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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

Marchiafava-Bignami Disease (MBD) is a rare, severe and usually fatal neurological disorder associated with chronic alcoholism. Previously, the definite diagnosis was confirmed at the autopsy. After the era of modern imaging technology, diagnosis was based on clinical profiles, history of alcoholism and specific location of pathology in corpus the callosum demonstrated by MRI. The authors reported a case of MBD in a 41 year-old alcoholic Thai male who presented with acute confusion and ataxia. MRI of the brain demonstrated demyelination, edema and necrosis of the corpus callosum with extensive symmetrical subcortical white matter lesions. He had a dramatic recovery after treatment with intravenous thiamine. Follow-up MRI revealed atrophic and cystic changes of the corpus callosum and almost complete resolution of the subcortical lesions. Recently, 15 cases of MBD with specific corpus callosal lesion, demonstrated by MRI, were published in the English literature. All had a favorable outcome after treatment with thiamine. Only one case had extensive extracallosal lesions and this case also had a good recovery after treatment. Now, MBD is not a fatal disease and early diagnosis and treatment are crucial.
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PMID:Marchiafava-Bignami disease: a case report. 1232 51

We report on a 16-month-old boy who presented with truncal ataxia and intermittent nystagmus. Magnetic resonance imaging (MRI) at 19 months showed a T (2)-hyperintensity of the splenium and the genu of the corpus callosum with extension into the adjacent frontal white matter. Diffusion tensor imaging (DTI) revealed a corresponding area of restricted diffusion, suggesting cytotoxic oedema. The extent and localisation of the signal abnormalities mimic tissue injury as seen in Marchiafava-Bignami disease (MBD). Metabolic investigations were normal. Follow-up imaging at 24 months showed a similar T (2)-hyperintensity of the corpus callosum and white matter while on DTI the cytotoxic oedema had resolved. Clinically a remaining truncal and gait ataxia, clumsiness and a developmental delay is seen. Goal of this case report is (a) to present a rare case of Marchiafava-Bignami-like injury of the corpus callosum in an infant and (b) to discuss the neuroradiological imaging findings including MRI and DTI.
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PMID:Marchiafava-Bignami-like injury of the corpus callosum in an infant. 1621 9

Marchiafava-Bignami disease (MBD) is a rare neurological disorder mostly seen in alcoholic and malnourished patients with a pathognomonic hallmark of corpus callosum demyelination. MBD in nonalcoholics without malnutrition has rarely been reported. We report a case of MBD in a diabetic patient, without alcoholism or malnutrition, caused by a wide range of glycemic level fluctuations. A 38-year-old man presented with sudden onset of alteration in speech and multiple falls in three days. Neurologic examination showed dysarthria, dysmetria, and ataxia but, otherwise, normal cranial nerves, motor and sensory functions, and tendon reflexes. Brain MRI showed symmetric abnormalities in the splenium of the corpus callosum. In addition, demyelination was also observed in bilateral posterior limbs of the internal capsule and brachium ponti. His symptoms significantly improved after stabilization and normalization of his plasma glucose level and administration of multivitamins and corticosteroids. The underlying pathophysiology of the development of MBD in our case is likely to be osmotic stress from a wide range of glycemic fluctuations causing structural and functional disturbance of oligodendrocytes, which may be reversible in its early stage.
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PMID:Marchiafava-bignami disease in a nonalcoholic diabetic patient. 2371 Mar 88

Marchiafava-Bignami disease (MBD) is a rare, toxic demyelinating disorder of the central nervous system associated with chronic alcoholism and malnutrition. The clinical presentation is varied and non-specific, including symptoms of acute dementia, impaired consciousness, dysarthria, hemiparesis, pyramidal tract signs, seizure activity, ataxia and signs of interhemispheric disconnection. The differential diagnosis of MBD may include Wernicke's encephalopathy, multiple sclerosis, encephalitis, infectious or paraneoplastic leucoencephalopathy, infarction, Alzheimer's disease, multi-infarct dementia and frontotemporal lobar degeneration (Pick) disease. The diagnosis of MBD is dependent on MRI findings of hyperintensity of the corpus callosum on T2 and fluid-attenuated inversion recovery T2 sequences, with or without extracallosal lesions. The use of MRI in diagnosis has allowed for early initiation of treatment with parenteral thiamine, and improved the prognosis of MBD from frequently fatal to a mortality of less than 8%. Administration of thiamine within 14 days of symptom onset has demonstrated statistically better outcomes over delayed treatment. We present a case report of MBD diagnosed in a 72-year-old woman who presented with ataxia and slurred speech, in an effort to highlight the importance of obtaining MRI in patients presenting with behavioural disturbance and neurological findings, as well as discuss the relationship between thiamine supplementation and demyelinating diseases in the central nervous system.
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PMID:Rare case of Marchiafava-Bignami disease due to thiamine deficiency and malnutrition. 3330 6