elsevier Inc.
Matsumoto, Tadahiko; Nishimoto, Katsutaro
The systemic mycoses are increasing in importance as opportunistic infections. Cutaneous lesions resulting from systemic mycoses may first alert clinicians to the presence of a life-threatening disorder, or even the presence of an unsuspected immunodeficiency state. Skin involvement is generally uncommon in disseminated aspergillosis, zygomycosis but is more common in systemic candidiasis (candidemia) and cryptococcosis. The blanket terms, hyalohyphomycosis and phaeohyphomycosis, cover the infections caused by diverse fungal opportunists. A variety of manifestations of skin lesions of the systemic mycoses are reviewed. These specific and/or non-specific lesions require early recognition, diagnosis, and aggressive antifungal treatment.
Alsasua del Valle, Angela
1. Preparations from Cannabis sativa (marijuana) have been used for many centuries both medicinally and recreationally. 2. Recent advances in the knowledge of its pharmacological and chemical properties in the organism, mainly due to Delta(9)-tetrahydrocannabinol, and the physiological roles played by the endocannabinoids have opened up new strategies in the treatment of neurological and psychiatric diseases. 3. Potential therapeutic uses of cannabinoid receptor agonists include the management of spasticity and tremor in multiple sclerosis/spinal cord injury, pain, inflammatory disorders, glaucoma, bronchial asthma, cancer, and vasodilation that accompanies advanced cirrhosis. CB(1) receptor antagonists have therapeutic potential in Parkinson's disease. 4. Dr. Julius Axelrod also contributed in studies on the neuroprotective actions of cannabinoids.
Kobayashi, Naoaki; Shimazaki, Haruyo; Hoshi, Yasutaka; Akatsuka, Jun-ichi (Jikei Univ., Tokyo (Japan). School of Medicine)
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).
Lizza, John P
This commentary challenges the conclusions reached by Franklin Miller and Robert Truog in their criticism of the President's Council's White Paper, "Controversies in the Determination of Death." I agree with much of Miller and Truog's criticism of the rationale offered by the President's Council for accepting neurological criteria for determining death but argue that they too quickly dismiss the alternative rationale of determining death by neurological criteria-i.e., the destruction of the psychophysical integrity of the human being that occurs when the potential for consciousness and every other mental function is lost due to catastrophic injury to the brain. By focusing on the death of bodies instead of human beings, their view absurdly implies that decapitation would not necessarily result in one's death. Since total brain failure is a form of physiological decapitation, the neurological criterion coheres perfectly well with the ordinary understanding of decapitation as death.
Saux, A; Niango, G; Charif, M; Morales, R; Mura, F; Bonafe, A; Mourand, I
Susac's syndrome (SS) is a rare, immune-mediated endotheliopathy affecting the microvasculature of the brain, the inner ear and the retina. Clinical presentation is characterised by a triad: encephalopathy, hearing loss and branch retinal artery occlusion (BRAO). Given the rarity of this disease, its natural history still remains partially unknown, but lethal cases appear to be extremely rare since there has never been, to our knowledge, a report of SS leading to death. We report 2 cases of SS illustrating the multiplicity of neurological symptomatology and its unpredictable course. One case is particularly unusual due to its severe neurological evolution, leading to death despite treatments. This report presents clinical and paraclinical findings contributory to SS diagnosis and offers an innovative perspective on disease management. These cases represent the potential severity of this disease. Early, aggressive treatment strategies may be warranted for SS in order to avoid neurological deterioration and lethal evolution. Copyright 2010 Elsevier B.V. All rights reserved.
Full Text Available The coexistence of neuronal degeneration, psychiatric manifestation, immune activation and malignant transformation has been documented in literature, suggesting a central dysfunction in the pathophysiology of these disorders. The isoprenoid pathway may be candidate in this respect, in view of the changes in the concentration of some products of this pathway in many of these disorders, however, no detailed study has been carried out in this respect. In view of this, a study was undertaken on the isoprenoid pathway in some of these disorders - primary generalized epilepsy, Parkinsonâ€s disease (PD, schizophrenia, manic depressive psychosis (MDP, CNS glioma, multiple sclerosis, subacute sclerosing panencephalitis (SSPEand a familial group with familial coexistence of schizophrenia, PD, primary generalized epilepsy, malignant neoplasia, rheumatoid arthritis and syndrome-X over three generations. The following parameters were studied in the patients of these disorders as compared to age and sex matched control subjects - ubiquinone dolichol, digoxin, activity of HMG CoA reductase in the plasma and erthyorcyte membrane Na -K--ATpase. Increase in the activity of HMG CoA reductase and in the concentration of plasma digoxin and dolichol was observed in most of these cases. On the other hand, there was decrease in the concentration of plasma ubiquinone. Decrease in the activity of erythrocyte membrane Na-K- ATpase activity for which digoxin is an inhibitor was also observed in all the cases studied. These results indicate an upregulation of the isoprenoid pathway in the neurological and psychiatric disorders studied. The implications of this change is discussed in details.
neoplasm prior to cervical fusion. This scan showed a marked gibbus deformity at C2-C3 due to the sublux- .... NF-1 is related to an abnormal gene occurring on the long arm of chro- mosome 17 whereas NF-2 results ... tumour suppression genes on chro- mosome 22 results in the clinical manifestations of NF-2. For further.
Central nervous system tuberculosis is a severe form of extra‑pulmonary tuberculosis. It mainly presents as meningitis or tuberculoma. Tubercular brain abscess (TBA) is a rare manifestation of tuberculosis in an immunocompetent patient. We report a case of TBA who presented to us due to the defective vision and ...
Singh, Bhupesh; Mohamed, Ashik; Chaurasia, Sunita; Ramappa, Muralidhar; Mandal, Anil Kumar; Jalali, Subhadra; Sangwan, Virender S
To study the various clinical manifestations associated with congenital aniridia in an Indian population. In this retrospective, consecutive, observational case series, all patients with the diagnosis of congenital aniridia seen at the institute from January 2005 to December 2010 were reviewed. In all patients, the demographic profile, visual acuity, and associated systemic and ocular manifestations were studied. The study included 262 eyes of 131 patients with congenital aniridia. Median patient age at the time of initial visit was 8 years (range: 1 day to 73 years). Most cases were sporadic and none of the patients had parents afflicted with aniridia. The most common anterior segment abnormality identified was lenticular changes. Cataract was the predominant lens finding, observed in 93 of 231 (40.3%) phakic eyes. Other lens abnormalities were subluxation, coloboma, posterior lenticonus, and microspherophakia. Corneal involvement of varying degrees was seen in 157 of 262 (59.9%) eyes, glaucoma was identified in 95 of 262 (36.3%) eyes, and foveal hypoplasia could be assessed in 230 of 262 (87.7%) eyes. Median age when glaucoma and cataract were noted was 7 and 14 years, respectively. None of the patients had Wilm's tumor. Congenital aniridia was commonly associated with classically described ocular features. However, systemic associations were characteristically absent in this population. Notably, cataract and glaucoma were seen at an early age. This warrants a careful evaluation and periodic follow-up in these patients for timely identification and appropriate management. Copyright 2014, SLACK Incorporated.
Neuville, M; Jondeau, G; Crestani, B; Taillé, C
Marfan's syndrome is a rare genetic disorder caused by a mutation of the gene FBN1, coding for the protein fibrillin-1. Cardiovascular, musculoskeletal and ophthalmic manifestations are the most commonly observed, but minor diagnostic criteria also include pulmonary manifestations. Pneumothorax, frequently relapsing, affects 5 to 11% of patients. Rib cage abnormalities (pectus excavatum or pectus carinatum) and apical blebs may contribute to their occurrence. Treatment does not require any specific procedure but there is an increased risk of recurrence. Pectus excavatum affects up to 60% of the patients, without any functional impairment in most cases. Surgery may be required (using the Nuss procedure) in case of cardiovascular or psychological symptoms. Marfan's syndrome is frequently associated with obstructive sleep apnoea, which may itself contribute to aortic dilatation. Some studies suggest a potential role of craniofacial abnormalities in the pathogenesis of sleep apnea in these patients. Pulmonologists should consider Marfan's syndrome when treating patients for recurrent spontaneous pneumothorax or rib cage abnormalities, since early detection of cardiac abnormalities improves the prognosis significantly. Copyright © 2014 SPLF. Published by Elsevier Masson SAS. All rights reserved.
Marques, Letícia Helena de Sousa; Martins, Daniela Vieira; Juares, Gabriel Liria; Lorenzetti, Fábio Tadeu Moura; Monsanto, Rafael da Costa
To describe and discuss otologic manifestations of Larsen syndrome, based on a case report and a systematic review of the literature. We performed a PubMED database search, and we selected studies reporting otolaryngologic manifestations secondary to Larsen syndrome. The selected articles were read in full, and three researchers independently extracted data from the studies. In parallel, we report the case of a 14-year-old patient who had hearing loss secondary to Larsen syndrome. Fifteen studies met our selection criteria. Seven studies reported hearing loss in patients with Larsen syndrome (4 had conductive hearing loss and 3 had mixed hearing loss). The conductive hearing loss may be secondary to ossicular malformations and/or middle ear effusions. Other causes for conductive hearing loss are mesenchymal remnants in the middle ear, Eustachian tube dysfunction, and cleft palate. Surgical management of the hearing loss is possible in selected cases, although the surgical and anesthetic risks should be considered. Hearing aids seem to be safe and effective treatment options for the hearing loss secondary to Larsen syndrome. Although rare, patients with Larsen syndrome may have hearing loss. The most frequent type of deficit is conductive, either secondary to malformation of the ossicles or middle ear effusion. Possible surgical correction of these abnormalities should be weighed against the anesthetic risks of these patients. Copyright © 2017 Elsevier B.V. All rights reserved.
