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.

La enfermedad de Marchiafava-Bignami: una realidad. Reporte de un caso / Marchiafava-Bignami Disease: a Reality. A Case Report

RESUMEN La enfermedad de Marchiafava-Bignami ha sido reconocida como un síndrome caracterizado por necrosis y desmielinización focal del cuerpo calloso. Fue descrita por primera vez en 1903 por los patólogos Marchiafava y Bignami en tres pacientes alcohólicos italianos, bebedores de vino tinto. Se reporta el caso de una paciente de 55 años, alcohólica, que acudió con crisis epilépticas generalizadas y deterioro del nivel de conciencia. La resonancia magnética de cráneo reveló desmielinización del cuerpo calloso, sustancia blanca y gris de los lóbulos parietales y frontales con lo que se diagnosticó una enfermedad de Marchiafava-Bignami aguda tipo A. La paciente falleció a causa de una neumonía intrahospitalaria a los 15 días luego del ingreso. Es necesario el conocimiento de esta enfermedad, porque a pesar del mal pronóstico de su forma aguda, se reportan casos de buena evolución con un diagnóstico y tratamiento oportunos. Se presenta el caso con el objetivo de evidenciar el diagnóstico de una enfermedad poco frecuente que se puede expresar de forma variable y califica como una urgencia médica.

ABSTRACT Marchiafava-Bignami disease has been recognized as a syndrome characterized by necrosis and focal demyelination of the corpus callosum. It was first described in 1903 by the pathologists Marchiafava and Bignami in three Italian alcoholic patients who drink red wine. The case of a 55-year-old patient is reported, alcoholic, who attended with generalized epileptic seizures and impaired level of consciousness. Magnetic resonance imaging of the skull revealed demyelination of the corpus callosum, white and gray matter of the parietal and frontal lobes, leading to the diagnosis of acute Marchiafava-Bignami type A disease. The patient died of hospital-acquired pneumonia 15 days later of income. Knowledge of this disease is necessary, because despite the poor prognosis of its acute form, cases of good evolution with timely diagnosis and treatment are reported. The case is presented with the aim of evidencing the diagnosis of a rare disease that can be expressed in a variable way and qualifies as a medical emergency.

Marchiafava-Bignami Disease in Crohn's Disease

No abstract available.

The diagnostic value of DTI and DKI technique in marchiafava bignami disease of chronic alcoholism / 实用放射学杂志

Objective To evaluate the diagnostic value of diffusion tensor imaging(DTI)and diffusion kurtosis imaging(DKI)in marchiafava bignami disease(MBD)of chronic alcoholism.The ROC curve was used to analyze the diagnostic significance of each parameter (FA,ADC and MK values).Methods Sixty-eight patients with chronic alcoholism were enrolled in this study,sixty normal middle-aged people were selected as the control group.The ROC curves of differentparameters in the diagnosis of chronic alcoholism were drawn,after whichthe thresholds were determined to evaluate the clinical significance of FA,ADC and MK values in the diagnosis of the disease.Results The FA and MK values of patients with chronic alcoholism were found to be significantly lower than those of the normal control group(P<0.01).ROC curve analysis showed that the critical values for the FA,ADC and MK parameters were 0.274,5.55 and 0.874,respectively.Conclusion Compared with the ADC and MK parameters,FA parameter has a high clinical significance in the diagnosis of MBD.

Clinical and imaging characteristics of acute marchiafava-bignami disease / 重庆医学

Objective To investigate the presentation and radiologic findings of acute marchiafava‐bignami disease(MBD) . Methods Three cases of acute MBD who were diagnosed and treated in our hospital were retrospectively analyzed ,including the clinical symptoms ,laboratory tests ,imaging examination(such as cranial CT ,magnetic resonance imaging(MRI) ,prognosis .Results Three cases were acute onset .The symptoms may be non‐specific ,such as consciousness disorder ,psychosis ,seizures ,delirium tremor and high fever .The imaging changes in the genu and splenium of corpus callosum could be found ,even in the bihemispheric white matter of all cases .CT revealed low‐density areas ,meanwhile MRI showed iso‐or hypo‐intensity on T1WI and ADC ,hyper‐in‐tensity on T2WI and fluid attenuated inversion recovery and restricted diffusion weighted imaging .The lesions involved in bihemi‐spheric brachium pontis in one case and in the body of corpus callosum in another case .Conclusion Acute MBD may present with various clinical forms ,but have characteristic imaging findings .

