Marchiafava- Bignami disease (MBD): A rare case report

S Sharmika,K Ajantha,T Peranantharajah,J Nalayini

doi:10.4038/jmj.v33i2.141

Abstract

Marchiafava-Bignami disease (MBD) is a rare neuro degenerative disease characterized by demyelination of corpus callosum. Clinical diagnosis of MBD is challenging due to its nonspecific neurological manifestations. It’s promptly diagnosed by brain Magnetic Resonance Imaging (MRI). Prompt treatment with high dose thiamine could be lifesaving. Here we report a 68 year old male with chronic alcoholism who was diagnosed to have MBD and successfully treated with high doses of intravenous thiamine administration.

Highlights

Marchiafava-Bignami disease (MBD) is a rare neuro degenerative disorder characterized by demyelination of corpus callosum
It is diagnosed by degeneration of corpus callosum in brain Magnetic Resonance Imaging (MRI)
Despite adequate correction of Sodium, he did not show improvement but deteriorated further. He was subjected to further imaging with MRI brain that revealed hyper intensity signals in T2W1 and significant atrophy of corpus callosum

Summary

Introduction

Marchiafava-Bignami disease (MBD) is a rare neuro degenerative disorder characterized by demyelination of corpus callosum. [1] It is most commonly seen in ill-fed chronic alcoholics, and observed in poorly nourished nondrinkers. [2] The clinical manifestations are nonspecific with a wide variation in neurological deterioration. Low Sodium levels were attributed to SIADH (Syndrome of Inappropriate Anti Diuretic Hormone secretion) due to alcoholism with low serum osmolality, high urine osmolality and increased urinary Sodium excretion with normal TSH and cortisol levels Despite adequate correction of Sodium, he did not show improvement but deteriorated further He was subjected to further imaging with MRI brain that revealed hyper intensity signals in T2W1 and significant atrophy of corpus callosum. Figure Axial T2 weighted image shows significant atrophy of genu (upward arrow) and hyper intensity of splenium (downward arrow) of corpus callosum. His thiamine dose was increased from 300 to 1500mg daily and continued for a week in the ward. Patient clinical condition markedly improved with that and he was able to walk and talk normally

Discussion

Conclusion