Viswanathan Shanthi, Arip Masita, Mustafa Norhazlin, Dhaliwal Jasbir S, Rose Norzainie, Muda Sobri, Puvanarajah Santhi Datuk, Rafia Mohammad Hanip, Wing Loong Mark Cheong
Department of Neurology, Kuala Lumpur Hospital, Kuala Lumpur, Malaysia.
Autoimmune Unit, Allergy & Immunology Research Centre, Institute for Medical Research (IMR), Kuala Lumpur, Malaysia.
Mult Scler Int. 2014;2014:568254. doi: 10.1155/2014/568254. Epub 2014 Nov 17.
Background. In the past the occurrence of neuromyelitis optica in Malaysia was thought to be uncommon and the frequency of anti-aquaporin-4 Ig G antibody was unknown. Objective. To evaluate the frequency of anti-aquaporin-4 Ig G antibody (Anti-AQP4 antibody) amongst patients with neuromyelitis optica (NMO) and its spectrum disorders (NMOSD) and the differences between the seropositive and seronegative groups. Methods. Retrospectively, 96 patients with NMO/high risk syndromes for NMOSD (HRS-NMOSD) were identified out of 266 patients with idiopathic inflammatory demyelinating disease from a single center hospital based registry. Anti-AQP4 seropositivity was found in 38/48 (79.2%) with NMO, 12/21 (57.1%) with brain involvement at high risk for NMOSD, 12/15 (80%) with transverse myelitis (i.e., 11/15 with relapsing transverse myelitis and one with monophasic transverse myelitis), and 3/7 (42.8%) with relapsing optic neuritis. Sixty-five out of 96 patients, that is, 67.7%, with NMO/HRS for NMOSD were seropositive. Seropositivity was significantly associated with female gender, a higher number of mean relapses, that is, 5.15 ± 4.42 versus 2.10 ± 1.68, longer length of spinal cord lesions, that is, 6.6 ± 4.9 versus 2.9 ± 2.5, vertebral bodies, higher EDSS, 4.5 ± 2.4 versus 2.4 ± 2.6, presence of paroxysmal tonic spasms, and blindness (unilateral/bilateral); P < 0.001. Longitudinally extensive cord lesions (contiguous or linear), presence of lesions in the cervical and thoracic regions, and involvement of the central gray matter or holocord regions on axial scans, were also significantly associated with seropositivity; P < 0.001. Conclusion. NMO and HRS for NMOSD are present in larger numbers than previously thought in Malaysia. More than 2/3rds are seropositive. Seropositive and seronegative NMO/NMOSD have differences that are useful in clinical practice.
背景。过去，人们认为视神经脊髓炎在马来西亚并不常见，抗水通道蛋白4 IgG抗体的频率也未知。目的。评估视神经脊髓炎（NMO）及其谱系障碍（NMOSD）患者中抗水通道蛋白4 IgG抗体（抗AQP4抗体）的频率，以及血清阳性和血清阴性组之间的差异。方法。回顾性地从一家单中心医院登记处的266例特发性炎性脱髓鞘疾病患者中，识别出96例NMO/ NMOSD高风险综合征（HRS-NMOSD）患者。在48例NMO患者中有38例（79.2%）抗AQP4血清阳性，21例有NMOSD高风险脑受累的患者中有12例（57.1%），15例横贯性脊髓炎患者中有12例（80%）（即15例复发型横贯性脊髓炎患者中有11例，单相横贯性脊髓炎患者中有1例），7例复发性视神经炎患者中有3例（42.8%）。96例NMO/HRS-NMOSD患者中有65例（即67.7%）血清阳性。血清阳性与女性性别、平均复发次数较多显著相关，即5.15±4.42次与2.10±1.68次，脊髓病变长度较长显著相关，即6.6±4.9节段与2.9±2.5节段，椎体受累、扩展残疾状态量表（EDSS）较高显著相关，即4.5±2.4与2.4±2.6，存在阵发性强直性痉挛以及失明（单侧/双侧）显著相关；P<0.001。纵向广泛脊髓病变（连续或线性）、颈段和胸段存在病变以及轴位扫描显示中央灰质或全脊髓区域受累，也与血清阳性显著相关；P<0.001。结论。在马来西亚，NMO和HRS-NMOSD的实际病例数比之前认为的要多。超过三分之二的患者血清阳性。血清阳性和血清阴性的NMO/NMOSD存在差异，这在临床实践中很有用。
