(Kiosk 6) The Efficacy of Multidisciplinary Treatment for Pediatric Dystonia: A Case Report

Case Diagnosis: A 16 year old male patient with dystonic quadriparetic cerebral palsy has significant dystonia relief from a multidisciplinary treatment regimen.

Case Description or Program Description: Patient is a 16-year-old male with a past medical history of dystonic quadriparetic cerebral palsy with Gross Motor Function Classification System V related to kernicterus as an infant. Patient has experienced episodes of status dystonicus with subsequent creatine kinase elevations and kidney injury. Patient has undergone intrathecal Baclofen (ITB) pump placement in the right subcostal region with catheter revision from spinal subarachnoid space to right frontal intraventricular, Botox injections every 12 weeks, and enteral medications including baclofen, atrane, valium, clonidine, and gabapentin. Despite these aforementioned therapies, patient’s dystonia remained severe, Barry Albright Dystonia Score 32/36. Neurosurgery completed stage 1 and 2 of Deep Brain Stimulation (DBS) with bilateral Globus Pallidus Internus electrode placement. Patient displayed no significant changes after multiple DBS programmings. On one occasion there was worsening dystonia, likely due to anxiety.

Setting: Riley Hospital for Children

Assessment/Results: Patient had decreased admissions for dystonic crises. Preceding stage 1 placement of the DBS in December 2021, the patient had 6 inpatient admissions. Following state 2 placement in June 2022, there were no admissions. Patient has sub-optimally controlled symptoms despite his multidisciplinary treatment regimen, as evidenced by patient's persistent dystonia.

Discussion (relevance): Typical dystonia treatments include ITB pumps, medications, and focal Botox injections. DBS is a treatment option for medically refractory dystonia in pediatric patients. Clinical improvement status-post DBS may take months to years. Some evidence suggests that DBS improves dystonia, pain/comfort, and quality of life; however, the Grading of Recommendations, Assessment, Development, and Evaluations is low. There is limited evidence to show that DBS results in full resolution of symptoms. Optimal results are obtained from frequent reprogramming sessions with incremental changes to avoid overstimulation. Psychosocial factors should be considered when treating dystonia, as anxiety can exacerbate symptoms.

Conclusions: Multidisciplinary dystonia treatment, consisting of pharmacological and neurosurgical interventions, is effective in reducing symptom severity and dystonic crises admissions. Placement of DBS did not cause complete symptom resolution.