Abstracts

RATIONALE: Physicians often have differing perspectives on utilizing Vagus Nerve Stimulation?with the NCP? device to treat medically refractory epilepsy including expected risks and benefits. This Abstract will examine the outcome for a 5 year old child with a progressive neurodegenerative disease of unknown etiology, myoclonic seizures and Status Epilepticus who was not expected to survive. After 3 months in the PICU, the parents and physicians made the decision to progress with VNS. The MD issues: expense of therapy in moribund child; lack of clinical data for efficacy in status or myoclonus. Parent issues: all therapy options had been exhausted, willing to persue small clinical improvements and quality of life gains. METHODS:Retrospective chart review, physician interviews, family interviews RESULTS:This unfortunate child was treated with pentobarb coma, midazolam, polytherapy AED's, Ketogenic diet with no sucess. Neurologic & genetic consults including tissue biopsies ruled out identifiable mitochondrial diseases. MRI/CT of the head showed atrophy,minimal corpus callosum,cerebellar atrophy. Invasive video EEG showed left central and bifrontal spike and wave discharges.SPECT/PET were non-localizing. VNS therapy began 3 months after admission and made no decernable change in the child's acute condition. The child was discharged on polytherapy AED's with stimulator parameters:0.5mAmp, 30 Hz,500 mSec, for chronic and magnet settings. on 30 seconds,off 5 minutes. The family was instructed to notify the team of the child's death so an autopsy could be obtained. Three months after discharge, the family reported that the child had not had any generalized seizures. Using the magnet, they could prevent the myoclonus from progressing to other seizure types. He became alert, and more interactive with his surroundings. CONCLUSIONS: MD's held that VNS had been minimally effective for this child. The family was very happy with the decrease in seizure frequency,intensity, improved recovery time and quality of life. Both views are important in making end of life decisions.More data on efficay in status is needed.