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. 2018 Sep 3;9(1):74–98. doi: 10.1016/j.jceh.2018.08.009

Table 7.

Global Assessment Scale for Wilson's Disease.

Tier Areas of assessment
Tier 1, domains scored from 0 to 5 in each domain based on impact on activities of daily living Domain
Liver* Clinical, biochemical, and abdominal ultrasound evidence for liver disease
Cognition and Behavior Intellectual decline, depression, and psychosis
Motor Motor impairment on examination
Osseomuscular Joint or muscle involvement by clinical examination and radiographs
Tier 2, items 1–13 scored from 0 to 4 based on clinical severity
  • 1.

    Wilson's facies

  • 2.

    Scholastic performance

  • 3.

    Depression

  • 4.

    Psychosis

  • 5.

    Dystonia

  • 6.

    Tremor

  • 7.

    Chorea
  • 8.

    Parkinsonism

  • 9.

    Speech

  • 10.

    Swallowing

  • 11.

    Salivation

  • 12.

    Posture and gait

  • 13.

    Kayser-Fleischer ring
Tier 2, item 14 is scored based on the presence or absence of uncommon manifestations (each scored as 1 point with a maximum of 4 points.) Emotional lability
Seizures over preceding 1 month
Myoclonus
Stereotypy
Tics
Pyramidal signs
Eye movement abnormalities

*In the liver domain, the scores range from no liver disease ever (L0) through active liver disease (L2), compensated (L3), decompensated liver disease (L4) to potentially life-threatening disease (L5).

Greater weightage is given to Wilson's facies and KF rings that are characteristic features of Wilson's disease.