Ivanović, Mirjana; Jovcić, Olivera; Mandić, Jelena; Bogetić, Dusko; Maddalone, Marcello
Acute leukaemia is the most common form of childhood cancer. The aim of this paper was to underline the importance of oral manifestations in children with acute leukaemia. The disease and its treatment can directly or indirectly affect oral health. Oral manifestations are gingival inflammation and enlargement. Leukaemic cells are capable of infiltrating the gingiva and the deeper periodontal tissues which leads to ulceration and infection of oral tissues. Gingival bleeding is a common sign in patients with leukaemia. Symptoms include local lymphadenopathy, mucous membrane Petechiae and ecchymoses. Cytotoxic drugs have direct effects like mucositis, involving atrophy, desquamation and ulceration of the mucosa, with increasing the risk for local and systemic infections. Leukaemia can directly influence dental care and dental treatment, while oral lesions may have life-threatening consequences. Knowledge and skills among dentists may also not be adequate to treat children with acute leukaemia. It is therefore imperative that all stomatologists be aware of dental problems that occur in leukaemia in order to be able to effectively carry out appropriate measures to mitigate these problems.
Full Text Available Acute leukaemia is the most common form of chilhood cancer. The aim of this paper was to underline the importance of oral manifestations in children with acute leukaemia. The disease and its treatment can directly or indirectly affect oral health. Oral manifestations are gingival inflammation and enlargement. Leukaemic cells are capable of infiltrating the gingiva and the deeper periodontal tissues which leads to ulceration and infection of oral tissues. Gingival bleeding is a common sign in patients with leukaemia. Symptoms include local lymphadenopathy, mucous membrane Petechiae and ecchymoses. Cytotoxic drugs have direct effects like mucositis, involving atrophy, desquamation and ulceration of the mucosa, with increasing the risk for local and systemic infections. Leukaemia can directly influence dental care and dental treatment, while oral lesions may have life-threatening consequences. Knowledge and skills among dentists may also not be adequate to treat children with acute leukaemia. It is therefore imperative that all stomatologists be aware of dental problems that occur in leukaemia in order to be able to effectively carry out appropriate measures to mitigate these problems.
to acknowledge the excellence of these two scholars by a double Festschrift, "A due". Both have been working at the Music Department of the University of Copenhagen and have collaborated with The Royal Library on various projects. This publication contains contributions from 44 colleagues, who thus - in topics...
Jørgensen, T M; Djurhuus, J C; Schrøder, H D
Symptomatology and clinical manifestations of detrusor sphincter dyssynergia are described in 23 patients without neurological disease. Their cardinal symptoms were recurrent cystitis, enuresis, frequent voiding, back pain during voiding and anal discomfort. The major objective finding...... was vesicoureteral reflux in 11 cases with kidney scarring in 10. Bladder trabeculation was found in 13 patients, bladder hyperreflexia in 8, and significant residual urine in 16 patients. The etiology of detrusor sphincter dyssynergia in non-neurological patients is discussed. By means of exclusion it is most...
Guardiola, Elena; Banos, Josep E
Popular medical literature attempts to discuss medical topics using a language that is, as far as possible, free of all medical jargon so as to make it more easily understandable by the general public. The very complexity of neurology makes it more difficult for the stories dealing with this specialty to be understood easily by an audience without any kind of medical training. This paper reviews the works written by Oliver Sacks involving the field of neurology aimed at the general public, and the main characteristics and the clinical situation discussed by the author are presented. Some biographical notes about Oliver Sacks are also included and the 11 books published by this author over the last 40 years are also analysed. In each case they are put into a historical context and the most outstanding aspects justifying what makes them an interesting read are commented on. In most cases, the genesis of the work is explained together with its most significant features. The works of Sacks contain a wide range of very interesting clinical situations that are usually explained by means of a language that is readily comprehensible to the general public. It also provides neurologists with a holistic view of different clinical situations, together with a discussion of their biographical, historical and developmental components.
Full Text Available Abstract Background Posterior reversible encephalopathy syndrome (PRES has been increasingly identified in patients with systemic lupus erythematosus (SLE owing to the advance in neuroimaging techniques. Prompt diagnosis is pivotal to improve its outcome. To analyze the clinical and radiographic profile of PRES in patients with SLE and search for the appropriate treatment strategy PRES in SLE. Methods SLE patients who fulfilled the diagnostic criteria for PRES from August 2008 to January 2011 were evaluated at baseline, and followed to determine clinical outcomes. Data were analysis on clinical characteristics, laboratory abnormalities, treatment details, and outcomes. Results Ten episodes of PRES in patients with SLE were identified. All patients were female, mean age of onset was 22.93 ± 2.48 years, and SLEDAI at the onset of PRES were 25.8 ± 5.7. All cases had acute onset of headache, altered mental status, stupor, vomiting, cortical blindness and seizures. Neurological symptoms were the initial manifestation of SLE in three cases. Head magnetic resonance imaging (MRI demonstrated posterior white matter edema involving the parietal, temporal and occipital lobes, which were more conspicuous on T2 weighted spin echo and diffusion-weighted MR imaging (DWI than on computed tomography (CT scan. Complete clinical and radiographic recovery was observed in 8 patients after prompt treatment with corticosteroids. Conclusions PRES might be due to lupus per se besides other traditional causative factors such as hypertension. PRES might be an underestimated variant of “reversible neurological deficits” in SLE. Prompt recognition and timely management is important to prevent permanent neurological deficits.
Full Text Available Neuropsychiatric manifestations of systemic lupus erythematosus are varied. Presently nineteen in number, they are classified as whether affecting the central or the peripheral compartments of the nervous system. Its diagnosis however remains difficult, more so when two or more of the syndromes are found concomitantly in the same patient and when they occur in absence of the more classical rash, serositis, and haematological manifestations. We present a case of lupus where myelopathy as well as demyelination existed simultaneously as the initial neurologic manifestation.
Ferrari, Teresa C A; Moreira, Paulo R R; Cunha, Aloísio S
The involvement of the central nervous system (CNS) by Schistosoma mansoni may or may not cause clinical manifestations. When symptomatic, neuroschistosomiasis mansoni (NSM) is one of the most severe presentations of this infection. The neurological manifestations are due to numerous granulomas grouped in confined areas of the spinal cord or the brain. Considering the symptomatic form, myelopathy is far more frequent than the cerebral disease. Spinal cord NSM presents as a low cord syndrome of acute/subacute progression usually associated with involvement of the cauda esquina roots. Lower limbs pain, weakness and sensory disturbance, and autonomic dysfunctions, particularly bladder dysfunction, are often present. Cerebrospinal fluid (CSF) examination generally shows an inflammatory pattern with or without eosinophils and/or IgG against schistosomal antigens. Magnetic resonance imaging (MRI) demonstrates signs of inflammatory myelopathy. Cerebral NSM presents as a slow-expanding intracranial tumor-like lesion. Its clinical manifestations are variable and depend on the increased intracranial pressure and on the site of the lesion. The diagnosis of spinal cord NSM is based on clinical evidence whereas the cerebral disease is usually diagnosed by biopsy of the nervous tissue. There is no consensus on the treatment of NSM. We discuss the literature data on this topic, and suggest a therapeutic approach based on our experience with 69 spinal cord NSM patients who have been followed up by a long period of time. Outcome is largely dependent on early treatment, particularly in the medullar disorder, and is better in cerebral NSM.
Tijmstra, Jesper; Hoijtink, Herbert; Sijtsma, Klaas
The assumption of latent monotonicity in item response theory models for dichotomous data cannot be evaluated directly, but observable consequences such as manifest monotonicity facilitate the assessment of latent monotonicity in real data. Standard methods for evaluating manifest monotonicity typically produce a test statistic that is geared toward falsification, which can only provide indirect support in favor of manifest monotonicity. We propose the use of Bayes factors to quantify the degree of support available in the data in favor of manifest monotonicity or against manifest monotonicity. Through the use of informative hypotheses, this procedure can also be used to determine the support for manifest monotonicity over substantively or statistically relevant alternatives to manifest monotonicity, rendering the procedure highly flexible. The performance of the procedure is evaluated using a simulation study, and the application of the procedure is illustrated using empirical data.
Full Text Available A study of 58 paitents of tinea faciei was conducted. Twenty five (43.1% patients had history of photosensitivity. Twenty eight (48.2% patients were applying topical steroids, 2 (3.4% patients were on 10 mg of prednisolone daily. Associated tinea of other sites were observed in 14 (24.13%. 23 (39.6% patients had typical circinate, arcuate, annular plaques with raised margin showing vesiculo-pustules. Atypical manifestations were in the form of arcuate plaques on the pinna in 4 patients, erythematous plaques full of vesiculo-pustules without central clearing in 3. Thirty two (55.17% patients had plaques with broad edges and indistinct central clearing. In 2 patients lesions resembled discoid lupus erythematosus. Skin scrapings for fungus was positive in 36 (62.06% cases. All patients responded to systemic griseofulvin 10mg/kg with 1% clotrimazole topicaly in 4-8 weeks.
Full Text Available Ce livre, paru chez Routledge en 2000 sous le titre Sociology beyond Societies , se veut le « manifeste d’une reformulation de la sociologie dans sa phase “post-sociétale” » (p. 15, c’est-à-dire d’une sociologie dont l’objet majeur ne serait plus les sociétés, mais « les diverses mobilités des peuples, des objets, des images, des informations et des déchets » (p. 15. L’enjeu est ainsi de penser la sociologie « des décennies à venir » (p. 31, de formuler ...
Torres Herrera, Eduardo Jonathan; Santos, Lea
A main feature of a chaotic quantum system is a rigid spectrum, where the levels do not cross. Dynamical quantities, such as the von Neumann entanglement entropy, Shannon information entropy, and out-of-time correlators can differentiate the ergodic from the nonergodic phase in disordered interacting systems, but not level repulsion from level crossing in the delocalized phase of disordered and clean models. This is in contrast with the long-time evolution of the survival probability of the initial state. The onset of correlated energy levels is manifested by a drop, referred to as correlation hole, below the asymptotic value of the survival probability. The correlation hole is an unambiguous indicator of the presence of level repulsion. EJTH is grateful to VIEP, BUAP for financial support through the VIEP projects program.
