ISSN:
1432-0533
Keywords:
Ataxia
;
Telangiectasia
;
Friedreich's ataxia
;
Peripheral sensory neuropathy
;
Sural nerve biopsy
;
Loss of large fibers
Source:
Springer Online Journal Archives 1860-2000
Topics:
Medicine
Notes:
Summary The bioptical morphometric and ultrastructural study of sural nerve in a 17-year-old boy with ataxia-telangiectasia (AT) is reported. Our findings include a loss of fibers, particularly of large ones, axonal degenerative changes, Schwann cell inclusions of various type, and rare signs of primary demyelination. Teased-fiber study showed paranodal myelin enlargements, segmental demyelination, shortening, and/or variability of internodal length. This picture is similar to that in Friedreich's ataxia (FA), although they differ in degree and time of onset. A correct neuropathologic diagnosis of AT cannot be made on the basis of sural nerve biopsy alone.
Type of Medium:
Electronic Resource
URL:
http://dx.doi.org/10.1007/BF00688296
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