癫痫的分类及外科治疗的术前评估.ppt

,*,单击此处编辑母版标题样式,单击此处编辑母版文本样式,第二级,第三级,第四级,第五级,癫痫分类及外科治疗的术前评估,北京天坛医院神经内科,丁 成 贇,一癫痫的定义,癫痫发作（,epileptic seizure,）癫痫发作是指大脑神经元异常和过度的超同步化放电所造成的临床现象。,癫痫（,epilepsy,）癫痫是一种脑部疾患，特点是持续存在能产生癫痫发作的易感性，并出现相应的神经生物学、认知、心理学以及社会等方面的后果。诊断癫痫至少需要一次以上的癫痫发作。（,2005,年国际抗癫痫联盟）,癫痫的定义,新的癫痫定义具有三个要素：,至少一次以上的癫痫发作史；,反复癫痫发作的倾向及易感性；,出现相应的神经生物学认知心理及社会等方面的障碍。,二 癫痫发作,癫痫及癫痫综合症 的分类,二 癫痫的分类,国际抗癫痫联盟（,ILAE,）在,1981,年提出的癫痫发作分类,ILAE 1989,年癫痫综合征的分类,2001,年,ILAE,提出了最新的,“,癫痫发作和癫痫诊断方案的建议,”,癫痫发作的分类,全面性发作（,generalized seizures,）,部分性发作（,partial seizures,）,难以分类的发作,特殊的发作形式或者类型,全面性发作,强直,-,阵挛性发作（,generalized tonic-clonic,seizure,）,失神发作（,absence seizure,）,强直发作,(tonic seizure),阵挛发作,(clonic,seizure),肌阵挛发作（,myoclonic,seizure,）,痉挛发作（,spasms,）,失张力发作（,atonic,seizure,）,部分性发作,简单部分性发作（,simple partial seizure,SPS,）,复杂部分性发作（,complex partial seizure,CPS,）,继发全面强直阵挛发作,(secondarily generalized tonic-clonic,seizure,SGTC),简单部分性发作,运动性发作,感觉性发作,自主神经性发作,精神性发作,运动性发作,仅为局灶性运动发作,杰克逊发作（,Jackson seizure,）,偏转性发作,姿势性发作,发音性发作,抑制性运动发作,失语性发作,简单部分性发作,运动性发作,感觉性发作,自主神经性发作,精神性发作,感觉性发作,躯体感觉性发作,视觉性发作,听觉性发作,嗅觉性发作,味觉性发作,眩晕性发作,简单部分性发作,运动性发作,感觉性发作,自主神经性发作,精神性发作,自主神经性发作,症状复杂多样：口角流涎、上腹部不适感或压迫感，,“,气往上冲,”,的感觉、肠鸣、呕吐、尿失禁、面色或口唇苍白或潮红、出汗、竖毛,极为少见，常常是继发或作为复杂部分性发作一部分,起源于岛叶、间脑及其周围（边缘系统等）,容易影响意识，继发复杂部分性发作,简单部分性发作,运动性发作,感觉性发作,自主神经性发作,精神性发作,精神性发作,发作性情感障碍,发作性记忆障碍,发作性认知障碍,发作性错觉,发作性结构性幻觉,部分性发作,简单部分性发作（,simple partial seizure,SPS,）,复杂部分性发作（,complex partial seizure,CPS,）,继发全面强直阵挛发作,(secondarily generalized tonic-clonic,seizure,SGTC),复杂部分性发作,仅表现为意识障碍,表现为意识障碍和自动症,常见的自动症包括：口咽自动症、姿势自动症、手部自动症、走动自动症、言语自动症,简单部分性发作演变为复杂部分性发作,起源：海马杏仁核（颞叶内侧）、额叶、颞叶外侧皮质,部分性发作,简单部分性发作（,simple partial seizure,SPS,）,复杂部分性发作（,complex partial seizure,CPS,）,继发全面强直阵挛发作,(secondarily generalized tonic-clonic,seizure,SGTC),继发全面强直阵挛发作,部分性发作继发全面强直阵挛发作仍属于部分性发作的范畴,鉴别：,有无,“,先兆,”,“,抽搐,”,的表现,“,失神,”,自动症,EEG,癫痫发作的分类,全面性发作（,generalized seizures,）,部分性发作（,partial seizures,）,难以分类的发作,特殊的发作形式或类型,因资料不全而不能分类的发作以及所描述的类型迄今尚无法归类者,癫痫发作的分类,全面性发作（,generalized seizures,）,部分性发作（,partial seizures,）,难以分类的发作,特殊的发作形式或者类型,特殊的发作形式或者类型,猝倒（,drop attack,）：表现为发作性的突发倒地，并不是一种具体的发作类型。,反射性发作（,reflex seizure,）：每次发作均为某种特定感觉刺激所诱发。发作符合癫痫发作的电生理和临床特征，但没有固定的发作形式。,癫痫综合征的定义,癫痫综合征：由一组体征和症状组成的特定的癫痫现象（发病年龄、发作类型、,EEG,病因）。,良性癫痫综合征：易于治疗或不需要治疗也能完全缓解，不留后遗症的癫痫综合征。