Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0004134 (ataxia)
 15,886 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Seven cases of an encephalitic brainstem syndrome are reported. All had an initial flu-like phase, with a febrile state. Then, all had eye movements disturbances and ataxia. Six had a variable dysfunction of other cranial nerves, five had dysmetria of limbs and three had pyramidal signs. In all, the topographic diagnosis was based on clinical, neurophysiological (electromyography, evoked potentials) and neuroradiological (swelled brainstem on CT scan) findings. Analysis of cerebro-spinal fluid revealed pleocytosis (mean; 165 cells/ml), without profile indicative of multiple sclerosis. Serologic tests for Adenovirus or Echovirus were positive in three cases. All recovered spontaneously. With a mean follow-up of three years there was no relapse and the clinical examination remained normal. These seven cases had, after exclusion of other brainstem syndromes, a Bickerstaff's encephalitis. Previous reports are discussed and a post-infectious origin is proposed to this benign syndrome.
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PMID:[Benign encephalitis of the brain stem]. 343 46

A young adult who presents with periodic vertigo, diplopia, and tinnitus, later followed by progressive ataxia, may not have multiple sclerosis as might initially be suspected, but rather may represent a type of familial spinocerebellar degeneration previously described by Farmer and Mustian as "vestibulocerebellar ataxia." We recently encountered a patient who presented with these symptoms, and who was found to have downbeat nystagmus, ocular dysmetria, skew deviation, optokinetic dissociation, and a vertical gain bias on horizontal eye movements. Although family involvement was specifically denied by the patient, seven members of her family representing four generations were examined, and found to have similar findings. The importance of a careful neuro-ophthalmologic examination in each available family member of any patient presenting with this constellation of symptoms is emphasized. A discussion of the spinocerebellar degenerations, differential diagnosis, and literature review are included.
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PMID:Neuro-ophthalmologic findings in vestibulocerebellar ataxia. 348 54

20 patients with movement disorders and spasticity were treated with SCS to decrease tonus and improve impaired motor function. 12 patients with multiple sclerosis had gait disorders and slight up to considerable increase in tonus. We observed quantitative changes in diminution of spasticity, voluntary motor function, bladder function, lessening of ataxia and pain relief. During a follow-up of up to 4 years no deterioration in the patients' condition could be observed. 11 patients reported a more fluent gait in 6 cases accompanied by a decrease in tonus. Increase in tonus and deterioration of gait pattern were observed after break down of the stimulation. Although the indications for electrostimulation still remains uncertain in some cases with definite diagnosis without any progressive character of the disease and without severe neurological disturbances improvement can be obtained.
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PMID:Application of SCS for movement disorders and spasticity. 349 59

The case of a 40-year-old woman with increasing ataxia is described. Although the clinical presentation and evoked response studies raised the possibility of multiple sclerosis, further investigation revealed multiple cystic intracranial lesions. Surgical excision of one of the lesions relieved the patient's symptoms. Histological examination revealed that this was an enterogenous cyst. Although single cysts of this type have rarely been reported occurring in the posterior cranial fossa, the occurrence of multiple lesions, some in the supratentorial compartment, appears to be unique.
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PMID:Multiple intracranial enterogenous cysts. 370 54

The dorsal (posterior) root ganglion is a relatively neglected part of the nervous system from the clinical point of view. In recent studies on the pathophysiology of experimental allergic encephalomyelitis (EAE), the main animal model of multiple sclerosis, the DRG of the rabbit was shown to be the site of extensive inflammation and demyelination and of focal conduction block in a high proportion of the large diameter afferents. The resulting severe functional peripheral deafferentation accounts for the postural disturbance, hypotonia, ataxia and areflexia in rabbits with EAE. The vulnerability of the DRG is due to a deficient blood-nerve barrier and possibly also to a susceptibility of the branch point of the ganglion neurone to demyelination-induced conduction block. These and other studies in experimental animals suggest that in man the DRG may be a preferential (but neglected) site of focal structural and functional abnormalities in inflammatory and also other neurological diseases.
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PMID:Vulnerability of the dorsal root ganglion in experimental allergic encephalomyelitis. 384 21

