Sánchez-Calderón M, de Santos T, Martín S, Angulo T, Careaga J, Campos-Castelló J
Departamento de Pediatria, Hospital Clincio San Carlos, Madrid, España.
Rev Neurol. 1998 Aug;27(156):237-41.
Multiple sclerosis (MS) is infrequent in childhood (0.3-2% of all cases of MS). At the present time more and more paediatric patients are being described. In this paper we describe our experience.
We review seven patients diagnosed as having MS before the age of 16, between 1984 and 1996. We have recorded: age, sex, personal and family history, form of onset and clinical course. CSF, neurophysiological and neuroimaging tests, treatment and diagnostic category according to the criteria of Poser.
Four boys and three girls aged between three and thirteen years at the onset of the disorder. In one case there was a family history of MS. The most frequent form of onset was hemiparesia, followed by oculomotor paralysis and cerebellar disorders. Six cases followed a recurrent-remittent course and one followed a secondarily progressive course. There were oligoclonal bands (OGB) in the CSF in three cases. Visual evoked potentials (VEP) were abnormal in six cases. Magnetic resonance (RM) was a great help in diagnosis and in six cases was very informative. Six patients were treated with corticosteroids during the acute phase and two with long-term azathioprine.
The commonest form of presentation, hemiparesia, makes the differential diagnosis with acute hemiplegia of childhood obligatory. Neurophysiological techniques, especially the VEP are very useful for initial assessment. RM is the most sensitive method, although it is not specific for diagnosis. The average follow-up period (4.5 years) is too short to determine the prognosis.
多发性硬化症(MS)在儿童期并不常见(占所有MS病例的0.3 - 2%)。目前,越来越多的儿科患者被报道。在本文中,我们描述了我们的经验。
我们回顾了1984年至1996年间16岁之前被诊断为MS的7例患者。我们记录了:年龄、性别、个人及家族史、起病形式和临床病程。脑脊液、神经生理学和神经影像学检查、治疗以及根据波塞尔标准的诊断类别。
疾病发作时年龄在3岁至13岁之间的有4名男孩和3名女孩。1例有MS家族史。最常见的起病形式是偏瘫,其次是动眼神经麻痹和小脑疾病。6例呈复发 - 缓解病程,1例呈继发进展病程。3例脑脊液中有寡克隆带(OGB)。6例视觉诱发电位(VEP)异常。磁共振成像(MRI)对诊断有很大帮助,6例提供了非常有价值的信息。6例患者在急性期接受了皮质类固醇治疗,2例接受了长期硫唑嘌呤治疗。
最常见的表现形式偏瘫,使得与儿童急性偏瘫的鉴别诊断成为必要。神经生理学技术,尤其是VEP,对初始评估非常有用。MRI是最敏感的方法,尽管它对诊断不具有特异性。平均随访期(4.5年)太短,无法确定预后。