發(fā)布時(shí)間：2018-05-09 05:57

  本文選題：兒童 + 多發(fā)性硬化��； 參考：《中國(guó)實(shí)用兒科雜志》2016年01期

【摘要】：目的探討兒童多發(fā)性硬化(multiple sclerosis,MS)臨床特點(diǎn)、治療及預(yù)后,以提高其診治水平。方法回顧性分析1993年11月至2014年12月北京大學(xué)第一醫(yī)院兒科84例住院MS患兒臨床資料及隨訪(fǎng)情況進(jìn)行總結(jié)。結(jié)果 84例主要臨床表現(xiàn)為視力下降、無(wú)力、頭痛、發(fā)熱、腦病癥狀、驚厥。腦脊液寡克隆區(qū)帶陽(yáng)性率為28.2%,抗髓鞘少突膠質(zhì)細(xì)胞糖蛋白抗體陽(yáng)性率為32.7%。頭顱磁共振成像(MRI)示92.9%存在皮質(zhì)下白質(zhì)脫髓鞘、86.9%存在腦室旁白質(zhì)病變、47.6%小腦病變、36.9%腦干病變、29.8%基底節(jié)病變、21.4%丘腦病變、16.7%胼胝體病變。急性期均給予糖皮質(zhì)激素治療,53例合用大劑量丙種球蛋白,69例首次發(fā)作治療后完全緩解。隨訪(fǎng)0.7~22.0年,平均復(fù)發(fā)次數(shù)(4.71±3.18)次,平均年復(fù)發(fā)(0.65±0.52)次/年,61例在首次發(fā)病1年內(nèi)復(fù)發(fā)。57例隨訪(fǎng)至2014年12月,12.3%有運(yùn)動(dòng)異常,29.8%有視力下降,39.6%有學(xué)習(xí)障礙。結(jié)論 10歲內(nèi)發(fā)病的MS并不少見(jiàn),常以視力下降、無(wú)力、頭痛、發(fā)熱、腦病、驚厥為首發(fā)癥狀;腦脊液寡克隆區(qū)帶陽(yáng)性率低;MRI顯示皮質(zhì)下、腦室旁白質(zhì)病變多見(jiàn);急性期糖皮質(zhì)激素治療有效,多在1年內(nèi)復(fù)發(fā),疾病修正治療(DMT)可延長(zhǎng)復(fù)發(fā)間隔,視力下降與學(xué)習(xí)障礙為其常見(jiàn)的后遺癥。
[Abstract]:Objective to investigate the clinical features, treatment and prognosis of multiple sclerosis in children. Methods from November 1993 to December 2014, the clinical data and follow-up data of 84 children with MS in pediatrics of Peking University first Hospital were analyzed retrospectively. Results the main clinical manifestations of 84 cases were visual acuity, weakness, headache, fever, encephalopathy and convulsion. The positive rate of oligoclonal bands in cerebrospinal fluid and anti-myelin oligodendrocyte glycoprotein antibody was 28.2and 32.7g respectively. Magnetic resonance imaging (MRI) showed that 92.9% had subcortical white matter demyelination and 86.9% had ventricular collateral leukoencephalopathy 47.6% cerebellar lesions and 29.8% basal ganglia lesions and 21.4% thalamic lesions and 16.7% corpus callosum lesions. In the acute phase, 53 cases were treated with glucocorticoid and 69 cases with high dose gamma globulin were completely relieved after the first attack treatment. During the follow-up period from 0.7 to 22.0 years, the average recurrence times were 4.71 鹵3.18 times, and the average annual recurrence frequency was 0.65 鹵0.52 times per year. 57 cases were followed up to 12.3% of the patients with motor abnormalities until December 2014. 39.6% of the patients had impaired visual acuity. Conclusion MS within the age of 10 years is not uncommon. The first symptoms are vision loss, weakness, headache, fever, encephalopathy, convulsion, and low positive rate of oligoclonal bands in cerebrospinal fluid (CSF). The treatment of glucocorticoid in acute stage was effective, most of them recurred within one year, the disease correction therapy could prolong the recurrence interval, the visual acuity and learning disability were the common sequelae.
【作者單位】： 北京大學(xué)第一醫(yī)院兒科;武漢市兒童醫(yī)院神經(jīng)內(nèi)科;
【分類(lèi)號(hào)】：R744.51

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