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  • Title: Peripheral neuropathy in late-onset Krabbe's disease: histochemical and ultrastructural findings.
    Author: Matsumoto R, Oka N, Nagahama Y, Akiguchi I, Kimura J.
    Journal: Acta Neuropathol; 1996 Dec; 92(6):635-9. PubMed ID: 8960324.
    Abstract:
    We performed histochemical and ultrastructural studies on a sural nerve biopsy specimen obtained from a 31-year-old man with late-onset Krabbe's disease. Myelin sheaths were uniformly thin for the fiber diameter, with a moderate reduction in the myelinated fiber population. Despite a few small onion-bulb formations, quantitative studies demonstrated uniformly thin myelin sheaths and less variability in internodal lengths. This suggests that the main pathophysiology of the peripheral neuropathy observed in late-onset Krabbe's disease is hypomyelination rather than segmental demyelination as in infantile Krabbe's disease. Ultrastructurally, curvilinear lamellar cytoplasmic inclusions were observed in Schwann cells and fibroblasts.
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