格林-巴利综合征的临床和腓肠神经活检病理特点  

Guilain┐Barr︺syndrome:aclinicalanalysisandpathologicalstudyon40casesofsuralnervebiopsy

作　　者：郭玉璞[1] 王翠娣[1] 高淑芳[1] 任海涛[1] 

机构地区：[1]中国医学科学院北京协和医院神经科

出　　处：《中华神经科杂志》1996年第4期225-229,共5页Chinese Journal of Neurology

摘　　要：为探讨格林－巴利综合征（ＧＢＳ）临床和神经活检病理特点，对４０例ＧＢＳ患者进行活检病理观察。４０例中急性起病２８例，亚急性和慢性起病各６例；单纯累及脊神经２２例，颅脊神经受累１４例，多颅神经麻痹２例和全植物神经功能不全２例。肌电图均为神经源性损害，ＣＳＦ蛋白细胞分离９１．９％，寡克隆重区带阳性６３．５％，ＩｇＧ合成率升高７５．０％。病理所见以脱髓鞘为主３８例，以轴索变性为主２例，脱髓鞘并发轻度轴索改变１３例。毛细血管通透性改变２５例和单核细胞增多１９例，葱头样肥大神经改变６例。对感觉障碍与神经活检病理特点相关性进行分析。结果显示：腓肠神经活检对ＧＢＳ诊断，特别对病程迁延和变异型诊断具有重要意义。针对近年报道轴索性ＧＢＳ、急性运动性索性神经病（ＡＭＡＮ），也就临床病理分型进行讨论。本组属经典型运动感觉型ＧＢＳ２２例，纯运动型７例，多颅神经麻痹型、纯感觉型和全植物神经功能不全各２例和Ｆｉｓｈｅｒ综合征１例。本组病理所见为感觉神经活检不能证明ＡＭＡＮ，但本组仅２例轴索变性，因此，我们认为轴索变性仍为少见的变异型。Theclinicalfeaturesandtheresultofsuralnervebiopsyof40casesofGuilain-Barrésyndrome(GBS)arereportedinthisarticle.Ofthe40cases,27weremales,13werefemales.Theiragesrangefrom14to77years.Thirty-sevenofthepatientswerebetween15～60years(92.5%).Theirpasthistorieswerewithsymptomsof“upperrespiratoryinfection”orcatchingcoldin25cases,withdiarheainthreecases,withotherilnessinninecases,andunknowninthreecases.Theclini-calfeaturesconsistedofacuteonsetin28cases,subacuteonsetinsixcasesandchroniconsetinsixcases.Therewerepurelyspinalnerveinvolvementin22cases,cranial-spinalnerveinvolvementin14cases,multicranialnerveparalysisintwocasesanddysautonomiaintwocases.Theelectrophysi-ologicalstudiesshowedneurogenicdamagesinalcases.CSFalbuminocytologicdissociationwasob-servedin91.9%ofthesecases.Therewereelevated24hourIgGsynthesisratein18of24cases(75.0%).CSFoligoclonalbandingwaspositivein19of30cases(63.7%).Thepathologicalfind-ings:inthe40suralnervebiopsycasesshowedthatdemyelinationwasthepredominantpathologicalchangein38cases,mildaxonaldegenerationwasalsosenin13ofthe38cases,prominentaxonaldegenerationwassenonlyintwocases,increasedmonocyteinfiltrationandvariedcapilaryperme-abilitywereobservedin19and25casesrespectively,andanonion-bulblikechangewasseeninsixcases.WestudiedthecorelationbetweenparesthesiaandthepathologicalfindingsofsuralnervebiopsyandconcludedthatthesuralnervebiopsywasveryusefulinthediagnosisofGBSanditsvari-ant.AstheaxonalGBS(AMAN)wasreportedinrecentyears,theclinico-pathologicaltypingwasalsodiscussedinthisarticle.Ofthe40cases,therearesensori-motorGBSin20cases,puremotorGBSin7cases,puresensoryGBSin2cases,multiplecranialnerveparalysisintwocases,pandysautonomiaintwocasesandFishersyndromeinonecases.ThepathologicalfindingsinthisgrouparebasedonthesensorynervebiopsywhichcannotbeusedinthediagnosisofAMAN,butinthe40cases.OfGBS,onlytwocasesofaxonaldegenerationwereobserved,soweconsideredthattheaxonaldegenerationwouldbeararevariantofGBS.

关 键 词：G-B综合征 临床 腓肠神经 活检 

分 类 号：R745.43[医药卫生—神经病学与精神病学]