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Acquired Hepatocerebral Degeneration

Summary

  • Neurological syndrome of extrapyramidal and neuropsychiatric symptoms in chronic liver disease
  • Occurs with portosystemic shunting and manganese accumulation
  • MRI shows symmetric T1 hyperintensity in the globus pallidus without corresponding T2 signal1

Pathophysiology

  • Manganese deposition in the basal ganglia due to impaired hepatobiliary excretion and portosystemic shunting
  • Alzheimer type II astrocytes in the basal ganglia and cortex
  • Oxidative stress and mitochondrial dysfunction contribute to neuronal injury

Demographics

  • Occurs in a minority of patients with cirrhosis, typically middle-aged to older adults with long-standing disease
  • Male predominance

Diagnosis

  • Extrapyramidal features (tremor, bradykinesia, rigidity), cognitive/personality change, ataxia and dysarthria
  • Deranged liver function; elevated serum ammonia
  • Exclude other causes (e.g. Wernicke encephalopathy, Wilson disease)

Imaging

  • MRI:
    • Symmetric T1 hyperintensity of the globus pallidus, extending to the subthalamic region and substantia nigra
    • No corresponding T2/FLAIR signal abnormality
    • Signal reflects paramagnetic manganese and usually regresses after transplantation
  • CT: typically normal; the T1 change is not attributable to calcification

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  • In a patient with alcoholic liver disease, there is symmetrical T1-hyperintensity in the globi pallidi and subganglionic region.

Treatment

  • Treat the underlying liver disease; symptoms and MRI changes may reverse after liver transplantation
  • Parkinsonian features respond poorly to levodopa

Differential diagnosis (symmetric T1 hyperintense globus pallidus)

Imaging differential Differentiating feature
Manganese toxicity (prolonged parenteral nutrition) Identical symmetric T1 GP hyperintensity; distinguished only by history
Wilson's Disease May show T1 GP signal but also T2 change in putamen/midbrain ("face of the giant panda")
Neurofibromatosis type 1 T1/T2 hyperintense foci (FASI) in the globus pallidus, with other NF1 features
Basal ganglia calcification (Fahr) Dense on CT and blooms on SWI, unlike paramagnetic manganese
Kernicterus Symmetric GP T1 hyperintensity in a neonate

  1. Meissner et al. Acquired hepatocerebral degeneration. 2011. Handbook of clinical neurology - Open in new tab. ↩