Xiong Hui, Zhang Yue-hua, Zhou Rui, Bao Xin-hua, Jiang Yu-wu, Wu Ye, Wang Shuang, Chang Xing-zhi, Fu Shang-qin, Qin Jiong, Wu Xi-ru
Department of Pediatrics, First Hospital, Peking University, Beijing 100034, China.
Zhonghua Er Ke Za Zhi. 2007 Aug;45(8):568-73.
Multiple sclerosis is a demyelinating disease frequently showing a relapsing-remitting disease course. Clinical manifestations of 25 inpatients with MS were summarized and analyzed so that the clinical features and therapeutic approaches to childhood multiple sclerosis (MS) were investigated in order to improve its diagnosis and management.
Clinical features and information during following-up of 25 cases with MS from June 1993 to May 2006 were collected and analyzed.
Among the 25 cases, 16 were female and the F:M ratio was 1.78:1. The relapsing-remitting type was seen in 21 cases, the secondary progressive MS in 3 cases and the classification was impossible in one case. The mean age of onset was 6.7 years (2-12) with various initial symptoms including visual loss (11 cases), cortical symptoms (8 cases with seizures, consciousness disturbance, aphasia and apraxia, etc.), myeleterosis (3 cases), symptoms of brainstem (2 cases) and cerebellar ataxia (1 case). Fever was present in 10 cases at the onset. Nine cases were monosymptomatic, while the other 16 had multiple symptoms. Visual loss occurred in 19 cases during the course of MS and 22 were found to have abnormal visual evoked potential (88%). The mean course of disease was 8.5 years (1.2-17.2) and 0-4 times of recurrences (0 means no new clinical attack occurred during following-up period).
MS is increasingly recognized as a disease affecting children though it is uncommon. Childhood MS possesses some manifestations different from those of adults. There was a female predominance. The most common finding at the onset of disease was optic neuritis. Other features include acute onset and shorter course of disease. Atypical demyelinating symptoms were often seen. White matter lesions on MRI are required for the diagnosis. CSF oligoclonal bands could be found less commonly than in adults. Neurological sequelae were less often seen than in adults MS even though optic nerve atrophy and visual loss were relatively common. Steroid and IVIG are effective in acute period treatment.
多发性硬化症是一种脱髓鞘疾病,常表现为复发-缓解型病程。总结并分析25例多发性硬化症住院患者的临床表现,以研究儿童多发性硬化症(MS)的临床特征及治疗方法,从而改善其诊断与管理。
收集并分析1993年6月至2006年5月期间25例多发性硬化症患者的临床特征及随访信息。
25例患者中,女性16例,男女比例为1.78:1。复发-缓解型21例,继发进展型3例,1例无法分类。平均发病年龄为6.7岁(2 - 12岁),首发症状多样,包括视力丧失(11例)、皮质症状(8例,表现为癫痫发作、意识障碍、失语和失用症等)、脊髓症状(3例)、脑干症状(2例)和小脑共济失调(1例)。10例患者起病时有发热。9例为单症状,其余16例有多种症状。病程中19例出现视力丧失,22例视觉诱发电位异常(88%)。平均病程为8.5年(1.2 - 17.2年),复发0 - 4次(0表示随访期间无新的临床发作)。
尽管多发性硬化症在儿童中并不常见,但越来越被认为是一种影响儿童的疾病。儿童多发性硬化症具有一些与成人不同的表现。女性居多。疾病发作时最常见的表现是视神经炎。其他特征包括起病急、病程短。常可见非典型脱髓鞘症状。MRI上的白质病变是诊断所必需的。脑脊液寡克隆带的发现率可能低于成人。尽管视神经萎缩和视力丧失相对常见,但神经后遗症的发生率低于成人多发性硬化症。类固醇和静脉注射免疫球蛋白在急性期治疗中有效。