婴儿和儿童期严重新皮质癫痫综合征的临床表现

该文献由北京癫痫病医院（）整理Infantsandchildrenwithsevereneocorticalepilepsysyndromeclinicalmanifestation(婴儿和儿童期严重新皮质癫痫综合征的临床表现)Typically,theinvolvementofchildreninthepriortotheonsetofnormal.Usuallywithfocalmotorseizures,partialstatusepilepticus,generalizedtonic-clonicseizures(GTCS)orwithautomatismincomplexpartialseizuresonset.WONcontinuousstatemayoccurin20%ofpatients.In50%ofcasesthebloodringattackintheprevious6months,withupperrespiratorytractinfections,otitismediaortonsillitishistory.典型的，受累儿童在发病前正常。通常以局灶性运动性发作、部分性癫痫持续状态、全面性强直阵挛发作〔GTCS)或不伴自动症的复杂部分性发作起病。WON持续状态可能出现于20%的患者中。50%的病例在血响发作前6个月左右，有上呼吸道感染、中耳炎或扁桃体炎史。Whenthedisease,focalmotorseizuresinvolvethedistalmuscles(athumb,finger,mouthoreyes),butastimeadvances,theaffectedareaspreadtoadjacentareas,havingawalkandamorediffuse,andsleepinthepersistenceof.Alongwiththeprogressof.Thereareavarietyofseizures,seizurefrequency,andgraduallyincreasedduringepisodesofprolonged,oftenaccompaniedbyseizuresafterhemiplegia.Partialepilepsytostatein60%patients,andcanlastforseveraldays,oftenmixedwithlateralitytonic-clonicseizures,butalsocandevelopasageneralizedtonic-clonicseizure.Appearsinepisodesafterinitialhemiparesis,shortduration,butgraduallydeveloppersistent.Inaddition,therearereportedintheliteratureinhistologicallyconfirmedKozhevvikovRssmussensyndromecases,butwithoutOARattack.发病时，局灶性运动性发作主要累及远端少数肌群(拇指、手指、嘴角或眼角)，但是随着时间进展，受累部位扩散至周围相邻区域，具有游走性和更加弥散，并且在睡眠中也持续存在。随着病程的进展。出现多种发作，并且发作频率逐渐增加、发作期延长，经常伴有发作后偏瘫。部分性癫痫待续状态见于60%的患者，并可以持续数天，经常混有偏侧强直一阵挛发作，也可发展为全面性强直一阵挛发作。出现于发作后的偏瘫，最初持续时间短暂，但逐步发展为持久性。另外，也有文献报进了经组织学确诊的Kozhevvikov一Rssmussen综合征病例，但是不伴有OAR发作。Theclinicalcourseisdividedintothreestages临床病程分三个阶段Thefirststage:thekeytosimplemotionorsomatosensoryasfeature.Thesituationisrelativelyraretopartialstatusepilepticus,notwithautomatismincomplexpartialseizuresorsecondaryGTCSonset,mayalsorepresenttheseizuretypecombination.Afterafewweeksormonths,graduallybecomefrequentepisodes.第一阶段:主要以简单运动或躯体感觉发作为特征。比较少见的情况是以部分性癫痫持续状态、不伴自动症的复杂部分性发作或继发性GTCS起病，也可能表现为这些发作类型的组合。经过数周或数月，发作逐渐地变得频繁。Secondstage:episodesofdeteriorationandtheintacthemisphereneurologicalsymptomsprogresstofeature.Thisphaseusuallybeginsabouttheoccurrenceofseizuresafter3months,butcanalsobedelayeduntilthediseasewasfirstsymptomsafter10years.Inthisphase,attacksbecomemorefrequent,theaffectedareaismoreextensive.Episodesoflongerduration.Atthesametime,asthediseaseprogressioninchildrenwithsharp,lastingpsychomotordefects,includingassociatedwithcognitiveandlanguageimpaired(aphasiaanddysarthria)ofhemiparesis,hemihypesthesia,hemianopia.Mostofthebraindamageoccurredin8aftera12month.第二阶段:以发作的恶化和受累半球的神经系统症状进展为特征。这一阶段通常大约开始于出现癫痫发作3个月后，但也可延迟至该病首次症状后10年之久。在此阶段，发作变得更加频繁，受累部位更加广泛。发作持续时间更长。同时，随着病情急剧进展，患儿出现持久的精神运动缺陷，包括伴有认知和语言受损(失语和构音障碍)的轻偏瘫、偏侧感觉减退、偏盲。大部分脑损害发生于起病后的8一12个月。Progressiveneurologicalandpsychomotorfunctiondegradation,eitherwithorwithoutdelusionseizuredeterioration,KoehevnikovisatypicalRssmusrxnsyndrome.进行性神经系统和精神运动功能退化，无论伴有或不伴有痴痫发作的恶化，是Koehevnikov一Rssmusrxn综合征的典型特征。Thethirdstage:thediseaseshowedthefrequencyofseizuresanddecreasedseverityofneurologicaldeficits,progressisslow,butremnantsinpatientswithseverecognitiveimpairment.第三阶段:该病表现出癫痫发作的频率和严重性降低，神经功能缺损的进展也减缓，但是患者遗留严重的神经认知功能损害。