R V Koranne
Full Text Available A study of 36 untreated patients with early syphilis revealed atypical variations namely; long incubation period of 101 days in I patient, more than 3 chancres in 1, undermined margin of the chancre along with tenderness in 1 and moderate to severe tenderness of the ulcers in 2 cases. In 3 patients there was no indurations of the ulcers. Three patients with primary syphilis had unilateral lymphadenitis, and in I case the lymph nodes were not only tender but showed tendency towardsmatingawell. Insecondarysyphilis, 11 out of 16 patients having condylomata lata had no other muco-cutaneous lesions. Concomitant presence of other venereal disease to account for the atypical manifestations was discounted- by appropriate laboratory tests, response to therapeutic agents and follow up.
Full Text Available Background: The phylum Cnidarians have over nine thousand species that approximately, one hundred species are dangerous for humans. Annually, a large number of deaths were reported due to jellyfish stings. The manifestations depend on their species and kind of venoms, and include the local and systemic manifestations. A number of methods and compounds were used and under investigation for management of injuries with jellyfishes. Due to the lack of an integrated systematic review, the current study was done. Materials and Methods: The PubMed data bank was searched for the term “Jellyfish”. A total of 1677 papers were found. These papers were divided into three categories: medical, biomedical and biotechnological fields. The medical category was further divided into three subcategories comprising systemic manifestations, cutaneous manifestations and treatments for the stings of jellyfishes. The biomedical category was further subdivided into genomics, proteomics, and biology of venoms, mechanisms of actions and products of biomedical significance. In this part of systematic review, the medical aspects of injuries with jellyfishes were evaluated. Results: The clinical manifestations in jellyfish envenomation depend on their species and the nature of venoms. The most common clinical manifestations of jellyfish stings are cutaneous presentations like urticasia, erythema, swelling, vesicles and severe dermonectoric manifestations. Systemic manifestations were seen in the stings of box jellyfishes, Portuguese man-of-war and in Irukandji syndrome. The most common recommendations for jellyfish envenomation managements include decreasing the local effects of venom, prevention of the venomous nematocysts release, and Controlling of systemic reactions. Application of commercial vinegar (4 - 6% acetic acid, hot water immersion (HWI (42 ° C for 20 minutes, ice packs, sea water rinsing for inactivating nematocysts, administration of topical and parenteral
Provaznikova, Dana; Geierova, Vera; Kumstyrova, Tereza; Kotlin, Roman; Mikulenkova, Dana; Zurkova, Kamila; Matoska, Vaclav; Hrachovinova, Ingrid; Rittich, Simon
Currently, the May-Hegglin anomaly (MHA), Sebastian (SBS), Fechtner (FTNS) and Epstein (EPS) syndrome are considered to be distinct clinical manifestations of a single disease caused by mutations of the MYH9 gene encoding the heavy chain of non-muscle myosin IIA (NMMHC-IIA). Manifestations of these disorders include giant platelets, thrombocytopenia and combinations of the presence of granulocyte inclusions, deafness, cataracts and renal failure. We examined 15 patients from 10 unrelated families on whom we performed immunostaining of NMMHC-IIA in blood samples. Polymerase chain reaction (PCR) analysis of selected exons of the MYH9 gene revealed mutations in nine samples with one novel mutation. Results of fluorescence and mutational analysis were compared with clinical manifestations of the MYH9 disorder. We also determined the number of glycoprotein sites on the surface of platelets. Most patients had an increased number of glycoproteins, which could be due to platelet size.
Tubbs, R Shane; Malefant, Jason; Loukas, Marios; Jerry Oakes, W; Oskouian, Rod J; Fries, Fabian N
The presence of a human tail is a rare and intriguing phenomenon. While cases have been reported in the literature, confusion remains with respect to the proper classification, definition, and treatment methods. We review the literature concerning this anatomical derailment. We also consider the importance of excluding underlying congenital anomalies in these patients to prevent neurological deficits and other abnormal manifestations. © 2016 Wiley Periodicals, Inc.
J Gordon Millichap
Full Text Available The clinical manifestations, outcome, and viral triggers in 137 children (<18 years old with multiple sclerosis (MS and 96 controls, enrolled between 2003 and 2006 from diverse multinational geographic regions, were studied by researchers from the Divisions of Neurology and Microbiology, The Hospital for Sick Children, Toronto; Stony Brook University Medical Center, USA; and other centers in Canada, USA, Argentina, Russia, Italy, and Sweden.
Guiraud-Chaumeil, Bernard; Pariente, Jérémie; Albucher, Jean-François; Loubinoux, Isabelle; Chollet, François
Stroke is one of the most common affliction of patients with neurological symptoms. Rehabilitation of stroke patients is a difficult task. Our knowledge on rehabilitation has recently improved with the emergence of data from new neuroimaging techniques. A prospective, double blind, cross over, placebo, controlled study on 8 patients with pure motor hemiparesia, is conducted to determine the influence of a single dose of fluoxetine on motor performance and cerebral activation of patients recovering from stroke. Each patient undergoes two functional magnetic resonance imaging (fMRI) examinations, one under fluoxetine and one under placebo. A single dose of fluoxetine is enough to modulate cerebral sensori-motor activation and significantly improves motor skills of the affected side. Further studies are required to investigate the effect of chronic administration of fluoxetine on motor function.
Full Text Available This state-of-the art paper focuses on the poorly explored issue of foreign language aptitude, attempting to present the latest developments in this field and reconceptualizations of the construct from the perspective of neuroscience. In accordance with this goal, it first discusses general directions in neurolinguistic research on foreign language aptitude, starting with the earliest attempts to define the neurological substrate for talent, sources of difficulties in the neurolinguistic research on foreign language aptitude and modern research methods. This is followed by the discussion of the research on the phonology of foreign language aptitude with emphasis on functional and structural studies as well as their consequences for the knowledge of the concept. The subsequent section presents the studies which focus on lexical and morphosyntactic aspects of foreign language aptitude. The paper ends with a discussion of the limitations of contemporary research, the future directions of such research and selec ed methodological issues.
Full Text Available Hashimoto%u2019s encephalopathy (HE is a rare disorder associated with autoimmune thyroiditis. Etiology of HE is not completely understood. High levels of serum antithyroid antibodies are seen in HE. Presentation with otoimmune thyroiditis, cognitive impairment, psychiatric and neurologic symptoms and absence of bacterial or viral enfections are characteristics of HE. HE is a steroid responsive encephalopathy. 60 years old male patient admitted to hospital with forget fulness continuing for 9 months and speech loss starting 2 days ago. Strong positivity of antithyroid antibodies increases the odds for HE. Thyroid function tests showed severe hypothyroidism. Electroencephalography and magnetic resonance imaging results were compatible with HE. HE is diagnosed with differantial diagnosis and exclusion of other reasons. This uncommon disorder is not recognised enough. High titres of serum antithyroid antiboides are always needed for diagnosis. Correct diagnosis requires awareness of wide range of cognitive and clinical presentations of HE.
Ribeiro, R A; Romano, A R; Birman, E G; Mayer, M P
Seventeen patients with a mean age of 7.33 (range 2.7-12.7) years with Rett syndrome (a progressive neurological disorder that occurs mainly in females) were evaluated for oral manifestations and habits. The most frequent habits were digit/hand sucking and/or biting (17/17), bruxism (14/17), mouth breathing (7/17), drooling (5/17), and tongue thrusting (5/17). Gingivitis (13/17) was the most common alteration of soft tissues. Only 2.7% of tooth surfaces were decayed. Nonphysiological dental attrition was present in 71% (12/17) of the children. Palatal shelving could be observed in 53% (9/17) of the children, probably related to the digit/hand sucking and/or biting habits. A high prevalence of anterior open bite (9/17) was observed. No patients exhibited anomalies of tooth number, size, form, structure, or eruption.
Full Text Available Varicella-zoster virus (VZV meningoencephalomyelitis is a rare but severe neurological complication of VZV reactivation in immunocompromised patients. We report the case of an HIV-infected individual who developed an acute and severe meningoencephalomyelitis accompanied by a disseminated cutaneous eruption due to VZV. The presence of VZV DNA in cerebrospinal fluid was confirmed by polymerase chain reaction (PCR technique. The patient started undergoing an intravenous acyclovir therapy with a mild recovery of neurological manifestations. Varicella-zoster virus should be included as a cause of acute meningoencephalomyelitis in patients with AIDS. Early diagnosis followed by specific therapy should modify the rapid and fulminant course for this kind of patients.
Full Text Available Abstract Background Dental assistants help the dentist in preparing material for filling teeth. Amalgam was the filling material mostly commonly used in Norway before 1980, and declined to about 5% of all fillings in 2005. Amalgam is usually an alloy of silver, copper, tin and mercury. Copper amalgam, giving particularly high exposure to mercury was used in Norway until 1994. Metallic mercury is neurotoxic. Few studies of the health of dental assistants exist, despite their exposure to mercury. There are questions about the existence of possible chronic neurological symptoms today within this working group, due to this exposure. The aim of this study was to compare the occurrence of neurological symptoms among dental assistants likely to be exposed to mercury from work with dental filling material, compared to similar health personnel with no such exposure. Methods All dental assistants still at work and born before 1970 registered in the archives of a trade union in Hordaland county of Norway were invited to participate (response rate 68%, n = 41, as well as a similar number of randomly selected assistant nurses (response rate 87%, n = 64 in the same age group. The participants completed a self-administered, mailed questionnaire, with questions about demographic variables, life-style factors, musculoskeletal, neurological and psychosomatic symptoms (Euroquest. Results The dental assistants reported significant higher occurrence of neurological symptoms; psychosomatic symptoms, problems with memory, concentration, fatigue and sleep disturbance, but not for mood. This was found by analyses of variance, adjusting for age, education, alcohol consumption, smoking and personality traits. For each specific neurological symptom, adjusted logistic regression analyses were performed, showing that these symptoms were mainly from arms, hands, legs and balance organs. Conclusion There is a possibility that the higher occurrence of neurological symptoms
Johnson, V Y; Hubbard, D; Vordermark, J S
To determine the character and prevalence of bladder dysfunction among persons with a history of polio, we conducted a survey of randomly selected polio survivors. The survey solicited information on the acute episode of polio and the nature of late-onset neurologic symptoms that could be attributed to postpolio syndrome. There were 242 female and 88 male respondents to the study. Symptoms attributable to postpolio syndrome were present in 87.2% of female subjects and 73.9% of male subjects. Respondents with postpolio syndrome had a significantly greater prevalence of urologic symptoms than seen among respondents without postpolio syndrome, although no dominant pattern of voiding dysfunction was noted. The early onset of erectile dysfunction was more common among male subjects with postpolio syndrome than among male subjects without postpolio syndrome. Genuine stress incontinence was seen in 36.3% of the survey population. Sixteen women with postpolio syndrome underwent surgical repair for urinary incontinence, with a success rate of 60.5%. Bladder disorders are common among persons with PPS, but further clinical and urodynamic data are necessary to define the nature and magnitude of this dysfunction.
