Marchiafava-Bignami disease - Computed tomographic scan, Tc-99m HMPAO-SPECT, and FLAIR MRI findings in a patient with subcortical aphasia, alexia, bilateral agraphia, and left-handed deficit of constructional ability

Objectives: To report and discuss the neuropsychological deficits and neuro imaging findings in a patient with probable Marchiafava-Bignami disease. Design and Method: A right-handed woman with chronic alcoholism demonstrate d mutism, impaired comprehension of spoken language, alexia, and right-hand ed agraphia. The syndrome of interhemispheric disconnection was manifested by left-handed deficit of constructional ability and agraphia. The patient underwent brain computed tomographic scans, technetium 99 hexylmethylpropyl ene amineoxime-single photon emission computed tomography, and magnetic res onance imaging (MRI) that also included fluid attenuated inversion recovery images. Setting: Clinical neurology department. Results: The patient's symptoms were related to scattered lesions of the co rpus callosum and to extensive symmetrical lesions of the centrum semiovale . Only the latter were detected by computed tomographic scans. Results of s ingle photon emission computed tomography did not show areas of focal hypop erfusion. Results of fast spin-echo MRI showed all lesions were hyperintens e in T-1-weighted images and hypointense in T-2-weighted images. Fluid atte nuated inversion recovery images revealed that periventricular lesions had a hypointense core surrounded by a hyperintense rim; callosal lesions were still hyperintense. Conclusions: We believe that our patient's symptoms are due to the disconti nuous affection of the corpus callosum and to the bilateral cutting of the outflow from the cortex. The MRI findings may be interpreted as indicating central necrosis and peripheral demyelination of periventricular lesions an d demyelination of the corpus callosum. The combined use of fast spin echo and fluid attenuated inversion recovery MRI reproduced with more accuracy t han fast spin echo MRI alone some features of Marchiafava-Bignami disease k nown from observations at autopsy.