Adult metachromatic leukodystrophy: three cases with normal nerve conduction velocities in a family

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Here, we report three cases of late onset metachromatic leukodystrophy (MLD) within a family. The patients presented with psychiatric disturbances and dementia. The arylsulphatase A (ASA) level in leucocytes was zero in all the patients. The cranial magnetic resonance imaging (MRI) revealed bilateral symmetrical demyelination but the nerve conduction velocities were normal in all three cases. The clinical, biochemical, imaging and electrophysiological data of the family has been discussed.

Keywords: Sjögren's syndrome; azathiopine; magnetic resonance imaging; myelopathy; treatment

Document Type: Research Article


Affiliations: 1: Department of Neurology, Faculty of Medicine, Ondokuz Mayıs University, Samsun, Turkey, 2: Department of Pathology, Faculty of Medicine, Ondokuz Mayıs University, Samsun, Turkey

Publication date: June 1, 2002

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