儿童多发性硬化的临床特点及MRI特征

目的 探讨中国儿童多发性硬化(MS)的临床孤立综合征( CIS)和复发时的临床及MRI特征.方法 回顾性分析16例MS患儿的首次发作及复发时临床及影像学资料.随访时间4个月至7年,期间患儿复发次数为1～5次.由1名儿科神经医师对CIS及复发的临床表现进行了归类总结.由1名资深神经影像学医师对患儿CIS及复发的头颅MRI表现进行分析,内容包括病灶的位置、大小、分布.病灶位置的分析包括皮层、皮层下白质、中央白质、脑室旁白质、深部灰质核团以及脑干和小脑.结果 (1)临床表现:儿童MS发病急,CIS以皮层症状及视觉障碍表现多见,14例1年以内复发,复发时皮层症状减少,而视觉症状仍较多,随访时康复良好.(2)颅脑MRI表现:CIS时,13例出现皮层下白质病灶,且大片融合,与中央白质病灶相连,好发部位依次为额、顶叶.皮层9例受累.10例可见中央白质病灶.6例可见脑室旁白质小病灶.4例可见对称性深部灰质核团病灶.5例可见脑干病灶.3例可见小脑病灶.3例可见视束或视神经肿胀、增粗.2例可见锥体束异常信号.1例可见胼胝体病灶.复发时,12例可见皮层下病灶,较CIS时数量增多,以小病灶为主.9例可见中央白质病灶,病灶大小较前减小.8例可见脑室旁病灶,病灶数量较CIS时增多.仅有2例出现皮层病灶.5例出现小脑病灶.4例可见脑干病灶.6例可见锥体束病灶,发生率较CIS时明显增多,且出现“轨道征”.结论 儿童MS的MRI表现具有一定特征,CIS时额、顶叶皮层下白质病灶融合成大片并常累及中央区白质,复发时有时可见“轨道征”,结合临床可以提高对儿童MS诊断的正确性.

Clinical and MRI features in pediatric multiple sclerosis

Objective To investigate the clinical and MRI features of multiple sclerosis in children,including the clinically isolated syndrome (CIS) and relapse.Methods In total,16 cases of pediatric multiple sclerosis were included in this study.Of them,11 patients were female and 5 were male,with the mean onset age of 10.1 years.They were followed up for 4 months to 7 years and found to have 1-5 relapses.The clinical manifestations of CIS and relapse were analyzed by a pediatric neurologist.An experienced neuroradiologist reviewed the MRI images of CIS and relapse.Information on the location,size,and pattern of the lesions was gathered.The location of lesions included subcortical,central,and periventricular white matter,cortex,deep gray matter,brain stem,and cerebellum.Results CIS episode presented acute onsets in 13/16 cases,with symptoms of cortice in 10 cases and visual impairment in 6 cases.Relapse occurred in 14/16 cases within one year.The incidence of symptoms of cortice was less frequent and severe in the second episode of MS,whereas the visual impairment had a high incidence.All patients had full recovery after the last episode.MRI of CIS showed confluent subcortical white matter lesions in 13/16 cases,abutting on central white matter lesions.The most frequently involved brain part was the frontal lobe,followed by the parietal lobe.Cortical involvement was observed in 9/16 cases.In 6 cases,periventricular white matter lesions were detected.Bilateral deep gray matter was abnormal in 4 cases.Other abnormalities included brain stem lesions in 5 cases,cerebellum lesions in 3 cases,optic nerve involvement in 3 cases,and pyramidal tract lesions in 2 cases.MRI of relapse revealed more small lesions in the subcortial and periventricular white matter in the patients.In the second episode,only 2 cases presented cortical involvement.Lesions were found in the brain stem in 4 cases and in the cerebellum in 5 cases.Pyramidal tract involvement was identified in 6 cases,serving as “railway track sign”.Conclusions CIS in children is characterized by large confluent lesions in subcortical white matter coalescing with central white matter.MRI of relapse showed distinctive “railway track sign”.It is important to integrate MRI and clinical features in the diagnosis of pediatric multiple sclerosis.