(743) A Rare Case of Severe Medication-Refractory Status Dystonicus in a Pediatric Patient

Introduction: Status dystonicus (SD) is a rare, life-threatening, and under-recognized complication presenting with sustained dystonia, autonomic dysfunction, and elevated CK with an incidence of 0.05 out of 1000 new admissions annually in children less than 12 years of age, and average mortality rate of 10%. Herein, we illustrate a case of severe refractory SD in a child with acquired generalized dystonia needing multiple medications, barbiturate coma, and deep brain stimulation (DBS).

Description: Patient is an eight-year-old girl with a history of ascending asymmetric spasticity and muscle weakness that progressed to spastic diplegia, dystonic choreoathetosis, oculomotor apraxia, and dysarthria. She was admitted to our hospital for worsening spasticity and dystonic posturing refractory to outpatient management. She was placed on a combination of trihexyphenidyl, clonidine, baclofen, gabapentin, tetrabenazine, and as needed doses of benzodiazepines with symptom easement. However, she developed a fever triggering medication refractory dystonia with CK elevation consistent with SD necessitating transfer to the PICU for midazolam infusion. Her SD persisted despite the addition of ketamine, dexmedetomidine, midazolam, and propofol infusions, in addition to vecuronium as needed. Symptomatic relief was achieved with pentobarbital dosed to burst suppression and simultaneous titration of enteral medications. Multiple attempts to wean pentobarbital resulted in reemergence of dystonic posturing. Therefore, she underwent DBS into the bilateral GPi. Four weeks after placement, her dyskinetic movements were improved over baseline, infusions stopped, and she was discharged to rehab.

Discussion: This case represents a severe and unique presentation of SD, that was treated by a multidisciplinary team including PICU, Neurology, Palliative, PM&R, and neurosurgery in a tertiary medical center. In SD, only 10% of patients respond to pharmacological treatment. Whereas, according to a meta-analysis published in 2018 on pediatric patients with SD treated with DBS, the median improvement in motor score was 54%, with 6 patients achieving over 85% improvement. This patient’s profound response to DBS underscores the need for early consideration of DBS for refractory SD.