A Case of Marchiafava Bignami Disease: Complete Recovery With Thiamine

To the Editor: A 53-year-old married man presented with acute-onset altered behavior in the form of disorientation, altered consciousness, loss of memory, decreased sleep, restlessness, and tremors for the last 15 days, with little spontaneous improvement over the last 2 or 3 days. He had history of alcohol consumption daily for the last 32 years, with heavy drinking, about 750 ml per day, for the last 5 or 6 years, amounting to alcohol dependence syndrome. On evaluation of higher mental functions, he was conscious and oriented, but had impairment of immediate and recent memory. On cortical function examination, he had apraxia, left–right disorientation, agraphia, finger anomia, difficulty in complex verbal and written calculations, impairment in writing and drawing (he could not draw or copy properly), perseveration, and absence of insight into his illness. His gait, power, and tone reflexes were normal, and bilateral plantars were down-going. Routine blood investigations including liver function tests and serum B12 levels were normal. MRI brain scan was done on the 7th day. Axial T1, Flair, and fast-spin echo T2W MRI brain were studied and were correlated with coronal and sagittal FSE T2 weighted scans. These revealed atrophy of the corpus callosum, with a linear band of bright signal intensity on T2-weighted images within substance, suggestive of demyelination and necrosis. A diagnosis of Marchiafava Bignami disease was made on the basis of the above findings, along with alcohol dependence syndrome. Abstinence from alcohol and injectable thiamine 100 mg intramuscularly for 7 days along with thiamine tablets, 75 mg per day were administered. The patient was evaluated after 15 days of treatment. On evaluation, there was improvement in his condition. There was no impairment of recent and immediate memory, and calculations, writing, and drawing were improved. There were no apraxias, right–left disorientation, or finger agnosia. He started gaining insight into his illness. The patient was maintained on oral thiamine supplementation along with motivation-enhancement therapy.

Marchiafava Bignami disease (MBD) was first described in three alcoholic patients in Northern Italy at the beginning of the 20th century on the basis of post-mortem findings. MBD may present in various clinical forms. Acute, subacute, and chronic forms of the disease have been described.¹ With the use of MRI to diagnose MBD, a large number of patients with a better prognosis have been identified, and the diagnosis has also become easier than before. The MR images of MBD on conventional spin-echo sequences are characterized by usually symmetrical lesions of the corpus callosum situated in the central layer, sparing the dorsal and ventral layers (sandwich sign). The corpus callosum degenerates and splits into three layers and is referred to as layered necrosis.² Localization in different parts of the corpus callosum may be correlated with the course of the disease (chronic, subacute, and acute forms). Two clinico-radiologic forms have been proposed, which have been reported to be pertinent for clinical practice: type A and type B, differing in type of onset, symptoms, and prognosis.³ Our case deserves mention in view of the facts that early recognition and treatment of this illness may lead to a favorable outcome. Further studies are warranted to assess the various prognostic factors for this fatal illness.