TY - JOUR
T1 - Autonomic neuropathy associated with sicca complex
AU - Wright, R. Alan
AU - Grant, Ian A.
AU - Low, Phillip A.
N1 - Funding Information:
Supported in part by grants from NINDS (PO1 NS3 2352) and Mayo Foundation funds.
PY - 1999/1/15
Y1 - 1999/1/15
N2 - We retrospectively studied 52 patients with sicca complex who presented primarily with peripheral neuropathic symptoms to assess the degree of autonomic involvement and its relationship to somatic neuropathy, serological tests and extraglandular disease. One patient presented with severe panautonomic dysfunction, with another six patients reporting prominent autonomic symptoms, and a further 40 patients having mild or asymptomatic disease. Eighty-seven percent of patients showed abnormalities on an autonomic reflex screen (ARS). Fifty-eight percent of patients showed a mixed pattern of autonomic dysfunction with abnormalities in two or more ARS subgroups of sudomotor, cardiovagal and adrenergic function. Three patients had a cholinergic autonomic neuropathy with sparing of adrenergic function. Tonic pupils were present in 13 patients. Autonomic neuropathy occurred in patients with a variety of somatic neuropathy subtypes, without a clear relationship between the type of autonomic neuropathy and the type of somatic peripheral neuropathy. Extraglandular disease was uncommon, occurring in only four patients. Serological abnormalities (extractable nuclear antigen [ENA], SS-A, SS-B) occurred in 20% of patients. We conclude that: (1) A wide spectrum of autonomic dysfunction is seen in sicca complex. (2) Severe autonomic involvement is uncommon, with most patients showing mildly symptomatic or asymptomatic disease. (3) Overall, characteristic patterns of autonomic dysfunction did not predominate, and there were no clear relationships with the different types of somatic peripheral neuropathy. (4) Clinical and serological evidence of non-neurological extraglandular disease is uncommon in this group of patients. Copyright (C) 1999 Elsevier Science B.V.
AB - We retrospectively studied 52 patients with sicca complex who presented primarily with peripheral neuropathic symptoms to assess the degree of autonomic involvement and its relationship to somatic neuropathy, serological tests and extraglandular disease. One patient presented with severe panautonomic dysfunction, with another six patients reporting prominent autonomic symptoms, and a further 40 patients having mild or asymptomatic disease. Eighty-seven percent of patients showed abnormalities on an autonomic reflex screen (ARS). Fifty-eight percent of patients showed a mixed pattern of autonomic dysfunction with abnormalities in two or more ARS subgroups of sudomotor, cardiovagal and adrenergic function. Three patients had a cholinergic autonomic neuropathy with sparing of adrenergic function. Tonic pupils were present in 13 patients. Autonomic neuropathy occurred in patients with a variety of somatic neuropathy subtypes, without a clear relationship between the type of autonomic neuropathy and the type of somatic peripheral neuropathy. Extraglandular disease was uncommon, occurring in only four patients. Serological abnormalities (extractable nuclear antigen [ENA], SS-A, SS-B) occurred in 20% of patients. We conclude that: (1) A wide spectrum of autonomic dysfunction is seen in sicca complex. (2) Severe autonomic involvement is uncommon, with most patients showing mildly symptomatic or asymptomatic disease. (3) Overall, characteristic patterns of autonomic dysfunction did not predominate, and there were no clear relationships with the different types of somatic peripheral neuropathy. (4) Clinical and serological evidence of non-neurological extraglandular disease is uncommon in this group of patients. Copyright (C) 1999 Elsevier Science B.V.
KW - Autonomic neuropathy
KW - Sicca complex
KW - Sjogren's syndrome
UR - http://www.scopus.com/inward/record.url?scp=0033555407&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=0033555407&partnerID=8YFLogxK
U2 - 10.1016/S0165-1838(98)00164-7
DO - 10.1016/S0165-1838(98)00164-7
M3 - Article
C2 - 9935271
AN - SCOPUS:0033555407
SN - 0165-1838
VL - 75
SP - 70
EP - 76
JO - Journal of the Autonomic Nervous System
JF - Journal of the Autonomic Nervous System
IS - 1
ER -