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目的探讨儿童多发性硬化(MS)的临床特点及治疗,以提高其诊治水平。方法对1993年6月至2006年5月我科收治的25例 MS 患儿的临床资料及随访情况进行分析。结果本组25例,女性16例,男性9例,男:女=1:1.78。其中复发-缓解型21例,继发进展型3例,1例未明确分型。平均起病年龄6.7岁(2～12岁)。以视力障碍为首发症状者11例;以皮层症状为主者8例,包括惊厥、意识障碍、失语、失用等;脊髓病变3例;脑干病变2例;小脑性共济失调1例。起病时有发热者10例。表现为神经系统单一部位病变者9例,多部位者16例。病程中有视力障碍者19例,视诱发电位异常22例(88%)。平均病程8.5年(1.2～17.2年),复发次数0～4次(0次者为随访期间无新的临床发作)。结论 MS 在儿童期少见,有其不同于成人的特点。女性多于男性,以视神经炎多见,起病急且病程短,常见不典型脱髓鞘疾病症状。头颅 MRI 提示白质病变是不可缺少的诊断标准之一。脑脊液寡克隆区带阳性率低于成人,与成人 MS 相比较少遗留神经系统后遗症,其中以视神经萎缩,视力障碍为常见。急性期激素和 IVIG 治疗有效。
Objective To investigate the clinical features and treatment of childhood multiple sclerosis (MS) in order to improve its diagnosis and treatment. Methods The clinical data and follow-up of 25 MS children admitted to our department from June 1993 to May 2006 were analyzed. Results The group of 25 cases, 16 females, 9 males, male: female = 1: 1.78. Among them, 21 cases were recurrence-remission type, 3 cases were secondary progression type, and 1 case was not definite type. The average age of onset of 6.7 years (2 to 12 years old). Among them, 11 cases were with visual impairment as their first symptom; 8 cases were mainly cortical symptoms including convulsions, disturbance of consciousness, aphasia and loss of function; 3 cases of spinal cord lesions; 2 cases of brain stem lesions; 1 case of cerebellar ataxia. 10 cases of fever when onset. Nine patients showed single-site nervous system diseases, multi-site in 16 cases. During the course of disease, there were 19 cases of visual impairment and 22 cases of abnormal visual evoked potentials (88%). The average course of disease was 8.5 years (1.2 to 17.2 years), and the number of recurrences was 0 to 4 times (0 times as no new clinical seizure during follow-up). Conclusion MS is rare in childhood and has characteristics different from those of adults. More women than men, more common in optic neuritis, acute onset and short course of disease, common atypical demyelinating disease symptoms. Head MRI suggests that white matter lesions are one of the indispensable diagnostic criteria. Cerebrospinal fluid oligoclonal band positive rate lower than adults, compared with adult MS less leftovers neurological sequelae, of which optic atrophy, visual impairment as common. Acute hormones and IVIG treatment effective.