TY - JOUR
T1 - Patterns of neuropathy and autonomic failure in patients with amyloidosis
AU - Wang, Annabel K.
AU - Fealey, Robert D.
AU - Gehrking, Tonette L.
AU - Low, Phillip A.
N1 - Funding Information:
This study was supported by grants from the National Institutes of Health (Grant Nos. NS3 2352, NS2 2352, NS3 9722, NS4 4233, NS4 3364, RR00585 ) and from Mayo Clinic.
PY - 2008/11
Y1 - 2008/11
N2 - OBJECTIVE: To define the clinical patterns of peripheral neuropathy and autonomic testing abnormalities in patients with amyloidosis. PATIENTS AND METHODS: A retrospective chart review was conducted of 65 patients who had biopsy-proven amyloidosis and autonomic function testing between January 1, 1985, and December 31, 1997, at Mayo Clinic's site in Rochester, MN. Patients were required to have neurologic evaluation, autonomic reflex screening, and tissue confirmation of amyloidosis. RESULTS: We identified 5 clinical patterns of peripheral neuropathy: (1) generalized autonomic failure and polyneuropathy with pain (40 patients [62%]), (2) generalized autonomic failure and polyneuropathy without pain (11 [17%]), (3) isolated generalized autonomic failure (7 [11%]), (4) polyneuropathy without generalized autonomic failure (4 [6%]), and (5) generalized autonomic failure and small-fiber (ie, autonomic and somatic C-fiber) neuropathy (3 [5%]). Moderately severe generalized autonomic failure, involving adrenergic, cardiovagal, or sudomotor domains, was found in all patients, including those without clinically manifested autonomic failure. The diagnosis of amyloidosis was delayed in patients who did not have initial symptoms of pain or generalized autonomic failure (48 months to diagnosis in patients with polyneuropathy without autonomic failure vs 12 months to diagnosis in patients with autonomic failure and small-fiber neuropathy; P=.57). CONCLUSION: Physicians should test for symptoms of generalized autonomic failure in patients who have peripheral neuropathy of unknown origin. Autonomic testing may give abnormal results in patients without overt symptoms of autonomic failure. Early recognition of autonomic failure may lead to earlier diagnosis of the underlying pathogenesis of amyloidosis, as well as earlier treatment for patients with this condition.
AB - OBJECTIVE: To define the clinical patterns of peripheral neuropathy and autonomic testing abnormalities in patients with amyloidosis. PATIENTS AND METHODS: A retrospective chart review was conducted of 65 patients who had biopsy-proven amyloidosis and autonomic function testing between January 1, 1985, and December 31, 1997, at Mayo Clinic's site in Rochester, MN. Patients were required to have neurologic evaluation, autonomic reflex screening, and tissue confirmation of amyloidosis. RESULTS: We identified 5 clinical patterns of peripheral neuropathy: (1) generalized autonomic failure and polyneuropathy with pain (40 patients [62%]), (2) generalized autonomic failure and polyneuropathy without pain (11 [17%]), (3) isolated generalized autonomic failure (7 [11%]), (4) polyneuropathy without generalized autonomic failure (4 [6%]), and (5) generalized autonomic failure and small-fiber (ie, autonomic and somatic C-fiber) neuropathy (3 [5%]). Moderately severe generalized autonomic failure, involving adrenergic, cardiovagal, or sudomotor domains, was found in all patients, including those without clinically manifested autonomic failure. The diagnosis of amyloidosis was delayed in patients who did not have initial symptoms of pain or generalized autonomic failure (48 months to diagnosis in patients with polyneuropathy without autonomic failure vs 12 months to diagnosis in patients with autonomic failure and small-fiber neuropathy; P=.57). CONCLUSION: Physicians should test for symptoms of generalized autonomic failure in patients who have peripheral neuropathy of unknown origin. Autonomic testing may give abnormal results in patients without overt symptoms of autonomic failure. Early recognition of autonomic failure may lead to earlier diagnosis of the underlying pathogenesis of amyloidosis, as well as earlier treatment for patients with this condition.
UR - http://www.scopus.com/inward/record.url?scp=55549111907&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=55549111907&partnerID=8YFLogxK
U2 - 10.4065/83.11.1226
DO - 10.4065/83.11.1226
M3 - Article
C2 - 18990321
AN - SCOPUS:55549111907
SN - 0025-6196
VL - 83
SP - 1226
EP - 1230
JO - Mayo Clinic proceedings
JF - Mayo Clinic proceedings
IS - 11
ER -