1/213. vitamin e deficiency ataxia associated with adenoma.Vitamin E is one of the most important lipid-soluble antioxidant nutrient. Severe vitamin e deficiency (VED) can have a profound effect on the central nervous system. VED causes ataxia and peripheral neuropathy that resembles Friedreich's ataxia. We report here a patient presenting this syndrome, but also a prolactin and FSH adenoma. Both the neurological syndromes and the adenoma regressed after treatment with alpha-tocopherol. Although, the presence of the prolactinoma in this patient may not be related to his vitamin e deficiency, alpha-tocopherol treatment seems to be beneficial and might usefully be tested in patients with hypophyseal secreting other forms of adenoma.- - - - - - - - - - ranking = 1keywords = ms (Clic here for more details about this article) |
2/213. A case of the subacute brainstem encephalitis.A case of brainstem encephalitis of undetermined etiology is reported in 66-year-old woman who had a sudden onset of illness with left abducens palsy, nystagmus and ataxia. The symptoms progressed to complete paralysis of eye movements, dysphagia and left hemiparesis with generalized hyperreflexia. Examination of CSF, CT scan and MRI of the brain were normal. The patient died 4 months after onset of disease. Neuropathologic study disclosed in the brainstem numerous perivascular and nodular inflammatory cell infiltrations composed predominantly of lymphocytes T and B. Most intensive inflammation concerned midbrain and pontine tegmentum and to a lesser degree medulla oblongata, pontine nuclei and cerebellar nuclei. basal ganglia, cerebral and cerebellar cortex were unaffected. Neuropathological finding was reminiscent of brainstem encephalitides related to viral infection or to paraneoplastic syndrome. However, HSV-1, EBV, and CMV antigens were not detected by immunohistochemistry, as well as evidences of malignancy were not present in this case.- - - - - - - - - - ranking = 0.5keywords = ms (Clic here for more details about this article) |
3/213. dextromethorphan- and pseudoephedrine-induced agitated psychosis and ataxia: case report.pseudoephedrine and dextromethorphan are therapeutic constituents of numerous commonly used, over-the-counter cough and cold preparations. Although this drug combination is generally considered quite safe if utilized in recommended doses, overmedication or overdose can result in serious neurologic and cardiovascular abnormalities that occasionally can be life-threatening. We present a case of a 2-year-old child who developed hyperirritability, psychosis, and ataxia after being overmedicated with a pseudoephedrine/dextromethorphan combination cough preparation, and discuss probable mechanisms of toxicity and risk factors for adverse events.- - - - - - - - - - ranking = 0.5keywords = ms (Clic here for more details about this article) |
4/213. intracranial hypotension with parkinsonism, ataxia, and bulbar weakness.OBJECTIVE: To describe a case of spontaneous intracranial hypotension with a previously unreported constellation of presenting features. DESIGN: Case report. SETTING: Tertiary care center. MAIN OUTCOME AND RESULTS: We describe a patient with intracranial hypotension who presented with a parkinsonian syndrome and later development of ataxia and prominent bulbar symptomatology. headache was not a feature of her initial presentation and was only reported after repeated questioning during later evaluations. magnetic resonance imaging of the patient's head revealed findings characteristic of intracranial hypotension. An [18F]fluoro-m-tyrosine positron emission tomographic scan showed normal striatal activity, suggesting intact presynaptic nigrostriatal function. Opening pressure on lumbar puncture was reduced at 40 mm H2O. A source of cerebrospinal fluid leakage was not identified on nuclear cisternography and the patient underwent lumbar epidural blood patching, which resulted in complete resolution of her signs and symptoms as well as in a marked improvement in her imaging findings. CONCLUSIONS: The clinical spectrum of intracranial hypotension can be broadened to include parkinsonism, cerebellar ataxia, and prominent bulbar dysfunction. As with more common manifestations of the disorder, these features may resolve after appropriate treatment.- - - - - - - - - - ranking = 0.5keywords = ms (Clic here for more details about this article) |
5/213. A case of paroxysmal tonic upgaze of childhood with ataxia.Paroxysmal tonic upgaze of childhood is a rare, distinctive, childhood syndrome that may be associated with ataxia and sometimes strabismus or amblyopia. Neurological examination as well as metabolic studies, electroencephalogram and neuroradiological investigations are normal in these patients. Although it has been considered as an age-related, dopa-sensitive dystonia, the exact pathogenetic mechanism is still unknown. Aggravation of attacks by fatigue, intercurrent infection or vaccination, and possible corticomesencephalic dysmaturation may underlie this abnormality. We report on a sporadic case of paroxysmal tonic upgaze with ataxia in which there was prompt aggravation of symptoms with sleep without response to levodopa treatment. This case suggests a different underlying pathogenetic mechanism from dopaminergic pathways for this syndrome.- - - - - - - - - - ranking = 0.5keywords = ms (Clic here for more details about this article) |
