Diagnóstico por imágenes de un síndrome de Wernicke Korsakoff con estigmas de Marchiafava-Bignami / maging diagnosis of Wernicke - Korsakoff syndrome with Marchiafava-Bignami stigmat

Las enfermedades de Marchiafava-Bignami y de Wernicke Korsakoff, se consideran complicaciones neuropsiquiátricas causadas por el consumo crónico de bebidas alcohólicas. Son encefalopatías poco frecuentes caracterizadas por una desmielinización y necrosis del cuerpo calloso, con la subsiguiente atrofia por daño en las partes bajas del cerebro (tálamo e hipotálamo). Se presenta un paciente masculino de 29 años, con antecedentes de alcoholismo, el cual acude a consulta de Oftalmología por presentar disminución de la visión del ojo derecho durante un año. Se le realizaron, tomografía simple y resonancia magnética con contraste endovenoso de cráneo, donde se observaron hallazgos radiológicos compatibles con el síndrome de Wernicke Korsakoff (ocasiona afectación de la memoria y el aprendizaje) con estigmas de Marchiafava-Bignami (enfermedad poco conocida). Es necesario el dominio de la epistemología de estas enfermedades, porque, a pesar del mal pronóstico en su forma aguda, se reportan casos con buena evolución, si se le realiza un diagnóstico y tratamiento oportunos.

Marchiafava-Bignami and Wernicke-Korsakoff diseases are considered neuropsychiatric complications caused by the chronic consumption of alcoholic beverages. They are rare encephalopathies characterized by demyelination and necrosis of the corpus callosum, with subsequent atrophy due to damage in the lower parts of the brain (thalamus and hypothalamus). We present a 29-year-old male patient with a history of alcoholism who went to the Ophthalmology consultation due to decreased vision in his right eye for a year. Simple tomography and magnetic resonance imaging with intravenous contrast of the skull were performed, observing radiological findings of Wernicke -Korsakoff syndrome (affect memory and learning) with Marchiafava-Bignami stigmata (little-known disease). Mastery of the epistemology of these diseases is necessary, because, despite the poor prognosis in its acute form, cases with good evolution are reported, if an opportune diagnosis and treatment is made.

Refractory Nonconvulsive Status Epilepticus with Favorable Outcome in a Patient with Marchiafava-Bignami Disease

No abstract available.

Wernicke Encephalopathy with Simultaneous Marchiafava-Bignami Disease

No abstract available.

Marchiafava-Bignami Disease in Crohn's Disease

No abstract available.

Marchiafava Bignami Disease Potentially Complicating Normal Pressure Hydrocephalus

Marchiafava-Bignami disease (MBD) is a rare disorder of demyelination or necrosis of the corpus callosum. Mainly, MBD is associated with alcohol and malnutrition. We report a 60-year-old woman with no history of alcohol consumption or malnutrition who had MBD as a possible complication of normal pressure hydrocephalus (NPH). The patient presented with a 2-month history of progressive gait unsteadiness, urinary incontinence, and forgetfulness, for which the patient underwent ventriculoperitoneal shunt surgery with remarkable improvement. Magnetic resonance imaging (MRI) demonstrated hyperintensity in the body and splenium of corpus callosum when she was brought to the hospital again with rapid deterioration of her mental ststus. It might be postulated that cerebrospinal fluid (CSF) tumor necrosis factor-alpha (TNF-alpha) might have contributed to the development of MBD although not measured in this patient, given that TNF-alpha, as a proinflammatory cytokine mediating demyelinating process have been found in be increased in the CSF of NPH.

Doença de Marchiafava-Bignami: uma rara entidade com prognóstico sombrio / Marchiafava-Bignami disease: a rare entity with a poor outcome

A doença de Marchiafava-Bignami é uma entidade rara, caracterizada por uma degeneração primária do corpo caloso, associada com o consumo crônico do etanol. A doença pode, ocasionalmente, ocorrer em pacientes não etilistas cronicamente desnutridos. Uma deficiência de vitaminas do complexo B é considerada como a hipótese etiopatogênica principal, uma vez que muitos pacientes obtiveram uma melhora após a administração desses compostos. Algumas vezes, entretanto, tal resposta terapêutica não foi observada. O diagnóstico definitivo da doença de Marchiafava-Bignami pode ser problemático e depende das características de estudos de neuroimagem, especialmente a ressonância magnética. Seu tratamento, dessa forma, é ainda controverso, com resultados variáveis. Como estão implicados fatores nutricionais, analogamente à encefalopatia de Wernicke, alguns autores recomendam a reposição de vitaminas do complexo B, particularmente da B1. O presente artigo relata a forma aguda da doença de Marchiafava-Bignami em um paciente masculino dependente do álcool, que apresentou discreta melhora após a administração parenteral das vitaminas do complexo B. Como consequência de suas más condições neurológicas e imunológicas, ele desenvolveu infecções pulmonares múltiplas e permaneceu, por longo tempo, na unidade de terapia intensiva. Seu óbito ocorreu por sepse causada por um fungo raro, o Rhodotorula mucilaginosa. O artigo é um relato clínico da evolução desse paciente, com a apresentação de seus dados de neuroimagem, acompanhada por uma revisão sobre doença de Marchiafava-Bignami e sobre as infecções por Rhodotorula dentro da perspectiva da unidade de cuidado intensivo.

