Dystonia due to cerebral palsy responds to deep brain stimulation of the globus pallidus internus.

BACKGROUND: Cerebral palsy is the most common cause of pediatric-onset dystonia. Deep brain stimulation is gaining acceptance for treating dystonias in children. There is minimal reported experience regarding the efficacy of deep brain stimulation in cerebral palsy. METHODS: Fourteen patients, including 8 younger than 16 years, received bilateral implants (13 patients) or a unilateral implant (1 patient) of the internal globus pallidus and were observed in a noncontrolled, nonblinded study for at least 6 months. Motor function was assessed using the Burke-Fahn-Marsden Dystonia Movement and Disability scales and the Barry Albright Dystonia Scale. RESULTS: By 6 months, significant improvement was observed in the Burke-Fahn-Marsden Dystonia Movement scale (P=.004), the Burke-Fahn-Marsden Dystonia Disability scale (P=.027), and the Barry Albright Dystonia Scale (P=.029) for the whole cohort (n=14) and in the patients treated before skeletal maturity (group 1; n=8): Burke-Fahn-Marsden Dystonia Movement scale, P=.012; Burke-Fahn-Marsden Dystonia Disability scale, P=.020; and Barry Albright Dystonia Scale, P=.027. CONCLUSIONS: Deep brain stimulation may offer an effective treatment option for cerebral palsy-related dystonia, especially in those treated before skeletal maturity.

Pallidal stimulation in children: comparison between cerebral palsy and DYT1 dystonia.

The authors compared the outcomes of 17 children aged 7 to 15 years with DYT1 dystonia or cerebral palsy following deep brain stimulation. While patients with cerebral palsy presented with significantly greater motor disability than the DYT1 cohort at baseline, both groups demonstrated improvement at 1 year (cerebral palsy = 24%; DYT1 = 6%). The group as a whole demonstrated significant improvement on the Barry-Albright Dystonia Scale across time. Gains in motor function were apparent in both axial and appendicular distributions involving both upper and lower extremities. Gains achieved by 6 months were sustained in the cerebral palsy group, whereas the DYT1 group demonstrated continued improvement with ongoing pallidal stimulation beyond 18 months. Young patients with dystonia due to cerebral palsy responded comparably to patients with DYT1 dystonia. The severity of motor impairment in patients with cerebral palsy at baseline and follow-up raises the issue of even earlier intervention with neuromodulation in this population to limit long-term motor impairments due to dystonia.

Deep brain stimulation for pediatric movement disorders.

Deep brain stimulation (DBS) has been used for the treatment of tremor and dystonia in adults since 1997. With more than 50,000 treated adults, it has become part of the standard care for pharmacoresistant tremor, Parkinson disease, and dystonias. Dystonias are a heterogeneous group of disorders with intrinsic (genetic) and extrinsic etiologic factors. In children and adults, DBS has been used for the treatment of both primary and secondary dystonias. Pediatric use has been more limited, with only a few experienced centers worldwide. Awake surgery can be safely performed with a dedicated multidisciplinary team approach to help ensure appropriate lead placement. It is incumbent upon us, as physicians, to advise patients and payers on the appropriate use of this technology. Neuromodulation of other disorders, including epilepsy, Tourette syndrome, obsessive-compulsive disorder, and depression, by DBS is under active investigation. Pediatric DBS is still in its early stages; experience will help us refine the indications and techniques for applying this complex technology to our most vulnerable patients, which should lead to our common goal of improving quality of life for our patients and their families. We review the role of DBS and our experience with establishing a dedicated pediatric DBS program.