6/213. Central neurocytoma of the fourth ventricle. Case report.The authors report on a 17-year-old boy who suffered from slowly progressive and long-standing symptoms of ataxia, neck pain, and headache. Computerized tomography (CT) and magnetic resonance (MR) imaging revealed a tumor arising from the floor of the fourth ventricle that resulted in a moderate hydrocephalus. A partial resection was performed. Histological and immunohistological findings led to the diagnosis of an atypical central neurocytoma of the fourth ventricle. The imaging features on CT scanning, MR imaging, and proton MR spectroscopy studies, the clinical picture, and the prognosis of this very unusual tumor are discussed. Three cases of neurocytomas in the posterior fossa have been described to date; however, in all three cases some atypical aspects were present. In the present case, with the exception of the very unusual location, both imaging findings and clinical history perfectly met the definition of this rare tumor.- - - - - - - - - - ranking = 0.5keywords = ms (Clic here for more details about this article) |
7/213. time constraints improve reaching movements in an ataxic patient.We report on a patient (AM) with a post-traumatic ataxia who has uncoordinated reaching movements to resting targets, but is able to catch moving objects. AM participated in three experiments to identify factors responsible for the favorable effect of object motion on her performance. In the first experiment, the task was to catch an object that moved away from AM. The speed of the object to be grasped (target object) varied. In experiment 2, the effect of time constraints on reaching performance was examined. AM had to reach for and grasp a stationary object and was allowed either 600 ms or 2000 ms to perform the task. In the third experiment, liquid crystal shutter glasses were used to manipulate the time that the subject was able to view the stationary target object and her reaching movements (vision-on time). While increased speed of the object, tighter time constraints, and short vision-on time hardly affected the performance of AM's unaffected left hand, they greatly improved her right-hand performance. These results are discussed in light of the hypothesis that the brain mechanisms controlling externally triggered movements differ from those controlling internally regulated movements.- - - - - - - - - - ranking = 1.5keywords = ms (Clic here for more details about this article) |
8/213. A neurological disease caused by an expanded CAG trinucleotide repeat in the TATA-binding protein gene: a new polyglutamine disease?To investigate whether the expansion of CAG repeats of the TATA-binding protein (TBP) gene is involved in the pathogenesis of neurodegenerative diseases, we have screened 118 patients with various forms of neurological disease and identified a sporadic-onset patient with unique neurologic symptoms consisting of ataxia and intellectual deterioration associated with de novo expansion of the CAG repeat of the TBP gene. The mutant TBP with an expanded polyglutamine stretch (63 glutamines) was demonstrated to be expressed in lymphoblastoid cell lines at a level comparable with that of wild-type TBP. The CAG repeat of the TBP gene consists of impure CAG repeat and the de novo expansion involves partial duplication of the CAG repeat. The present study provides new insights into sporadic-onset trinucleotide repeat diseases that involve de novo CAG repeat expansion.- - - - - - - - - - ranking = 1keywords = ms (Clic here for more details about this article) |
9/213. Subacute encephalopathy in a 5-year-old boy.A 5-year-old boy presented with an acute ataxia and altered mental status. Although he initially recovered from these symptoms, he presented a second time with myoclonus and seizures and rapidly became vegetative. cerebrospinal fluid studies, magnetic resonance imaging, and brain biopsy all confirmed the presence of subacute sclerosing panencephalitis. Despite courses of therapy with cimetidine, amantadine, ribavirin, and inosine, no clinical improvement has been seen. Clinicians need to be alert to the possibility of subacute sclerosing panencephalitis even in the vaccinated child in the appropriate clinical setting.- - - - - - - - - - ranking = 0.5keywords = ms (Clic here for more details about this article) |
10/213. Acute axonal polyneuropathy in chronic alcoholism and malnutrition.In contrast to the classic, slowly progressive polyneuropathy in alcoholic patients, acute forms, clinically mimicking guillain-barre syndrome, are rare. We present a patient who developed motor weakness and sensory loss in all four limbs within four days. Laboratory data were consistent with long-term alcohol abuse and documented thiamine deficiency. Repeated cerebrospinal fluid examinations were normal. Electrophysiological studies showed an acute sensorimotor polyneuropathy with predominantly axonal involvement. We conclude that acute alcoholic neuropathy has to be distinguished from guillain-barre syndrome and other forms of acute polyneuropathy by using clinical, laboratory, and electrophysiological data. Both ethanol toxicity and vitamin deficiency could play a role in the pathogenesis.- - - - - - - - - - ranking = 1keywords = ms (Clic here for more details about this article) |
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