Wilson's disease
ICD-10 E83.0 · ICD-11 5C64.00
Managing Dystonia in Wilson's Disease
Clinical Scenario
Wilson's disease can present with dystonia — sustained or repetitive muscle contractions that cause abnormal postures and movements. Dystonia may be focal, segmental, or generalised, and its management is a distinct treatment priority in affected patients.
Treatment Approach
First-line treatment targets the distribution of dystonia: focal forms are typically addressed with localised injections into the most affected muscles, while broader presentations may require oral pharmacotherapy. The full selection criteria, agents, and escalation pathway are in the protocol.
Clinical goal: reduction and control of dystonic symptoms.
References
- Focal dystonias are usually effectively treated with BTX injections and segmental or generalized dystonias are treated with oral drugs alone or in combination with BTX injected into the most affected muscles.
- Anticholinergic drugs show moderate efficacy with well-known side effects to watch for (confusion, urinary retention, blurred vision, etc.) (trihexyphenidyl with a starting dose of 2 mg, slowly increased up to a maximum of 30 mg/day, or biperiden with a starting dose of 6 mg/day in three doses, slowly increased up to 16 mg/day).