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)

Cerebral infarction after minor trauma to the neck has rarely been reported. A case is presented of a child with trauma to the vertebrobasilar artery resulting in stroke. Computerized tomography scan and angiography results are presented. Despite two subsequent, separate transient episodes of vertigo, the child had good functional recovery with complete restoration of language and cognitive function. After 28 months, residual impairments identified were a mild right-sided ataxia and hemiparesis.
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PMID:Childhood stroke after minor neck trauma: case report. 222 63

Magnetic resonance images obtained before and 2 months after surgical decompression for Arnold-Chiari type 1 malformation and an arachnoid cyst revealed retraction of the cerebellar tonsils from the spinal canal into the posterior fossa. This was associated with prominent functional recovery of the patient's ataxia and nystagmus. Such an observation may reflect a benign prognosis. Magnetic resonance imaging is the test of choice for the evaluation of this type of lesion.
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PMID:Spontaneous retraction of cerebellar tonsils after surgery for Arnold-Chiari malformation and posterior fossa cyst. 333 49

Twenty-seven cases of neosporosis in European dogs are described. The disease was confirmed by immunohistochemistry, electron microscopy, or a favourable response to treatment in the dogs with appropriate clinical signs, and by the presence of antibodies to Neospora caninum but not to Toxoplasma gondii. The affected dogs were two days to seven years old, and of 13 different breeds. Both sexes were affected and in most cases littermates remained normal. Twenty-one cases had an initial hindlimb paresis or ataxia, in which muscle atrophy was the most consistent clinical sign. Rigid hyperextension developed in approximately half of the cases. Anorexia and pyrexia were rare. Other clinical signs included forelimb ataxia, head tremors with tetraparesis and sudden collapse due to myocarditis. Titres of > or = 1:800 in the N caninum indirect fluorescent antibody test were detected in the 20 cases from which serum samples were taken. Such high titres are rare in healthy dogs and strongly suggest a diagnosis of neosporosis. Sixteen of the dogs received appropriate antiprotozoal treatment with clindamycin, potentiated sulphonamides and/or pyrimethamine; 10 made a full or functional recovery. Recovery was less likely in peracute cases with severe clinical signs, and when the treatment was delayed.
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PMID:Clinical aspects of 27 cases of neosporosis in dogs. 893 Dec 99

We tested the potential of insulin-like growth factor I (IGF-I) to induce functional recovery in an animal model of cerebellar ataxia because this motor impairment is accompanied in humans and rodents by distinct changes in several components of the IGF-I trophic system. Rats rendered ataxic by deafferentation of the cerebellar cortex with 3-acetylpyridine recovered motor function after IGF-I was administered, as determined by behavioral and electrophysiological tests. When treated with IGF-I, inferior olive neurons, the targets of the neurotoxin, were rescued to various degrees (from 92 to 27% of surviving neurons), depending on the time that treatment with IGF-I was initiated. Furthermore, full recovery was obtained regardless of the route by which the trophic factor was administered (intraventricular or subcutaneous) even in rats with severe neuronal loss. These results suggest that human ataxia could be treated with IGF-I by a simple procedure.
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PMID:Insulin-like growth factor I restores motor coordination in a rat model of cerebellar ataxia. 944 18

We report an unusual case of tobramycin-induced bilateral high-frequency vestibular toxicity with subsequent clinical and objective evidence of functional recovery. In those patients with a clinical presentation suggestive of aminoglycoside-induced bilateral vestibular toxicity (ataxia and oscillopsia) and normal low-frequency (ENG-caloric) responses, high-frequency rotation chair testing should be performed to exclude a high-frequency vestibular deficit.
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PMID:Reversible tobramycin-induced bilateral high-frequency vestibular toxicity. 1081 Feb 61

Wernicke's encephalopathy (WE) is most commonly associated with alcoholism, although other causes have also been implicated. In the years 1994-1997, 9 patients with no history of alcohol abuse presented with acute signs of ophthalmoplegia or nystagmus and ataxia which resolved within 48 h after intravenous thiamine. There were 7 women and 2 men aged 17-57 (7 below the age of 30). Precipitating events included vomiting 2, drastic weight-reducing diet 2, renal colic in a postpartum woman 1, colonic surgery 2 and chronic hemodialysis 1. In 2 patients there was no obvious precipitating event but their history was suggestive of a genetic predisposition. Mental changes were slight or absent in all patients and all of them made good functional recovery. These cases suggest that the diagnosis of WE should be considered more often in nonalcoholics in various clinical settings.
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PMID:Thiamine-responsive acute neurological disorders in nonalcoholic patients. 1115 Aug 38

