Antemortem diagnosis of Baló concentric sclerosis: correlative MR imaging and pathologic features.

Abstract

To report a case of a rare demyelinating disease called Baló concentric sclerosis. A 32-year-old woman had left hemiparesis that progressed to hemiplegia, hemihypesthesia, and left hemianopsia. Laboratory evaluation was notable only for a mildly elevated erythrocyte sedimentation rate and an elevated level of myelin basic protein in cerebrospinal fluid. The antemortem diagnosis was made on the basis of magnetic resonance (MR) imaging of the brain, which showed numerous areas of concentric demyelination alternating with "spared" white matter. With the exception of a temporary improvement with high-dosage intravenous steroid therapy, the clinical course was monophasic and relentless, leading to death approximately 1 year after the onset of symptoms. Pathologic examination helped confirm the diagnosis. Because MR imaging shows alternating ringlike lesions involving the deep and superficial white matter, which correspond to pathologic findings, it may play a central role in antemortem diagnosis of this rare disease, leaving more invasive diagnostic procedures for evaluation of equivocal cases.