Case of the Week
Section Editors: Matylda Machnowska1 and Anvita Pauranik2
1University of Toronto, Toronto, Ontario, Canada
2BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada
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May 5, 2016
Marchiafava-Bignami Disease
- Background:
- Marchiafava-Bignami disease is a rare disorder characterized by demyelination and necrosis of the corpus callosum, commonly seen in patients with alcoholism.
- The central layers of the corpus callosum are affected, with sparing of the dorsal and ventral layers, producing sandwich sign.
- Other white matter areas involved are the anterior and posterior commissures, cortico-spinal tract, hemispheric white matter, and middle cerebellar peduncles.
- Clinical Presentation:
- Cognitive impairment, gait disturbance, seizures, coma
- Key Diagnostic Features:
- A patient with alcoholism with MRI showing hyperintensity of the corpus callosum on T2WI and T2-FLAIR
- MR spectroscopy may be a useful adjunct technique, which may reveal NAA/Cr ratio declining during the first 4 months after the onset of disease, followed by a lactate peak and, finally, a lipid peak. These findings reverse following clinical recovery.
- Differential Diagnosis:
- Corpus callosum glioma: Produces enlargement of the corpus callosum with mass effect
- Demyelination: Lesions are usually discrete and shows perpendicular arrangement called Dawson’s fingers
- Vascular lesions: Lesions are usually more discrete
- Wernicke encephalopathy: Typically affects hypothalamus, thalamus, and periaqueductal gray matter
- Treatment:
- No specific proven treatment
- Parenteral thiamine is usually administered.
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