Dopamine-Responsive Dystonia Treatment & Management

Updated: Mar 25, 2019
  • Author: Nirjal K Nikhar, MD, FRCP; Chief Editor: Amy Kao, MD  more...
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Treatment

Approach Considerations

All patients with dopamine-responsive dystonia (DRD) should be treated with the levodopa/carbidopa combination. Early treatment can prevent morbidity and contracture formation. In patients with autosomal recessive TH or SR deficiency, early treatment with levodopa may also reduce the motor and intellectual developmental delay.

Surgical care

A fixed equinovarus foot deformity has been corrected surgically after treating the dystonia with levodopa. [46]

Consultations

Physical therapy is particularly important if the patient has a contracture or chronic gait disturbance.

Diet

An in vitro study suggested that oral BH4 supplementation could have a potential benefit in disorders associated with TH misfolding, such as DRD [47] ; however, this awaits further confirmation.

Follow-up

Regular outpatient follow-up is required for patients with DRD to assess the efficacy of treatment and to adjust the dopamine dose accordingly. Although uncommon, dyskinesias and chorea may develop in treated patients. Monitor patients carefully for these conditions.