Bilateral Subthalmic Nucleus Deep Brain Stimulation With Microelectrode Recordings In The Setting Of Mild Inherited Hemophilia B: A Case Report

Hemophilia B is an X linked recessive deficiency of factor IX that presents with a range of clinical severity that co-relates with factor levels. Although guidelines exist to guide perioperative hemostasis in such patients, there is scarce data on elective high-risk neurosurgeries, resulting in a reluctance to offer these patients elective neurosurgeries. These patients thus rarely if ever undergo such procedures. We report a unique case of undiagnosed mild hemophilia B in a gentleman that was found incidentally at age 64 during pre-operative workup. This gentleman had intractable Parkinson's disease for which subthalmic deep brain stimulation was indicated. He was found to have a prolonged APTT on initial lab testing. After subsequent workup, and having excluded the presence of inhibitors, he was diagnosed with Hemophilia B. With the use of Factor IX concentrates (AlphaNine (R)) and close clinical, laboratory, and radiological monitoring a plan was made for this patient to undergo this procedure. Our patient successfully underwent subthalmic deep brain stimulation with microelectrode recordings and intraoperative test stimulation in a two-step procedure, followed by single channel implantable neurostimulator and extension wire implantations 2weeks later. The successful peri-operative course of this patient using this novel approach is described, and the need for future data in this regard is emphasized.