【摘要】 目的 分析急性播散性脑脊髓炎的临床特点，提高诊疗。 方法 收集1999年1月-2010年1月住院的急性播散性脑脊髓炎患者42例，对其临床症状体征、实验室检查、影像学改变及治疗进行全面回顾性分析。 结果 42例患者中5～14岁者11例（26.19%）；15～40岁者20例（47.62%），感染后引起的23例（54.76%），无明显诱因占15例（35.71%）；脑脊液23例（23/34，67.65%）异常；脑电图异常者27例（27/32，84.38%）；CT检查阳性率26例（26/40，65.00%），MRI阳性率25例（25/28，89.29%）；糖皮质素、丙种球蛋白治疗有效。 结论 急性播散性脑脊髓炎是一组临床表现多样的免疫介导的炎性疾病，脑脊液、MRI和脑电图有重要诊断价值。急性期大剂量皮质素、静脉丙种球蛋白治疗均有较好疗效。【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.
Objective To observe the clinical characteristics of demyelinating optic neuritis (DON) in Chinese children under the age of 16. Methods A retrospective review of the medical charts of 42 pediatric patients with DON was conducted in this study. Twenty-two patients (52.4%) were male, and 20 patients (47.6%) were female. The patients aged from 3 to 15 years, with the mean age of (9.5±2.3) years. There were 35 bilateral patients and 7 unilateral patients. Twenty-seven patients (64.3%) had prodromal symptoms before onset. All patients underwent visual function and imaging tests, such as best corrected visual acuity (BCVA), fundus photography, visual evoked potential (VEP), visual field, MRI. The patients were tested for serum levels of antibodies for aquaporin 4 (AQP4) and myelin oligodendrocyte glycoprotein (MOG) with a cell-based assay. All patients were received corticosteroid therapy. The mean follow-up was (1.17±0.42) years. The children who had coordination ability and with BCVA≥0.3 were received examination of Humphery automatic perimeter. Data were collected on the age, gender, clinical features, neuroimaging, serological specific antibodies, treatment and vision prognosis. Results 23.8% of the children were bilateral optic neuritis in onset stages. 64.2% were recurrent optic neuritis and 83.3% exhibited bilateral diseases eventually. BCVA had decreased to ≤0.1 in 87.0%% eyes and disc swelling was observed in 77.9% eyes during the onset stages. All eyes had visual field defects and abnormal VEP exam results, with delayed latency of P100 and P2, and varying degrees of amplitude reduction. Serum AQP4 antibody and MOG antibody were tested by cell-based assay, 2/42 children (4.7%) were positive for AQP4 antibody and 5/24 children (20.8%) were positive for MOG antibody. All of anti-AQP4+ and anti- MOG+ cases relapsed. All children underwent orbital magnetic resonance imaging (MRI), 40 cases (95.2%) showed demyelination features of optic nerve, and 5 cases (11.9%) showed long segments lesion (more than 1/2 length of the optic nerve). There were 2 anti-AQP4+ cases and 3 anti- MOG+ cases from the 5 cases with long segments lesion. MRI also showed brain demyelinating lesions in 4 children (3 of them were anti- MOG+) or spinal cord demyelinating lesions in 3 children (2 of them were anti- MOG+). After treatment with glucocorticoid, visual acuity improved in all eyes, of which 84.4% with BCVA≥0.5. Forty-eight eyes of 26 children accept dynamic visual field during the course of treatment, showed the vision abnormalities associated with optic nerve damage. Conclusions Children under the age of 16 with DON can experience severe visual impairment, higher recurrence tendencies, and higher rate of disc involvement, but good response to glucocorticoid therapy. AQP4 or MOG antibodies positive might be concurrent with brain and (or) spinal cord demyelinating lesions and indicated a poorer prognosis.