Session Information
Date: Monday, June 20, 2016
Session Title: Parkinsonism, MSA, PSP
Session Time: 12:30pm-2:00pm
Objective: To determine the effect of liver transplantation (LT) on neurological symptoms in patients with chronic acquired hepatocerebral degeneration (CAHD).
Background: CAHD is a rare neurological condition characterized by parkinsonism and cognitive impairment. It is a complication of advanced liver disease with portal hypertension and portosystemic shunt. Improvement of neurological symptoms after LT has been reported.
Methods: We included patients with CAHD submitted to LT. We reviewed and compared data obtained from clinical examinations, neuropsychological evaluations, ammonia and manganese determinations and brain MRI, realized previously and 6 months after LT.
Results: Ten patients (7 men) with CAHD with several etiologies were submitted to LT. Mean duration of liver disease was 9,9±4,7 years and of neurological manifestations was 5,2±2,5 years. Previously, all patients had cognitive symptoms. Six patients had extrapyramidal signs (5 tremor, 4 bradykinesia, 1 dystonia, 1 rigidity and 1 orofacial dyskinesia), 3 had impaired ocular motricity, 1 gait disorder and 1 pyramidal signs. Concomitant episodes of hepatic encephalopathy were reported in 5 patients. Clinical improvement was documented in every patient after LT. Extrapyramidal symptoms and hepatic encephalopathy immediately disappeared. Cognitive impairment progressively improved. One patient died because of failure of the transplanted organ. Neuropsychological evaluations demonstrated significant improvement in memory and psychomotor velocity tests in every patient. Ammonia and manganese levels, as well as pallidal T1 MRI hyperintensity reduced significantly.
Conclusions: Our study suggests that LT is an effective treatment for neurological symptoms in CAHD, especially for extrapyramidal and neuropsychological symptoms. Clinical improvement was documented along with normalization of manganese levels and attenuation of pallidal T1 MRI hyperintensity.
To cite this abstract in AMA style:
J. Martins, J. Alves, S. Cavaco, J. Gandara, S. Ferreira, H. Pessegueiro, M. Magalhães. Chronic acquired hepatocerebral degeneration: Efficacy of liver transplatation in 10 patients [abstract]. Mov Disord. 2016; 31 (suppl 2). https://www.mdsabstracts.org/abstract/chronic-acquired-hepatocerebral-degeneration-efficacy-of-liver-transplatation-in-10-patients/. Accessed November 4, 2026.« Back to 2016 International Congress
MDS Abstracts - https://www.mdsabstracts.org/abstract/chronic-acquired-hepatocerebral-degeneration-efficacy-of-liver-transplatation-in-10-patients/