Abstract
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 atrophy, axonal spheroids and corpora amylacea; (2) neuronal lipofuscin accumulation in the third cortical layer of the cerebral cortex, thalamus, lateral geniculate body, twelfth and ambiguus nuclei, spinal horns and posterior root ganglia. Ultrastructurally, the lipopigments were of uniform granularity without lipid droplets.
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Received: 20 May 1996 / Revised, accepted: 6 October 1996
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Larnaout, A., Belal, S., Zouari, M. et al. Friedreich’s ataxia with isolated vitamin E deficiency: a neuropathological study of a Tunisian patient. Acta Neuropathol 93, 633–637 (1997). https://doi.org/10.1007/s004010050662
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DOI: https://doi.org/10.1007/s004010050662