Marchiafava-Bignami disease is a rare affliction characterized by primary degeneration of the corpus callosum associated with chronic consumption of ethanol. The disease may occasionally occur in patients who are not alcoholics but are chronically malnourished. A complex deficiency of group B vitamins is the main etiopathogenic hypothesis, and many patients improve after the administration of these compounds. However, a good response is not always observed. The definitive diagnosis of Marchiafava-Bignami disease can be problematic and is based on features of neuroimaging studies, especially magnetic resonance imaging. Its treatment is still controversial and shows variable results. Because nutritional factors are implicated, as in Wernicke's encephalopathy, some authors claim that replacement of B vitamins is beneficial. The present article is a case report of a severe acute form of Marchiafava-Bignami disease in an alcohol-dependent male patient who improved after the administration of parenteral B vitamins. As a consequence of his neurological and immunologic conditions, he developed multiple pulmonary infections and had a protracted course in the intensive care unit. He eventually died of sepsis associated with an uncommon fungus, Rhodotorula mucilaginosa. The present article reports the clinical and neuroimaging data from this patient and contains a review of Marchiafava-Bignami disease and Rhodotorula infections in the intensive care unit.

A Case of Marchiafava-Bignami Disease with Symmetrical Lesions of the Putamen in a Patient with Chronic Alcohol Dependence

Marchiafava-Bignami disease(MBD) is a rare complication of chronic alcoholism characterized by symmetrical demyelination of the corpus callosum. We report a case of MBD in a Korean patient having chronic alcohol dependence. The patient exhibited mental depression, weakness of all four limbs and dysarthria. Laboratory data showed mild hyponatremia. Magnetic resonance imaging(MRI) revealed unusual symmetrical resolving lesions of the putamen in addition to the typically observed lesion of the corpus callosum. The neurologic disturbances were gradually improved with the normalization of low plasma sodium levels. Marked improvement of abnormal MRI findings was noted after thiamine supplement, though the patient still exhibited severe cognitive impairment.

Alcohol-Related Neurologic Disorders: Ten Years of Experiences

Alcohol-related disorders are among the most costly health problems worldwide. Ingested alcohol is mainly metabolized by an oxidative pathway in the liver. Alcohol and its metabolic products (e.g., acetaldehyde and reactive oxygen species) have toxic effects on multiple organs, especially the nervous system. The diverse mechanisms of alcohol-related neurologic disorders include the direct toxic effects of alcohol, the alcohol withdrawal effect, nutritional deficiency secondary to alcoholism, and abnormalities of serum electrolytes and osmolality. We analyzed 156 cases of alcoholrelated neurologic disorders among admitted patients that had been referred in Korea during the previous 10 years. The duration of alcohol consumption ranged from 0.5 to 47 years (mean=17.8 years) and the mean amount of alcohol intake per day was 245.5 g. The 156 patients had the following diseases: Wernicke's encephalopathy (n=81, 51.9%), peripheral neuropathy (n=68, 43.6%), delirium tremens (n=59, 37.8%), Rum fit (n=31, 19.9%), pellagra encephalopathy (n=29, 18.6%), Korsakoff's psychosis (n=22, 14.2%), cerebellar atrophy (n=11, 7.0%), and alcoholic myopathy (n=6, 3.8%). We report on these cases and review the literature on alcohol-related neurologic disorders.

Callosal and Cortical Involvement in Acute Marchiafava-Bignami Disease

No abstract available.

Marchiafava-Bignami disease.

Marchiafava- Bignami disease is the symmetrical demyelination of the middle portion of the corpus callosum observed in people with chronic alcoholism. We report two male patients who had history of chronic alcoholism, different clinical presentation and MRI findings consistent with the diagnosis of Marchiafava-Bignami disease.

Acute Marchiafava-Bignami Disease With Typical White Matter Involvement on Diffusion Weighted MRI

Marchiafava-Bignami disease (MBD) is characterized by cerebral white matter lesions associated with chronic alcoholism. Premortem diagnosis of MBD is usually based on history and clinical manifestations. We report a case of acute MBD in which diffusion-weighted MRI (DWI) showed high signal intensities along the white matter including the corpus callosum. DWI may be useful in premortem diagnosis of acute MBD.

Acute Marchiafava-Bignami Disease with Widespread Callosal and Cortical Lesions

Marchiafava-Bignami disease (MBD) is a rare alcohol-related disorder that results in progressive demyelination and necrosis of the corpus callosum. The process may extend to the optic chiasm and tracts, cerebellar peduncle, subcortical resion, neighboring white matter, and rarely, cortical gray matter. We report a case of MBD in which fluid-attenuated inversion recovery and diffusion magnetic resonance imaging studies revealed symmetrical hyperintense lesions in the cerebral cortex in addition to the callosal lesions.

