Chuan Liu, Roumin Wang, Xiaoyan Li, Wang Ni, GongLu Liu, Yi Dong
RATIONALE: Acquired hepatocerebral degeneration (AHD) is a rare neurological complication of chronic liver disease or portosystemic shunting, typically characterized by extrapyramidal symptoms and manganese accumulation in the basal ganglia. Its diagnosis is particularly challenging in patients with occult cirrhosis and complex exposure histories, especially when traditional Chinese medicines (TCM) and natural health products (NHPs) contribute to hepatotoxicity. PATIENT CONCERNS: A 66-year-old woman presented with a 2-year history of progressive postural and intention tremor, with marked worsening over the preceding 2 months, interfering with daily activities. DIAGNOSES: Laboratory tests indicated hepatic dysfunction and cirrhosis, while neuroimaging revealed bilateral T1 hyperintensity in the basal ganglia. Extensive evaluation excluded viral hepatitis, alcohol-related liver disease, autoimmune liver disease, Wilson disease, and occupational toxic exposure. Whole-blood manganese was markedly elevated. Detailed history-taking revealed long-term use of hepatotoxic TCM (notably Psoralea corylifolia) and manganese-containing supplements. A diagnosis of manganese-related AHD secondary to cirrhosis, likely induced by chronic TCM/NHPs exposure, was established. INTERVENTIONS: Management included discontinuation of hepatotoxic agents and manganese-rich supplements, implementation of a low-manganese diet, hepatoprotective therapy, ammonia-lowering treatment, symptomatic control of tremor (clonazepam and trihexyphenidyl), and chelation therapy with dimercaptosuccinic acid. OUTCOMES: At follow-up, liver function and ammonia levels improved, whole-blood manganese levels decreased but remained elevated, and tremor severity was significantly reduced with corresponding functional improvement. LESSONS: This case highlights that AHD may arise in the setting of occult cirrhosis related to long-term TCM/NHPs use, with additional manganese exposure acting as a precipitating factor. Clinicians should routinely screen for liver dysfunction in patients with unexplained extrapyramidal symptoms, incorporate manganese testing and basal ganglia magnetic resonance imaging into diagnostic evaluation, and systematically inquire about TCM/NHPs use. Multidisciplinary management is crucial, particularly in regions with widespread use of TCM and NHPs.