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

A 21 year old male, well-nourished and non-alcoholic, died after five weeks illness. He had suffered epileptic fits, bilateral internuclear ophthalmoplegia, bulbar and pontine paralysis, tetraparesia, ataxia and dystonia. A CT brain scan showed low density lesions of the striatum bilaterally. Post-mortem studies revealed pathological anomalies compatible with Leigh's disease, although the presence of haemorrhages and involvement of the mamillary bodies could also suggest Wernicke's encephalopathy.
 ...
PMID:Necrotising haemorrhagic encephalomyelopathy in an adult: Leigh's disease. 357 37

Chronic thiamine deprivation in the rat leads to selective neuropathological damage to pontine structures. Onset of neurological symptoms of thiamine deprivation (ataxia, loss of righting reflex) was accompanied by selective decreases (of the order of 30%) in the activity of alpha-ketoglutarate dehydrogenase (alpha KGDH) in lateral vestibular nucleus and hypothalamus. Enzyme activities were decreased to a lesser extent in medulla oblongata, striatum and hippocampus and were unchanged in other brain structures. No changes in alpha KGDH occurred prior to the onset of neurological signs of thiamine deprivation. Administration of the central thiamine antagonist, pyrithiamine, results within 3 weeks in loss of righting reflex and convulsions and in more widespread neuropathological changes than those observed following thiamine deprivation. alpha KGDH activities were found to be substantially diminished in all brain regions studied following pyrithiamine treatment with most severe changes occurring in brain regions found to be vulnerable to pyrithiamine (lateral vestibular nucleus, hypothalamus, midbrain, medulla-pons). In some cases, alpha KGDH changes preceded the appearance of neurological symptoms of pyrithiamine treatment. Such decreases in alpha KGDH may explain previous findings of region-selective changes in energy metabolism and of decreased synthesis of glucose-derived neurotransmitters (acetylcholine, GABA, glutamate) in pyrithiamine-treated rat brain. Thiamine administration to symptomatic pyrithiamine treated rats resulted in reversal of neurological signs of encephalopathy and in normalisation of defective alpha KGDH activity in all brain regions. These findings suggest that the reversible neurological symptoms associated with Wernicke's Encephalopathy in man likely result from region-selective impairment of alpha KGDH.
 ...
PMID:Activities of thiamine-dependent enzymes in two experimental models of thiamine-deficiency encephalopathy. 2. alpha-Ketoglutarate dehydrogenase. 372 63

Wernicke's encephalopathy has been associated rarely with fasting. We describe a middle-aged patient who presented in a subacute fashion with features of thiamin deficiency after marked weight reduction. Associated features were chest pain, possibly related to cardiac involvement, and a proximal myopathy. The Wernicke-Korsakoff syndrome has been described in the literature for around 100 years. However, the classic triad of ataxia, ophthalmoplegia and impaired conscious state that occurs in a person with chronic alcoholism is by no means its exclusive presentation.
 ...
PMID:Wernicke's encephalopathy after prolonged fasting. 373 79

Thirty-two cases of Wernicke's encephalopathy were admitted to hospital for treatment in a period of 33 months; this represented a relatively low incidence in total hospital admissions. Thiamin status was deficient, borderline and normal in 21 (66%), five (16%) and six (19%) patients, respectively, and responded immediately to treatment in those who had abnormal thiamin status. Ophthalmoplegia responded rapidly to treatment. Nystagmus, ataxia, disturbance of mental function and peripheral neuropathy responded incompletely to treatment in both the short-and the long-term. The overall setting for the development of Wernicke's encephalopathy appears to be chronic alcohol abuse, accompanied by cerebral "atrophy" and liver disease, but often without gross evidence of malnutrition.
 ...
PMID:Wernicke's encephalopathy in a metropolitan hospital. A prospective study of incidence, characteristics and outcome. 394 14