,反射性癫痫综合征：全部癫痫性发作都是由一定的感觉刺激所诱发的综合征。,特发性癫痫综合征：除了癫痫，没有大脑结构性损伤和其他神经系统症状与体征的综合征。,症状性癫痫综合征：癫痫发作是由大脑一个或多个可证实的损伤引起的综合征。,癫痫综合征,良性家族性新生儿惊厥（,Benign familial neonatal convulsion,BFNC,）和良性新生儿惊厥（,Benign neonatal convulsion,BNC,）,早发性肌阵挛脑病（,early myoclonic,encephalopathy,）,大田原综合征（,Ohtahara,综合征）,癫痫综合征,良性婴儿肌阵挛癫痫（,benign myoclonic,epilepsy in infancy,）,婴儿严重肌阵挛癫痫（,Dravet,综合征）,婴儿痉挛（,West,综合征）,Lennox-Gastaut,综合征（,LGS,）,肌阵挛,-,站立不能性癫痫（,epilepsy with myoclonic astatic,seizures,）,癫痫综合征,失神癫痫,(absence epilepsy),儿童良性癫痫伴有中央颞部棘波（,benign childhood epilepsy with centrotemporal,spike,）,儿童良性枕叶癫痫（,benign childhood occipital epilepsy,）,获得性癫痫性失语,(acquired epileptic aphasia),慢波睡眠中持续棘慢复合波的癫痫（,ECSWS/ESESS,）,癫痫综合征,Rasmussen,综合征,青少年肌阵挛癫痫,(juvenile myoclonic,epilepsy),觉醒期全身强直阵挛发作的癫痫,肌阵挛失神癫痫,全面性癫痫伴热性惊厥附加症,癫痫综合征,颞叶癫痫,额叶癫痫,顶叶癫痫,枕叶癫痫,常染色体显性遗传夜发性额叶癫痫,家族性颞叶癫痫,特殊类型的癫痫综合征,进行性肌阵挛癫痫（,progressive myoclonic,epilepsies,）,反射性癫痫,(reflex epilepsies),边缘叶癫痫和新皮质癫痫,(limbic epilepsies and neocortical epilepsies),热性惊厥（,febrile convulsion,）,癫痫性脑病,(epileptic encephalopathies,),2001,年国际抗癫痫联盟,新提出的癫痫发作类型,肌阵挛失神,(myoclonic,absence seizures),：表现为失神发作，同时伴有肢体的肌阵挛动作。,负性肌阵挛,(negative myoclonus,),：没有预先的肌阵挛而出现的强直性肌肉活动的中断，时间小于,500,毫秒。,眼睑肌阵挛,(eyelid myoclonus,),：眼睑肌阵挛往往是突发性，节律性的快速眼睑肌阵挛抽动，每次发作中往往有三次以上的眼睑抽动，并且可以伴有轻微的意识障碍。,痴笑发作（,gelastic,seizures,）：为发作性的发笑，内容空洞,三 癫痫外科手术治疗的术前评估,所有的癫痫都适合外科手术治疗吗,?,所有的难治性癫痫都适合外科手术治疗吗,?,外科手术治疗是癫痫的,”,根治,”,方法吗,?,问题的提出,?,手术治疗癫痫,“,方法,”,还是,“,底线,”,？,手术治疗癫痫还是难治性癫痫,?,什么是难治性癫痫,?,如何界定难治性癫痫,?,目前癫痫治疗的方法,药物治疗,外科手术治疗,迷走神经刺激上术,颅内刺激,饮食疗法,经颅磁刺激,生物反馈疗法,其他方法,选择手术治疗的理由？,药物难治性癫痫？,手术治疗方法本身的优势？,癫痫疾病本身的特质？,其他因素,如何理解药物难治性癫痫的概念？,基于临床药物疗效的概念？,有无统一的标准及如何界定？,是否存在医源性难治性癫痫？,非癫痫性发作,癫痫发作和癫痫分类错误,抗癫痫药物的使用不当,抗癫痫药物,Older:,Phenobarbital,Phenytoin,Carbamazepine,Valproic,Acid,Ethosuximide,Newer:,Lamotrigine,Oxcarbazepine,Zonisamide,Felbamate,Topiramate,Levetiracetam,Gabapentin,Tiagabine,Pregabalin,Generalized,Partial,抗癫痫药物的选择,Tonic-,clonic,PHT,CBZ,PB,GBP,TGB,OCBZ,ACTH,TPM?TGB?VGB?,Tonic,Myoclonic,Atonic,InfantileSpasms,Absence,ESX,VPA,LTG,TPM,(FBM),ZNS,LEV,Simple,Complex,Secondarygeneralized,*,Kwan&Brodie.N Engl,J Med 2000;342:314-9.,手术可治性癫痫综合症,颞叶内侧癫痫,伴局灶性结构性损害,伴弥漫性皮层损害,特殊癫痫综合症,Landau-Klefner,Syndrome,Rasmussen,s Encephalitis,Sturge,-Weber Syndrome,Lennox-Gastaut,Syndrome,术前评估中应该尽量去,认识的一些术语,Epileptogenic lesion,Seizure onset zone,Irritative zone,Symptomatogenic zone,Functional deficit zone,Epileptogenic zone,致癎区以及相关概念,致癎区：是大脑皮质兴奋抑制功能失常的区域，并且这种失常的强度足以引起大多数的临床发作，手术切除后可以获得完全的临床缓解。