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Fulminant case of hereditary neuropathy with liability to pressure palsy
Author(s) -
Crum Brian A.,
Sorenson Eric J.,
Abad Gregorio A.,
Dyck P. James B.
Publication year - 2000
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/(sici)1097-4598(200006)23:6<979::aid-mus23>3.0.co;2-0
Subject(s) - medicine , mononeuropathy , fulminant , nerve biopsy , palsy , differential diagnosis , electromyography , muscle biopsy , pediatrics , peripheral neuropathy , surgery , physical medicine and rehabilitation , biopsy , pathology , diabetes mellitus , alternative medicine , endocrinology
Hereditary neuropathy with liability to pressure palsy (HNPP) is typified as isolated nerve palsies caused by trivial compression or trauma. It rarely presents in two extremities and even more infrequently affects all four limbs simultaneously. We present a patient who concurrently experienced right shoulder, left hand, and bilateral foot weakness mimicking several multifocal conditions. Electromyography suggested HNPP and subsequent nerve biopsy and genetic testing were confirmatory. The case demonstrates that HNPP can present in a fulminant manner and should be included in the differential diagnosis of acute multiple mononeuropathies. The possible causes for such a rapid clinical course in our patient are discussed. © 2000 John Wiley & Sons, Inc. Muscle Nerve 23: 979–983, 2000