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)

Neurologic complications may appear before the primary disease, alcoholism, is recognized. The common syndromes are polyneuropathy, the withdrawal syndrome and the combination of Wernicke's encephalopathy and Korsakoff's psychosis. Other conspicuous clinical pictures include ataxia of cerebellar origin, convulsions, acute hallucinosis, myopathy and coma. Rarer disorders are Marchiafava-Bignami disease and central pontine myelinolysis.
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PMID:The neurologic complications of alcoholism. 18 91

Two children with osteosarcoma are presented in whom Wernicke encephalopathy with vomiting occurred during the chemotherapy. One of the children died with symptoms of toxic cardiomyopathy. Autopsy revealed Wernicke encephalopathy. The other child had similar symptoms (ocular signs, ataxia, somnolence). Parenteral thiamine had been given and after this therapy the child recovered from the encephalopathy. The authors emphasize the importance of the recognition of this neurological disorder occurring rarely in childhood: it can be cured with parenteral thiamine. Without thiamine treatment this condition is lethal.
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PMID:[Wernicke encephalopathy in childhood osteosarcoma]. 140 86

Clinical characteristics were examined in 5 elderly patients whose brain showed typical features of Wernicke's encephalopathy at the autopsy. All 5 were females with a mean age of 67 +/- 4 years old. The pathological diagnosis of Wernicke's encephalopathy was based on the presence of bleeding or atrophy of bilateral mammilary bodies, proliferation of capillaries and increase of macrophages in mammilary bodies, midbrain periaqueductal gray matter and periventricular area, with relatively intact neurons. Wernicke's encephalopathy was diagnosed clinically only in one case. The remaining four had no clinical diagnosis of Wernicke's encephalopathy. Underlying diseases were varied including neurological, metabolic, gastrointestinal disorders and malignancy. The predominant symptom, consciousness disturbance, was seen in 4 cases. Two of them showed a comatose state. Ocular symptoms and ataxia were observed in 2 cases. Laboratory findings revealed leukocytosis and anemia in 3 cases, hypoproteinemia in 4 cases. One case was alcoholic, but the other four were non-alcoholics and developed the disease after prolonged malnutrition. At the onset of the disease, 4 cases were receiving glucose and electrolyte infusion without vitamins, at the onset of the disease. We propose that in elderly patients with consciousness disturbance of unknown cause, Wernicke's encephalopathy should be taken into consideration even in non-alcoholics, and thiamine infusion should be commenced at once when the disease is suspected even when typical symptoms are lacking.
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PMID:[Clinical characteristics of Wernicke's encephalopathy in the elderly]. 156 Jun 7

A pilot study of 31 consecutive alcohol-related deaths over an 8-month period revealed the presence of histologically diagnosed Wernicke's encephalopathy in 17 cases. Analysis of the clinical records revealed that a disturbance of the mental state was the commonest finding and neurological signs were present in only 2 of the 17 cases (ataxia 1, peripheral neuropathy 1). Analysis of 22 ward admissions for Wernicke's encephalopathy during the same 8-month period revealed that the diagnosis is easily made when neurological deficits (ophthalmoplegia, ataxia) accompany mental changes and when Wernicke's encephalopathy is the predominant illness. In patients with established alcohol-related disease attention is often directed to the presenting illness so that Wernicke's encephalopathy may easily be overlooked as a cause of deterioration in the mental state in these patients. It is recommended that routine management of patients with alcohol-related disease should include thiamine even if neurological signs are absent.
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PMID:Wernicke's encephalopathy and alcohol-related disease. 177 21

Wernicke encephalopathy and Korsakoff psychosis are two facets of the same disease with well-determined cerebral lesions caused by thiamine deficiency. The disease occurs mainly in alcoholics, but other conditions (malabsorption or severe malnutrition) also predispose to the risk of Wernicke-Korsakoff syndrome. The incidence in Denmark is unknown. In the period 1.1.1979-31.12.1988, 24 patients (18 men and six women) were discharged from Rigshospitalet with the diagnosis Wernicke encephalopathy or Korsakoff psychosis. This represents about 0.05% of all admissions in the period. Eighteen out of the 24 cases (75%) were admitted in the past three years (1986-88). The mean age was 55 years. Twenty patients admitted alcohol abuse. The presenting symptoms and the patients' complaints showed great variety and were often related to other alcoholic complications, which could mask the disease. The classic symptom combination: eye movement abnormalities- ataxia and disorders of consciousness were found in seven patients (29%). Sixteen patients had disorders of consciousness or orientation. All the patients were treated with thiamine. The eye-movement disorder has recovered in eight out of ten known cases (80%), nystagmus--in six out of seven cases (86%) while ataxia, disorders of orientation and confabulation recovered in about 50% of cases. The average duration of hospitalisation was 50 days. Altogether nine patients died during the observation period. The condition is most probably underdiagnosed and the traditional diagnostic criteria are considered too rigid. The diagnosis should be considered in alcoholics who present even only one of the classical symptoms and in patients with alcohol dementia. Thiamine should be given on wide indications.
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PMID:[Wernicke-Korsakoff syndrome at the Rikshospitalet in 1979-1988. A retrospective study]. 192 15

