MOG-IgG in NMO and related disorders: a multicenter study of 50 patients. Part 2: Epidemiology, clinical presentation, radiological and laboratory features, treatment responses, and long-term outcome.
MOG-IgG in NMO and related disorders: a multicenter study of 50 patients. Part 2: Epidemiology, clinical presentation, radiological and laboratory features, treatment responses, and long-term outcome.
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DOI：
10.1186/s12974-016-0718-0
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发表时间：
2016-09-27
影响因子：
9.3
通讯作者：
in cooperation with the Neuromyelitis Optica Study Group (NEMOS)
中科院分区：
文献类型：
--
作者：
Jarius S;Ruprecht K;Kleiter I;Borisow N;Asgari N;Pitarokoili K;Pache F;Stich O;Beume LA;Hümmert MW;Ringelstein M;Trebst C;Winkelmann A;Schwarz A;Buttmann M;Zimmermann H;Kuchling J;Franciotta D;Capobianco M;Siebert E;Lukas C;Korporal-Kuhnke M;Haas J;Fechner K;Brandt AU;Schanda K;Aktas O;Paul F;Reindl M;Wildemann B;in cooperation with the Neuromyelitis Optica Study Group (NEMOS)
已显示患有视神经肌萎缩谱系疾病（NMOSD）的患者的子集对髓鞘少突胶质细胞糖蛋白抗体（MOG-IgG）呈血清阳性。描述MOG-IgG阳性的视神经炎（ON）和/或肌炎患者（n = 50）的流行病学、临床、影像学、脑脊液（CSF）和电生理学特征以及发作和长期治疗结局。回顾性多中心研究。男女性别比为1：2.8。发病时的中位年龄为31岁（范围6-70岁）。80%的患者（首次复发的中位时间为5个月;年复发率为0.92）出现多期病程，40%的患者（平均随访时间为75 ± 46.5个月）出现严重残疾，最常见的长期后遗症为重度视力损害或功能性失明（36%）和麻痹或共济失调引起的明显弱视（25%）。约70%的患者在至少一次ON发作期间观察到单眼或双眼功能性失明。几个病人出现视周强化。除急性四肢轻瘫外，急性肌无力患者常伴有感觉迟钝和疼痛（70%）.纵向广泛的脊髓病变是常见的，但短病变发生至少一次在44%。41%的人有同时ON和myeloid的病史。50%的患者临床或影像学表现为脑、脑干或小脑受累;视神经脊髓外症状包括顽固性恶心、呕吐和呼吸功能不全（1例死亡）。70%的患者出现CSF细胞增多（部分嗜酸性），仅13%出现寡克隆条带，32%出现血-CSF屏障功能障碍。静脉注射甲基强的松龙（IVMP）和长期免疫抑制通常有效;然而，在许多患者中注意到治疗失败导致残疾的快速累积以及类固醇停药后的突然发作。在某些情况下，通过血浆置换实现了完全恢复，包括IVMP失败后。硫唑嘌呤下的突破性发作与药物特异性潜伏期和缺乏口服类固醇的联合治疗有关。甲氨蝶呤在5/6例患者中有效。干扰素β与持续或增加的疾病活动有关。利妥昔单抗和奥法木单抗在一些患者中有效。然而，在几例病例中，利妥昔单抗治疗后出现早期复发;首次输注后9-12个月出现剂量终末复发。共存的自身免疫性是罕见的（9%）。符合Wingerchuk 2006年和2015年NMO（SD）标准以及Barkhof和McDonald多发性硬化症（MS）标准的患者分别为28%、32%、15%和33%; 36%的患者疑似MS。在一些病例中，疾病发作或复发之前是感染、接种疫苗或怀孕/分娩。我们的研究结果从一个主要是白人队列强烈反对的概念MOG-IgG表示一个温和的，通常是双相变异的NMOSD。主要是复发性和经常严重的疾病过程和较短的中位时间，以第二次发作支持使用预防性长期治疗的患者MOG-IgG阳性ON和/或肌萎缩症。
A subset of patients with neuromyelitis optica spectrum disorders (NMOSD) has been shown to be seropositive for myelin oligodendrocyte glycoprotein antibodies (MOG-IgG). To describe the epidemiological, clinical, radiological, cerebrospinal fluid (CSF), and electrophysiological features of a large cohort of MOG-IgG-positive patients with optic neuritis (ON) and/or myelitis (n = 50) as well as attack and long-term treatment outcomes. Retrospective multicenter study. The sex ratio was 1:2.8 (m:f). Median age at onset was 31 years (range 6-70). The disease followed a multiphasic course in 80 % (median time-to-first-relapse 5 months; annualized relapse rate 0.92) and resulted in significant disability in 40 % (mean follow-up 75 ± 46.5 months), with severe visual impairment or functional blindness (36 %) and markedly impaired ambulation due to paresis or ataxia (25 %) as the most common long-term