Neuromuscular dysfunction and ultrastructural pathology in children with chronic cholestasis and vitamin E deficiency. Ann Neurol 1983 Mar;13(3):291-6
Date
03/01/1983Pubmed ID
6847142DOI
10.1002/ana.410130311Scopus ID
2-s2.0-0020671904 (requires institutional sign-in at Scopus site) 30 CitationsAbstract
The ultrastructural pathology of nerve and muscle and the neurological dysfunction in children with cholestatic liver disease and vitamin E deficiency have not been previously correlated. We studied two children with this syndrome. One child, 11 years of age, had severe hyporeflexia and decreased vibratory sense. Nerve conduction was delayed. The second child, 2 years of age, was neurologically normal. Both children showed ultrastructural evidence of damage to the sural nerve and accumulation of electron-dense deposits in the muscle fibers. Abnormalities of the nerves included disruption of the myelin sheath and separation and degeneration of the inner and outer components of the Schmidt-Lanterman incisure.
Author List
Werlin SL, Harb JM, Swick H, Blank EAuthor
Steven L. Werlin MD Emeritus Professor in the Pediatrics department at Medical College of WisconsinMESH terms used to index this publication - Major topics in bold
BiopsyChild
Cholestasis
Female
Humans
Infant
Male
Microscopy, Electron
Muscles
Nerve Fibers, Myelinated
Neuromuscular Diseases
Sural Nerve
Vitamin E Deficiency
