Hamdy Sherif M, Abdel-Naseer Maged, Shalaby Nevin M, Elmazny Alaa, Girgis Marian, Nada Mona A, Hassan Amr, Mourad Husam S, Hegazy Mohamed I, Abdelalim Ahmed, Kishk Nirmeen A, Abokrysha Noha T, Genedy Shaimaa A, Essawy Ehab A, Shehata Hatem S
Neurology Department, Cairo University, Cairo, Egypt.
Pediatric Department, Cairo University, Cairo, Egypt.
Neuropsychiatr Dis Treat. 2018 Feb 23;14:631-640. doi: 10.2147/NDT.S160060. eCollection 2018.
Although the frequency of pediatric-onset multiple sclerosis (POMS) has increased in recent decades, it is still highly uncommon, which creates a need for the involvement of more registries from various clinical centers.
To characterize the demographic, clinical, and paraclinical features of Egyptian patients with POMS.
A retrospective chart review study was undertaken on 237 Egyptian patients with demyelinating events which started before the age of 18 years who attended one of five tertiary referral centers in Cairo, Egypt.
Multiple sclerosis was diagnosed in 186 patients, 47 (25.27%) patients had disease onset before the age of 12 years; "early-onset pediatric multiple sclerosis (EOPMS)". The mean age of disease onset was (14.13±2.49 years), with a female:male ratio of 1.62:1, none of the enrolled patients had a primary progressive course (PPMS), whereas 10 patients (5.38%) had a secondary progressive form. Approximately two-thirds of the patients had monofocal disease onset, and less than 10% presented with encephalopathy; most of them had EOPMS. Motor weakness was the presenting symptom in half of the patients, whereas cerebellar presentation was detected in 34.95%, mainly in EOPMS. Seizures (not related to encephalopathy) were more frequent in those with EOPMS. Initial brain magnetic resonance images were positive in all patients, with detected atypical lesions in 29.03%, enhanced lesions in 35.48%, black holes in 13.98%, and infratentorial in 34.41%. Cervical cord involvement was found in 68.28%. More than two-thirds of the patients received either immunomodulatory or immunosuppressant (IS) treatment throughout their disease course, and about half of them received their treatment within the first year from symptoms onset, with a more favorable outcome, and patients with highly active disease received natalizumab, fingolimod, or other IS.
The results from this registry - the largest for MS in the Arab region to date - are comparable to other registries. Immunomodulatory therapies in POMS are well tolerated and efficacious and they can improve the long-term outcome in children.
尽管近几十年来儿童期多发性硬化症（POMS）的发病率有所上升，但仍然极为罕见，这就需要更多不同临床中心的登记处参与其中。
描述埃及POMS患者的人口统计学、临床和辅助检查特征。
对237例在18岁之前出现脱髓鞘事件的埃及患者进行了一项回顾性病历审查研究，这些患者就诊于埃及开罗的五家三级转诊中心之一。
186例患者被诊断为多发性硬化症，47例（25.27%）患者在12岁之前发病，即“早发性儿童多发性硬化症（EOPMS）”。疾病发病的平均年龄为（14.13±2.49岁），女性与男性的比例为1.62:1，所有纳入患者均无原发进展型病程（PPMS），而10例患者（5.38%）有继发进展型。约三分之二的患者为单病灶发病，不到10%的患者表现为脑病，其中大多数为EOPMS。一半患者以运动无力为首发症状，34.95%的患者有小脑表现，主要见于EOPMS。癫痫发作（与脑病无关）在EOPMS患者中更为常见。所有患者的初始脑磁共振成像均为阳性，29.03%检测到非典型病变，35.48%有强化病变，13.98%有黑洞，34.41%有幕下病变。68.28%的患者发现颈髓受累。超过三分之二的患者在整个病程中接受了免疫调节或免疫抑制（IS）治疗，约一半患者在症状出现后的第一年内接受治疗，预后较好，疾病高度活跃的患者接受了那他珠单抗、芬戈莫德或其他IS治疗。
该登记处（迄今为止阿拉伯地区最大的多发性硬化症登记处）的结果与其他登记处相当。POMS中的免疫调节疗法耐受性良好且有效，可改善儿童的长期预后。