Lavely, James A
The neurologic examination in the puppy or kitten can be a challenging experience. Understanding the development of behavior reflexes and movement in puppies and kittens enables us to overcome some of these challenges and to recognize the neurologically abnormal patient. Subsequently,we can identify the neuroanatomic localization and generate a differential diagnosis list. This article first reviews the pediatric neurologic examination and then discusses diseases unique to these individuals.
George, Reena; Andronikou, Savvas; Plessis, Jaco du; Plessis, Anne-Marie du; Maydell, Arthur [University of Stellenbosch, Department of Radiology, Tygerberg Academic Hospital, Cape Town (South Africa); Toorn, Ronald van [University of Stellenbosch, Department of Paediatrics and Child Health, Tygerberg Academic Hospital, Cape Town (South Africa)
Vertically transmitted HIV infection is a major problem in the developing world due to the poor availability of antiretroviral agents to pregnant women. HIV is a neurotrophic virus and causes devastating neurological insults to the immature brain. The effects of the virus are further compounded by the opportunistic infections and neoplasms that occur as a result of the associated immune suppression. This review focuses on the imaging features of HIV infection and its complications in the central nervous system. (orig.)
Full Text Available Sleep problems are frequently addressed as a primary or secondary concern during the visit to the pediatric neurology clinic. Sleep disorders can mimic other neurologic diseases (e.g., epilepsy and movement disorders, and this adds challenges to the diagnostic process. Sleep disorders can significantly affect the quality of life and functionality of children in general and those with comorbid neurological diseases in particular. Understanding the pathophysiology of sleep disorders, recognizing the implications of sleep disorder in children with neurologic diseases and behavioral difficulties, and early intervention continue to evolve resulting in better neurocognitive outcomes.
Faurholt-Jepsen, Daniel; Range, Nyagosya; PrayGod, George Amani
Objective: Diabetes is associated with pulmonary tuberculosis (TB), possibly due to impaired immunity, and diabetes may exacerbate the clinical manifestations of TB. Our aim was to assess the role of diabetes in the clinical manifestations of TB. Methods: We studied 1250 patients with pulmonary T...
Ghapanchi, Janan; Rezazadeh, Fahimeh; Kamali, Fereshte; Rezaee, Mostafa; Ghodrati, Maryam; Amanpour, Sara
The effect of asthma on oral health is the subject of debate among dental practitioners. The current study was planned to investigate the oral manifestations of asthmatics compared to healthy subjects. The study group composed of 100 asthmatics and 100 age-matched healthy controls. The caries status based on Decayed/Missing/Filled Teeth (DMFT) criteria and oral lesion were evaluated in all subjects. The mean age of the asthmatics group was 47.5±3.5 years and in the control group it was 43.5±3.0 years. Asthmatics included 45(45%) males and 55(55%) females. There was no statistical difference between caries prevalence in both groups. The most prevalent oral lesions in asthmatics group were geographic tongue 10(10%), fissured tongue 13(13%), chronic atrophic candidiasis13(13%), and in the control group were fissured tongue(11%) and lichenoid reaction(2%). The dental professional must be familiar with all signs and symptoms of this disease in order to offer effective and safe treatment.
M. Yu. Bobylоva
Full Text Available Autism spectrum disorders in children are attracting the increasing attention of general practitioners, which is due to the specification of diagnostic criteria and to the recognition of the fact that there is atypical autism that is a syndrome of many different diseases. The authors consider in detail the theory of autism and the clinical manifestations of atypical autism, including possible neurological and somatic disorders. Individual attention is placed on the association of autistic disorder with epilepsy or epileptiform activity on the electroencephalogram. An emphasis is made on the need for an interdisciplinary approach and on the interaction of clinicians of different specialties when treating patients with autistic disorder.
G. Yu. Evzikov
Full Text Available The paper describes spinal dural arteriovenous fistulas (SDAVF, the most common type of spinal cord vascular anomalies. SDAVFs account for 60–80% of the spinal cord vascular anomalies. The causes of SDAVFs, the specific features of their hemodynamics, and their classification remain the subject matter of disputes.SDAVFs form in dura mater tissue, on the dorsal surface of radicular cuffs. The pathogenesis of neurological disorders in SDAVF has determined the name «venous hypertensive myelopathy», a spinal cord injury occurring in their presence. Pain and paresthesias, cacesthesia (more commonly in their distal parts, and motor disorders as flail legs are observed at the onset of SDAVF in typical cases. On average, 12 to 44 months elapse to establish its diagnosis. In addition of motor and sensory disorders, sphincter impairments and sexual dysfunction are detected in the patients at the time of diagnosis. By this time, most patients have already neurological disability.The paper presents the history of studying SDAVF, the existing classifications of arteriovenous malformations and fistulas, the clinical manifestations of venous hypertensive myelopathy in SDAVF, neuroimaging findings, and treatment options.
Gröber, Uwe; Kisters, Klaus; Schmidt, Joachim
Vitamin B12 is a cofactor of methionine synthase in the synthesis of methionine, the precursor of the universal methyl donor S-Adenosylmethionine (SAMe), which is involved in different epigenomic regulatory mechanisms and especially in brain development. A Vitamin B12 deficiency expresses itself by a wide variety of neurological manifestations such as paraesthesias, skin numbness, coordination disorders and reduced nerve conduction velocity. In elderly people, a latent Vitamin B12 deficiency can be associated with a progressive brain atrophy. Moderately elevated concentrations of homocysteine (>10 µmol/L) have been associated with an increased risk of dementia, notably Alzheimer’s disease, in many cross-sectional and prospective studies. Raised plasma concentrations of homocysteine is also associated with both regional and whole brain atrophy, not only in Alzheimer’s disease but also in healthy elderly people. Clinician awareness should be raised to accurately diagnose and treat early Vitamin B12 deficiency to prevent irreversible structural brain damage. PMID:24352086
Otávio Valério Carvalho
Full Text Available Canine distemper is a highly contagious viral disease caused by the canine distemper virus (CDV, which is a member of the Morbillivirus genus, Paramyxoviridae family. Animals that most commonly suffer from this disease belong to the Canidae family; however, the spectrum of natural hosts for CDV also includes several other families of the order Carnivora. The infectious disease presents worldwide distribution and maintains a high incidence and high levels of lethality, despite the availability of effective vaccines, and no specific treatment. CDV infection in dogs is characterized by the presentation of systemic and/or neurological courses, and viral persistence in some organs, including the central nervous system (CNS and lymphoid tissues. An elucidation of the pathogenic mechanisms involved in canine distemper disease will lead to a better understanding of the injuries and clinical manifestations caused by CDV. Ultimately, further insight about this disease will enable the improvement of diagnostic methods as well as therapeutic studies.
Chalifoux, Sara L; Konyn, Peter G; Choi, Gina; Saab, Sammy
Primary biliary cholangitis (PBC) is an autoimmune liver disease characterized by progressive destruction of the intrahepatic bile ducts, leading to cholestasis. PBC is known to have both hepatic and extrahepatic manifestations. Extrahepatic manifestations are seen in up to 73% of patients with PBC, with the most common being Sjogren’s syndrome, thyroid dysfunction and systemic sclerosis. It is thought that patients with PBC are at increased risk of developing these extrahepatic manifestation...
Brennan, Michael T; Valerin, Manuel A; Napeñas, Joel J; Lockhart, Peter B
Lupus erythematosus manifests as cutaneous variants, such as discoid lupus erythematosus or systemic lupus erythematosus. Systemic lupus erythematosus is a multisystem autoimmune disease characterized by general autoantibody production and a wide range of mucocutaneous, renal, neuropsychiatric, cardiovascular, infectious, and hematologic manifestations. This article discusses the prevalence of and considerations for oral mucosal lesions in lupus erythematosus and the impact of the various disease manifestations of systemic lupus erythematosus on dental management.
Full Text Available
Aim of the study: to evaluate the effciency of detection for HIV-infection in patients of neurological departments of Saratov. Materials and methods. We retrospectively analyzed 25 250 medical histories of patients hospitalized into neurological departments of Saratov hospitals between January 2007 and April 2012. Results. Blood samples of 2010 patients (7,96 % were tested for the presence of HIV-antibodies. 37 patients were HIV-positive (1,84 % of examined patients and 0,15 % of the total number of patients. Conclusion. Increasing popularity and variety of clinical manifestations of HIV-infection requires the expansion of neurological patients whom serum test for antibodies against HIV is needed.
Doherty, Mia Aagaard; Hertel, Niels Thomas; Hove, Hanne Buciek
Aim To investigate the prevalence of neurological symptoms and the types of complications in a cohort of Danish patients with mutation verified achondroplasia and hypochondroplasia and compare the results with previously reported findings. Methods Retrospective descriptive study by chart review...... of patients followed in three outpatients clinics in the period 1997-2014. Forty-eight patients with achondroplasia and a median age of 9,5 years old and 20 patients with hypochondroplasia and a median age of 12 years old were enrolled. Neurological manifestations, epidemiological variables and clinical data...... for referral to an MRI scan or neurosurgery. Conclusion Through investigation of phenotypes and genotypes in patients with achondroplasia and hypochondroplasia we report the frequencies of neurological symptoms, foramen magnum stenosis, spinal cord compression and neurosurgery in Danish patients. Variation...
