Modifications of longitudinally extensive transverse myelitis and brainstem lesions in the course of neuromyelitis optica (NMO): a population-based, descriptive study.
Modifications of longitudinally extensive transverse myelitis and brainstem lesions in the course of neuromyelitis optica (NMO): a population-based, descriptive study.
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DOI：
10.1186/1471-2377-13-33
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发表时间：
2013-04-08
期刊：
影响因子：
2.6
通讯作者：
Kyvik KO
中科院分区：
文献类型：
--
作者：
Asgari N;Skejoe HP;Lillevang ST;Steenstrup T;Stenager E;Kyvik KO
视神经肌炎包括横肌炎、视神经炎和脑病变。最近的研究表明，脑干是NMO的重要发病部位。纵行广泛横肌萎缩（LETM）是NMO临床诊断的重要组成部分。脑干和LETM病变的频率、LETM随时间的变化以及NMO过程中的临床后果仅很少研究。该研究是一项基于人群的回顾性病例系列研究，对35例确诊NMO和复发缓解期患者进行了临床和磁共振成像（MRI）随访。脑干病变25例，其中延髓18例（最后区11例）。脑桥、中脑和间脑病变分别为10例、7例和7例。病变在延髓和脑桥有症状，在中脑和间脑无症状。抗水通道蛋白-4（AQP-4）抗体阳性的脑干病变明显多于血清阴性的患者（p < 0.002）。30/36例患者的脊髓MRI证实了LETM，其中23/30例患者进行了脊髓随访MRI。在5例患者中观察到复发性LETM。在9例患者中，LETM在缓解或治疗期间变为多个病变。在12/23例（52%）患者中观察到脊髓萎缩，与扩展残疾状态量表相关（r = 0.88，p < 0.001）。NMO患者脑干病变和LETM多见。脑干病变与抗AQP 4抗体阳性相关。LETM病变随着时间的推移而分化，结局包括复发、碎裂和萎缩。脊髓萎缩与神经功能障碍之间存在相关性。
Neuromyelitis optica (NMO) includes transverse myelitis, optic neuritis and brain lesions. Recent studies have indicated that the brainstem is an important site of attack in NMO. Longitudinally extensive transverse myelitis (LETM) is an important component of the clinical diagnosis of NMO. The frequency of brainstem and LETM lesions, changes over time of LETM and the clinical consequences in the course of NMO have only been sparsely studied. The study was a population-based retrospective case series with clinical and magnetic resonance imaging (MRI) follow-up of 35 patients with definite NMO and a relapsing-remitting course. Brainstem lesions were observed in 25 patients, 18 in medulla oblongata (11 in area postrema). Lesions in the pons, mesencephalon and diencephalon occurred in 10, 7 and 7 patients, respectively. Lesions were symptomatic in medulla oblongata and pons, asymptomatic in mesencephalon and diencephalon. Brainstem lesions were observed significantly more often in anti-aquaporin-4 (AQP-4) antibody positive than in seronegative patients (p < 0.002). LETM was demonstrated by MRI of the spinal cord in 30/36 patients, 23/30 of whom had follow-up MRI of the spinal cord. Recurrent LETM was observed in five patients. In nine patients the LETM changed into multiple lesions during remission or treatment. Spinal cord atrophy was observed in 12/23 (52%) patients, correlating to Expanded Disability Status Scale (r = 0.88, p < 0.001). NMO patients had frequent occurrence of brainstem lesions and LETM. Brainstem lesions were associated with anti-AQP4 antibody positivity. LETM lesions differentiated over time and the outcome included relapses, fragmentation and atrophy. Correlation was observed between spinal cord atrophy and neurological disability.
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影响因子：
9.3
作者：
Jarius S;Franciotta D;Paul F;Ruprecht K;Bergamaschi R;Rommer PS;Reuss R;Probst C;Kristoferitsch W;Wandinger KP;Wildemann B
通讯作者：
Wildemann B
影响因子：
3.7
作者：
Lu Z;Zhang B;Qiu W;Kang Z;Shen L;Long Y;Huang J;Hu X
通讯作者：
Hu X
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：
11.2
作者：
Weinshenker, BG;Wingerchuk, DM;Lennon, VA
通讯作者：
Lennon, VA
影响因子：
11
作者：
Takahashi, T.;Miyazawa, I.;Itoyama, Y.
通讯作者：
Itoyama, Y.