Infantile neuroaxonal dystrophy and giant axonal neuropathy: are they related?

Abstract:

:Light and electron microscopic findings from two sural nerve biopsies obtained at a one-year interval from a patient with the clinical features of Seitelberger's disease are described. Ballooned axons with accumulations of membranous profiles, vesicles, mitochondria, and a homogeneous center were present, and there were masses of 90 A filaments in endothelial, endoneurial, perineurial, and Schwann cells. These pathological alterations were less prominent in the second nerve biopsy, which showed a more pronounced decrease in myelinated fibers. The case shows that a generalized increase of 90 A filaments in structures of the peripheral nervous system is not a phenomenon exclusively occurring in patients with giant axonal neuropathy and, furthermore, that it may be a transitory feature.

journal_name

Ann Neurol

journal_title

Annals of neurology

authors

Begeer JH,Houthoff HJ,van Weerden TW,de Groot CJ,Blaauw EH,le Coultre R

doi

10.1002/ana.410060614

subject

Has Abstract

pub_date

1979-12-01 00:00:00

pages

540-8

issue

6

eissn

0364-5134

issn

1531-8249

journal_volume

6

pub_type

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