Peripheral and Central Myelinopathy in Cockayne's Syndrome. Report of 3 Siblings
- 1 August 1982
- journal article
- case report
- Published by Georg Thieme Verlag KG in Neuropediatrics
- Vol. 13 (03) , 161-167
- https://doi.org/10.1055/s-2008-1059617
Abstract
Three siblings with Cockayne's syndrome are reported. Sural nerve biopsies revealed segmental de- and remyelination with onion-bulb formation. Disturbed visual and brainstem auditory evoked responses indicated demyelination of the central nervous system. The peripheral and central myelinopathy increased with age, suggesting a progressive disorder. Our observations support the theory of Cockayne's syndrome being a leukodystrophy.Keywords
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