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September 1988

Computed Tomography and Magnetic Resonance Imaging in Adult-Onset Leukodystrophy

Author Affiliations

From the Department of Neurology, Veterans Administration (VA) Medical Center, Washington, DC (Dr Schwankhaus); the Department of Radiology, Georgetown University Hospital, Washington, DC (Dr Patronas); the Department of Radiology, Medical Center Hospital of Vermont, Burlington (Dr Dorwart); the Neuroepidemiology Branch (Dr Eldridge) and the Neuroimmunology Branch (Dr McFarland), National Institute of Neurological and Communicative Disorders and Stroke, Bethesda, Md; and the Interinstitute Medical Genetics Program, Warren Grant Magnuson Clinical Center, Bethesda, Md (Ms Schlesinger).

Arch Neurol. 1988;45(9):1004-1008. doi:10.1001/archneur.1988.00520330094015

• Five clinically affected and nine atrisk members of a kindred with an autosomal dominant adult-onset leukodystrophy simulating chronic progressive multiple sclerosis were studied with computed tomography (CT) and magnetic resonance imaging (MRI). Computed tomographic scans showed white matter lucencies occurring earliest and most prominently in the frontoparietal region. The lesions were nondiscrete, diffuse, and bilaterally symmetric. These changes were more clearly visualized as areas of increased signal intensity with T2-weighted MRI. Magnetic resonance imaging also showed increased signal intensity in the brain stem, cerebellar white matter, or both of four patients. Both MRi and CT differentiated this entity from multiple sclerosis, but MRI was superior to CT in detailing the extent of white matter involvement.

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