ISSN:
1432-0533
Keywords:
Key words Late-onset Krabbe’s disease
;
Polyneuropathy
;
Hypomyelination
;
Cytoplasmic
;
inclusion body
Source:
Springer Online Journal Archives 1860-2000
Topics:
Medicine
Notes:
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.
Type of Medium:
Electronic Resource
URL:
http://dx.doi.org/10.1007/s004010050573