Visual evoked potentials (VEPs) were delayed in 11 out of 18 patients with Friedreich's ataxia, in 1 out of 8 patients with Strumpell's hereditary spastic ataxia, in 2 out of 5 cases with cerebellar atrophy and in 42 out of 50 patients with multiple sclerosis (MS). Responses were normal in 5 cases with Pierre Marie's disease. Amplitude and temporal dispersion were statistically analyzed in the above-mentioned groups of patients with respect to controls. An abnormal temporal dispersion, also considered as interpeak N1P2, was frequently found in MS but only occasionally in spinocerebellar ataxias. Amplitude was statistically reduced in Friedreich's ataxia group, where an inverse relationship between latency and amplitude was found. No relation was found between VEP delay and duration of the disease, in any group considered.
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PMID:Comparative study of visual evoked potentials in spinocerebellar ataxias and multiple sclerosis. 399 30

In recent studies we have demonstrated that staphylococcal alpha-toxin can specifically bind to rabbit vagus nerves and cause disruption of myelin sheaths in this peripheral nerve in vitro. We report here that staphylococcal alpha-toxin, incubated in vitro with brain slices or injected intracerebrally into mice, can induce disruption of myelin sheaths in central nervous tissue. Intracerebral injection of alpha-toxin is followed by a characteristic and reproducible syndrome involving ataxia followed by a severe contraction of the limbs on the side contralateral to the injection and a maximal extension of the opposing limbs. At 1.1 micrograms of toxin injected, death occurs within 20 min. Histopathologic examination reveals extensive demyelination with minimal involvement of the axons. It is possible that staphylococcal alpha-toxin may play a role in the etiology of multiple sclerosis.
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PMID:Disruption of myelin sheaths in mouse brain in vitro and in vivo by staphylococcal alpha-toxin. 408 75

In an unselected series of 644 cases of multiple sclerosis, 25 cases with acquired pendular nystagmus were found. Ten additional cases of pendular nystagmus in multiple sclerosis were investigated, and four cases from the literature are analysed. Acquired pendular nystagmus is purely sinusoidal in form, ceases with eye closure, is accompanied by oscillopsia, often monocular and vertical in direction, and never accompanied by optokinetic inversion. This is different from congenital nystagmus. Acquired pendular nystagmus in multiple sclerosis shows a high correlation with holding tremor of head and arm and with trunk ataxia, and must therefore be viewed as a result of lesions of cerebellar nuclei or their fibre connections with the brain-stem. Supporting evidence is discussed. The results fit into a theory of cerebellar function according to which the cerebellar nuclei are involved in the maintenance of positions.
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PMID:Acquired pendular nystagmus with oscillopsia in multiple sclerosis: a sign of cerebellar nuclei disease. 483 52

In the epidemiological area of Southern Lower Saxony 92 patients with clinically definite or probable diagnosis of multiple sclerosis (MS) were interviewed and examined. This group contained a remarkably high percentage of benign cases (52%) in comparison with a sample of hospitalized patients. Neurological examination revealed spasticity and pareses to be the most important disturbances followed by ataxia and bladder/bowel problems. After a mean duration of 18.4 years, 52% received a pension and about 30% were still working full time. The pension was granted too early to 11 patients and vocational rehabilitation services would be required for 13 men. Although only half of the patients had an acceptable income, the socioeconomic situation of the families was adequate in 71%; 80% lived with their own families and could stay there in case more ambulant services were offered. Psychotherapeutic measures are required among these to relieve the stress within the families (present in 42%)and to improve the coping behavior (unsatisfactory in 60%).
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PMID:Rehabilitation for patients with multiple sclerosis? 616 30

Based on one patient's enthusiastic report, eight patients with multiple sclerosis, seriously disabled with tremor and ataxia, were given oral tetrahydrocannabinol. Two demonstrated improved motor coordination.
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PMID:Tetrahydrocannabinol for tremor in multiple sclerosis. 630 74


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