Wilson disease (CNS manifestations)

Dr Matthew Jarvis and Dr Henry Knipe et al.

Wilson disease, also known as hepatolenticular degeneration, is a multisystem disease due to abnormal accumulation of copper. It is characterized by early onset liver cirrhosis with CNS findings most frequently affecting the basal ganglia and midbrain.

This article aims to discuss the central nervous system manifestations of this condition. For a broad view on the condition, please refer to the main article on Wilson disease.

Clinical presentation

Common neurological clinical features include dysarthria, dystonia, tremor, parkinsonism, choreoathetosis, and ataxia and gait anomalies. Kayser-Fleischer rings are also seen in nearly all patients with neurological manifestations of Wilson disease 1.

Radiographic features

Neuroimaging features of Wilson disease may vary depending on whether the disease is treated or untreated. The basal ganglia and midbrain are the most frequently affected sites 2.


May demonstrate atrophic changes in the basal ganglia, cortical, and cerebellar regions 3:

  • non-enhanced: copper deposition does not increase density on CT
  • post-contrast: lesions do not enhance

Hyperintensity in lentiform nuclei and mesencephalic regions on T1 have been described as most common initial MRI abnormality 4.  

T2 hyperintensity typically involves the 5

There may be T2 hyperintensity in the outer rim of the deep gray matter and/or T1 hyperintensity in cases of copper toxicosis. Axial T2 MR at midbrain level can show a face of the giant panda sign, a characteristic finding of Wilson disease. Axial T2 MR at pons may also show the face of a miniature panda sign (cub of the giant panda). This combination is referred to as the double panda sign 6.

Diffusion restriction may be seen early in the course of the disease 7.

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Article information

rID: 39667
Synonyms or Alternate Spellings:

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Cases and figures

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