TY - JOUR
T1 - Autonomic dysfunction in Wilson's disease - A clinical and electrophysiological study
AU - Meenakshi-Sundaram, Salvadeeswaran
AU - Taly, Arun B.
AU - Kamath, Vikram
AU - Arunodaya, G. R.
AU - Rao, Shivaji
AU - Swamy, H. S.
PY - 2002
Y1 - 2002
N2 - Wilson's disease is known for its protean manifestations; however involvement of the autonomic nervous system has not received much attention. Fifty patients with varying duration and severity of illness were evaluated for autonomic dysfunction clinically and electrophysiologically, using sympathetic skin response (SSR) and RR interval variability (RRIV) on deep breathing. The change in heart rate (ΔHR) was calculated from RRIV. Five patients had at least one autonomic symptom and one asymptomatic patient had significant postural hypotension. Absent SSR and abnormal ΔHR were noted in seven patients each. Overall, 13 patients had electrophysiological dysautonomia and an additional six had clinical dysautonomia. All had normal peripheral conductions and all but one had normal hepatic functions. Dysautonomia was more common among patients with neurological presentation (12/32) than non-neurological (1/18) (p = .012). Dysautonomia, often subclinical is common in Wilson's disease and is probably of central origin. It is more frequent among those with neurological presentation. Sympathetic and parasympathetic functions are equally affected.
AB - Wilson's disease is known for its protean manifestations; however involvement of the autonomic nervous system has not received much attention. Fifty patients with varying duration and severity of illness were evaluated for autonomic dysfunction clinically and electrophysiologically, using sympathetic skin response (SSR) and RR interval variability (RRIV) on deep breathing. The change in heart rate (ΔHR) was calculated from RRIV. Five patients had at least one autonomic symptom and one asymptomatic patient had significant postural hypotension. Absent SSR and abnormal ΔHR were noted in seven patients each. Overall, 13 patients had electrophysiological dysautonomia and an additional six had clinical dysautonomia. All had normal peripheral conductions and all but one had normal hepatic functions. Dysautonomia was more common among patients with neurological presentation (12/32) than non-neurological (1/18) (p = .012). Dysautonomia, often subclinical is common in Wilson's disease and is probably of central origin. It is more frequent among those with neurological presentation. Sympathetic and parasympathetic functions are equally affected.
KW - Dysautonomia
KW - R-R interval variability
KW - Sympathetic skin response
KW - Wilson's disease
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U2 - 10.1007/s10286-002-0038-6
DO - 10.1007/s10286-002-0038-6
M3 - Article
C2 - 12269551
AN - SCOPUS:0036036944
SN - 0959-9851
VL - 12
SP - 185
EP - 189
JO - Clinical Autonomic Research
JF - Clinical Autonomic Research
IS - 3
ER -