,发作起始区：临床发作起始的区域。,刺激区：是由于各种原因造成的大脑中兴奋抑制功能失常的区域，这种失常的强度主要表现为发作间歇期的放电。,致癎病灶：导致癫癎形成的责任性、结构异常性病灶。,致癎区以及相关概念,临床症状产生区：是由于受癫癎样放电的刺激而能够产生发作症状的皮质区域,功能缺损区：在发作间歇期表现为功能失常的皮质区域,可表达功能的皮质区域：该区域是负责某种功能的大脑皮质。,癫癎外科的术前综合评估,致癎区以及相关概念,定位评估手段,神经心理学评估,评估程序,定位评估手段,临床发作症状分析,简单部分性发作的定位价值,复杂部分性发作的定位价值,发作症状学分类的定位价值,癫癎外科的术前综合评估,致癎区以及相关概念,定位评估手段,神经心理学评估,评估程序,神经心理学评估,神经心理学评估内容：包括智力、注意力、运动、感觉、语言、记忆、视空间能力、执行功能等,评估检查介绍,评估检查注意事项,评估检查介绍,Wada,试验,卵圆孔电极,硬膜外,/,下电极,深部电极,皮质电极,皮质电刺激,评估检查注意事项,根据具体情况以获得满意的定位资料为标准选择相关检查。,定位是多项检查的综合。,必须要求获得发作期的脑电变化。发作期的监测要记录到至少,3,次以上与平时发作一致的自然发作。,对于内侧型颞叶癫癎，包括蝶骨电极在内的脑电图记录等，均能够很好的定侧定位。而对于新皮质癫癎，头皮脑电图往往不能满足手术 要求。,癫癎外科的术前综合评估,致癎区以及相关概念,定位评估手段,神经心理学评估,评估程序,评估程序,步骤二：以侵袭性手段为主，包括颅内电极的放置及监测，,WADA,实验等，采用有创性检查,术中检查：包括术中皮质脑电图和皮质电刺激,步骤一（以非侵袭性手段定位为主）,可选择性进行的检查：,MEG,、,fMRI,、,MRS,、,TMS,、体感诱发。,致痫区定位,头皮录象,EEG,：发作间歇期,EEG,及发作期,EEG,，录象可对发作症状进行分析。,结构影像学检查：头颅,CT,、,MRI,。,功能影像学检查：,PET,、发作期和发作间歇期,SPECT,检查。,功能区定位,MEG,、,fMRI,运动、感觉、语言优势定侧。,神经心理学评估。,如何认识这些相关术语,EP.,lesi,potentially epileptogenic,lesion on anatomical,neuroimaging,topographical relationship to the other zones.,Seiz,.,Ons,area of cortex from which the seizure originates according to ictal,EEG recording,Irrit,.,zon,area of cortex that produces interictal,spikes in the EEG,Symp.gen,area of cortex which when activated by an epileptiform,discharge,produces the initial ictal symptomatology,Func,.,defi,area of cortex,which by functional neuroimaging,neuropsychological testing,neurologic,examination,or other tests shows functional abnormalities,Ep,.,gen,be indispensable to the generation of clinical epileptic seizures.can only be defined theoretically.,Surgical resection,步骤二：以侵袭性手段为主，包括颅内电极的放置及监测，,WADA,实验等，采用有创性检查,术中检查：包括术中皮质脑电图和皮质电刺激,手术治疗的目标,长期无发作,长期安全,功能保留,术前评估方法,神经电生理,神经影象学,临床症候学,心理学测试,Positron Emission Tomography(PET),Measures glucose metabolism,Use 2-,18,Ffluro-2-deoxyglucose(FDG)or flumazenil,Interictal PET identifies epileptogenic,region,Single Photon Emission Computerized Tomography(SPECT),Utilize