Article

Acute Marchiafava-Bignami disease with selective involvement of the precentral cortex and splenium: a serial magnetic resonance imaging study.

Department of Neurology, College of Medicine, Kangwon National University, Chuncheon-si, Kangwon-do, Republic of Korea.
The Neurologist (Impact Factor: 1.48). 07/2011; 17(4):213-7. DOI: 10.1097/NRL.0b013e31821a25ae
Source: PubMed

ABSTRACT Marchiafava-Bignami disease (MBD) is defined pathologically as callosal degeneration associated with chronic alcoholism. We report a patient with MBD with acute lesions confined in the precentral cortex and splenium. Various magnetic resonance imaging (MRI) modalities were performed sequentially to elucidate the pathophysiology.
A 52-year-old man with chronic alcoholism developed acute confusion and dysarthria. He improved incompletely with nutritional supplementation. Diffusion-weighted imaging MRI disclosed the presence of reversible lesions with low apparent diffusion coefficient values in the precentral cortex and splenium. Perfusion-weighted imaging revealed that the cerebral blood volume and flow decreased and subsequently increased, and the mean transit time and time to peak were persistently prolonged. On magnetic resonance spectroscopy, choline was initially normal and became elevated during the recovery period, whereas N-acetylaspartate was low initially and after the resolution of the lesion on conventional MRI.
The precentral cortex and splenium are the most vulnerable areas in acute MBD. The lesions have reversible restricted diffusivity and hypoperfusion. The magnetic resonance spectroscopy findings correlate well with the clinical status.

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    ABSTRACT: Marchiafava-Bignami disease (MBD) is a rare condition mainly associated with alcoholism, although it may be mimicked by several other disorders that cause corpus callosum lesions. Our objective was to identify helpful features for differential diagnosis and assess whether any treatment can be recommended. We reviewed 122 reports containing data on 153 subjects with confirmed MBD that was associated with either alcoholism or malnutrition, and 20 reports with data on 53 subjects with conditions mimicking MBD. All the cases had been verified antemortem by brain imaging. Unconditional logistic regression was used to demonstrate factors that were associated with the outcome of MBD. The mimicking conditions were differentiated from MBD by the occurrence of solitary and rapidly disappearing splenial lesions; fewer signs and symptoms with exception of seizures, hemiparesis and tetraparesis; nystagmus; and rapid and complete recovery. MBD occurred most frequently among alcoholics, but it was also reported in 11 non-alcoholics (7.2% of all the MBD cases). A better outcome was observed among those who were treated within 2 weeks after onset of symptoms with parenteral thiamine (p=0.033). As thiamine deficiency is frequently associated with alcoholism, malnutrition and prolonged vomiting; we recommend prompt treatment of MBD with parenteral thiamine in such subjects. Recovery should be followed by repeated neuropsychological and MRI examinations, preferably using diffusion tensor imaging.
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