Simona Roxana Georgescu
Full Text Available Inflammatory bowel diseases have a high frequency in Europe. They are chronic disorders that evolve with relapses and remissions. Clinical features include the signs of underlying inflammatory bowel disease and also signs of extraintestinal manifestations. Cutaneous disorders are the most common extraintestinal manifestations associated with inflammatory bowel diseases, which can be dependent on or independent of gastrointestinal disease activity. The main cutaneous disorders are erythema nodosum and pyodermagangrenosum. The pathogenic mechanisms are not fully understood but it seems that related mechanisms are involved in the development of inflammatory bowel diseases and extraintestinal manifestations. Treatment should be aimed at both the cutaneous manifestations and the bowel inflammation
du Moulin, M; Koehn, A F; Golsari, A; Dulz, S; Atiskova, Y; Patten, M; Münch, J; Avanesov, M; Ullrich, K; Muschol, N
Fabry disease (FD) is a multisystem lysosomal storage disorder caused by mutations in the GLA gene. The clinical significance of the mutation p.D313Y is still under debate. Retrospective chart analysis of clinical (neurological, cardiac, renal, and ophthalmological), genetic, and biochemical (lyso-globotriaosylsphingosine, lyso-Gb3; enzyme activity) data was performed in all our patients carrying the p.D313Y mutation. Fourteen patients from 5 families (10 female, 4 male; age range 10-51) were included. Symptoms and organ manifestations compatible with FD could be identified in 10 patients. Cerebrovascular events occurred in 4 females. Seven patients reported pain or acroparaesthesia. Cornea verticillata was found in 1 patient, mild retinal vascular tortuosity in 5 patients. Lyso-Gb3 was elevated in 2 females with cerebrovascular involvement. Classical cardiac, renal or skin manifestations could not be identified. The mutation p.D313Y in the GLA gene may lead to organ manifestations and elevation of the Fabry-specific biomarker lyso-Gb3. Neurological symptoms (stroke and pain) and ocular manifestations seem to be the leading findings. Annual routine visits are recommended for patients carrying the p.D313Y mutation. Enzyme replacement therapy might be considered in symptomatic patients. © 2017 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.
Nicholas S. Kurek; Sathees B. Chandra
Nanotechology involves the application, analysis and manipulation of nanomaterials. These materials have unique and medically useful properties due to their nanoscale parameters. Nanotechnology based treatments and diagnostics might eventually bring great relief to people suffering from neurological disorders including autism spectrum disorders, Alzheimers disease and Parkinsons disorders. A large variety of nonmaterials such as viruses, carbon nanotubes, gold and silica nanoparti...
influence of one or another of the divisions of the ANS, which served as the basis for determining the severity of the impact of the components of autonomic regulation. Autonomic reactivity was assessed on the basis of reflex of samples Danin-Aschner and Chermak-Goering in statistical analysis for Galu. Support for the activities was set according to clinortotation samples. UNDCT diagnosis and severity was Dagestanis on the basis of criteria proposed by T. Milkovsky-Dimitrova (1985 in modification of L. N. Abakumova. Results. Therefore, children with neurological pathology on the background of undifferentiated connective tissue dysplasia have a deeper down-the vegetative, the severity of which correlates with the degree of NDCT (P<0.05. The prevalence parasympathicotonia and low sympathetic reactivity in children with UDCT, perhaps due to metabolic disorders: increased average potassium-calcium ratio, which serves as one of the criteria the predominance of vagotonia, and lower levels of total calcium and inorganic phosphorus, which point to insufficient provision of vitamin D. Inadequate functioning of the sympathetic division of ANS leads to decrease of adaptive&trophic processes in situations requiring intense mental activity, and hence the adaptive capacity of the organism. Patients with insufficient vegetative support amid NDCT form a group of psychological risk, which is characterized by reduced subjective assessment of their own capabilities, emotional instability, and repressive conformism. Conclusion. Deficit vegetative maintenance in children with NDCT is potentially dangerous during physical paranavitana in the untrained conditions, dysplastic modified myocytes, including cardiomyocytes, which may poyasnite ciselements (deficiency of vitamin D3, hypocalcaemia and gphotospace, which, in turn, contributes to a violation of biochemically-colloidal processes and bioenergetic reactions in children and more severe manifestations of the UNDCT. Given the above, children
Full Text Available Karine Pelc1, Guy Cheron2, Bernard Dan1,21Department of Neurology, Hôpital Universitaire des Enfants Reine Fabiola, Université Libre de Bruxelles (ULB, Brussels, Belgium; 2Laboratory of Neurophysiology and Movement Biomechanics, Université Libre de Bruxelles (ULB, Brussels, BelgiumAbstract: Angelman syndrome has been suggested as a disease model of neurogenetic developmental condition with a specific behavioral phenotype. It is due to lack of expression of the UBE3A gene, an imprinted gene located on chromosome 15q. Here we review the main features of this phenotype, characterized by happy demeanor with prominent smiling, poorly specific laughing and general exuberance, associated with hypermotor behavior, stereotypies, and reduced behavioral adaptive skills despite proactive social contact. All these phenotypic characteristics are currently difficult to quantify and have been subject to some differences in interpretation. For example, prevalence of autistic disorder is still debated. Many of these features may occur in other syndromic or nonsyndromic forms of severe intellectual disability, but their combination, with particularly prominent laughter and smiling may be specific of Angelman syndrome. Management of problematic behaviors is primarily based on behavioral approaches, though psychoactive medication (eg, neuroleptics or antidepressants may be required.Keywords: Angelman syndrome, UBE3A, chromosome 15, behavioral phenotypes, autism, neurogenetics
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
Boersma, Isabel; Miyasaki, Janis; Kutner, Jean; Kluger, Benzi
Palliative care is an approach to the care of patients and families facing progressive and chronic illnesses that focuses on the relief of suffering due to physical symptoms, psychosocial issues, and spiritual distress. As neurologists care for patients with chronic, progressive, life-limiting, and disabling conditions, it is important that they understand and learn to apply the principles of palliative medicine. In this article, we aim to provide a practical starting point in palliative medicine for neurologists by answering the following questions: (1) What is palliative care and what is hospice care? (2) What are the palliative care needs of neurology patients? (3) Do neurology patients have unique palliative care needs? and (4) How can palliative care be integrated into neurology practice? We cover several fundamental palliative care skills relevant to neurologists, including communication of bad news, symptom assessment and management, advance care planning, caregiver assessment, and appropriate referral to hospice and other palliative care services. We conclude by suggesting areas for future educational efforts and research. © 2014 American Academy of Neurology.
Al-Araji, A; Sharquie, K; Al-Rawi, Z
Objectives: To determine the prevalence of neurological involvement in Behcet's disease in a prospective study, and to describe the clinical patterns of neurological presentation in this disease in patients attending a multidisciplinary clinic in Baghdad. Methods: All patients attending the clinic who fulfilled the international study group criteria for the diagnosis of Behcet's disease were studied during a two year period starting in April 1999. Patients were assessed neurologically by a neuro-Behcetologist. All those with clinical neurological manifestations were sent for CSF examination, cranial magnetic resonance imaging, and magnetic resonance venography and were followed up to explore the patterns of neurological relapse. Results: 140 patients with Behcet's disease were studied. Their mean age was 34.2 years (range 16 to 66); 105 (75%) were men and 35 (25%) were women. The mean duration of the disease was 4.2 years (range 0.4 to 26). Twenty patients (14%) had neurological involvement (neuro-Behcet's disease); 14 of these (70%) were men and six (30%) women. The mean age at the first neurological presentation was 34.1 years. The mean duration of follow up of patients with neuro-Behcet's disease was 20.7 months. Ten patients with neuro-Behcet's disease (50%) presented with parenchymal CNS involvement, six (30%) with intracranial hypertension, and four (20%) with a mixed pattern of both parenchymal CNS involvement and intracranial hypertension. Conclusions: Careful neurological assessment of patients with Behcet's disease may show a relatively high prevalence of neuro-Behcet features, and though the clinical patterns of presentation are characteristic a mixed pattern may occur. PMID:12700303
Cacioppo, Stephanie; Capitanio, John P.; Cacioppo, John T.
Social isolation has been recognized as a major risk factor for morbidity and mortality in humans for more than a quarter century. The brain is the key organ of social connections and processes, however, and the same objective social relationship can be experienced as caring and protective or as exploitive and isolating. We review evidence that the perception of social isolation (i.e., loneliness) impacts brain and behavior and is a risk factor for broad-based morbidity and mortality. However, the causal role of loneliness on neural mechanisms and mortality is difficult to test conclusively in humans. Mechanistic animal studies provide a lens through which to evaluate the neurological effects of a member of a social species living chronically on the social perimeter. Experimental studies show that social isolation produces significant changes in brain structures and processes in adult social animals. These effects are not uniform across the brain or across species but instead are most evident in brain regions that reflect differences in the functional demands of solitary versus social living for a particular species. The human and animal literatures have developed independently, however, and significant gaps also exist. The current review underscores the importance of integrating human and animal research to delineate the mechanisms through which social relationships impact the brain, health, and well-being. PMID:25222636
Ranieri, Roberta; Laezza, Chiara; Bifulco, Maurizio; Marasco, Daniela; Malfitano, Anna M
Several studies support the evidence that the endocannabinoid system and cannabimimetic drugs might have therapeutic potential in numerous pathologies. These pathologies range from neurological disorders, atherosclerosis, stroke, cancer to obesity/metabolic syndrome and others. In this paper we review the endocannabinoid system signaling and its alteration in neurodegenerative disorders like multiple sclerosis, Alzheimer's disease, Parkinson's disease and Huntington's disease and discuss the main findings about the use of cannabinoids in the therapy of these pathologies. Despite different etiologies, neurodegenerative disorders exhibit similar mechanisms like neuro-inflammation, excitotoxicity, deregulation of intercellular communication, mitochondrial dysfunction and disruption of brain tissue homeostasis. Current treatments ameliorate the symptoms but are not curative. Interfering with the endocannabinoid signaling might be a valid therapeutic option in neuro-degeneration. To this aim, pharmacological intervention to modulate the endocannabinoid system and the use of natural and synthetic cannabimimetic drugs have been assessed. CB1 and CB2 receptor signaling contributes to the control of Ca2+ homeostasis, trophic support, mitochondrial activity, and inflammatory conditions. Several studies and patents suggest that the endocannabinoid system has neuro-protective properties and might be a target in neurodegenerative diseases.
