Unusual MRI findings in a case of Marchiafava Bignami disease

Abstract

Twenty-four hours after surgery for acute aortic dissection, a 51-year-old man with alcoholism developed agitation, decreased consciousness, mutism, and upbeat nystagmus on upward gaze. He also showed left-sided weakness with pyramidal signs and spastic limb ataxia. After 4 weeks treatment with thiamine supplementation, his neurologic and general condition improved. MRI findings are shown in the figure.Marchiafava Bignami disease is associated with chronic alcoholism and nutritional deficiency. 1,2 Although etiopathogenesis is unknown, alcohol toxicity and osmotic disorders are in relation with typical corpus callosum demyelination. Other CNS structures such us white matter tracts and middle cerebellar peduncles may be involved, but far less frequently.S. Bellido, MD, I. Navas, MD, M.A. Aranda, MD, R. Ginestal, MD, B. Venegas, MD, Madrid, Spain Author contributions: Dr. Bellido: drafting/revising the manuscript, study concept or design, analysis or interpretation of data. Dr. Navas: drafting/revising the manuscript, study concept or design, analysis or interpretation of data. Dr. Aranda: drafting/revising the manuscript, study concept or design, analysis or interpretation of data. Dr. Ginestal: drafting/revising the manuscript, study concept or design, analysis or interpretation of data. Dr. Venegas: drafting/revising the manuscript, study concept or design, analysis or interpretation of data.The authors report no disclosures relevant to the manuscript. Go to Neurology.org for full disclosures. This article, along with others on similar topics, appears in the

Clinical and radiological features of Marchiafava–Bignami disease

“…Autopsy reports of alcoholic patients with MBD found that the third layer of the cortex was primarily affected. [ 23 ] Some researchers have proposed that the cortex and the corpus callosum are the most vulnerable regions of acute MBD. The cortical lesions might not be caused by heavy alcohol consumption but by deficiency of thiamine.…”

Marchiafava-Bignami disease with haemophagocytic lymphohistiocytosis as a postoperative complication of cardiac surgery

“…In addition, histological examination of the CNS was consistent with MBD. Speaking about the spreading of lesions, Bellido et al reported unusual MRI findings in a patient who had onset of MBD in the early postoperative period of acute aortic dissection 8. They reported that the middle cerebellar peduncles and white matter tracts were included in MBD.…”

Reversible Hyperintensity in Middle Cerebellar Peduncles, an infrequent finding in Marchiafava-Bignami Disease

“…MBD is an infrequent pathology (although probably underdiagnosed), affecting most frequently middleaged male (40-60 years of age) 1 . Its pathophysiology is unknown, it is associated with chronic alcoholism and malnutrition (including vitamin de ciencies, especially thiamine) 2 producing demyelinating lesions involving the corpus callosum 1,3 . The few cases described demonstrate a variable clinical presentation 4 , including ataxia, impairment of mental status, etc.…”

“…Given the non-speci c symptomatology, the diagnosis in the past was based on postmortem pathological examination, and currently is based on the characteristic neuroimaging ndings (symmetric lesions of the Corpus Callosum). Extracallosal lesions such as cortical/subcortical white matter lesions are well documented, but the MCP-sign is rare, with only a few cases reported 1,3,5 . Treatment includes vitamin reconstitution and supportive care 4,6 .…”