Department of Pediatric Neurology, Dr. Behçet Uz Children's Hospital, İzmir, Turkey.
Department of Pediatric Neurology, Hacettepe University Faculty of Medicine, Ankara, Turkey.
Eur J Paediatr Neurol. 2017 Nov;21(6):864-872. doi: 10.1016/j.ejpn.2017.06.004. Epub 2017 Jun 29.
To document the clinical and paraclinical features of pediatric multiple sclerosis (MS) in Turkey.
Data of MS patients with onset before age 18 years (n = 193) were collected from 27 pediatric neurology centers throughout Turkey. Earlier-onset (<12 years) and later-onset (≥12 years) groups were compared.
There were 123 (63.7%) girls and 70 (36.3%) boys aged 4-17 years, median 14 years at disease onset. Family history of MS was 6.5%. The first presentation was polysymptomatic in 55.4% of patients, with brainstem syndromes (50.3%), sensory disturbances (44%), motor symptoms (33.2%), and optic neuritis (26.4%) as common initial manifestations. Nineteen children had facial paralysis and 10 had epileptic seizures at first attack; 21 (11%) were initially diagnosed with acute disseminated encephalomyelitis (ADEM). Oligoclonal bands were identified in 68% of patients. Magnetic resonance imaging revealed periventricular (96%), cortical/juxtacortical (64.2%), brainstem (63%), cerebellum (51.4%), and spinal cord (67%) involvement. Visual evoked potentials (VEP) were abnormal in 52%; serum 25-hydroxyvitamin D levels were low in 68.5% of patients. The earlier-onset group had a higher rate of infection/vaccination preceding initial attack, initial diagnosis of ADEM, longer interval between first 2 attacks, and more disability accumulating in the first 3 years of the disease.
Brainstem and cerebellum are common sites of clinical and radiological involvement in pediatric-onset MS. VEP abnormalities are frequent even in patients without history of optic neuropathy. Vitamin D status does not appear to affect the course in early disease. MS beginning before 12 years of age has certain characteristics in history and course.
记录土耳其儿科多发性硬化症(MS)的临床和辅助检查特征。
从土耳其 27 家儿科神经病学中心收集了发病年龄在 18 岁以下(n=193)的 MS 患者的数据。比较了早发(<12 岁)和晚发(≥12 岁)组。
患者年龄为 4-17 岁,中位数为 14 岁,其中 123 例(63.7%)为女孩,70 例(36.3%)为男孩。MS 家族史为 6.5%。55.4%的患者首发症状为多系统症状,常见的初始表现为脑干综合征(50.3%)、感觉障碍(44%)、运动症状(33.2%)和视神经炎(26.4%)。19 例患儿首发时有面瘫,10 例有癫痫发作,21 例(11%)最初被诊断为急性播散性脑脊髓炎(ADEM)。68%的患者检测到寡克隆带。磁共振成像显示脑室周围(96%)、皮质/皮质下(64.2%)、脑干(63%)、小脑(51.4%)和脊髓(67%)受累。52%的患者视觉诱发电位(VEP)异常;68.5%的患者血清 25-羟维生素 D 水平较低。早发组首发前感染/疫苗接种率较高、首发诊断为 ADEM、首次发作至第 2 次发作间隔时间较长、疾病前 3 年内残疾累积较多。
脑干和小脑是儿童发病 MS 常见的临床和影像学受累部位。即使在没有视神经病变病史的患者中,VEP 异常也很常见。维生素 D 状态似乎不会影响早期疾病的病程。12 岁之前发病的 MS 在病史和病程上具有某些特征。