Hereditary Neuropathy with Liability to Pressure Palsies Masquerading as Slowly Progressive Polyneuropathy
作者:
Kevin J. Felice,
Michael Poole,
Mila Blaivas,
James W. Albers,
期刊:
European Neurology
(Karger Available online 1994)
卷期:
Volume 34,
issue 3
页码: 173-176
ISSN:0014-3022
年代: 1994
DOI:10.1159/000117033
出版商: S. Karger AG
关键词: Tomaculous neuropathy;Hereditary neuropathy;Pressure palsies
数据来源: Karger
摘要:
Hereditary neuropathy with liability to pressure palsies (HNLPP) is a peripheral nerve disorder in which recurrent pressure palsies are associated with pathological swelling (‘tomacula’) of myelinated nerve internodes. We report a patient with a slowly progressive polyneuropathy associated with substantial slowing of conduction velocities initially suggestive of an acquired or hereditary demyelinating polyneuropathy. However, repeat electrodiagnostic studies demonstrated that conduction slowing and partial conduction block were localized primarily to common sites of nerve compression and entrapment. Sural nerve biopsy revealed multiple tomacula consistent with the diagnosis of HN
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