Three Cases of Chronic Acquired Hepatocerebral Degeneration

ABSTRACT

BACKGROUND: Chronic acquired hepatocerebral degeneration (CAHD), a slowly progressive neurologic disease characterized by chronic intermittent hepatic encephalopathy, is seen sporadically in patients with chronic hepatic disease. The spectrum of clinical presentations could include neuropsychiatric (apathy, lethargy, excessive somnolence), a movement disorder (ataxia, tremor, chorea, Parkinsonism, myoclonus, dystonia) or both. Unfortunately only a few reports are available concerning the neurological manifestations as well as the findings of brain Magnetic resonance image (MRI) in patients with CAHD in spite of high prevalence of chronic liver disease in Korea. METHODS: We reviewed clinical or laboratory data of 3 patients with CAHD. All patients had evaluations such as neurological examination, mini-mental status examination, liver function test and brain MRI. From sagittal image of their brain MRI, we calculated pallidal signal intensity. RESULTS: The most common neurological findings were cognitive dysfunction, dysarthria, and parkinsonism including tremor, rigidity and bradykinesia. The brain MRI of all three patients showed hyperintensity signal in globus pallidus on T1 weighted image. Some patients also showed similar signal intensity on their brainstem. CONCLUSIONS: We describe three cases of CAHD with clinical, radiological feature.