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Clinical variability in type I familial amyloid polyneuropathy (Val30Met): Comparison between late‐ and early‐onset cases in Portugal
Author(s) -
Conceição Isabel,
De Carvalho Mamede
Publication year - 2007
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/mus.20644
Subject(s) - polyneuropathy , medicine , age of onset , amyloidosis , neuropathic pain , pediatrics , family history , disease , anesthesia
We compared early‐ with late‐onset cases in 86 patients with familial amyloidotic polyneuropathy type I (FAP). Among these patients, 43 presented before age 50 (early‐onset) and 43 after this age (late‐onset). Sex and geographical distribution were similar, although a family history of the disorder was more frequent in early‐onset cases. In late‐onset disease, autonomic dysfunction was less frequent, but organ involvement and severe neuropathic pain were more frequent. Neurophysiological measurements were similar. FAP is a cause of neuropathy in elderly patients, in particular in those presenting with neuropathic pain. Muscle Nerve, 2006