Urriola Nicolás X, Helou Jacob, Maamary Joel, Pogson Jacob, Lee Frederick, Parratt Kaitlyn, Gillis David, Fulham Michael J, Halmágyi G Michael
Departments of Neurology and Immunology, Royal Prince Alfred Hospital, Sydney, Australia.
Department of Neurology, Royal Prince Alfred Hospital, Sydney, Australia.
J Clin Neurosci. 2018 Dec;58:203-204. doi: 10.1016/j.jocn.2018.10.011. Epub 2018 Oct 16.
Opsoclonus-myoclonus syndrome (OMS) is a brainstem/cerebellar syndrome producing disabling multi-directional saccadic oscillations with oscillopsia, with or without somatic myoclonus and cerebellar ataxia (Wong et al., 2001; Armangué et al., 2016). OMS is presumed to have an autoimmune basis and patients with it are tested for antineuronal antibodies and have imaging to locate any tumors. Here we report a unusual case of a young woman who had NMDAR antibody (NMDAR-ab) positive, teratoma-related, isolated OMS without encephalopathy. Removal of her ovarian teratoma, and immunotherapy with steroids, intravenous immunoglobulin (IVIg), plasma exchange (PLEX), and ultimately with B-cell depletion with rituximab resulted in total recovery after 3 months. Patients with teratoma-related OMS very rarely have NMDAR-ab which suggests that it is not the NMDAR-ab per se that causes the OMS.
眼阵挛-肌阵挛综合征（OMS）是一种脑干/小脑综合征，可产生伴有视振荡的致残性多向眼球震颤，伴或不伴有躯体肌阵挛和小脑共济失调（Wong等人，2001年；Armangué等人，2016年）。OMS被认为具有自身免疫基础，患有该综合征的患者会接受抗神经元抗体检测，并进行影像学检查以定位任何肿瘤。在此，我们报告一例年轻女性的罕见病例，该患者N-甲基-D-天冬氨酸受体抗体（NMDAR-ab）呈阳性，与畸胎瘤相关，为孤立性OMS且无脑病。切除她的卵巢畸胎瘤，并采用类固醇、静脉注射免疫球蛋白（IVIg）、血浆置换（PLEX）进行免疫治疗，最终使用利妥昔单抗进行B细胞清除，3个月后完全康复。与畸胎瘤相关的OMS患者很少有NMDAR-ab，这表明并非NMDAR-ab本身导致OMS。
