Background
Deep brain stimulation (DBS) has been utilized for over two decades to treat medication refractory dystonia in children. Short-term benefit has been demonstrated for inherited, isolated and idiopathic cases, with less efficacy in heredodegenerative and acquired dystonias. The ongoing publication of long-term outcomes warrants a critical assessment of available information as pediatric patients are expected to live most of their lives with these implants.
Summary
We performed a review of the literature for data describing motor and neuropsychiatric outcomes, in addition to complications, five or more years after DBS placement in patients undergoing DBS surgery for dystonia at an age younger than 21. We identified 20 articles including individual data on long-term motor outcomes after DBS for a total of 78 patients. In addition, we found five articles reporting long-term outcomes after DBS in nine patients with status dystonicus. The majority of patients were implanted within the globus pallidus internus, with only a few cases targeting the subthalamic nucleus and ventrolateral posterior nucleus of the thalamus. The average follow-up was 8.5 years, with a range up to 22 years. Long-term outcomes showed a sustained motor benefit, with median Burke-Fahn-Marsden dystonia rating score improvement ranging from 2.5% to 93.2% in different dystonia subtypes. Patients with inherited, isolated and idiopathic dystonias had greater improvement than those with heredodegenerative and acquired dystonias. Sustained improvements in quality of life were also reported, without the development of significant cognitive or psychiatric co-morbidities. Late adverse events tended to be hardware-related, with minimal stimulation-induced effects.
Key Messages
While data regarding long-term outcomes is somewhat limited, particularly with regards to neuropsychiatric outcomes and adverse events, improvement in motor outcomes appear to be preserved more than 5 years after DBS placement.