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Ultrastructure of peripheral nerve in Cockayne's syndrome
Authors:S. Roy  R. N. Srivastava  P. C. Gupta  G. Mayekar
Affiliation:(1) Department of Pathology, All-India Institute of Medical Sciences, Ansar Nagar, New Delhi 16, India;(2) Department of Pediatrics, All-India Institute of Medical Sciences, New Delhi, India;(3) Department of Neurology, All-India Institute of Medical Sciences, New Delhi, India
Abstract:Summary The ultrastructure of sural nerve biopsies was studied in two sisters with Cockayne's syndrome. Both had severe physical and mental retardation and evidence of peripheral neuropathy. Striking alterations in the myelin sheath with relative preservation of the axis cylinder were noted in both. There were also electron dense bodies in the Schwann cells. These findings support the suggestion that Cockayne's syndrome may be a form of leukodystrophy.
Keywords:Cockayne's Syndrome  Peripheral Nerve  Electron Microscopy  Demyelination
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