A Rare Case Of Sub-Acute Form Of Marchiafava-Bignami Disease Presenting Predominantly With Psychotic Symptoms

Objective: The objective is to present a rare case of sub-acute form of Marchiafava-Bignami disease presenting with psychosis, minimal cognitive impairment and no clinical neurological signs. Methods: This is a case report of a patient with Marchiafava-Bignami disease presenting to a tertiary care psychiatric hospital in Singapore. A review of the literature of the condition is also presented. Results: The patient presented with prominent psychotic symptoms in the context of chronic alcohol abuse. He also had minimal cognitive impairment and clinically no neurological signs. A working diagnosis of alcohol induced psychotic disorder was made. His psychotic symptoms seemed to be resistant to treatment with antipsychotic medications initially and this led to further investigation by MRI scan of brain which revealed atrophy of corpus callosum and no other significant abnormalities. His psychotic symptoms gradually improved with treatment. He is now placed in an intermediate care setting in the hospital while waiting for a suitable community placement. Conclusions: It is rare for Marchiafava-Bignami disease to present with prominent psychotic symptoms with minimal cognitive impairment and no neurological signs clinically. In patients with a history of chronic alcohol abuse presenting with psychotic disorder, Marchiafava-Bignami disease should be suspected as a diagnostic possibility.

Application of diffusion weighted imaging on diagnosis and therapy of acute marchiafava-bignami disease / 实用放射学杂志

Objective To study the value of diffusion weighted imaging (DWI)in Marchiafava-Bignami disease.Methods (1)12 cases of Marchiafava-Bignami disease (MBD)patients with 6 month follow-up and 12 hedthy adults were clone MRI DWI;(2)MR imaging characteristics of 12 patients were observed on the corpus callosum and the other gray-white matters;(3)The ADC values of the central part and marginal area of the corpus callosum and the other gray-white matters were measured,data analysis were carried out completely by random design.Results Hyperintensity on the corpus callosum were showed in 12 patients on DWI,typical“sandwich sign”was seen on the sagittal T2 WI in 1 1 cases,and gray-white matters beside the corpus callosum were involved other in 6 cases;The ADC values of central and marginal area of the corpus callosum and the other gray-white matters had significant differ-ence between the improved clinical symptom group and,unimproved clinical symptom group and the control group (P < 0.05 ). There were no significant differences in the ADC values for the other white matters.Conclusion DWI can be used to reflect the change of MBD.Low ADC values in the corpus callosum and cortex are associated with a poor prognosis.

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.

Enfermedad de Marchiafava-Bignami, a propósito de un caso clínico-imagenológico / Clinical and imaging issues - Marchiafava-Bignami’s disease, case report

RESUMEN: Arch Med Interna 2013 - 35(3):105-108 La Enfermedad de Marchiafava-Bignami descripta a principios del siglo XX en bebedores de vino tinto y posteriormente en consumidores de otras bebidas alcohólicas, es una de las complicaciones menos frecuentes de los alcoholistas crónicos, su reconocimiento ha sido puesto en evidencia en los últimos tiempos debido a la Resonancia Magnética (RM). Lamentablemente el pronóstico, no ha variado sustancialmente debido a que no existe una terapéutica específica.

ABSTRACT: Arch Med Interna 2013 - 35(3):105-108 The Marchiafava-Bignami disease described at the beginning of the twentieth century, in red wine drinkers and consumers subsequently spirits, is a less common complication of chronic alcoholics, their recognition has been highlighted in recent times due to the Magnetic Resonance (MR). Unfortunately the prognosis has not changed substantially since there is no specific therapy.

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.

Imaging and clinical characteristics of Marchiafava-Bignami disease: six cases / 中华神经科杂志

Objective To investigate the presentation and radiologic findings of MarchiafavaBignami disease (MBD).Methods Six cases of MBD who were diagnosed and treated in our hospital were retrospectively analyzed,including the imaging examination(such as cranial CT,magnetic resonance imaging (MRI)),laboratory tests,clinical symptoms and prognosis.Results Six cases of MBD disease were presented with swelled,thickened corpus callosum,iso-or hypo-intensity on T1WI,hyper-intensity on T2WI and fluid attenuated inversion recovery and restricted diffusion on diffusion weighted imaging.Coma of acute onset was the major clinical finding in case 1,case 4 and case 6,which showed the lesions in the entire corpus callosum and extended to the white matter,and the prognosis of these cases were worse.Slow reaction and memory decrease were the clinical findings in case 2 and case 3,which showed the genu and splenium of corpus callosum involvement on MRI and CT,and returned to normal with aggressive treatment.Numbness and weakness of the lower lambs were the major clinical finding in case 5,which showed that the lesion was limited in the splenium and body of corpus callosum on MRI.The patient recovered after treatment.Conclusions MBD may present with various clinical forms,but have characteristic imaging findings.Outset form and neuroimaging characteristics of MBD are critical for improvement of the clinical outcome.

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.

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: Diffusion-Weighted MRI in Cortical and Callosal Involvement

Marchiafava-Bignami disease (MBD) is a fatal disorder characterized by demyelination of the corpus callosum. MRI, suggestive of corpus callosum demyelination with associated white matter involvement in both cerebral hemispheres, indicates a diagnosis of MBD. In this case, MR diffusion-weighted findings taken at an acute stage of MBD revealed lesions not only in the corpus callosum but also in the cerebral cortex. Lower apparent diffusion coefficient values of the corpus callosum and cortical lesions were associated with poor clinical outcome.

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 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.