Süsskind, D; Berneburg, M; Rohrbach, J M
The aim of this study was to evaluate ocular changes in patients with xeroderma pigmentosum (XP) and Cockayne syndrome (CS). Both diseases belong to the progeroid syndromes caused by single gene mutations with premature aging. Both syndromes are very rare autosomal recessive diseases caused by a gene defect leading to deficient DNA repair. 12 patients (4 female, 8 male) with XP between 3 and 67 years old and a 39-year-old female patient with CS were examined. The examination included visual acuity testing, slit lamp biomicroscopy, funduscopy and performance of a Schirmer test. Changes of the lids in the XP group included blepharitis in 7 patients, distichiasis in 2, and madarosis in 3 patients. 8 patients had multiple lentigines solares of the lids. One patient showed scars of the lower lids after excision of a squamous cell carcinoma and a basal cell carcinoma. Conjunctival lesions comprised a tumour of unknown origin of the conjunctiva in 1 patient, teleangiectasia of the conjunctiva in 5 patients, pterygia in 4, and pinguecula in 1 patient. Two patients had an intraepithelial melanosis of the conjunctiva, and one showed conjunctival nevi. Two patients had corneal scars and corneal vascularisation, another a significant conjunctivalisation of the cornea. A Schirmer test was feasible only in 3 patients. Tear production was markedly reduced in all these patients. Break-up time was shortened significantly in 6 patients examined. The patient with the CS showed atrophy of the pupillary rim and a subcapsular cataract. Funduscopically there were pigment epithelial changes. Patients with XP often exhibit ocular changes. Beside the light-dependent degenerative and inflammatory manifestations at the lids, the conjunctiva and the cornea patients with XP also develop malignancies early in life. The majority of patients suffer from dry eye symptoms. © Georg Thieme Verlag KG Stuttgart · New York.
Ayangco, Lilibeth; Rogers, Roy S
Erythema multiforme is a reactive mucocutaneous disorder in a disease spectrum that comprises a self-limited, mild, exanthematic, cutaneous variant with minimal oral involvement (EM minor) to a progressive, fulminating, severe variant with extensive mucocutaneous epithelial necrosis (SJS and TEN). Significant differences exist among EM minor, EM major, SJS, and TEN with regards to severity and clinical expression; however, all variants share two common features: typical or less typical cutaneous target lesions and satellite-cell or more widespread necrosis of the epithelium. These features are considered to be sequelae of a cytotoxic immunologic attack on keratinocytes expressing non-self-antigens. These antigens are primarily microbial (viruses) or drugs and in rare instances histocompatibility antigens . Although the precise pathogenesis is unknown, there is a tendency to consider EM both minor and major as part of one spectrum that is most often triggered by viral infections, and SJS and TEN as a separate one most often elicited by drugs with EM major and SJS representing a bridge in the continuum of EM. The oral manifestations of the spectrum of EM range from tender superficial erythematous and hyperkeratotic plaques to painful deep hemorrhagic bullae and erosions. Other mucosal surfaces including ocular, nasal, pharyngeal, laryngeal, upper respiratory, and anogenital may be involved. Scarring sequelae from ocular and pharyngeal involvement cause morbidity. The oral EM variant is an underrecognized form of EM. Most patients have chronic or recurrent oral lesions only, but one third have oral and lip lesions and one quarter have oral, lip, and skin lesions. This variant is a reaction pattern similar to EM minor, EM major, SJS, and TEN. The diagnosis of oral EM is one of exclusion. Careful clinical evaluation for other chronic mucocutaneous diseases, such as pemphigus, paraneoplastic pemphigus, mucous membrane pemphigoid, and lichen planus, is a necessary
Full Text Available Background: The aim of this study was to evaluate and compare the clinical and electrocardiographic (ECG manifestations of benzodiazepines (BZs. Methods: In this retrospective study, all BZ-poisoned patients hospitalized at Loghman Hakim Hospital between September 2010 and March 2011 were evaluated. Patients’ information including age, sex, time elapsed between the ingestion and presentation, and type of the BZ used were extracted from the patients' charts and recorded. ECGs on presentation to the emergency department (ED were evaluated and parameters such as PR interval, QRS duration, corrected QT, amplitude of S wave in lead I, height of R wave and R/S ratio in the lead aVR were also measured and recorded. Results: Oxazepam, chlordiazepoxide, lorazepam, alprazolam, diazepam, and clonazepam were ingested by 9 (3%, 13 (4.4%, 29 (9.9%, 105 (35.8%, 65 (22.2%, and 72 (24.6% patients, respectively. Mean PR interval was reported to be 0.16 ± 0.03 sec and PR interval of greater than 200 msec was detected in 12 (4.5% patients. Mean QRS duration was 0.07 ± 0.01sec and QRS≥120 msec was observed in 7 (2.6% cases. Conclusion: Diazepam is the only BZ that does not cause QRS widening and oxazepam is the only one not causing PR prolongation. It can be concluded that if a patient refers with a decreased level of consciousness and accompanying signs of BZ toxicity, QRS widening in ECG rules out diazepam, whereas PR prolongation rules out oxazepam toxicity.
Defining educational objectives is the key to achieving the goal of professional competence in students. The technique of task analysis was selected to determine components of competence in clinical neurology appropriate to the needs of primary care. A survey of neurological problems in general practice revealed that ...
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A non medical person suspected and confirmed neurological dysfunction in an individual, based only on e mail messages sent by the individual. With email communication becoming rampant “peculiar” email messages may raise the suspicion of neurological dysfunction. Organic pathology explaining the abnormal email ...
Soupart, Alain; Pochet, Roland; Brion, Jean Pierre
Osmotic demyelination syndrome is a devastating neurologic condition that occurs after rapid correction of serum sodium in patients with hyponatremia. Pathologic features of this injury include a well-demarcated region of myelin loss, a breakdown of the blood–brain barrier, and infiltration of microglia. The semisynthetic tetracycline minocycline is protective in some animal models of central nervous system injury, including demyelination, suggesting that it may also protect against demyelination resulting from rapid correction of chronic hyponatremia. Using a rat model of osmotic demyelination syndrome, we found that treatment with minocycline significantly decreases brain demyelination, alleviates neurologic manifestations, and reduces mortality associated with rapid correction of hyponatremia. Mechanistically, minocycline decreased the permeability of the blood–brain barrier, inhibited microglial activation, decreased both the expression of IL1α and protein nitrosylation, and reduced the loss of GFAP immunoreactivity. In conclusion, minocycline modifies the course of osmotic demyelination in rats, suggesting its possible therapeutic use in the setting of inadvertent rapid correction of chronic hyponatremia in humans. PMID:21051736
Full Text Available AIM: To analyze the characteristics of neurological ophthalmology manifestation of patients with neurosyphilis.METHODS: Retrospective and nonrandomized case analysis were used. Totally 22 cases of 39 eyes were included. They were 17 males and 5 females, aged from 34 to 65 years old. The average age were 49.6 years old. RESULTS: The optic nerve atrophy presented in 11 cases of 22 eyes. One eye of them accompanied by left eye oculomotor nerve palsy; 5 eyes in 3 cases expressed as optic neuritis acute phase; neuroretinitis appeared in 4 cases of 6 eyes; 1 case of 2 eyes expressed as chorioretinitis accompanied by optic disc edema; central retinal artery occlusion were found in 1 case of 1 eye. Argyll-Robertson pupil was as only manifestation in 2 cases of 3 eyes. In all cases, Argyll-Robertson pupil signs can be seen in 19 eyes. Treponema pallidum particle agglutination test(TPPAwere positive in all 22 cases. Syphilis rapid plasma reactin test(RPRwere positive in 19 of 21 cases. All patients underwent lumbar puncture and cerebrospinal fluid were detected for RPR, cerebrospinal fluid protein, white blood cell count. Cerebrospinal fluid RPR were positive in 13 cases. Cerebrospinal fluid protein were greater than 450mg/L in 18 cases. Cerebrospinal fluid white blood cell count were greater than 5/mm3 in 13 cases. CONCLUSION: Neurosyphilis involving neuro-ophthalmology often occurs in middle-aged men and subacute onset. Both eyes can suffered from optic nerve disease simultaneously or sequencely. A few can be expressed as other cranial nerve palsy, which may lead to misdiagnosis. Considering medical history, clinical manifestations, ophthalmic examination, serum and cerebrospinal fluid laboratory tests can improve the diagnostic rate.
Albert, Dara V F; Patel, Anup D; Behnam-Terneus, Maria; Sautu, Beatriz Cunill-De; Verbeck, Nicole; McQueen, Alisa; Fromme, H Barrett; Mahan, John D
The aim of this study was to evaluate whether the current state of child neurology education during pediatric residency provides adequate preparation for pediatric practice. A survey was sent to recent graduates from 3 pediatric residency programs to assess graduate experience, perceived level of competence, and desire for further education in child neurology. Responses from generalists versus subspecialists were compared. The response rate was 32%, half in general pediatric practice. Only 22% feel very confident in approaching patients with neurologic problems. This may represent the best-case scenario as graduates from these programs had required neurology experiences, whereas review of Accreditation Council of Graduate Medical Education-accredited residency curricula revealed that the majority of residencies do not. Pediatric neurologic problems are common, and pediatric residency graduates do encounter such problems in practice. The majority of pediatricians report some degree of confidence; however, some clear areas for improvement are apparent.
