Disclosures: Rachel H. Anderson: No financial relationships or conflicts of interest

Case Diagnosis: A 7-year-old boy with Pantothenate Kinase-Associated Neurodegeneration (PKAN) in Refractory Status Dystonicus

Case Description: Patient is a 7-year-old male with a history of PKAN managed with multiple enteral medications who presented to an acute care hospital with severe dystonia. He developed status dystonicus with respiratory distress and rhabdomyolysis, ultimately requiring intubation with high-dose sedatives and paralytics to manage his refractory dystonia. He received a 100 mcg baclofen test dose with robust results at peak concentration effect, therefore the decision was made to proceed with implantation. A 40 mL pump was placed with a starting rate of 200 mcg/day. Throughout the next 10 days, the patient underwent sequential increases to control his movement disorder. Concurrently, sedative and paralytic drips were weaned off, and he was extubated. His home medication regimen was consolidated. At discharge, his total pump rate was 800 mcg/day with two 60 mcg boluses programmed to help with morning routine and sleep. Upon discharge, patient was voiding regularly, tolerating tube feeds, and his dystonia had improved compared to baseline.

Setting: Pediatric Acute Care Children’s HospitalAssessment/

Results: At 1-month post-implantation, patient exhibited much improved tone control and had no issues with the baclofen pump. His mother noted that he had been doing extremely well at home. Due to the progressive nature of the dystonia, multiple adjustments have been required to both enteral medications and pump doses. Through utilization of the baclofen pump, side effects from enteral medications have been mitigated.

Discussion: Medically refractory status dystonicus is a known complication of PKAN. We successfully placed an emergent baclofen pump for a patient with PKAN in status dystonicus, which decreased his enteral medication burden and his side effect profile.

Conclusion: Early consideration for intrathecal baclofen may be a successful palliative option for patients with refractory dystonia, especially in those with known progressive pathologies.

Level of Evidence: Level V

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PM&R Meeting Abstracts - https://pmrjabstracts.org/abstract/emergent-baclofen-pump-placement-for-refractory-status-dystonicus-in-a-patient-with-pantothenate-kinase-associated-neurodegeneration-a-case-report/