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Query: UMLS:C0004134 (ataxia)
 15,886 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report four cases of childhood ataxia with and without encephalopathies which were successfully managed with thiamine supplementation and diagnosed in retrospect as being caused by thiamine deficiency. The clinical presentations were similar to those previously reported amongst adolescents and adults who ingested the larvae of Anaphe venata, a local delicacy among the Ijeshas of south-western Nigeria, during the rainy season. This has been called seasonal ataxia syndrome. The cases were of interest because the disorder was not described in children in the previous report, which might indicate a worsening nutritional status in these communities. This report is to alert child health physicians in the developing world to the possibility of a pre-existing thiamine deficiency progressing into ataxic disorders when certain foods are ingested.
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PMID:Thiamine deficiency: a cause of childhood ataxia not to be ignored. 1572 Aug 94

Wernicke encephalopathy consists of a triad of ophthalmoplegia, ataxia, and altered mental status. It is caused by thiamine deficiency and although it is commonly seen in alcoholics, patients undergoing gastric bypass surgery for morbid obesity could be a new group of patients to watch for. Florid papilloedema and optic neuropathy are not commonly seen but are still consistent with the diagnosis of Wernicke encephalopathy. The most striking feature remains the dramatic recovery after replacement of thiamine.
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PMID:You are what you eat. 1596 92

Seven Kuvasz puppies from 2 same-parentage litters developed weakness and ataxia. Six necropsied dogs had lesions in caudate nucleus, cerebellar nuclei and folia, and spinal cord. Lesions seen were felt to be familial or due to the effects of an amprolium-induced thiamine deficiency on the developing brains of these puppies.
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PMID:A degenerative encephalomyelopathy in 7 Kuvasz puppies. 1601 62

A 6-year-old dog, a 4-year-old dog and three 7-week-old puppies were diagnosed with thiamine deficiency caused by feeding sulphite treated meat. The 6-year-old dog presented with a history of inappetence, weight loss and vomiting that rapidly progressed to signs of multifocal intracranial disease including mental dullness, paresis, seizures, spontaneous nystagmus and strabismus. Thiamine pyrophosphate effect was elevated at 58% and magnetic resonance imaging revealed bilaterally symmetrical hyperintensity of the caudate nucleus and rostral colliculi. The dog recovered with thiamine supplementation. The 4-year-old dog and three 7-week-old puppies also presented with rapidly progressive multifocal central nervous system signs including ataxia, paresis, increased muscle tone, seizures, nystagmus and exophthalmos. The 4-year-old dog made a rapid recovery with thiamine supplementation. Euthanasia and necropsy of a puppy revealed malacia of multiple brainstem nuclei and oedema of the cerebral cortex. These findings were consistent with thiamine deficiency.
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PMID:Thiamine deficiency in dogs due to the feeding of sulphite preserved meat. 1603 79

Wernicke's encephalopathy (WE) is an acute neuropsychiatric condition due to thiamine deficiency (vitamin B1) most commonly associated with chronic alcohol abuse. WE is difficult to diagnose because the classical triad of signs (confusion, ataxia and ophthalmoplegia) occurs in only 10% of cases. The presentation is often one of a non-specific confusional state which may easily be attributed to intoxication, alcohol withdrawal or to a concurrent morbidity such as head injury. To improve the outcome, it is important to make a presumptive diagnosis of WE and treat the patients as soon as possible with high-dose parenteral thiamine. Patients with an alcohol problem associated with malnutrition should all be offered a preventive treatment with parenteral thiamine in view of the impaired oral thiamine absorption.
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PMID:[Prevalence, prophylaxis and treatment of Wernicke encephalopathy. Thiamine, how much and how do we give it?]. 1611 48

Wernicke encephalopathy is a neurologic disorder that results from thiamine deficiency. It is associated with a classic triad of symptoms consisting of ataxia, ocular motor cranial neuropathies, and changes in consciousness. We report 3 cases of Wernicke encephalopathy in which MR imaging, including diffusion-weighted imaging, was performed at the onset and during follow-up. MR imaging findings were correlated with the clinical status of both the acute and chronic stage of Wernicke encephalopathy.
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PMID:MR imaging with diffusion-weighted imaging in acute and chronic Wernicke encephalopathy. 1621 37