A young woman developed Wernicke's encephalopathy after gastroplasty for morbid obesity. Her clinical presentation of nystagmus and ocular gaze palsies, ataxia, and a sensorimotor neuropathy is described. A small number of previously described patients are compared with respect to clinical features, response to treatment, and investigative procedures.
 ...
PMID:Wernicke's encephalopathy after gastroplasty for morbid obesity. 396 9

A case of coma due to Wernicke's encephalopathy complicated by respiratory failure is described. Ventilation and thiamine administration lead to recovery, although Korsakoff's psychosis and ataxia persisted. A review of similar cases of coma emphasizes the absence of diagnostic features, but that if structural disease is excluded the presence of pupillary and ocular signs may support a diagnosis of Wernicke's encephalopathy. The response to thiamine may be diagnostic as in 8 of these patients who received it, but the long-term morbidity remains high.
 ...
PMID:Reversible coma in Wernicke's encephalopathy. 402 93

Thiamine deficiency in the monkey is the animal counterpart of Wernicke's disease in humans. In the present study, thiamine deficiency was induced in 11 monkeys while three monkeys were given paired feedings supplemented by thiamine hydrochloride and three monkeys were maintained on regular chow. The typical clinical symptoms were apathy, inattention to peripheral stimuli, ataxia, ptosis, mydriasis progressing to pupillary areflexia, nystagmus, and ophthalmoparesis progressing to total ophthalmoplegia. With thiamine treatment, recovery was prompt and complete in mild to moderate cases but delayed and incomplete in severe cases. The animals were killed six or more months after discontinuance of the experiments to determine the chronic effects of treated thiamine deficiency. The significant abnormalities in the brain stem were symmetric gliosis and neuronal loss in the inferior colliculi, the regions of the third and sixth nerve nuclei, and the medial vestibular nuclei. White matter was characteristically spared. With the exception of the inferior colliculi, the target sites for neuropathologic changes were the centers for ocular motor control.
 ...
PMID:Ocular signs in thiamine-deficient monkeys and in Wernicke's disease in humans. 402 52

Brain stem auditory evoked responses (BAERs) were performed on 25 alcoholic patients with Wernicke-Korsakoff syndrome, 56 alcoholic patients without Wernicke-Korsakoff syndrome, 24 of whom had cerebellar ataxia, and 37 control subjects. Abnormal BAERs were found in 48% of patients with Wernicke-Korsakoff syndrome, in 25% of alcoholic patients without Wernicke-Korsakoff syndrome but with cerebellar ataxia, and in 13% of alcoholic patients without Wernicke-Korsakoff syndrome or ataxia. The mean value of the I-V interval was prolonged in all patient groups. There were more patients with prolonged I-V and I-III intervals in the Wernicke-Korsakoff syndrome group than in the group without the syndrome. The I-III interval was prolonged in 32% of those with Wernicke-Korsakoff syndrome but in only 6% of alcoholics without the syndrome. These abnormalities improved following thiamine treatment and abstinence from alcohol. The presence of prolonged I-III interval in an alcoholic should raise the possibility of Wernicke's encephalopathy.
 ...
PMID:Brain stem auditory evoked responses in chronic alcoholics. 407 76

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.
 ...
PMID:Neurologic deficit following gastric partitioning: possible role of thiamine. 623 85

A case of Wernicke's encephalopathy with ataxia, confusion, memory loss, partial seizures of complex behavior and hypothermia, subsequent to thiamine depletion due to chronic malnourishment and triggered by an episode of acute vomiting and diarrhea, is reported, Computerized tomography (CT-scan) depicted small bilateral lesions in areas adjacent to the walls of the third ventricle, common location of the lesions seen in autopsy material of Wernicke's encephalopathy. Early diagnosis and treatment with vitamin B complex supplemented with intensive mnemonic and cognitive therapy led to complete recovery in a ten day period.
 ...
PMID:Wernicke's encephalopathy. A case report with neurophysiologic and CT-scan studies. 686 52


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>