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Sympathetic skin responses in hereditary sensory and autonomic neuropathy and familial amyloid neuropathy are different
Author(s) -
Shivji Zaitoon M.,
Ashby Peter
Publication year - 1999
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(199909)22:9<1283::aid-mus19>3.0.co;2-#
Subject(s) - autonomic neuropathy , sensory neuropathy , medicine , sensory system , amyloid (mycology) , amyloidosis , neuroscience , pathology , psychology , biology , genetics , cell culture , neuroblastoma
We compared the clinical and electrophysiological findings in seven patients with familial amyloid polyneuropathy (FAP) and six with hereditary sensory and autonomic neuropathy type I (HSAN). Both groups had progressive loss of pain and temperature appreciation, beginning distally in the feet. In HSAN, the median sensory nerve action potentials (SNAP) were lost early, but the sympathetic skin responses (SSR) were always preserved. In FAP, the SSR were lost at an early stage. The SSR may thus help to distinguish between these two causes of small‐fiber neuropathy early in the course of the disorder. © 1999 John Wiley & Sons, Inc. Muscle Nerve 22: 1283–1286, 1999