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 Neuropatholjournal_title
Acta neuropathologicaauthors
Carpenter S,Karpati G,Rothman S,Watters G,Andermann Fdoi
10.1007/BF00690973subject
Has Abstractpub_date
1978-05-24 00:00:00pages
91-7issue
2eissn
0001-6322issn
1432-0533journal_volume
42pub_type
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