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Neuropediatrics 1985; 16(4): 194-201
DOI: 10.1055/s-2008-1059536
© Georg Thieme Verlag KG Stuttgart · New York

Peripheral Nerve Involvement in Children with Chronic Cholestasis and Vitamin E Deficiency

A Clinical, Electrophysiological and Morphological StudyP.  Landrieu1 , Jacqueline  Selva2 , F.  Alvarez3 , Angéle  Ropert4 , Stéphane  Métral4
  • 1Service de Neuropédiatrie, Hôpital de Bicêtre, F-94270 Bicêtre, France, and Laboratoire de Neuropathologie experimentale de la Faculte Parix XI
  • 2Laboratoire d'Histoembryologie-cytogenetique, Hôpital de Bicêtre, F-94270 Bicêtre, France
  • 3Unité de recherce en Hepatologie infantile INSERM U 56, Hôpital de Bicêtre, F-94270 Bicêtre, France
  • 4Laboratoire de Neurophysiologie, Hôpital de Bicêtre, F-94270 Bicêtre, France
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Publication History

Publication Date:
30 April 2008 (online)

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Abstract

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.

Key words

Vitamin E deficiency - Peripheral nerve - Ultrastructure - Morphometry - Cholestasis

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