Cardiovascular and sudomotor autonomic dysfunction in Wilson's disease--limited correlation with clinical severity.
BACKGROUND
Wilson's disease, a disorder of copper metabolism, results in abnormal accumulation of copper in liver, brain, kidney and cornea giving rise to protean manifestations. Wilson's disease predominantly affects the basal ganglia and brain stem nuclei which may cause autonomic dysfunction. Disturbances of autonomic nervous system have not received attention in Wilson's disease due to its rarity. The aim of this study was to evaluate autonomic cardiovascular reflexes in patients of Wilson's disease and to look for any relationship between autonomic nervous system disturbances and clinical severity of Wilson's disease.


METHODS
Cardiovascular autonomic reflexes were evaluated clinically and electrophysiologically in 30 patients of Wilson's disease with neurological onset and compared with equal number of age and gender matched healthy controls.


RESULTS
Significantly abnormal response to the Valsalva maneuver and RR interval variation was seen in patients compared to controls (p<0.05). Latency for sympathetic skin response was also significantly (p<0.02) prolonged in patient group. No specific correlation with clinical severity of Wilson's disease and autonomic dysfunction could be established.


CONCLUSIONS
Autonomic dysfunction occurs in Wilson's disease and affects parasympathetic more than the sympathetic functions.
Soni
Dilip
D
Department of Neurology, All India Institute of Medical Sciences, Ansari Nagar, New Delhi 110029, India.
Shukla
Garima
G
Singh
Sumit
S
Goyal
Vinay
V
Behari
Madhuri
M
D000293:Adolescent; D000328:Adult; D001341:Autonomic Nervous System / Q000503:physiopathology*; D001342:Autonomic Nervous System Diseases / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology*; D001933:Brain Stem / Q000378:metabolism / Q000503:physiopathology; D002320:Cardiovascular Physiological Phenomena; D002648:Child; D003300:Copper / Q000378:metabolism; D018450:Disease Progression; D005260:Female; D005712:Galvanic Skin Response / Q000502:physiology; D006321:Heart / Q000294:innervation / Q000503:physiopathology; D006527:Hepatolenticular Degeneration / Q000150:complications* / Q000503:physiopathology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D011930:Reaction Time / Q000502:physiology; D012018:Reflex / Q000502:physiology; D012720:Severity of Illness Index; D013545:Sweat Glands / Q000503:physiopathology; D014636:Valsalva Maneuver / Q000502:physiology; D055815:Young Adult
D003300:Copper