Wernicke's encephalopathy should be considered as a possible diagnosis in comatose and hypothermic patients. The classic triad of confusion, ophthalmoplegia (or nystagmus) and ataxia may be absent, and the history of alcohol abuse or other causes of thiamine deficiency may be unknown. Left untreated, acute Wernicke's encephalopathy has a 17 percent mortality rate. Since the morbidity from Wernicke's encephalopathy is potentially reversible with parenteral thiamine, and large doses of thiamine can be given without documented ill effects, it is recommended that all comatose or hypothermic patients, as well as those with more classic presentations of Wernicke's encephalopathy, be given parenteral thiamine before administration of glucose.
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PMID:Wernicke's encephalopathy. 218 37

A 15-year-old boy with non-T ALL in early 2nd remission was autografted using a regimen with busulphan 4 mg/kg/day, po, from day -9 to -6, and cyclophosphamide 50 mg/kg/day, iv, from day -5 to -2. During busulphan administration he experienced a few generalized seizures, and starting on day 25 post ABMT he developed a progressively severe neurological symptomatology characterized by nystagmus, right VIth cranial nerve palsy, truncal ataxia and, finally, confusion and coma. MRI showed lesions in the periaqueductal gray matter, thalamus, mammillary bodies and putamen. Within 24 hours of treatment with thiamine he improved dramatically, but during the following weeks permanent neurologic damage with memory deficit, truncal ataxia and nystagmus became evident. To our knowledge this is the first case of Wernicke's encephalopathy reported after BMT. We suspect in this case a contribution of busulphan to the development of the syndrome.
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PMID:Wernicke-like encephalopathy after autologous bone marrow transplantation. 222 27

Lesions of the brain stem and cerebellum due to toxic substances or nutritional deficiencies are mostly seen in chronic alcohol abuse and, more rarely, in severe malnutrition. Four clinical entities are described: Wernicke's encephalopathy is the most frequent of these, with progressive development of disorders of consciousness, oculomotor palsies and ataxia. The condition regresses under massive vitamin BA therapy, but it often leaves neuropsychological sequelae (e. g. Korsakoff's syndrome). The best treatment is prevention by giving thiamine to alcoholics and to malnourished subjects who receive glucose infusions. Cerebellar atrophy of alcoholic origin is responsible for subacute ataxia which, as a rule, is definitive. Central pontine myelinolysis is rare and presents as sudden quadriplegia with pseudobulbar palsy and sometimes "locked-in" syndrome. Beside alcohol, a frequent cause is major ionic disorders, such as hyponatraemia or its excessively rapid correction. Pellagra is a classical disease rarely seen in this country. It is due to nicotinamide (vitamin PP) deficiency.
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PMID:[Lesions of the brain stem and cerebellum of alcoholic and nutritional deficiency origin]. 234 51

Wernicke encephalopathy is a disorder caused by a deficiency of thiamine which is a cofactor of several metabolic enzymes. The symptoms include mental confusion, ataxia, and ocular signs in adults, infants, and children. Patients often have somnolence and weakness combined with ophthalmoplegia. Alcoholics are the best known risk group; however, Wernicke encephalopathy occurs in poorly nourished patients of all ages. We present 2 children with malignant disease in whom Wernicke encephalopathy--an underdiagnosed and potentially fatal, but preventable and treatable disease--was diagnosed postmortem.
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PMID:Wernicke encephalopathy--a preventable cause of death: report of 2 children with malignant disease. 267 84

Records of the emergency medical admissions to a large teaching hospital over a one year period were examined for evidence of Wernicke's encephalopathy or Korsakoff's syndrome. It was found that only 0.4% of the population studied had the classical triad of Wernicke's encephalopathy, namely confusion, ophthalmoplegia, and ataxia. If two of these three criteria are allowed in the absence of other causes then 2.2% of the population had this limited Wernicke's encephalopathy or Korsakoff's syndrome. It is concluded that the diagnosis of Wernicke's encephalopathy should not rely on the presence of all three criteria; any two of the three in the absence of other causes will suffice for the diagnosis.
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PMID:Diagnosis of Wernicke's encephalopathy. 346 66


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