【摘要】 目的 分析合并免疫指標異常的視神經脊髓炎臨床特點。 方法 回顧性分析2009年5月-2010年11月收治的62例視神經脊髓炎患者中24例合并免疫指標異常患者的臨床資料。24例均為女性，發病年齡14~53歲。對其臨床表現、視覺誘發電位、影像學檢查結果、免疫檢查結果進行分析。 結果 所有患者均有脊髓和視神經同時或先后受累的表現。24例視覺誘發電位檢查23例異常。脊髓MRI顯示病變集中于頸段、上胸段脊髓。頸段和胸段脊髓同時受累17例，單純頸段脊髓受損6例，單純胸段脊髓受損1例。所有患者抗核抗體滴度均≥1∶100，合并抗SSA抗體陽性14例（55.5％），同時合并抗SSB抗體陽性11例（45.8％），合并抗Rib抗體陽性1例，合并抗SCL-70抗體陽性1例，合并抗dsDNA抗體1例。 結論 視神經脊髓炎合并免疫指標異常的患者以女性較為多見，易復發，青壯年患者發病率最高。脊髓MRI示病變集中于頸段、上胸段脊髓，表現為長節段脊髓損害。視神經脊髓炎患者合并結締組織病的病例較多。【Abstract】 Objective To analyze the clinical features of neuromyelitis optica (NMO) combined with abnormal immune parameters. Methods We retrospectively reviewed the clinical data of 24 patients with NMO and abnormal immune parameters among the 62 NMO patients who were admitted into our department between May 2009 and November 2010. All patients were female, aged from 14 to 53 years. We analyzed their clinical manifestations, visual evoked potentials, imaging results, and immunological examinations. Results All patients had simultaneous or successive spinal cord and optic nerve involvement. Twenty-three patients had abnormal visual evoked potential. MRI showed that the lesions focused on the cervical and upper thoracic spinal cord. Both cervical and thoracic spinal cord were involved in 17 cases; there were 6 cases of simple cervical spinal cord injury and 1 case of simple thoracic spinal cord damage. Antinuclear antibody titer of all the patients was ≥1∶100. Combined positive anti-SSA antibody occurred in 14 patients (55.5%); Concomitant positive anti-SSB antibodies occurred in 11 patients (45.8%); Combined positive anti-Rib antibodies occurred in 1 patient; Combined positive anti-SCL-70 antibody occurred in 1 patient; and combined positive anti-dsDNA antibodies occurred in 1 patient. Conclusions NMO combined with abnormal immune parameters mainly occurs in female patients, especially in young people. Recurrence rate is high. MRI shows that the lesions focus mainly on the cervical and upper thoracic spinal cord, manifesting the characteristic of long segment damage. And NMO is frequently combined with connective tissue disease.
Plasma exchange (PE) is a therapeutic blood component replacement method. The blood of patients is first separated into plasma and blood cell components using a blood cell separator in vitro, the plasma containing harmful pathogenic substances is then discarded and replaced with the same volume of exchange solution. Finally the separated blood cells together with the exchange solution are returned back to the blood circulation of patients. By reducing the circulating antibodies, abnormal plasma proteins or cytokines and other pathogenic molecules, PE can block the disease process. PE has a good therapeutic effect on neuromyelitis optica-related optic neuritis (NMO-ON), which shows resistant to glucocorticoid therapy for the first onset. The American Society for Apheresis guideline evaluates PE for acute optic neuritis as a recommended grade 1B, type II indication. In the implementation of PE treatment for NMO-ON and other diseases, indications and contraindications should be strictly adhered to the guideline, treatment procedures and protocols should be optimized, common adverse events and its prevention and management should be known and alerted. It is important to conduct multi-center clinical cooperation and a high standard clinical randomized controlled study, to find out the optimal time window, the best protocol, and the associated factors for the efficacy and prognosis of PE in NMO-ON.
