title: Autonomic dysfunction in Wilson's disease --a clinical and electrophysiological study.
abstract: 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 (AHR) was calculated from RRIV. Five patients had at least one autonomic symptom and one asymptomatic patient had significant postural hypotension. Absent SSR and abnormal AHR 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.
authors.affiliation: (NIMHANS), National Institute of Mental Health & Neurosciences, Bangalore, India.
authors.forename: Salvadeeswaran
authors.initials: S
authors.lastname: Meenakshi-Sundaram
authors.forename: Arun B
authors.initials: AB
authors.lastname: Taly
authors.forename: Vikram
authors.initials: V
authors.lastname: Kamath
authors.forename: G R
authors.initials: GR
authors.lastname: Arunodaya
authors.forename: Shivaji
authors.initials: S
authors.lastname: Rao
authors.forename: H S
authors.initials: HS
authors.lastname: Swamy
mesh_terms: D000293:Adolescent; D000328:Adult; D001341:Autonomic Nervous System / Q000503:physiopathology*; D001342:Autonomic Nervous System Diseases / Q000209:etiology; D002648:Child; D004594:Electrophysiology; D005260:Female; D006339:Heart Rate; D006527:Hepatolenticular Degeneration / Q000503:physiopathology*; D006801:Humans; D007024:Hypotension, Orthostatic / Q000209:etiology; D008297:Male; D008875:Middle Aged; D015656:Respiratory Mechanics; D012867:Skin / Q000294:innervation; D013564:Sympathetic Nervous System / Q000503:physiopathology