A case of wilson's disease showing palialia as an initial symptom

J Korean Neurol Assoc > Volume 15(2); 1997 > Article

Journal of the Korean Neurological Association 1997;15(2): 413-416.

동어반복증을 첫 증상으로 보인 윌슨씨병 1례

윤성민, 이상원, 김광수, 유경무

고신대학교 의학부 신경과

A case of wilson's disease showing palialia as an initial symptom

Sung Min Yoon, M.D., Sang Won Yi, M.D., Kwang Soo Kim, M.D., Kyung Mu Yoo, M.D.

Department of Neurology, Kosin Medical College

Abstract

Wilson's disease is an autosomal recessive disorder resulting from an excessive accumulation of copper in the liver, cornea, kidneys, and in the basal ganglia of the brain. The prominent speech disturbances of Wilson's disease include monopitch, monoloudness, slow rate, low pitch, delayed in initiating speech and rarely palilalia. A19-year-old woman developed palilalia which was characterized by compulsive repetition of a phrase with increasing rapidity and with a decrescendo of voice volume. Although she had been suffering from liver cirrhosis for the past 8 months, the palilalia was the only neurological sign at initial examination. She showed a low serum ceruloplasmin, low serum copper, increased urinary copper excretion, and Kayser-Fleischer rings. Brain magnetic resonance images showed high signals in the bilateral basal ganglia in T2-weighted images, and slight cortical atrophy.