Friedreich’s ataxia with isolated vitamin E deficiency: a neuropathological study of a Tunisian patient

@article{Larnaout1997FriedreichsAW,
  title={Friedreich’s ataxia with isolated vitamin E deficiency: a neuropathological study of a Tunisian patient},
  author={Abdelmajid Larnaout and Samir Belal and Mourad Zouari and Moncef Fki and Christiane Ben Hamida and Hans Hilmar Goebel and M. Mehdi Ben Hamida and Fayçal Hentati},
  journal={Acta Neuropathologica},
  year={1997},
  volume={93},
  pages={633-637}
}
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… CONTINUE READING