FRIEDREICHS ATAXIA WITH ISOLATED VITAMIN-E-DEFICIENCY - A NEUROPATHOLOGICAL STUDY OF A TUNISIAN PATIENT

The neuropathological findings in a Tunisian patient with Friedreich's ataxia with vitamin E deficiency are reported. The main histological changes are: (1) spinal sensory system demyelination with neuronal atr ophy, axonal spheroids and corpora amylacea; (2) neuronal lipofuscin a ccumulation in the third cortical layer of the cerebral cortex, thalam us, lateral geniculate body, twelfth and ambiguus nuclei, spinal hems and posterior root ganglia. Ultrastructurally, the lipopigments were o f uniform granularity without lipid droplets.