University Department of Medicine, Queen Mary Hospital, LKS Faculty of Medicine, The University of Hong Kong, Hong Kong; Neuroimmunology and Neuroinflammation Research Laboratory, LKS Faculty of Medicine, The University of Hong Kong, Hong Kong; Research Center of Heart, Brain, Hormone and Healthy Aging, LKS Faculty of Medicine, The University of Hong Kong, Hong Kong.
J Neuroimmunol. 2013 Sep 15;262(1-2):100-5. doi: 10.1016/j.jneuroim.2013.06.004. Epub 2013 Jul 6.
Classical multiple sclerosis (CMS) and neuromyelitis optica spectrum disorders (NMOSD) are distinct central nervous system inflammatory demyelinating disorders (CNS IDD). Early diagnosis of CNS IDD is important as appropriate immunotherapies to optimize prognosis. We studied the diagnoses of CNS IDD among Hong Kong Chinese in a hospital-based setting. Consecutive Chinese patients who presented to our hospital with clinically isolated syndrome and subsequently diagnosed to have CNS IDD from 1980 to 2010 were reviewed. Patients with known diagnosis of CNS IDD referred for further care were excluded. Serial sera were assayed for aquaporin-4 autoantibodies (AQP4 Ab), at least 3 assays within 2-5years. A total of 210 patients diagnosed to have CNS IDD with disease duration of at least 2years were studied. Among 198 patients with serial sera available, 40 (20.2%, 20 had NMO and 20 other NMOSD) were AQP4 Ab-positive. Four patients who were AQP4 Ab-negative on the initial assay converted to AQP4 Ab-positive on repeated assays. The diagnoses of 210 patients were CMS in 88 (41.9%), NMOSD 47 (22.4%, 27 NMO, 20 other NMOSD), single attack of myelitis 23 (11.0%), single attack of optic neuritis 21 (10.0%), relapsing myelitis 10 (4.8%), acute disseminated encephalomyelitis (ADEM) 9 (4.3%), relapsing optic neuritis in 6 (2.9%), opticospinal multiple sclerosis 3 (1.4%) and single attack of brainstem encephalitis 3 (1.4%). Compared to CMS, NMOSD patients had older onset age, lower frequencies of brain MRI abnormalities and CSF OCB, higher frequency of LETM, higher CNS inflammation attack frequency in the first 2years, worse clinical outcome with higher EDSS score and mortality rate. This hospital-based study suggests that CMS (41.9%) and NMOSD (22.4%) are the most common CNS IDD among Hong Kong Chinese. NMOSD has worse clinical outcome than CMS. Detection of AQP4 Ab facilitates early diagnosis and prompts immunotherapies of NMOSD.
经典多发性硬化症（CMS）和视神经脊髓炎谱系疾病（NMOSD）是两种不同的中枢神经系统炎症性脱髓鞘疾病（CNS IDD）。早期诊断 CNS IDD 非常重要，因为适当的免疫疗法可以优化预后。我们在医院环境中研究了香港中文人群中的 CNS IDD 诊断。回顾了 1980 年至 2010 年间因临床孤立综合征就诊并随后被诊断为 CNS IDD 的连续中文患者。排除了已知 CNS IDD 诊断并接受进一步治疗的患者。对至少 3 次在 2-5 年内进行的血清进行水通道蛋白 4 自身抗体（AQP4 Ab）检测。共研究了 210 例至少有 2 年病程的 CNS IDD 患者。在可获得 198 例连续血清的患者中，40 例（20.2%，20 例为 NMO，20 例为其他 NMOSD）为 AQP4 Ab 阳性。4 例初次检测为 AQP4 Ab 阴性的患者在重复检测中转为 AQP4 Ab 阳性。210 例患者的诊断为 CMS 88 例（41.9%）、NMOSD 47 例（27 例 NMO，20 例其他 NMOSD）、单发性脊髓炎 23 例（11.0%）、单发性视神经炎 21 例（10.0%）、复发性脊髓炎 10 例（4.8%）、急性播散性脑脊髓炎（ADEM）9 例（4.3%）、复发性视神经炎 6 例（2.9%）、视神经脊髓多发性硬化症 3 例（1.4%）和单发性脑干脑炎 3 例（1.4%）。与 CMS 相比，NMOSD 患者的发病年龄较大，脑 MRI 异常和 CSF OCB 频率较低，LETM 频率较高，前 2 年 CNS 炎症发作频率较高，EDSS 评分和死亡率较高，临床预后较差。这项基于医院的研究表明，CMS（41.9%）和 NMOSD（22.4%）是香港中文人群中最常见的 CNS IDD。NMOSD 的临床预后比 CMS 差。AQP4 Ab 的检测有助于早期诊断并促使 NMOSD 进行免疫治疗。
