Neuromyelitis optica spectrum disorders with non opticospinal manifestations as initial symptoms: a long-term observational study.
Neuromyelitis optica spectrum disorders with non opticospinal manifestations as initial symptoms: a long-term observational study.
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以非视神经脊髓表现为首发症状的视神经脊髓炎谱系疾病：一项长期观察研究
DOI：
10.1186/s12883-021-02059-1
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发表时间：
2021-01-25
期刊：
影响因子：
2.6
通讯作者：
Yang Z
中科院分区：
文献类型：
--
作者：
Li R;Lu D;Li H;Wang Y;Shu Y;Chang Y;Sun X;Lu Z;Qiu W;Yang Z
背景以非视神经脊髓炎为首发症状的早期视神经脊髓炎视谱性疾病(NMOSD)易误诊，但有关其全部症状的资料有限。此外，这些患者的临床特征和长期结果尚不清楚。我们试图分析以非视神经脊髓症状为首发症状的NMOSD的临床特征、影像特征和远期转归。结果在471例NMOSD患者中，43例(9.13 %)诊断为非视髓首发症状。其中，88.37%的 在平均6.33年的随访期内发展为视神经炎/脊髓炎。非视脊髓症状均为脑/脑干症状。大多数症状和相关的脑部病变是可逆的。这些患者的发病年龄(P<； 0.001)、血清水通道蛋白4(AQP4)抗体滴度(P= 0.030)、发病时和随访时的扩展残疾状态量表评分(P<； 0.001)和随访(P= 0.041)均低于有视脊髓首发症状的NMOSD患者。结论非视髓首发症状的非视神经功能障碍患者发病年龄较早，血清水通道蛋白4抗体滴度较低，临床转归较好(P= 0.028)。
BackgroundEarly stage neuromyelitis optica spectrum disorders (NMOSD) with non-opticospinal manifestations as initial symptoms are easily misdiagnosed; however, data on the full symptom profile are limited. Moreover, the clinical characteristics and long-term outcomes of these patients remain unknown. We sought to analyze the clinical characteristics, imaging features, and long-term outcomes of NMOSD with non-opticospinal manifestations as initial symptoms.MethodsWe retrospectively included relevant patients from our center. Clinical, demographic, magnetic resonance imaging, treatment, and outcome data were compared according to the non-opticospinal vs. opticospinal initial symptoms.ResultsWe identified 43 (9.13 %) patients with non-opticospinal initial symptoms among 471 patients with NMOSD. Of these, 88.37 % developed optic neuritis/myelitis during an average follow-up period of 6.33 years. All the non-opticospinal symptoms were brain/brainstem symptoms. Most of the symptoms and associated brain lesions were reversible. These patients had a younger onset age (P< 0.001), lower serum aquaporin-4 (AQP4) antibody titers (P= 0.030), and a lower Expanded Disability Status Scale (EDSS) score at onset (P< 0.001) and follow-up (P= 0.041) than NMOSD patients with opticospinal initial symptoms. In addition, EDSS scores reached 3.0 (indicating moderate disability) later than in patients with opticospinal initial symptoms (P= 0.028).ConclusionsPatients with NMOSD with non-opticospinal initial symptoms have a younger onset age, lower serum AQP4 antibody titers, and better clinical outcomes.
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影响因子：
9.9
作者：
Wingerchuk DM;Banwell B;Bennett JL;Cabre P;Carroll W;Chitnis T;de Seze J;Fujihara K;Greenberg B;Jacob A;Jarius S;Lana-Peixoto M;Levy M;Simon JH;Tenembaum S;Traboulsee AL;Waters P;Wellik KE;Weinshenker BG;International Panel for NMO Diagnosis
通讯作者：
International Panel for NMO Diagnosis
影响因子：
9.9
作者：
Wingerchuk, D. M.;Lennon, V. A.;Weinshenker, B. G.
通讯作者：
Weinshenker, B. G.
影响因子：
14.5
作者：
Kitley, Joanna;Leite, M. Isabel;Palace, Jacqueline
通讯作者：
Palace, Jacqueline
影响因子：
168.9
作者：
Lennon, VA;Wingerchuk, DM;Weinshenker, BG
通讯作者：
Weinshenker, BG
中国人群中 AQP4 血清阳性患者的迟发性视神经脊髓炎谱系障碍
影响因子：
2.6
作者：
Mao Z;Yin J;Zhong X;Zhao Z;Qiu W;Lu Z;Hu X
通讯作者：
Hu X