One Case of Chronic Acquired Hepatocerebral Degeneration caused by a Spontaneous Spleno-renal Shunt

자발성 비신단락으로 유발된 만성후천성간뇌퇴행 1례

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 $level(226{\mu}g/dl)$ 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.

간경변이 없이 자발성 비신단락으로 인해 발생한 만성후천성간뇌퇴행으로 진단된 1례를 경험하였기에 문헌고찰과 함께 보고하였다. 본 증례는 만성후천성간뇌퇴행의 임상양상인 인지기능 저하, 의식변화, 기면, 구음장애, 진전, 보행장애 등의 증상을 가지면서도, 간질환이 없이 자발성 문맥-체순환 단락에 의한 것이란 점과, 피질하 혈관성 치매가 동반되었다는 점에서 그 진단에 주목할 만하다. 대개 만성후천성간뇌퇴행이 일반적인 간성 혼수 치료에 잘 반응하지 않는다고 알려져 있으나, 한약 치료를 통해 의식변화, 진전, 보행장애 등의 증상에 좋은 반응을 관찰할 수 있었다.