We report a clinical and neuroradiological description of a severe case of Wernicke's encephalopathy in a surgical patient. After colonic surgery for neoplasm, he was treated for a long time with high glucose concentration total parenteral nutrition. In the early post-operative period, the patient showed severe encephalopathy with ataxia, ophthalmoplegia and consciousness disorders. We used magnetic resonance imaging (MRI) to confirm the clinical suspicion of Wernicke's encephalopathy. The radiological feature showed hyperintense lesions which were symmetrically distributed along the bulbo-pontine tegmentum, the tectum of the mid-brain, the periacqueductal grey substance, the hypothalamus and the medial periventricular parts of the thalamus. This progressed to typical Wernicke-Korsakoff syndrome with ataxia and memory and cognitive defects. Thiamine deficiency is a re-emerging problem in non-alcoholic patients and it may develop in surgical patients with risk factors such as malnutrition, prolonged vomiting and long-term high glucose concentration parenteral nutrition.
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PMID:Wernicke's encephalopathy in a malnourished surgical patient: clinical features and magnetic resonance imaging. 1622 8

Acute Wernicke's encephalopathy (WE) is caused by profound vitamin B1 (thiamine) deficiency and commonly presents with the classic clinical triad of mental confusion, ataxia, and ophthalmoplegia. This characteristic presentation results from the propensity of acute thiamine deficiency to preferentially injure specific brain regions: the dorsomedial thalamus, periaqueductal gray, and mamillary bodies. In these regions, abnormal magnetic resonance signaling on conventional sequences has been well described; however, diffusion restriction has only recently been reported. The authors demonstrate diffusion-weighted imaging (DWI) abnormalities of the splenium of the corpus callosum in a patient with acute WE, which has not been reported previously, and suggest a potential pathological mechanism. With the recent addition of DWI, MRI is becoming more sensitive to the changes in acute WE. Furthermore, the use of apparent diffusion coefficient mapping to evaluate the extent of likely underlying cytotoxic injury may help determine long-term response to vitamin therapy and, thus, disability.
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PMID:Restricted diffusion of the splenium in acute Wernicke's encephalopathy. 1625 4

Wernicke encephalopathy (WE) is a rare but known complication of severe hyperemesis gravidarum caused by thiamine deficiency. This article presents an unusual case that occurred at our institution and reviews the 48 previously published cases of WE in pregnancy. Considering all the 49 cases, the mean (+/-standard deviation) patients' age was 26.7 +/- 4.9 years, the mean gestational age when WE manifested was 14.3 +/- 3.4 weeks, and the mean duration of vomiting and feeding difficulties was 7.7 +/- 2.8 weeks. Wernicke's classic triad (confusion, ocular abnormalities, and ataxia) manifested in only 46.9% (23 of 49) of the patients. Confusion affected 63.3% (31 of 49) of the patients, ocular signs 95.9% (47 of 49) and symptoms 57.1% (28 of 49), and ataxia 81.6% (40 of 49). Deterioration of consciousness affected 53.1% (26 of 49) of the subjects and memory impairment 61.2% (30 of 49). Complete remission of the disease occurred in only 14 of 49 cases. Symptom resolution required months and permanent impairments were common. The overall pregnancy loss rate, directly (spontaneous fetal loss) and indirectly (planned abortion) attributable to WE, was 47.9% (23 of 49). The diagnosis of WE is clinical and can be rapidly confirmed by magnetic resonance imaging. We emphasize the importance of thiamine supplementation to women with prolonged vomiting in pregnancy, especially before intravenous or parenteral nutrition. We also underline the necessity to promptly replace vitamin B1 when neurologic symptoms and/or signs develop in a patient with hyperemesis gravidarum.
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PMID:Hyperemesis gravidarum complicated by Wernicke encephalopathy: background, case report, and review of the literature. 1655 77

A 1-yr-old intact male African lion (Panthera leo) fed only beef muscle meat was evaluated for episodes of hypermetric ataxia, generalized weakness and tonic-clonic front limb movements. A hemogram, biochemical profile, blood lead, electrocardiogram, survey radiographs, and brain computed tomography were normal. Cerebral spinal fluid analyses suggested mild inflammation. Acetylcholine receptor antibody and serologic tests for all infectious agents tested were negative. Clinical signs resolved completely 9 days after instituting oral thiamine (3 mg/kg/day) and a completely nutritional diet. This lion's pretreatment thiamine blood value (11 nmol/L) was markedly lower than that of a healthy lion (191 nmol/L) and a proposed reference range for adult African lions (160-350 nmol/L). The lion remained clinically normal 2 yr later when his blood thiamine value was 340 nmol/L. African lions can develop clinical primary thiamine deficiency and may respond favorably when thiamine treatment and adequate diet are instituted prior to irreversible neuronal necrosis.
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PMID:Presumed primary thiamine deficiency in a young African lion (Panthera leo). 1731 74


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