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

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.