Author:
Lamarche J.,Luneau C.,Lemieux B.
Abstract
ABSTRACT:We report the preliminary study of a dorsal root ganglion obtained at biopsy during a surgical intervention in a patient with Friedreich’s ataxia. There was an apparent decrease in the number of large myelinated fibers without necrosis, but with numerous axonal swellings consisting mainly of dense accumulated neurofilaments. Large amounts of lipofuscin were also found as well as some onion bulb formations suggesting a process of axonal atrophy.
Publisher
Cambridge University Press (CUP)
Subject
Clinical Neurology,Neurology,General Medicine
Reference13 articles.
1. Elias E. , Muller D.P.R. , and Scott J. (1981). Association of spino-cerebellar disorders with cystic fibrosis or chronic childhood cholestasis and very low serum vitamin E. The Lancet: 1319–1321.
2. Dyck P.J. and Lais A.C. (1973). Evidence for segmental demyelination secondary to axonal degeneration in Friedreich’s ataxia. In: Kakulas B. (ed) Clinical Studies in Myology. Int. Congr. Series. Amsterdam K. , Excerpta Medica: 252–263.
3. Pathomechanics and Management of Scoliosis in Friedreich Ataxia Patients: Preliminary Report
4. Does a defect of energy metabolism in the nerve fiber underlie axonal degeneration in polyneuropathies?
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