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Related Experiment Videos

Optic nerve in globoid leukodystrophy (Krabbe's disease). Ultrastructural changes.

S Brownstein, K Meagher-Villemure, R C Polomeno

    Archives of Ophthalmology (Chicago, Ill. : 1960)
    |May 1, 1978
    PubMed
    Summary

    Globoid leukodystrophy, or Krabbe's disease, involves progressive neurological decline and optic atrophy. Autopsy revealed abnormal myelin metabolism, leading to retinal degeneration due to optic nerve damage.

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    Area of Science:

    • Neurology
    • Ophthalmology
    • Biochemistry

    Background:

    • Globoid leukodystrophy (Krabbe's disease) is a rare, inherited lysosomal storage disorder.
    • It is characterized by progressive demyelination and severe neurological impairment.
    • Ocular manifestations, including optic atrophy, are common and contribute to visual loss.