PDF(Pubmed)
Abstract：
BACKGROUND: 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% to 3% risk of hemorrhage, a risk that can be much higher in patients with concomitant coagulopathy. Magnetic resonance imaging-guided focused ultrasound (MRgFUS) thalamotomy is a surgical alternative that is highly effective in treating ET, with no reports of intracranial hemorrhage to date.
METHODS: This is the first documented case of successful MRgFUS thalamotomy in a patient with von Willebrand disease (VWD). A 60-year-old left-handed male had medically refractory ET, VWD type 2B, and a family history of clinically significant hemorrhage after DBS. He underwent right-sided MRgFUS thalamotomy and received a perioperative course of VONVENDI (recombinant von Willebrand factor) to ensure appropriate hemostasis. Postprocedure imaging confirmed a focal lesion in the right thalamus without evidence of hemorrhage. The patient reported 90% improvement of his left-hand tremor and significant improvement in his quality of life without obvious side effects.
CONCLUSIONS: MRgFUS thalamotomy with peri- and postoperative hematological management is a promising alternative to DBS for patients with underlying coagulopathies.
METHODS: This is the first documented case of successful MRgFUS thalamotomy in a patient with von Willebrand disease (VWD). A 60-year-old left-handed male had medically refractory ET, VWD type 2B, and a family history of clinically significant hemorrhage after DBS. He underwent right-sided MRgFUS thalamotomy and received a perioperative course of VONVENDI (recombinant von Willebrand factor) to ensure appropriate hemostasis. Postprocedure imaging confirmed a focal lesion in the right thalamus without evidence of hemorrhage. The patient reported 90% improvement of his left-hand tremor and significant improvement in his quality of life without obvious side effects.
CONCLUSIONS: MRgFUS thalamotomy with peri- and postoperative hematological management is a promising alternative to DBS for patients with underlying coagulopathies.
摘要：
背景：原发性震颤（ET）是世界范围内最常见的运动障碍之一。在医学难治性ET中，丘脑腹侧中间核的深部脑刺激（DBS）是当前的护理标准。然而,DBS具有固有的2％至3％的出血风险，合并凝血病患者的风险可能更高。磁共振成像引导的聚焦超声（MRgFUS）丘脑切开术是一种非常有效的治疗ET的手术方法，到目前为止还没有颅内出血的报告.
方法：这是首例有文献记载的血管性血友病（VWD）患者成功进行MRgFUS丘脑切开术的病例。一名60岁的左撇子男性患有医学难治性ET，VWD2B型,以及DBS术后有明显出血的家族史。他接受了右侧MRgFUS丘脑切开术，并接受了围手术期的VONVENDI（重组vonWillebrand因子）以确保适当的止血。术后影像学证实右丘脑局灶性病变，无出血迹象。患者报告其左手震颤改善了90％，生活质量得到了显着改善，而没有明显的副作用。
结论：MRgFUS丘脑切开术与围手术期和术后血液学管理是潜在凝血障碍患者DBS的有希望的替代方法。
方法：这是首例有文献记载的血管性血友病（VWD）患者成功进行MRgFUS丘脑切开术的病例。一名60岁的左撇子男性患有医学难治性ET，VWD2B型,以及DBS术后有明显出血的家族史。他接受了右侧MRgFUS丘脑切开术，并接受了围手术期的VONVENDI（重组vonWillebrand因子）以确保适当的止血。术后影像学证实右丘脑局灶性病变，无出血迹象。患者报告其左手震颤改善了90％，生活质量得到了显着改善，而没有明显的副作用。
结论：MRgFUS丘脑切开术与围手术期和术后血液学管理是潜在凝血障碍患者DBS的有希望的替代方法。
