Objective: To highlight acquired hepatocerebral degeneration as an underrecognized cause of secondary parkinsonism and to emphasize the diagnostic value of serial MRI in movement disorder practice.

Background: Acquired hepatocerebral degeneration related to portosystemic shunting may mimic degenerative parkinsonism, particularly when overt hepatic encephalopathy is absent. Delayed recognition may lead to misdiagnosis and inappropriate treatment.

Method: Single-patient case report with longitudinal follow-up. We performed a focused movement-disorder examination, cognitive screening, targeted laboratory evaluation including exclusion of Wilson’s disease, and longitudinal review of serial brain MRI.

Results: A 59-year-old woman with long-standing portal hypertension after portal vein thrombosis due to hereditary thrombophilia and subsequent portosystemic shunting developed progressive bilateral upper-limb postural kinetic tremor with a dystonic component, more pronounced on the left, mild symmetric parkinsonism, and intermittent head tremor. Cognition was preserved (MoCA 29/30). Plasma ammonia was elevated, whereas blood manganese was within the reference range. Serial brain MRI over five years demonstrated stable bilateral T1 hyperintensity of the globus pallidus without progression. Under ammonia-lowering therapy, parkinsonian rigidity and bradykinesia partially improved, while MRI abnormalities persisted.

Conclusion: Acquired hepatocerebral degeneration should be considered in patients with symmetric or atypical parkinsonism accompanied by dystonic tremor, especially in the setting of portal hypertension or portosystemic shunting. Persistent pallidal T1 hyperintensity is a practical imaging clue, and the combination of stable MRI abnormalities with partial clinical reversibility illustrates clinicoradiological dissociation that may help prevent misdiagnosis as degenerative parkinsonism.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/portosystemic-shunt-related-parkinsonism-in-acquired-hepatocerebral-degeneration-with-persistent-pallidal-t1-hyperintensity/