MRgFUS Thalamotomy for Essential Tremor in a Patient with von Willebrand Disease: Perioperative Optimization for Patients with Coagulopathies

Introduction: Essential tremor (ET) is one of the most common movement disorders worldwide. In medically refractory ET, deep brain stimulation (DBS) of the ventral intermediate nucleus of the thalamus is the current standard of care; however, DBS carries an inherent 2-3% risk of hemorrhage. In patients with acquired or genetic coagulopathies, the risk of hemorrhage can be much higher. MRgFUS thalamotomy is a surgical alternative that is highly effective in treating ET and has a favorable safety profile with no reports of intracranial hemorrhage to date. In this case-report, we share the first documented case of MRgFUS in a patient with von Willebrand Disease (VWD), the most common autosomal bleeding disorder. Based on our experience, we subsequently propose a framework through which to approach surgical management of ET in patients with VWD and other coagulopathies.

Methods: We present a case report of a 60-year-old left-handed male with VWD Type 2b, medically refractory ET, and a family history of hemorrhage secondary to DBS surgery.

Results: Our patient successfully underwent right sided MRgFUS thalamotomy. Perioperatively, he received a course of VonVendi (a recombinant von Willebrand Factor) to ensure appropriate coagulative function. Post-procedure imaging confirmed a focal lesion targeted to the right thalamus without any evidence of hemorrhage. At time of follow-up, the patient reported 90% improvement of his left hand tremor and marked (“100%” per his description) improvement in his quality of life without any obvious side effects.

Conclusion : This case report demonstrates that MRgFUS thalamotomy is a promising surgical alternative to DBS in the treatment of medically refractory ET, especially in patients with underlying coagulopathies. With proper perioperative and postoperative management of coagulopathies, the risk of intracranial hemorrhage is further decreased, thus optimizing these patients’ outcomes.