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.

Advances in management of movement disorders in children.

Movement disorders in children are causally and clinically heterogeneous and present in a challenging developmental context. Treatment options are broad ranging, from pharmacotherapy to invasive neuromodulation and experimental gene and stem cell therapies. The clinical effects of these therapies are variable and often poorly sustained, and only a few of the management strategies used in paediatric populations have been tested in randomised controlled studies with age-appropriate cohorts. Identification of the most appropriate treatment is uniquely challenging in children because of the incomplete knowledge about the pathophysiology of movement disorders and their influence on normal motor development; thus, effective therapeutic options for these children remain an unmet need. It is vital to transfer the expanding knowledge of the movement disorders into the development of novel symptomatic or, ideally, disease-modifying treatments, and to assess these therapeutic strategies in appropriately designed and well done trials.

Epilepsy: habilitation and rehabilitation.

Rehabilitation represents not only a distinct field of medicine, but also a philosophical and practical treatment approach that can be applied to a variety of chronic disorders. Neurology encompasses many chronic disorders, making it ideal for the application of rehabilitation principles in daily practice. Epilepsy offers a unique opportunity to incorporate rehabilitation principles into the management of a complex medical disorder. Epilepsy is an evolving disease process that changes with the maturation of the central nervous system. The rehabilitative model provides the framework for a dynamic treatment plan to meet the changing needs of the child with epilepsy through the social and developmental changes of childhood, adolescence, and adulthood. The development of epilepsy may complicate the recovery from many acute and chronic conditions that affect the central nervous system. The rehabilitation process must address these many aspects of the disease process and its sequelae. This makes neurologists uniquely qualified to manage the rehabilitation team. The impact of the therapeutic milieu on the recovery process may be as important as any specific medical or surgical intervention.

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.