Segmental demyelination of peripheral nerves in Krabbe's disease.

Abstract

THE current literature contains many references to peripheral neuropathies in a variety of conditions1–4. This communication describes a peripheral neuropathy associated with Krabbe's globoid cell leucodystrophy which is a progressive heredofamilial neurological disorder of infancy, and is usually diagnosed by either brain biopsy or at necropsy by the demonstration of characteristic “globoid” cells in the white matter. Bachhawat et al.5 ave described a deficiency of cerebroside sulphotransferase in the brain of two cases. Clinical descriptions and details of investigations of a series of patients suffering from this rare condition are in preparation.