本文選題：Miller-Fisher綜合征　切入點(diǎn)：Bickerstaff腦炎　出處：《臨床兒科雜志》2017年06期

【摘要】：目的探討兒童Miller-Fisher綜合征(MFS)與Bickerstaff腦炎(BBE)、典型吉蘭-巴雷綜合征(GBS),吉蘭-巴雷變異型即咽-頸-臂吉蘭-巴雷綜合征(PCB-GBS)重疊的概率和時(shí)間。方法回顧分析確診的128例MFS患兒的臨床資料。結(jié)果 128例MFS患兒中,60例為單純MFS(眼肌麻痹、共濟(jì)失調(diào)、反射減弱或消失,不伴有肢體無(wú)力和嗜睡,實(shí)驗(yàn)室檢查提示有腦脊液蛋白-細(xì)胞分離和/或血清抗-GQ1b抗體陽(yáng)性),28例發(fā)展為MFS/PCB-GBS(符合MFS診斷標(biāo)準(zhǔn),并伴有咽部、頸部和上肢無(wú)力,上肢反射減弱或消失,不伴下肢無(wú)力),22例發(fā)展為MFS/GBS(符合MFS診斷標(biāo)準(zhǔn),并伴有四肢無(wú)力),18例發(fā)展為MFS/BBE(符合MFS診斷標(biāo)準(zhǔn),并伴有嗜睡、椎體束征陽(yáng)性)。四組患兒發(fā)病年齡、起病至開(kāi)始治療時(shí)間、Hughes功能分級(jí)評(píng)分以及有前驅(qū)感染病史、血清抗-GQ1b抗體陽(yáng)性比例、腦脊液蛋白-細(xì)胞分離比例均無(wú)統(tǒng)計(jì)學(xué)意義(P0.05)。MFS患兒發(fā)展為BBE、PCB-GBS、GBS的時(shí)間在起病后10天內(nèi)。結(jié)論 MFS患兒重疊PCB-GBS、BBE、GBS的概率為50%,一般發(fā)生在癥狀起始后10天內(nèi),臨床醫(yī)師需要注意時(shí)間窗,合理調(diào)整用藥。
[Abstract]:Objective to investigate the probability and time of overlap between Miller-Fisher syndrome in children and Bickerstaff encephalitis, typical Guillain-Barre syndrome and Guillain-Barre syndrome.Methods the clinical data of 128 children with MFS were retrospectively analyzed.Results among the 128 cases of MFS, 60 cases were simple MFSs (ophthalmoplegia, ataxia, weakening or disappearance of reflex, no limb weakness and somnolence).Laboratory examination showed that 28 patients with cerebrospinal fluid protein-cell separation and / or serum anti-GQ1b antibody positive had developed into MFS / PCB-GBS (in accordance with MFS diagnostic criteria, accompanied by weakness of pharynx, neck and upper limb, weakening or disappearance of upper limb reflex).Twenty-two patients without lower limb weakness developed into MFS / GBS (in accordance with MFS diagnostic criteria and 18 with limb weakness) (MFS diagnostic criteria, somnolence and spinal tract sign positive).In the four groups, the age of onset, the time from the onset of the disease to the beginning of treatment, the score of Hughes functional grading, the history of pre-infection, and the positive rate of anti-GQ1b antibody in serum were observed in the four groups.Conclusion the probability of overlapping PCB-GBS BBEBS in children with MFS is 50, which usually occurs within 10 days after the onset of symptoms. Clinicians should pay attention to the time window and adjust the use of drugs reasonably.
【作者單位】： 華中科技大學(xué)同濟(jì)醫(yī)學(xué)院附屬武漢兒童醫(yī)院;湖北省新華醫(yī)院神經(jīng)內(nèi)科;
【基金】：武漢市科技創(chuàng)新平臺(tái)——兒童神經(jīng)疾病臨床醫(yī)學(xué)研究中心資助項(xiàng)目(No.武科計(jì)2014-160號(hào))
【分類號(hào)】：R745.43
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本文編號(hào)：1706373