Department of Neurology, Hallym University Sacred Heart Hospital, Hallym University College of Medicine, Anyang, South Korea.
Hallym Neurological Institute, Hallym University College of Medicine, Anyang, South Korea.
BMC Neurol. 2020 May 21;20(1):201. doi: 10.1186/s12883-020-01784-3.
Stress cardiomyopathy (Takotsubo cardiomyopathy) is very rare in the central nervous system (CNS) demyelinating disorders. Although this dysfunction of the heart-brain axis has been reported in several case series related to multiple sclerosis (MS), stress cardiomyopathy by neuromyelitis optica (NMO), which is rarer CNS demyelinating disorder than MS, is extremely rare. Herein, we report a case of stress cardiomyopathy associated with a medullary lesion as a presentation of NMO.
A 30-year-old woman was treated by veno-arterial extracorporeal membrane oxygenation due to catastrophic cardiopulmonary dysfunction after prolonged and unexplained nausea, vomiting, and cough. Myoclonus on the limbs developed afterward. Taken with suspicion of area postrema syndrome (APS), the brain MRI showed a demyelinating lesion in the medulla oblongata. APS and severe heart failure by stress cardiomyopathy were completely resolved by ECMO and hydrocortisone therapy. However, the CNS demyelinating lesion recurred after 1 month. The patient was diagnosed with NMO evident by the presence of aquaporin-4 antibody; Steroid therapy improved her symptoms.
NMO should be considered as one of the differential diagnoses in patients with APS preceding severe cardiopulmonary distress.
在中枢神经系统(CNS)脱髓鞘疾病中,应激性心肌病(Takotsubo 心肌病)非常罕见。尽管这种心脏-大脑轴的功能障碍已在与多发性硬化症(MS)相关的几例系列病例中报告,但神经脊髓炎(NMO)的应激性心肌病(比 MS 更为罕见的 CNS 脱髓鞘疾病)则极为罕见。在此,我们报告了一例与延髓病变相关的应激性心肌病病例,表现为 NMO。
一名 30 岁女性因长时间不明原因的恶心、呕吐和咳嗽后发生灾难性心肺功能障碍而接受静脉-动脉体外膜肺氧合治疗。随后出现四肢肌阵挛。怀疑为延髓后极区综合征(APS),脑 MRI 显示延髓脱髓鞘病变。APS 和应激性心肌病引起的严重心力衰竭通过 ECMO 和氢化可的松治疗完全缓解。然而,1 个月后 CNS 脱髓鞘病变再次发生。患者被诊断为 NMO,存在水通道蛋白-4 抗体;类固醇治疗改善了她的症状。
在严重心肺窘迫之前出现 APS 的患者中,应考虑 NMO 作为鉴别诊断之一。