Pohodich, Amy E; Zoghbi, Huda Y
Loss-of-function mutations in the X-linked gene Methyl-CpG-binding protein 2 (MECP2) cause a devastating pediatric neurological disorder called Rett syndrome. In males, these mutations typically result in severe neonatal encephalopathy and early lethality. On the other hand, owing to expression of the normal allele in ∼50% of cells, females do not suffer encephalopathy but instead develop Rett syndrome. Typically females with Rett syndrome exhibit a delayed onset of neurologic dysfunction that manifests around the child's first birthday and progresses over the next few years. Features of this disorder include loss of acquired language and motor skills, intellectual impairment and hand stereotypies. The developmental regression observed in patients with Rett syndrome arises from altered neuronal function and is not the result of neurodegeneration. Maintenance of an appropriate level of MeCP2 appears integral to the function of healthy neurons as patients with increased levels of MeCP2, owing to duplication of the Xq28 region encompassing the MECP2 locus, also present with intellectual disability and progressive neurologic symptoms. Despite major efforts over the past two decades to elucidate the molecular functions of MeCP2, the mechanisms underlying the delayed appearance of symptoms remain unclear. In this review, we will highlight recent findings that have expanded our knowledge of MeCP2's functions, and we will discuss how epigenetic regulation, chromatin organization and circuit dynamics may contribute to the postnatal onset of Rett syndrome. © The Author 2015. Published by Oxford University Press. All rights reserved. For Permissions, please email: firstname.lastname@example.org.
BACKGROUND: Comparable data on the global and country-specific burden of neurological disorders and their trends are crucial for health-care planning and resource allocation. The Global Burden of Diseases, Injuries, and Risk Factors (GBD) Study provides such information but does not routinely......-specific prevalence, mortality, disability-adjusted life-years (DALYs), years of life lost (YLLs), and years lived with disability (YLDs) for various neurological disorders that in the GBD classification have been previously spread across multiple disease groupings. The more inclusive grouping of neurological...... aggregate results that are of interest to clinicians specialising in neurological conditions. In this systematic analysis, we quantified the global disease burden due to neurological disorders in 2015 and its relationship with country development level. METHODS: We estimated global and country...
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Full Text Available Syphilis is an infectious disease presenting stages associated with specific oral lesions. Therefore, health professionals should be familiar with the different syphilis oral manifestations at each stage and be prepared to refer any suspected patient for further evaluation. This report describes the most important clinical factors of each stage, emphasizing the oral manifestations.
Hong, Bujung; Hermann, Elvis J; Feuerhake, Friedrich; Krauss, Joachim K
Secondary spinal manifestations of esthesioneuroblastoma are rare. A 67-year-old woman was presented with an extradural spinal manifestation at the vertebra Th7 within 8 weeks after resection of an esthesioneuroblastoma. Subtotal removal of the epidural tumour was achieved combined with dorsal transpedicular stabilization. Early screening for distant metastases may be considered in patients with esthesioneuroblastoma.
K C Shah
Full Text Available Cutaneous manifestations are found in 60 to 70% cases of tuberous sclerosis and consist of adenoma sebaceum, periungual fibromatas, cafe au lait spots, shagreen patches and white macules. Our patient showed unusual skin manifestations like spotty pigmentation on the chest, back and abdomen and hyperkeratosis palmaris et plantaris.
Dammeyer, Jesper Herup
Objective: Several hypotheses of different medical and psychological delayed manifestations among people who have congenital rubella syndrome (CRS) have been discussed. This study tests some of these hypotheses of delayed manifestations. Methods: Gathering information about 35 individuals who hav...... which people with CRS face must primarily be understood in relation to congenital deafblindness and dual sensory and communicative deprivation....
Penders, John; Thijs, Carel; van den Brandt, Piet A.; Kummeling, Ischa; Snijders, Bianca; Stelma, Foekje; Adams, Hanne; van Ree, Ronald; Stobberingh, Ellen E.
BACKGROUND AND AIMS: Perturbations in intestinal microbiota composition due to lifestyle changes may be involved in the development of atopic diseases. We examined gut microbiota composition in early infancy and the subsequent development of atopic manifestations and sensitisation. METHODS: The
Gunderson, Carl H; Daroff, Robert B
Between December 1965 and December 1971, the United States maintained armed forces in Vietnam never less than 180,000 men and women in support of the war. At one time, this commitment exceeded half a million soldiers, sailors, and airmen from both the United States and its allies. Such forces required an extensive medical presence, including 19 neurologists. All but two of the neurologists had been drafted for a 2-year tour of duty after deferment for residency training. They were assigned to Vietnam for one of those 2 years in two Army Medical Units and one Air Force facility providing neurological care for American and allied forces, as well as many civilians. Their practice included exposure to unfamiliar disorders including cerebral malaria, Japanese B encephalitis, sleep deprivation seizures, and toxic encephalitis caused by injection or inhalation of C-4 explosive. They and neurologists at facilities in the United States published studies on all of these entities both during and after the war. These publications spawned the Defense and Veterans Head Injury Study, which was conceived during the Korean War and continues today as the Defense and Veterans Head Injury Center. It initially focused on post-traumatic epilepsy and later on all effects of brain injury. The Agent Orange controversy arose after the war; during the war, it was not perceived as a threat by medical personnel. Although soldiers in previous wars had developed serious psychological impairments, post-traumatic stress disorder was formally recognized in the servicemen returning from Vietnam. © 2016 S. Karger AG, Basel.
Kawamura, Mitsuru; Sugimoto, Azusa; Kobayakawa, Mutsutaka; Tsuruya, Natsuko
To discuss the neurological basis of facial recognition, we present our case reports of impaired recognition and a review of previous literature. First, we present a case of infarction and discuss prosopagnosia, which has had a large impact on face recognition research. From a study of patient symptoms, we assume that prosopagnosia may be caused by unilateral right occipitotemporal lesion and right cerebral dominance of facial recognition. Further, circumscribed lesion and degenerative disease may also cause progressive prosopagnosia. Apperceptive prosopagnosia is observed in patients with posterior cortical atrophy (PCA), pathologically considered as Alzheimer's disease, and associative prosopagnosia in frontotemporal lobar degeneration (FTLD). Second, we discuss face recognition as part of communication. Patients with Parkinson disease show social cognitive impairments, such as difficulty in facial expression recognition and deficits in theory of mind as detected by the reading the mind in the eyes test. Pathological and functional imaging studies indicate that social cognitive impairment in Parkinson disease is possibly related to damages in the amygdalae and surrounding limbic system. The social cognitive deficits can be observed in the early stages of Parkinson disease, and even in the prodromal stage, for example, patients with rapid eye movement (REM) sleep behavior disorder (RBD) show impairment in facial expression recognition. Further, patients with myotonic dystrophy type 1 (DM 1), which is a multisystem disease that mainly affects the muscles, show social cognitive impairment similar to that of Parkinson disease. Our previous study showed that facial expression recognition impairment of DM 1 patients is associated with lesion in the amygdalae and insulae. Our study results indicate that behaviors and personality traits in DM 1 patients, which are revealed by social cognitive impairment, are attributable to dysfunction of the limbic system.
Schwarz, Heidi B; Fritz, Joseph V; Govindarajan, Raghav; Penfold Murray, Rebecca; Boyle, Kathryn B; Getchius, Thomas S D; Freimer, Miriam
There are many factors driving health care reform, including unsustainable costs, poor outcomes, an aging populace, and physician shortages. These issues are particularly relevant to neurology. New reimbursement models are based on value and facilitated by the use of multidisciplinary teams. Integration of advanced practice providers (APPs) into neurology practice offers many advantages with new models of care. Conversely, there are many and varied challenges financially and logistically with these practice models. The American Academy of Neurology has formed a Work Group to address the needs of both neurologists and neurologic APPs and monitor the effect of APPs on quality and cost of neurologic care.
Full Text Available Multiple myeloma is considered a hypercoagulable state due to several mechanisms such as the increased IL-6 and immunoglobulins production, the defective fibrinolytic mechanism, and the acquired resistance to activated protein C that are involved in the pathogenesis and clinical futures of the disease. We describe a case of a female patient who presented to the hospital with pulmonary embolism as the first manifestation of the hypercoagulability of multiple myeloma.
Hemoglobinopathies are among the most common inherited diseases around the world. They have become much more common recently in northern and central Europe, including Germany, due to immigration. Selective review of the literature with consideration of national guidelines. The hemoglobinopathies encompass all genetic diseases of hemoglobin. They fall into two main groups: thalassemia syndromes and structural hemoglobin variants (abnormal hemoglobins). α- and β-thalassemia are the main types of thalassemia; the main structural hemoglobin variants are HbS, HbE and HbC. There are many subtypes and combined types in each group. The highly variable clinical manifestations of the hemoglobinopathies range from mild hypochromic anemia to moderate hematological disease to severe, lifelong, transfusion-dependent anemia with multiorgan involvement. Stem-cell transplantation is the preferred treatment for the severe forms of thalassemia. Supportive, rather than curative, treatment consists of periodic blood transfusions for life, combined with iron chelation. Drugs to treat the symptoms of sickle-cell disease include analgesics, antibiotics, ACE inhibitors and hydroxyurea. Blood transfusions should be given only when strictly indicated. More than 90% of patients currently survive into adulthood. Optimally treated patients have a projected life span of 50 to 60 years. Hemoglobinopathies are a public health issue in today's multiethnic German population. Adequate care of the affected patients requires a wide variety of diagnostic and therapeutic measures.
Linari, Silvia; Castaman, Giancarlo
Gaucher disease is a multisystemic metabolic disorder due to a genetic deficiency of the lysosomal enzyme glucocerebrosidase, which leads to the accumulation within the lysosomes of macrophages of its natural substrate, glucosylceramide and its deacylated product glucosylsphingosine. The most prevalent form of the disease is the so-called non-neuronopathic form (type 1) characterized by anemia, thrombocytopenia, enlargement of liver and/or spleen, skeletal abnormalities. Etiology of anemia and thrombocytopenia may be multifactorial and not necessarily predicted by the degree of splenomegaly. Bleeding diathesis may not always be related to absolute platelet count but may be influenced by abnormal platelet function or coagulation factor deficiencies. A significant increased risk of severe hematological co-morbidities, including multiple myeloma and B-cell lymphoma, has been reported. Accumulation of glucosylceramide and glucosylsphingosyne in macrophages and the resulting chronic inflammation with the secretion of cytokines leading to polyclonal and monoclonal B cell proliferation up to multiple myeloma, as a continuum clonal expansion, is a key pathophysiological mechanism. Enzyme replacement therapy has been shown to be effective in reducing glucosylceramide storage burden and the deleterious effects caused by its accumulation, including hematological manifestations.
