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Nervous and Ocular Disorders in Children with Cholestasis and Vitamin A and E Deficiencies

✍ Scribed by Fernando Alvarez; Pierre Landrieu; Paul Laget; Frédérique Lemonnier; Michel Odièvre; Daniel Alagille

Publisher
John Wiley and Sons
Year
2007
Tongue
English
Weight
583 KB
Volume
3
Category
Article
ISSN
0270-9139

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✦ Synopsis

Thirteen children, aged 10 months to 20 years, presenting with chronic cholestasis from the first month of life and with low serum levels of vitamins A and/or E, have been investigated for neurological and ophthalmological symptoms. Clinical findings consisted of 4 types: peripheral neuropathy; cerebellar dysfunction; abnormalities of eye movement, and retinal degenerative changes. The results of electrophysiological and morphological studies of muscle and nerves were consistent with neurono-axonal degeneration. Electrical abnormalities of the retina, especially a decrease of the b wave of electroretinogram, appear to be the first sign of the syndrome, allowing early detection. Evidence for vitamin deficiency (E or E+A) suggests substitutive parenteral treatment in such patients.

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