Deep brain stimulation in a pediatric dystonia patient with cochlear implants and mitochondrial disorder: novel application of a frameless stereotactic system and navigating the anesthesia choice and neurosurgical complexities. Illustrative case

• DOI: 10.3171/case23128
• Publication Date: 2023-08-08T14:09:27Z
• Authors: Audrey Grossen; Helen H. Shi; Mallory Schenk; Amber Stocco; Justin Ramsey; Suneet Sahgal; Andrew K. Conner; Virendra R. Desai
• Keywords: Subthalamic Nucleus; Dexmedetomidine

BACKGROUND This report presents a case of medically refractory dystonia in pediatric patient successfully treated with bilateral subthalamic nucleus (STN) deep brain stimulation (DBS) while under general anesthesia by using microelectrode recordings (MERs) intraoperative computed tomography (CT). OBSERVATIONS The was an 18-year-old female primary secondary to mitochondrial Leigh syndrome. Her past medical history significant for complex partial epilepsy and hearing loss cochlear implants. implants precluded anatomical targeting via magnetic resonance imaging. Additionally, the could not tolerate awake surgery MER. decision made proceed STN DBS CT anesthesia. patient’s standard frame placement difficult, so navigation performed Nexframe system. Recordings were obtained ketamine, dexmedetomidine, remifentanil. At 3- 6-month follow-ups, demonstrated marked improvement without neurological complications. LESSONS is first syndrome DBS. authors describe novel use lead patient. demonstrates that MER can be safe effective method treating certain patients.