Neuromyelitis optica-related optic neuritis (NMO-ON) is a kind of severe optic nerve disease, which always leads to replase, poor prognosis, and even blindness. Aquaporin 4 antibody (AQP4-IgG) is the main diagnostic biomarker for neuromyelitis optica with high specificity. Serum myelin oligodendrocyte glycoprotein antibody (MOG-IgG) is helpful for the diagnosis of AQP4-IgG negative patients. The study of biomarkers is helpful to deeply understand the pathogenesis of NMO-ON, help the diagnosis of the disease, and finally make precise treatment. Orbital MRI can help to differentiate MOG-IgG positive from AQP4-IgG positive neuromyelitis optica and optic neuritis, which is very important for the diagnosis of NMO-ON. At present, the standardized treatment of NMO-ON can be divided into two clinical stages: acute stage and remission stage. Corticosteroids and plasma exchange are the main treatments in acute stage, aiming at alleviating acute inflammatory reaction and improving prognosis. Immunosuppressive agents and biological agents are the main treatments in remission stage, aiming at preventing or reducing recurrence. With the development of the diagnosis and treatment of NMO-ON, we find that it is more and more important to strengthen the construction of neuro-ophthalmology team in China, establish clinical epidemiological database of NMO-ON, and carry out multi-centre, large-sample, prospective clinical control studies in China to provide evidence-based medicine for Chinese people. In addition, we need to strengthen efforts to establish and improve the diagnostic criteria for NMO-ON and the promotion of diagnostic and therapeutic criteria, and strive to improve the clinical diagnosis and treatment level of NMO-ON in China.
【摘要】 目的 分析急性播散性腦脊髓炎的臨床特點，提高診療。 方法 收集1999年1月-2010年1月住院的急性播散性腦脊髓炎患者42例，對其臨床癥狀體征、實驗室檢查、影像學改變及治療進行全面回顧性分析。 結果 42例患者中5～14歲者11例（26.19%）；15～40歲者20例（47.62%），感染后引起的23例（54.76%），無明顯誘因占15例（35.71%）；腦脊液23例（23/34，67.65%）異常；腦電圖異常者27例（27/32，84.38%）；CT檢查陽性率26例（26/40，65.00%），MRI陽性率25例（25/28，89.29%）；糖皮質素、丙種球蛋白治療有效。 結論 急性播散性腦脊髓炎是一組臨床表現多樣的免疫介導的炎性疾病，腦脊液、MRI和腦電圖有重要診斷價值。急性期大劑量皮質素、靜脈丙種球蛋白治療均有較好療效。【Abstract】 Objective To analysis the clinical features of acute disseminated encephalomyelitis so as to improve medical treatment. Methods From January, 1999 to January, 2010, 42 inpatients with acute disseminated encephalomyelitis were collected and their clinical data were analyzed retrospectively. Results Out of these 42 patients, 11 (26.19% ) were within 5 to 14 years, 20 (47.62%) ithin 15 to 40 years; 23 (54.76%) had definite infection, and 15 (35. 71%)had no any causes; 23 (23/34, 67.65%) had abnormal cerebrospinal fluid; 27 (27/32, 84.38%) had abnormal electro-encephalograph; 26 (26/40, 65.00%) were CT positive, 25 (25/28, 89.29%) MRI positive; corticosteroids and gamma globulin were effective in the treatment of disseminated encephalomyelitis. Conclusion Acute disseminated encephalomyelitis is a kind of inflammatory disease with various clinical manifestation and mediated by immune. Cerebrospinal fluid, MRI, and electro-encephalograph have important roles in its diagnosis. Large dose of corticosteroids and gamma globulin are effective in the treatment of acute disseminated encephalomyelitis.
Neuromyelitis optica (NMO) is a kind of demyelinating disorder that preferentially affects the optic nerves and spinal cord and results in permanent vision loss. NMO pathogenesis is thought to involve binding of anti-aquaporin-4 (AQP4) auto-antibodies to astrocytes, which causes complement-dependent cytotoxicity (CDC) and downstream inflammation leading to oligodendrocyte and neuronal injury. Vasculocentric deposition of activated complement is a prominent feature of NMO pathology. In recent years, a number of groups have found complements play an important role in the pathogenesis of NMO, and basic researches in NMO therapy due to its specificity and uniformity. Its inhibition would protect against proteins in the classical complement pathway so that cure the disease. This review will expound the the role of complement signaling pathway in the pathogenesis of NMO, and provide reference for a more in-depth understanding and clinical treatments of NMO.
Neuromyelitis optica spectrum disorders (NMOSD) are a group of inflammatory disorders of the central nervous system characterized by episodes of immune-mediated demyelination and axonal damage mainly involving optic nerves and spinal cord. Neuromyelitis optica related optic neuritis (NMO-ON) is a common neuro-ophthalmic disease which often results in permanent blindness. The discovery of aquaporin 4 antibodies confirms that neuromyelitis optica is a distinct disease entity different from multiple sclerosis. In patients with NMO-ON, the correct therapeutic approach has to recognize two distinct clinical situations: treatment of the acute attacks and prevention of the relapses. With the in-depth study of the pathogenesis of NMOSD, new treatments are emerging in different targets of the disease. This review gives an update of latest treatment of NMO-ON, emphasizing both current situation and future immunotherapy strategies.
