Pathological involvement of primary sensory neurons in Werdnig-Hoffmann disease.

Abstract:

:Seven patients with the typical clinical picture and muscle biopsy findings of classical Werdnig-Hoffmann disease showed Wallerian degeneration in their biopsied sural nerves. In dorsal root ganglia of one patient there were residual nodules and several chromatolytic neurons. By electron microscopy the changes of chromatolysis were confirmed and found to be consistent with an axonal reaction. Involvement of the primary sensory neuron is probably a regular pathologic feature of Werdnig-Hoffmann disease. The type of abnormality suggests in initial failure of the axon distal to the nerve roots.

journal_name

Acta Neuropathol

journal_title

Acta neuropathologica

authors

Carpenter S,Karpati G,Rothman S,Watters G,Andermann F

doi

10.1007/BF00690973

subject

Has Abstract

pub_date

1978-05-24 00:00:00

pages

91-7

issue

2

eissn

0001-6322

issn

1432-0533

journal_volume

42

pub_type

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