There is accumulated evidence that cortical reorganization plays an important role in motor recovery after supratentorial stroke. However neural mechanisms underlying functional recovery of ataxia after infratentorial stroke remain unclear. We investigated cortical activations during ataxic gait in patients with infratentorial stroke to test the hypothesis that cerebral cortices were involved in compensatory mechanisms for ataxic gait. Twelve patients with infratentorial stroke (mean duration+/-S.D. from the onset: 88.3+/-44.8 days) and 11 age-matched healthy subjects participated in this study. All patients had predominant ataxia without severe hemiparesis. We measured cortical activation as assessed by task-related increase of oxygenated hemoglobin during gait on a treadmill using functional near-infrared spectroscopy. Task consisted of three repetitions of gait period alternated with rest period. In controls, cortical activations in the lateral and medial prefrontal cortex during the acceleration phase tended to be attenuated during the steady phase of the gait period while these activations were sustained throughout the gait period in ataxic patients. Repeated measures ANOVA for cortical activation revealed significant interactions (p<0.005) between phase (acceleration/steady) and group (control/stroke) in the medial and lateral prefrontal regions. These results suggest that sustained prefrontal activation during ataxic gait might be relevant to compensatory mechanisms for ataxic gait after infratentorial stroke.
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PMID:Sustained prefrontal activation during ataxic gait: a compensatory mechanism for ataxic stroke? 1768 49

The objective of this study was to describe a case of sensory neuronopathy syndrome (SNS) with Ro antibodies who had nearly complete functional recovery with combination immunosuppression. Plasma exchange, azathioprine, and hydroxychloroquine were used in combination. The gait ataxia, kinesthetic sensation, and sensory response amplitudes showed considerable recovery with excellent functional outcomes. Prompt combined therapy with azathioprine and hydroxychloroquine is a promising therapy for patients with sensory neuronopathy syndrome and Ro antibodies.
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PMID:Sensory neuronopathy with Ro antibodies: response to combination immunosuppression. 2021 84

We describe a patient presenting with progressive upper limb numbness and sensory ataxia of the 4 limbs. Motor nerve conduction studies were completely normal. Sensory electrophysiology showed reduced/absent upper limb sensory action potentials (SAPs). In the lower limbs, SAPs were mostly normal. Sensory conduction velocities were normal. Forearm sensory conduction blocks were present for both median nerves on antidromic testing. The maximal recordable sural SAP was preserved in comparison to maximal recordable radial SAP, consistent with an "abnormal radial normal sural" pattern. Somatosensory evoked potentials were unrecordable for tibial and median nerves. Cerebrospinal fluid protein was raised (0.99 g/L). The patient worsened on oral corticosteroids but subsequently made substantial functional recovery on intravenous immunoglobulins. This case is different to those previously reported of sensory chronic inflammatory demyelinating polyradiculoneuropathy, given its exclusive sensory electrophysiologic presentation, presence of predominant upper limb reduced sensory amplitudes, and detection of sensory conduction blocks. These electrophysiologic features were of paramount importance in establishing diagnosis and effective therapy.
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PMID:Chronic inflammatory pure sensory polyradiculoneuropathy: a rare CIDP variant with unusual electrophysiology. 2253 10

Sequelae in children following cerebellar tumor removal surgery are well defined, and predictors for poor recovery include lesions of the cerebellar nuclei and the inferior vermis. Dynamic reorganization is thought to promote functional recovery in particular within the first year after surgery. Yet, the time course and mechanisms of recovery within this critical time frame are elusive and longitudinal studies are missing. Thus, a group of children and adolescents (n = 12, range 6-17 years) were followed longitudinally after cerebellar surgery and compared to age- and gender-matched controls (n = 11). Patients were examined (1) within the first days, (2) 3 months, and (3) 1 year after surgery. Each time behavioral tests of balance and upper limb motor function, ataxia rating, and a MRI scan were performed. Data were used for subsequent lesion-symptom mapping of cerebellar function. Behavioral improvements continued beyond 3 months, but were not complete in all patients after 1 year. At that time, remaining deficits were mild. Within the first 3 months, cerebellar lesion volumes were notably reduced by vanishing edema. Reduction in edema affecting the deep cerebellar nuclei but not reduction of total cerebellar lesion volume was a major predictor of early functional recovery. Persistent impairment in balance and upper limb function was linked to permanent lesions of the inferior vermis and the deep cerebellar nuclei.
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PMID:Location and restoration of function after cerebellar tumor removal-a longitudinal study of children and adolescents. 2256 48


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