자발성 비신단락으로 유발된 만성후천성간뇌퇴행 1례
One Case of Chronic Acquired Hepatocerebral Degeneration caused by a Spontaneous Spleno-renal Shunt
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Abstract
Chronic acquired hepatocerebral degeneration(CAHD) is a heterogenous that can occur with a primary neurologic, hepatic, or combined presentation. Symptoms and signs of that included progressive dementia, dysarthria, involuntary movements(including tremor, asterixis, and choreoathetosis), ataxia of limb and gait, typically in a patient with chronic liver cirrhosis. Characteristic radiologic findings is high signal on globus pallidus on T1W1 MRI. Recently, we experienced a patients, a 73-year-old female with CAHD presenting mental change, cognitive deficits, and various involuntary movement. In our patient, T1 weighted MRI of the brain showed symmetric high signal intensity in both basal ganglia. Increased ammonia level(226㎍/㎗) in whole blood and a multiple anomalous vessels with spleno-renal shunt on abdominal CT were found. But, liver cirrhosis is absent. In admission care, these mental change and involuntary movements had a good response to herbal medication. We report on patient with CAHD which had a spontaneous spleno-renal shunt without liver disease.
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