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)

In the course of multiple episodes of thiamine deficiency in the rhesus monkey, the triad of anorexia, apathy, and hind limb weakness is the earliest clinical manifestation. In later episodes, nystagmus, abducens paresis, midline ataxia, dysmetria, and congestive heart failure are also seen. With the exception of dysmetria, the neurologic signs promptly respond to thiamine administration. Pair-fed controls showed no clinical signs. Neither peripheral neuropathy nor edema was observed. Thiamine-deficiency in the experimental animals was confirmed by blood transketolase assays.
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PMID:Clinical manifestations of chronic thiamine deficiency in rhesus monkey. 40 80

Thiamin is one of the marginally adequate nutrients in the Australian diet. The incidence and prevalence of Wernicke-Korsakoff syndrome in this country may be the highest in the world. Homeless men could be at risk for low intakes of thiamin in association with irregular high alcohol intakes. A sample of 107 homeless men from two hostels and one clinic for homeless persons in inner Sydney was investigated for nutritional status; their thiamin status is reported here. By means of 24-hour recall methods, their mean dietary thiamin intake--0.76 mg per day--was less than the National Health and Medical Research Council's recommended dietary intake of 1.1 mg per day; at 0.076 mg/MJ it was even less than the minimal requirement of 0.08 mg/MJ. It was much lower than the mean intake of 1.38 mg per day that was found in the 1983 National Dietary Survey of adults and the distribution of thiamin intakes in this study was skewed positively, with the largest intake being in the range of 0-0.1 mg per day. On clinical examination we found a high prevalence of signs that were consistent with thiamin deficiency. Twenty-four per cent of the subjects showed three-or-more of the signs of the Wernicke-Korsakoff syndrome (ophthalmoplegia, nystagmus, ataxia, peripheral neuropathy and global confusion). In assaying for red-cell transketolase levels, this subgroup showed higher thiamin pyrophosphate effects than did the whole sample. Thirty-six per cent of the whole sample showed subnormal thiamin status by the thiamin pyrophosphate effect. Thus, in this sample, homeless men showed a high prevalence of dietary, biochemical and clinical features to indicate subclinical or early clinical thiamin deficiency.
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PMID:Thiamin status of a sample of homeless clinic attenders in Sydney. 204 82

Eleven alcoholics with cerebellar degeneration (eight with computerized tomography confirmation of cerebellar atrophy) were matched with nonataxic alcoholics and nonalcoholics. There were no laboratory or physiological markers for ataxia, including hemoglobin A1a + b, red blood cell transketolase, liver function enzymes, and measures of reaction time and hand-eye coordination. Acetaldehyde-modified hemoglobin levels (as hemoglobin A1a + b) did not, as previously reported, distinguish between alcoholics and nonalcoholics. There was 24% less annual alcohol consumption in ataxic alcoholics compared with nonataxic alcoholics, 9% less lifetime consumption in ataxic alcoholics, and 33% less maximal daily intake. The finding that ataxic alcoholics do not have higher alcohol consumption than nonataxic alcoholics suggests that alcoholic cerebellar degeneration is not a dose-dependent phenomenon, and that alcoholics with cerebellar degeneration may have an idiosyncratic sensitivity to the neuronal effects of alcohol.
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PMID:Alcoholic cerebellar degeneration is not a dose-dependent phenomenon. 330 8

