Sustained relief of generalized dystonia despite prolonged interruption of deep brain stimulation.

BACKGROUND: Pallidal deep brain stimulation (DBS) is an established treatment for disabling, medication-refractory generalized dystonia. Patients typically regress to their preoperative baseline when stimulation is discontinued.

METHODS: Presented are case reports of 2 dystonia patients.

RESULTS: Two patients with primary generalized dystonia (1 with the DYT1 mutation) who were treated successfully with bilateral pallidal DBS for periods of 18 months and 5 years retained motor benefit for several months after inadvertent interruption of stimulation. Stimulation was interrupted unilaterally for 3 and 7 months and bilaterally for 2 days and 2 months, respectively. Symptoms of dystonia returned only partially during the period of therapy interruption and rapidly and completely resolved after resuming stimulation.

CONCLUSIONS: We report unexpected and prolonged retention of motor benefits despite transient cessation of pallidal DBS in 2 dystonia patients. Factors that appear to differentiate these individuals are young age, short duration of disease, and chronic DBS therapy with relatively low energy of stimulation.