Zgorzalewicz Małgorzata, Michałowska-Wender Grazyna, Losy Jacek, Wender Mieczysław
Katedra i Klinika Neurologii Wieku Rozwojowego AM 60-355 Poznań, ul. Przybyszewskiego 49.
Przegl Lek. 2003;60 Suppl 1:1-4.
Multiple sclerosis (MS) in children and adolescents is a rare disease. In such cases additional evidence from visual evoked potential (VEP) recordings, cerebrospinal fluid (CSF) analysis may be helpful in attaining security about the definite diagnosis of this disease. The VEP assessment and the immunological investigation of CSF were performed in 42 patients with MS at the age of 4-22 years. The diagnosis of this disease was based on the clinical and MR findings according to the recommended criteria by Mc Donald and al. VEP recordings were performed with Multiliner equipment in accordance to the IFCN recommendations and CSF analysis including IgG index and the oligoclonal IgG bands by using isoelectric focusing. Abnormal VEP typical of MS (delayed but with well-preserved wave form) was observed in 100% of MS patients with optic neuritis (ON). Changes in VEP indicating clinically silent lesions in the visual pathway were found at the initial period of disease in 86% of patients in whom a diagnosis of clinically definite MS was established. The increased IgG index, testifying the intrathecal IgG synthesis was found only in 52% cases, whereas the oligoclonal IgG bands were visible in 50% of patients. This indicated the limited value of immunological studies of CSF in diagnosis of childhood and juvenile MS in contrast to VEP, which was the most helpful method especially in the detection of clinically silent lesions in the visual pathway. Nevertheless, only close and careful evaluation of the neurological symptoms together with paraclinical investigations may be fundamental for early diagnosis of the disease in young patients.
儿童和青少年多发性硬化症(MS)是一种罕见疾病。在这类病例中,视觉诱发电位(VEP)记录、脑脊液(CSF)分析等额外证据可能有助于明确诊断该疾病。对42例4至22岁的MS患者进行了VEP评估和CSF免疫学检查。该疾病的诊断依据是根据麦克唐纳等人推荐的标准所做的临床和磁共振成像(MR)检查结果。VEP记录使用多线设备按照国际临床神经生理联合会(IFCN)的建议进行,CSF分析包括通过等电聚焦检测IgG指数和寡克隆IgG带。100%患有视神经炎(ON)的MS患者观察到典型的MS异常VEP(潜伏期延长但波形保存良好)。在最终确诊为临床确诊MS的患者中,86%在疾病初期发现VEP变化,提示视觉通路存在临床无症状性病变。仅52%的病例发现IgG指数升高,证实鞘内IgG合成,而50%的患者可见寡克隆IgG带。这表明与VEP相比,CSF免疫学检查在儿童和青少年MS诊断中的价值有限,VEP是最有用的方法,尤其有助于检测视觉通路的临床无症状性病变。然而,只有密切仔细地评估神经症状并结合临床旁检查,才可能是早期诊断年轻患者该疾病的基础。