Chronic thiamine deprivation in the rat leads to ataxia, loss of righting reflex and neuropathological damage to lateral vestibular nucleus. Before onset of neurological symptoms, transketolase (TK) activities were found to be selectively reduced by 25% in lateral vestibular nucleus and surrounding pons. Further progression of thiamine deprivation resulted in a generalized reduction in TK activity. Measurement of enzyme activity in the presence of added TPP cofactor in vitro did not lead to normalisation of enzyme activities suggesting loss of apoenzyme. Administration of thiamine to symptomatic thiamine-deprived rats resulted in reversal of neurological symptoms and to normalisation of defective TK activities in less vulnerable structures such as cerebral cortex, striatum and hippocampus; reduction of TK activity, however, persisted in brainstem and cerebellar regions. Pyrithiamine treatment results, within 3 weeks, in loss of righting reflex, convulsions and more widespread neuropathological damage compared to that observed following thiamine deprivation. TK activity was found to be significantly decreased before the onset of neurological symptoms in all brain regions and appearance of symptoms was accompanied by more severe reductions of TK. In contrast to chronic thiamine deprivation, TK activities following pyrithiamine treatment were: equally reduced in magnitude in vulnerable and non-vulnerable brain structures, unchanged following reversal of neurological abnormalities by thiamine administration.
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PMID:Activities of thiamine-dependent enzymes in two experimental models of thiamine deficiency encephalopathy: 3. Transketolase. 358

Yearling rainbow trout (Salmo gairdneri) were fed a purified diet with and without thiamin supplementation for 30 wk, at which time overt signs of thiamin deficiency appeared in the deficient group. Overt signs of thiamin deficiency were anorexia, darkening and ataxia. Death rapidly followed the development of overt thiamin deficiency. Transketolase activity and thiamin pyrophosphate levels were measured monthly in erythrocyte and liver samples. Significant differences in erythrocyte transketolase activity between fish fed the thiamin-deficient and control diets were measured after 24 wk of feeding. No significant difference in liver transketolase activity was found between trout fed diets with or without thiamin supplementation. Thiamin pyrophosphate levels were significantly lower in erythrocytes and liver of fish fed the thiamin-deficient diet after 16 wk of feeding. Thiamin pyrophosphate levels in erythrocytes and liver were found to be a more sensitive indicator of thiamin status of rainbow trout than erythrocyte or liver transketolase activity.
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PMID:Comparison of transketolase activity and thiamin pyrophosphate levels in erythrocytes and liver of rainbow trout (Salmo gairdneri) as indicators of thiamin status. 362 13

Chronic thiamine deprivation in the rat leads to selective neuropathological damage in brainstem structures whereas treatment with the central thiamine antagonist, pyrithiamine, results in more widespread damage. In order to further elucidate the neurochemical mechanisms responsible for this selective damage, the thiamine-dependent enzyme complex pyruvate dehydrogenase (PDHC) was measured in 10 brain structures in the rat during progression of thiamine deficiency produced by chronic deprivation or by pyrithiamine treatment. Feeding of a thiamine-deficient diet to adult rats resulted in 5-7 weeks in ataxia and loss of righting reflex accompanied by decreased blood transketolase activities. PDHC activities were selectively decreased by 15-30% in midbrain and pons (lateral vestibular nucleus). Thiamine treatment of symptomatic rats led to reversal of neurological signs and to concomitant reductions of the cerebral PDHC abnormalities. Daily pyrithiamine treatment led within 3 weeks to loss of righting reflex and convulsions and to decreased blood transketolase of a comparable magnitude to that observed in chronic thiamine-deprived rats. No significant regional alterations of PDHC, however, were observed in pyrithiamine-treated rats.
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PMID:Activities of thiamine-dependent enzymes in two experimental models of thiamine-deficiency encephalopathy: 1. The pyruvate dehydrogenase complex. 406 11

Gastric partitioning has been proposed as an alternative to jejunoileal bypass for the control of morbid obesity because of the relative ease of performance and the lack of metabolic complications. From our series of 250 patients who underwent gastric partitioning, one of them developed a neurologic syndrome consistent with Wernicke's disease. The clinical findings were characterized by ataxia, confusion, impaired extra-ocular movements with diplopia, and weakness. A second patient with a similar condition was referred to us for evaluation and treatment. Pretreatment thiamine levels were normal in one case and revealed thiamine deficiency in the second. The study group of 15 patients had thiamine and red cell transketolase levels determined before, 6 wk, and 1 yr after gastric partitioning. A significant drop in values was seen at 6 wk postoperatively. Gastric partitioning is a safe and effective alternative to jejunoileal bypass in the control of morbid obesity. However, severe permanent neurologic deficits, probably related to thiamine deficiency, may occur.
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PMID:Neurologic deficit following gastric partitioning: possible role of thiamine. 623 85