Full Text Available We present a case of an incidental diagnosis of panhypopituitarism in a 68-year-old African American man admitted to our psychiatric inpatient unit with symptoms suggestive of schizophrenia. The case was unusual as a first-episode psychosis given the patient’s age. In the course of his admission, the patient’s clinical condition deteriorated culminating in a sudden altered mental status which prompted a transfer to the medical floors and further investigations. A head CT scan and a pituitary MRI revealed a near total resection of the pituitary while laboratory investigations revealed hyponatremia and a grossly low hormone profile. The progression of these events casts doubts on our admitting diagnosis as the primary cause of the patient’s symptoms. The patient’s clinical condition improved only when his endocrinopathy was treated with hormone replacement, fluids, and electrolyte correction in addition to antipsychotics. An inability to verify the patient’s psychiatric history and a remote history of pituitary resection several decades earlier, unknown to the treating team, added to the diagnostic conundrum. We revised the diagnosis to neuropsychiatric manifestations secondary to an organic brain syndrome due to a partial pituitary resection. The patient was discharged with no symptoms of psychosis, good insight, judgment, and good reality testing.
Jeyakumar, Ajouka; Balakumar, Thyarapan; Raghu, Alyssa; Chang, Katherine I.; Soewono, Katarina; Gustave, Mario; Jolayemi, Ayodeji
We present a case of an incidental diagnosis of panhypopituitarism in a 68-year-old African American man admitted to our psychiatric inpatient unit with symptoms suggestive of schizophrenia. The case was unusual as a first-episode psychosis given the patient's age. In the course of his admission, the patient's clinical condition deteriorated culminating in a sudden altered mental status which prompted a transfer to the medical floors and further investigations. A head CT scan and a pituitary MRI revealed a near total resection of the pituitary while laboratory investigations revealed hyponatremia and a grossly low hormone profile. The progression of these events casts doubts on our admitting diagnosis as the primary cause of the patient's symptoms. The patient's clinical condition improved only when his endocrinopathy was treated with hormone replacement, fluids, and electrolyte correction in addition to antipsychotics. An inability to verify the patient's psychiatric history and a remote history of pituitary resection several decades earlier, unknown to the treating team, added to the diagnostic conundrum. We revised the diagnosis to neuropsychiatric manifestations secondary to an organic brain syndrome due to a partial pituitary resection. The patient was discharged with no symptoms of psychosis, good insight, judgment, and good reality testing. PMID:28567321
C. Miller Fisher MD, one of the great neurologists in the 20th century, died in April 2012. Born in Canada, he studied medicine at the University of Toronto. As a Canadian Navy medical doctor he participated in World War II and was a war prisoner from 1941 to 1944. He did a residency in neurology at the Montreal Neurological Institute between 1946 and 1948, and later on was a Fellow in Neurology and Neuropathology at the Boston City Hospital. In 1954 he entered the Massachusetts General Hospital as a neurologist and neuropathologist, where he remained until his retirement, in 2005. His academic career ended as Professor Emeritus at Harvard University. His area of special interest in neurology was cerebrovascular disease (CVD). In 1954 he created the first Vascular Neurology service in the world and trained many leading neurologists on this field. His scientific contributions are present in more than 250 publications, as journal articles and book chapters. Many of his articles, certainly not restricted to CVD, were seminal in neurology. Several concepts and terms that he coined are currently used in daily clinical practice. The chapters on CVD, in seven consecutive editions of Harrison's Internal Medicine textbook, are among his highlights. His death was deeply felt by the neurological community.
Drogan, O.; Manno, E.; Geocadin, R.G.; Ziai, W.
Objective: Limited information is available regarding the current state of neurocritical care education for neurology residents. The goal of our survey was to assess the need and current state of neurocritical care training for neurology residents. Methods: A survey instrument was developed and, with the support of the American Academy of Neurology, distributed to residency program directors of 132 accredited neurology programs in the United States in 2011. Results: A response rate of 74% (98 of 132) was achieved. A dedicated neuroscience intensive care unit (neuro-ICU) existed in 64%. Fifty-six percent of residency programs offer a dedicated rotation in the neuro-ICU, lasting 4 weeks on average. Where available, the neuro-ICU rotation was required in the vast majority (91%) of programs. Neurology residents' exposure to the fundamental principles of neurocritical care was obtained through a variety of mechanisms. Of program directors, 37% indicated that residents would be interested in performing away rotations in a neuro-ICU. From 2005 to 2010, the number of programs sending at least one resident into a neuro-ICU fellowship increased from 14% to 35%. Conclusions: Despite the expansion of neurocritical care, large proportions of US neurology residents have limited exposure to a neuro-ICU and neurointensivists. Formal training in the principles of neurocritical care may be highly variable. The results of this survey suggest a charge to address the variability of resident education and to develop standardized curricula in neurocritical care for neurology residents. PMID:22573636
Alterações psiquiátricas após corticoterapia em paciente com rara manifestação neurológica de Síndrome de Behçet e o papel da interconsulta psiquiátrica Behavior disturbances after corticotherapy in a patient with uncommom neurological manifestation of Behcet Syndrome and the role of the consultation liaison psychiatry
Amilton Santos Júnior
Full Text Available A Interconsulta Psiquiátrica (IP trata-se do estudo da relação entre a psiquiatria e todas as outras áreas dos conhecimentos do processo saúde-doença, visando, sob uma perspectiva biopsicossocial, atender sua demanda clínica (prestação da assistência ao paciente e institucional (relacionada aos serviços. É descrito o caso de uma paciente que apresentou rara manifestação fisiopatológica da Síndrome de Behçet e que evoluiu com transtorno psiquiátrico após a instituição de terapêutica com corticoesteróides. Apesar da remissão dos sintomas mentais e comportamentais com tratamento psicofarmacológico de curta duração, a evolução do quadro demandou a reintrodução de corticoterapia, com recrudescência de quadro psiquiátrico e necessidade de instituição de tratamento de manutenção para seu manejo. Além de ilustrativo, no sentido de discutir uma rara condição clínica, o caso descrito exemplifica os benefícios da atuação conjunta e planejada entre psiquiatras e outros profissionais na assistência integral ao paciente.Consultation Liaison Psychiatry studies the relationship between psychiatry and all other areas of knowledge of the health-disease process and intends to propose solutions, under a biopsychosocial perspective, to the clinical (assistant or institutional (service-related problems. It is described the case of a patient who presented unusual pathophysiologic manifestation of the Behcet's syndrome and also developed mental disorder after the pharmacological treatment with corticosteroids. Despite the remission of mental and behavioral symptoms with psychopharmacological treatment of short duration, her clinical outcome made it need the reintroduction of corticotherapy, with recrudescence of the psychiatric disorder and the need for maintenance treatment to assure its management. Besides illustrating a rare clinical condition, the case described exemplifies the benefits of joint and planned actions
Joseph E. Safdieh
Full Text Available Background: The neurologic examination is a challenging component of the physical examination for medical students. In response, primarily based on expert consensus, medical schools have supplemented their curricula with standardized patient (SP sessions that are focused on the neurologic examination. Hypothesis-driven quantitative data are needed to justify the further use of this resource-intensive educational modality, specifically regarding whether using SPs to teach the neurological examination effects a long-term benefit on the application of neurological examination skills. Methods: This study is a cross-sectional analysis of prospectively collected data from medical students at Weill Cornell Medical College. The control group (n=129 received the standard curriculum. The intervention group (n=58 received the standard curriculum and an additional SP session focused on the neurologic examination during the second year of medical school. Student performance on the neurologic examination was assessed in the control and intervention groups via an OSCE administered during the fourth year of medical school. A Neurologic Physical Exam (NPE score of 0.0 to 6.0 was calculated for each student based on a neurologic examination checklist completed by the SPs during the OSCE. Composite NPE scores in the control and intervention groups were compared with the unpaired t-test. Results: In the fourth year OSCE, composite NPE scores in the intervention group (3.5±1.1 were statistically significantly greater than those in the control group (2.2±1.1 (p<0.0001. Conclusions: SP sessions are an effective tool for teaching the neurologic examination. We determined that a single, structured SP session conducted as an adjunct to our traditional lectures and small groups is associated with a statistically significant improvement in student performance measured 2 years after the session.
Full Text Available The objective of this article is to highlight some of the most important pioneering books specifically focused on the neurological examination and their authors. During the XIX Century, Alexander Hammond, William Gowers and Charles Mills pioneered the neurological literature, followed in the XX Century by Aloysio de Castro, Monrad-Krohn, Derek Denny-Brown, Robert Wartenberg, Gordon Holmes, and Russel DeJong. With determination and a marked sense of observation and research, they competently developed and spread the technique and art of the neurological exam.
Lyme disease (Lyme borreliosis) is a systemic infectious disease that can present in a variety of clinical manifestations. The disease is caused by a group of spirochaetes--Borrelia burgdorferi sensu lato or Lyme borrelia--that are transmitted to humans by the bite of Ixodes ticks. Lyme disease is the most common arthropode-borne infectious disease in many European countries including Germany. Early localized infection is typically manifested by an erythema migrans skin lesion, in rarer cases as a borrelial lymphocytoma. The most common early disseminated manifestation is (early) neuroborreliosis. In adults, neuroborreliosis appears typically as meningoradiculoneuritis. Neuroborreliosis in children, however, is typically manifested by meningitis. In addition, multiple erythema migrans lesions and Lyme carditis occur relatively frequently. The most common manifestation oflate Lyme disease is Lyme arthritis. Early manifestations (and usually also late manifestations) of Lyme disease can be treated successfully by application of suitable antibacterial agents. For the treatment of Lyme disease, doxycycline, certain penicillins such as amoxicillin and some cephalosporins (ceftriaxone, cefotaxime, cefuroxime axetil) are recommended in current guidelines. A major challenge is the treatment of chronic, non-specific disorders, i. e., posttreatment Lyme disease syndrome and "chronic Lyme disease". Prevention of Lyme disease is mainly accomplished by protecting against tick bites. Prophylactic administration of doxycycline after tick bites is generally not recommended in Germany. There is no vaccine available for human beings.