神經(jīng)節(jié)苷脂治療后吉蘭巴雷綜合征參考幻燈片.ppt

1、,1,Guillain-Barre syndrome (GBS),AIDP AMAN AMSAN MFS,2,Ganglioside,神經(jīng)節(jié)苷脂廣泛分布于全身各組織細(xì)胞的外表面,在神經(jīng)系統(tǒng)中含量豐富,在周圍神經(jīng)系統(tǒng)中主要存在于軸索，而在膠質(zhì)和髓鞘中濃度較低 根據(jù)其糖基結(jié)合唾液酸分子的數(shù)目及部位分為GM、GD、GT 及GQ,3,Pathology,AMAN and AMASN are associated with Campylobacter jejuni enteritisand antibodies against gangliosides,4,chemical structure of g

2、angliosides,5,不同種類的神經(jīng)節(jié)苷脂在周圍神經(jīng)系統(tǒng)的分布具有各自不同的特點,GM1除廣泛分布于周圍神經(jīng)的軸索，還分布于郎飛氏節(jié)及施萬細(xì)胞近軸索側(cè)細(xì)胞膜，在運動神經(jīng)髓鞘內(nèi)GM1的含量多于其在感覺神經(jīng)髓鞘內(nèi)的含量 GD1b在后根神經(jīng)節(jié)大感覺神經(jīng)元分布較多 GQ1b在動眼、滑車、外展神經(jīng)的髓鞘中含量高于其他顱神經(jīng),6,特定抗神經(jīng)節(jié)苷脂抗體和具有某些特異臨床特點的神經(jīng)系統(tǒng)疾病相關(guān),抗GM1抗體見于急性運動軸索性神經(jīng)病（AMAN）及脫髓鞘性GBS，IgM類GM1抗體見于多灶性運動神經(jīng)?。∕MN），癥狀學(xué)研究提示GM1抗體陽性的患者更多出現(xiàn)肢體無力，而感覺障礙相對較少 抗GD1b抗體與感覺性共

3、濟失調(diào)等感覺障礙為主的疾病相關(guān) GQ1b與Miller-Fisher 綜合征（MFS),Bickerstaff腦干腦炎（BEE）等存在眼肌麻痹或球部肌肉受累的疾病相關(guān),7,After exogenous gangliosides injection,High titers of anti-GM1 antibodies were found in patients who developed GBS following exogenous gangliosides injection , leading to the suspicion that exogenous gangliosides m

4、ight be foreign to humans and may act as an immunogenic agent.,8,GBS following intravenous use of gangliosides in Europe severaldecades ago led to its withdrawal from European market,Figueras A, Morales-Olivas FJ, Capella D, Palop V, Laporte JR (1992) Bovinegangliosides and acute motor polyneuropath

5、y. BMJ 305:13301331.,9,We identified 7 patients who developed GBS after intravenous use of gangliosides,Department of Neurology of the First Hospital of Jilin University 2013 Gangliosides as an exclusive component or part of a compoundhave never been used in our department and all the enrolled patie

6、nts were referred to our department from other departments or from other hospitals.,10,11,Grouping,Enrolled subjects were divided into the ganglioside+ group(ganglioside-associated) and the ganglioside- group (non-ganglioside-associated) according to whether they received exogenousgangliosides befor

7、e disease onset.,12,13,14,Evaluation of clinical severity and functional impairment,Motor function deficits of patients were scored by the Hughes Functional Grading Scale(HFGS) score Neurologic function was also evaluated by using the Medical Research Council (MRC) sum score of six bilateral muscles

8、 in arms and legs, ranging from 0 (tetraparalytic) to 60 (normal strength),15,16,17,The association between exogenous gangliosides and GBS,high titers of anti-GM1 antibodies were found in some of the patients who developed GBS after receiving a ganglioside therapy, leading to the suspicion that exog

9、enous gangliosides might be foreign to humans and might be neuritogenic in humans The association between exogenous gangliosides and GBS wasfurther evidenced by the positivity of anti-GM1 and anti-GT1aantibodies in CSF and plasma,18,Sum,In sum, exogenous gangliosides may be associated withdevelopment of GBS due to incompletely recognized pathogenesis.Ganglioside-associ