sequelae. Functional blindess in one or both eyes was noted during at least one ON attack in around 70 %. Perioptic enhancement was present in several patients. Besides acute tetra-/paraparesis, dysesthesia and pain were common in acute myelitis (70 %). Longitudinally extensive spinal cord lesions were frequent, but short lesions occurred at least once in 44 %. Fourty-one percent had a history of simultaneous ON and myelitis. Clinical or radiological involvement of the brain, brainstem, or cerebellum was present in 50 %; extra-opticospinal symptoms included intractable nausea and vomiting and respiratory insufficiency (fatal in one). CSF pleocytosis (partly neutrophilic) was present in 70 %, oligoclonal bands in only 13 %, and blood-CSF-barrier dysfunction in 32 %. Intravenous methylprednisolone (IVMP) and long-term immunosuppression were often effective; however, treatment failure leading to rapid accumulation of disability was noted in many patients as well as flare-ups after steroid withdrawal. Full recovery was achieved by plasma exchange in some cases, including after IVMP failure. Breakthrough attacks under azathioprine were linked to the drug-specific latency period and a lack of cotreatment with oral steroids. Methotrexate was effective in 5/6 patients. Interferon-beta was associated with ongoing or increasing disease activity. Rituximab and ofatumumab were effective in some patients. However, treatment with rituximab was followed by early relapses in several cases; end-of-dose relapses occurred 9-12 months after the first infusion. Coexisting autoimmunity was rare (9 %). Wingerchuk’s 2006 and 2015 criteria for NMO(SD) and Barkhof and McDonald criteria for multiple sclerosis (MS) were met by 28 %, 32 %, 15 %, 33 %, respectively; MS had been suspected in 36 %. Disease onset or relapses were preceded by infection, vaccination, or pregnancy/delivery in several cases. Our findings from a predominantly Caucasian cohort strongly argue against the concept of MOG-IgG denoting a mild and usually monophasic variant of NMOSD. The predominantly relapsing and often severe disease course and the short median time to second attack support the use of prophylactic long-term treatments in patients with MOG-IgG-positive ON and/or myelitis.
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影响因子：
5.8
作者：
Elsone, Liene;Kitley, Joanna;Jacob, Anu
通讯作者：
Jacob, Anu
影响因子：
--
作者：
Jacob, Anu;Weinshenker, Brian G.;Cree, Bruce A. C.
通讯作者：
Cree, Bruce A. C.
DOI：
10.1177/1352458514567216
发表时间：
2015-05
期刊：
Multiple sclerosis (Houndmills, Basingstoke, England)
影响因子：
--
作者：
Bennett JL;de Seze J;Lana-Peixoto M;Palace J;Waldman A;Schippling S;Tenembaum S;Banwell B;Greenberg B;Levy M;Fujihara K;Chan KH;Kim HJ;Asgari N;Sato DK;Saiz A;Wuerfel J;Zimmermann H;Green A;Villoslada P;Paul F;GJCF-ICC&BR
通讯作者：
GJCF-ICC&BR
影响因子：
11
作者：
Baumann, M.;Sahin, K.;Rostasy, K.
通讯作者：
Rostasy, K.
影响因子：
14.5
作者：
Barkhof, F;Filippi, M;Valk, J
通讯作者：
Valk, J