Cortical Involvement of Marchiafava-Bignami Disease: A Case Report

Marchiafava-Bignami disease is a rare complication of chronic alcoholism and this malady typically manifests as callosal lesion. I report here on one patient with Marchiafava-Bignami disease (MBD) who has symmetric restricted diffusion in both lateral-frontal cortices, in addition to the callosal lesion.

Marchiafava-Bignami disease with only slowly progressive cognitive impairment / Journal of Rural Medicine

We report on a right-handed 43-year-old policeman with atypical Marchiafava-Bignami disease (MBD). The typical clinical manifestations of MBD are reduced consciousness, confusion, seizures, psychotic and emotional symptoms, hemiparesis, dysarthria, ataxia, and coma and death. However, our patient had not developed any of the above symptoms except for slowly progressive cognitive impairment mimicking that of Alzheimer disease. The incidence of MBD may be higher and its prognosis less severe than generally believed. MBD has been underdiagnosed and underreported, and nonspecific general symptoms and encephalopathy in an alcoholic might indicate undiagnosed MBD.

Marchiafava-Bignami Disease with Abnormal PET Findings: Case Report

We report the FDG PET findings in a patient with Marchiafava-Bignami disease (MBD) in whom there was diffusely reduced metabolism in the whole brain cortex and strongly decreased metabolism in the thalami. The use of FDG PET helps provide an understanding of the neurologic manifestations and prognosis of MBD.

A Case of Marchiafava-Bignami Disease with Personality Change / 대한정신약물학회지

Marchiafava-Bignami Disease is a rare disorder characterized pathologically by demyelination of the corpus callosum. This disease could be related with chronic alcoholism although a proven etiology has not been reported. Marchiafava-Bignami Disease (MBD) could cause the fatal acute phase symptoms (seizure, coma, and death), various neurological symptoms (tremor, dysarthria, gait disturbance, apraxia), and cognitive impairments (memory impairment, disorientation). It is also reported that MBD causes a dementia. Approximately 250 cases have been reported regarding the MBD since it was first reported in 1903. However, only 20 cases have revealed a favorable prognosis. We found a case of MBD with personality change and chronic alcoholism subsequent to the repeated improvement and aggravation for 4 years. This case is demonstrated a atrophy of splenium of corpus callosum by Magnetic Resonance Imaging. The case also is diagnosed the personality changes, such as emotional irritability, impulsivity, and indignation-expose due to general medical condition, as described in Diagnostic and Statistic Manual of Mental Disorder 4th edition (DSM-IV). However, in early phase this diagnosis did not revealed. The patient was improved in impulse control and behavior by treatment with the Carbamazepine. We investigate the etiology, pathogenesis, symptom &sign, and treatment regarding the MBD in neuropsychiatric aspect.

A Case of Reversible Marchiafava-Bignami Disease Showing Severe Thalamic Hypometabolism in PET

No abstract available.

A Case of Marchiafava-Bignami Disease with Abnormal SPECT Findings

Marchiafa-Bignami disease(MBD) is a rare complication of chronic alcoholism and is pathologically characterized by a symmetrical primary demyelination of the corpus callosum with or without focal necrosis. The clinical features of the disease are quite variable, and the course is usually progressive and fatal. We present a case with characteristic clinical features and imaging findings, including SPECT, of MBD. A 51-year-old man was admitted due to mental confusion, violent behavior, and slowness of motion for 2 months. He had been a heavy alcoholic for about 20 years. He was in a stuporous state for 1 week beginning 2 weeks after his admission.. He then showed alertness but was still in a mute state. His cooperation and responses to noxious stimuli were very poor. A brain MRI showed focal cystic change in the enlarged corpus callosum and diffused high-signal intensity in the subcortical white matter, thalamus, and corpus callosum in T2WI. 99mTc-ECD SPECT revealed diffusely decreased cerebral perfusion in both frontal, temporal and pari-eto-occipital lobes.

A Case of Marchiafava-Bignami Disease with Reversible Brain MRI Findings of Corpus Callosal Lesions

Marchiafava-Bignami disease(MBD), characterized by the primary degeneration of the corpus callosum, is a rare complication of chronic alcoholism. Recently, a few cases of MBD with reversible neuro-imaging abnormalities were reported. A 58-year-old, chronic alcoholic man was admitted with mental change, dysarthria, and a seizure attack. A T2-weighted Brain magnetic resonance imaging demonstrated high signal intensities in the body and splenium of the corpus callosum, multiple white matter, and cortical gray matter. Treatment with a multiple vitamin complex resulted in a near complete recovery of neurological manifestation. A brain MRI obtained four weeks after admission revealed a dramatic resolution of previous imaging abnormalities. We report a case of Marchiafava-Bignami disease with reversible neuro-imaging abnormalities.

Two Cases of Marchiafava-Bignami Disease

We described 2 patients with Marchiafava-Bignami disease, a neurological disorder associated with chronic alcohol consumption. Patient 1 expressed general weakness and confusion, while patient 2 expressed dysphagia and seizures. Lesions involving the corpus callosum were identified with T2 weighted MR imaging in which there was a hyperintensity in the splenium of patient 1 and in the entire corpus callosum of patient 2. While the conditions of both patients were improved by with nutritional support, they were left with cognitive impairment.