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Adult metachromatic leukodystrophy with an unusual relapsing-remitting course.
  1. M. Sadeh,
  2. A. Kuritzky,
  3. E. Ben-David,
  4. Y. Goldhammer
  1. Department of Neurology, Chaim Sheba Medical Center, Tel Hashomer, Israel.

    Abstract

    A 46 year old woman had a relapsing-remitting course of hemiparesis, disorientation, paraparesis and seizures, followed by progressive dementia, spasticity and ataxia. Computed tomography at onset showed a parietotemporal hypodense area with diffuse mottled enhancement obliterating the lateral ventricle. Subsequent scans demonstrated symmetric periventricular non-enhancing hypodensities, progressive ventricular enlargement and atrophy. Adult metachromatic leukodystrophy was diagnosed on the basis of low leukocyte arylsulphatase A level and metachromatic material accumulation at neural nerve biopsy.

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