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Vitamin E deficiency in Werdnig-Hoffmann disease

Y Shapira, R Amit, E Rachmilewitz

    Annals of Neurology
    |September 1, 1981
    PubMed
    Summary
    This summary is machine-generated.

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    Infants with Werdnig-Hoffmann disease (WHD) show significantly lower vitamin E levels, suggesting a potential role for this deficiency in the disease

    Area of Science:

    • Neurology
    • Biochemistry

    Background:

    • Neuromuscular disorders affect infants and children.
    • Vitamin E is a crucial antioxidant.

    Purpose of the Study:

    • To investigate vitamin E levels in children with neuromuscular disorders.
    • To explore the relationship between vitamin E deficiency and Werdnig-Hoffmann disease (WHD).

    Main Methods:

    • Plasma vitamin E levels were measured in infants and children.
    • Patients included those with WHD, congenital myopathies, and muscular dystrophy.
    • A therapeutic trial of vitamin E was conducted in WHD patients.

    Main Results:

    • Seven out of eight infants with WHD exhibited significantly lower plasma vitamin E levels compared to controls (p < 0.01).

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  • Vitamin E deficiency in WHD was not attributed to malabsorption.
  • Vitamin E supplementation did not alter the disease's progression in three WHD patients.

    • Conclusions:

    • Low vitamin E levels are a characteristic finding in Werdnig-Hoffmann disease.
    • Vitamin E deficiency may be implicated in the pathogenesis of WHD.
    • Further research is needed to understand the role of vitamin E in WHD.