Patterns of neuropathy and autonomic failure in patients with amyloidosis

Annabel K. Wang, Robert D. Fealey, Tonette L. Gehrking, Phillip Anson Low

Research output: Contribution to journalArticle

58 Citations (Scopus)

Abstract

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.

Original languageEnglish (US)
Pages (from-to)1226-1230
Number of pages5
JournalMayo Clinic Proceedings
Volume83
Issue number11
DOIs
StatePublished - 2008

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Amyloidosis
Polyneuropathies
Peripheral Nervous System Diseases
Pain
Unmyelinated Nerve Fibers
Adrenergic Agents
Nervous System
Reflex
Early Diagnosis
Physicians
Biopsy

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Patterns of neuropathy and autonomic failure in patients with amyloidosis. / Wang, Annabel K.; Fealey, Robert D.; Gehrking, Tonette L.; Low, Phillip Anson.

In: Mayo Clinic Proceedings, Vol. 83, No. 11, 2008, p. 1226-1230.

Research output: Contribution to journalArticle

Wang, Annabel K. ; Fealey, Robert D. ; Gehrking, Tonette L. ; Low, Phillip Anson. / Patterns of neuropathy and autonomic failure in patients with amyloidosis. In: Mayo Clinic Proceedings. 2008 ; Vol. 83, No. 11. pp. 1226-1230.
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