Acquired hepatocerebral degeneration and hepatic encephalopathy: One or two entities?

Background: Acquired hepatocerebral degeneration (AHD) and hepatic encephalopathy (HE) are neurological complications of chronic liver disease (CLD) with portosystemic shunt. While HE is common, AHD is a rare entity and the clinical-imaging relationships observed in small series, lack validation in large patient cohorts.

Objectives: To characterize a cohort of AHD patients and to explore possible associations with HE coexistence.

Methods: Retrospective analysis of patients with a working AHD diagnosis, between 2008 and 2019. Clinical, laboratorial, imagiological, and neuropsychological results at first neurological observation were reviewed and compared between "AHD" and "AHD with HE" groups.

Results: A total of 76 patients were recruited. The most frequent neurological manifestations were neuropsychiatric (93.4%) and extrapyramidal (84.2%). Only 38% of patients had hypermanganesemia. Compared with "AHD," the "AHD with HE" group had more hyperkinetic movement disorders (71.4% vs 38.5%; p=0.05), higher number of patients in the dementia spectrum (57.7% vs 20%; p=0.04), higher median ammonia levels (p=0.014) and more widespread cortico-subcortical and pyramidal involvement on brain MRI. Nineteen patients underwent liver transplantation (LT), with a significantly improved survival (p=0.006).

Discussion: HE and AHD often coexist in the same patient. Seventy-six patients with CLD and AHD were evaluated, making this one of the largest reported AHD cohorts. Blood manganese level was a weak diagnostic marker in AHD. Early liver function restoration through LT improved survival. Our report provides a detailed description of the phenotype and long-term outcome of AHD, with relevance for diagnosis and treatment.

Keywords: Acquired hepatocerebral degeneration; chronic liver disease; hepatic encephalopathy; liver transplantation; manganese.