ANTEMORTEM DIAGNOSIS OF BALO CONCENTRIC SCLEROSIS - CORRELATIVE MR-IMAGING AND PATHOLOGICAL FEATURES

PURPOSE: To report a case of a rare demyelinating disease called Ba16 concentric sclerosis. MATERIALS AND METHODS: A 32-year-old woman had l eft hemiparesis that progressed to hemiplegia, hemihypesthesia, and le ft hemianopsia. Laboratory evaluation was notable only for a mildly el evated erythrocyte sedimentation rate and an elevated level of myelin basic protein in cerebrospinal fluid. RESULTS: The antemortem diagnosi s was made on the basis of magnetic resonance (MR) imaging of the brai n, which showed numerous areas of concentric demyelination alternating with ''spared'' white matter. With the exception of a temporary impro vement with high-dosage intravenous steroid therapy, the clinical cour se was monophasic and relentless, leading to death approximately 1 yea r after the onset of symptoms. Pathologic examination helped confirm t he diagnosis. CONCLUSION: Because MR imaging shows alternating ringlik e lesions involving the deep and superficial white matter, which corre spond to pathologic findings, it may play a central role in antemortem diagnosis of this rare disease, leaving more invasive diagnostic proc edures for evaluation of equivocal cases.