Two young females with severe morbid obesity presented with Wernicke's syndrome after Roux-en-Y gastro-jejunum bypass had been performed. The first patient had recurrent vomiting and dyplopia two months post-surgery. Physical examination indicated bilateral ophthalmoparesia with conserved convergence and ataxia. The second patient had frequent vomiting episodes over the previous three months together with lower limb hypotonia, myoclonia and generalised tonicoclonic seizures on two occasions within one year of surgery. In both cases routine blood test, ion levels (sodium, potassium, calcium, phosphates), electroencephalogram and CT scan were normal. Thiamine therapy was instigated on the basis of clinical intuition and the first patient achieved complete remission within 24 hours while the second improved gradually in that two years later only mild lower limb hypotonia and a slight cognitive deficit remains. Erythrocyte transketolase activity determinations were abnormal on two separate occasions for this second patient. Vitamin B1 determinations were not available for the first patient. In conclusion, the restriction in energy intake and the persistent vomiting together with malabsorption induced by the surgical intervention could explain the vitamin deficiency causing Wernicke's encephalopathy. This indicates a need for close monitoring and systematic vitamin supplementation in those patients who undergo bariatric surgery.
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PMID:Wernicke's syndrome after bariatric surgery. 1103 Oct 78

Polioencephalomalacia (PEM), hereafter used to refer to the specific lesion of cerebrocortical necrosis, developed in 11 of 110 mature cattle on pasture in central Saskatchewan. The primary water source contained a markedly elevated level of sodium sulfate (7200 ppm). The significant clinical findings of the herd investigation included depression, ataxia, cortical blindness, dysphagia, and death. Diagnosis of PEM was confirmed by histopathological evidence of cerebrocortical and subcortical necrosis with microvascular fibrinoid necrosis predominantly in the thalamic region of three affected cattle. The histopathology of sulfate-associated PEM observed in this herd appears to be unique and its features are presented and discussed. Mean levels for serum transketolase, copper, red blood cell transketolase activity, and thiamine (vitamin B(1)) in all exposed young (n = 100) and mature (n = 99) animals did not reveal evidence of deficiencies. Although the blood thiamine status of the seven surviving, affected animals was not evaluated before treatment with exogenous thiamine, 199 members of the herd had blood thiamine levels within the reference range at the time of the outbreak. The outbreak resolved after cattle were moved to a water source containing acceptable levels of sodium sulfate.
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PMID:Polioencephalomalacia in cattle consuming water with elevated sodium sulfate levels: A herd investigation. 1742 82

Thiamine (vitamin B(1)) is an essential component of a number of metabolic pathways and thiamine deficiency results in a progressive encephalopathy in both humans and animals. Confirming thiamine deficiency is problematic and relies on demonstrating reduced red blood cells transketolase activity, or indirect methods including urinary organic acid analysis and dietary analysis. The characteristic and selective vulnerability of different brain regions in carnivores has been demonstrated by magnetic resonance (MR) imaging in the dog and cat as an aid to diagnosis. A 2-year-old, female, domestic shorthair cat was presented with an acute onset of seizures and ataxia. MR imaging was consistent with thiamine deficiency and supplementation resulted in a progressive clinical improvement. Repeated MR imaging 4 days after starting thiamine supplementation revealed near complete resolution of the MR abnormalities. Repeated MR imaging following appropriate therapy may be useful to further confirm thiamine deficiency.
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PMID:Thiamine deficiency in a cat: resolution of MRI abnormalities following thiamine supplementation. 2067 25


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