Two Cases of Chronic Acquired Hepatocerebral Degeneration with Parkinsonian Symptoms

J Korean Neurol Assoc > Volume 17(4); 1999 > Article

Journal of the Korean Neurological Association 1999;17(4): 579-584.

파킨슨양 증세를 보인 만성 간뇌 퇴행증 환자 2예

신원철, 성강주,노용우,구본대*,장대일,정경천

경희대학교 의과대학 신경과학교실,분당차병원 신경과*

Two Cases of Chronic Acquired Hepatocerebral Degeneration with Parkinsonian Symptoms

Won Chul Shin, Kang Ju Sung, Yong Woo Noh, Bon Dae Ku, M.D*., Dae-Il Chang, Kyung-Cheon Chung

Department of Neurology, College of Medicine, Kyung Hee University Department of Neurology, Pundang CHA General Hospital*

Abstract

Chronic acquired hepatocerebral degeneration (CAHD) is a progressive or episodic neurologic syndrome that occurs occasionally in patients who have chronic liver disease or portocaval shunt. The clinical features of CAHD include action and postural tremors, generalized chorea, asterixis, myoclonus, dystonia, resting tremor, gait ataxia, and variable impairments of intellectual function. We experienced 2 cases of CAHD with unusual neurologic manifestations, which were hypokinetic parkinsonian symptoms. Both cases had a history of liver cirrhosis and the same symptoms. They had masked faces, cogwheel rigidiy in both wrists, slurred speech, tongue tremor, bradykinesia, and gait ataxia. They had normal mental status and no pathologic reflex. Brain MRI showed abnormal, increased signal on T1-weighted images in the globus pallidus and mesencephalon bilaterally. The hypokinetic parkinsonian symptoms disappeared when given doses levodopa. J Kor Neurol Ass 17(4):579~584, 1999 Key Words : Chronic Acquired Hepatocerebral Degeneration, Hypokinetic Parkinsonian Symptoms