Peripheral nerve involvement in children with chronic cholestasis and vitamin E deficiency. A clinical, electrophysiological and morphological study.
Seven children with early onset cholestasis who developed signs of peripheral neuropathy were investigated before and after one and a half to three years of treatment by vitamin E. This neuropathy appeared to be due to neuronoaxonal degeneration, but unusual Schwann cell inclusions were also observed. Although the treatment stabilized or improved the condition of all patients, no striking changes were noted neither in the EMG nor in the second nerve biopsy of a patient treated for three years. As the pathological process concerned it is probably both a developmental disorder and a degenerative phenomenon, substitutive vitamin E treatment should be proposed very early in life.
D000293:Adolescent; D002648:Child; D002779:Cholestasis / Q000473:pathology / Q000503:physiopathology*; D002908:Chronic Disease; D004576:Electromyography; D005500:Follow-Up Studies; D006801:Humans; D009132:Muscles / Q000294:innervation; D009413:Nerve Fibers, Myelinated / Q000648:ultrastructure; D009431:Neural Conduction; D010525:Peripheral Nerves / Q000473:pathology; D010523:Peripheral Nervous System Diseases / Q000473:pathology / Q000503:physiopathology*; D010543:Peroneal Nerve / Q000503:physiopathology; D012583:Schwann Cells / Q000648:ultrastructure; D014811:Vitamin E Deficiency / Q000473:pathology / Q000503:physiopathology*