isotope technetium,99,(,99m,T,C,),99m,T,C,rapidly fixed into the brain tissue,Reflect cerebral perfusion at the time of the injection,Ictal SPECT mostly helpful in lateralizing epileptogenic,region,SPECT,Magnetoencephalography(MEG),Recording of the magnetic fields generated by the electrical activity of the brain,Analyze spatial distribution of magnetic field to localize its source,Magnetoencephalography,(MEG),Noninvasive,Direct measurement of the neuronal function,High temporal resolution,High spatial resolution,Magnetic source imaging,Fig.1.(a)Preoperative MRI,localizations;(b)resection cavity;(c)preoperative dipole localizations projected onto postoperative MRI scan.,*,Genow A,et al.Neuroimage,.2004 Jan;21(1):444-9,Functional MRI,Provide image of cerebral activation,Utilize blood flow oxygen level-dependent(BOLD)contrast,High spatial and temporal resolution,Neuropsychological Testing,Apply a battery of tests,Testing of memory,speech,non-verbal skills,planning.,Identify pathological areas,Presurgical evalution,Wada Test,Unilateral carotid amobarbital injection,Assess the risk of temporal lobe surgery,HM had total amnestic syndrome after bilateral temporal lobectomy for epilepsy,Wada J,Rasmussen T.Intracarotid injection of sodium amytal for the lateralization of cerebral speech dominance.J Neurosurg,1960;17:266-82,Scoville WB,Milner B.Loss of recent memory after bilateral hippocampal,lesions.,J Neurol Neurosurg Psychiatr,1957;20:11-21,Cortical surface before and after grid removal,Completed Functional Map,Sylvian Fissure,Completed Functional Map,3-D Reconstruction of Functional Map,Tingling right leg,Motor responses,right hand to leg,Speech,naming and,motor activity block,Speech block,Laughter,Fried et al.,Nature,1998,39 yo man with generalized tonic-clonic seizures,started 22 yo,.Normal MRI,Normal PET,39 yo man with generalized tonic-clonic seizures,started 22 yo,.Normal MRI,Normal PET,额叶癫痫,功能复杂,联系广泛,Clinical features of frontal lobe epilepsy,SPS or CPS with or without secondary generalization(more common),CPS:brief,frequency,abrupt(onset&termination),Auras are often nonspecific,Bizarre bimanual/bipedal activity,often from onset,Vocalizations or speech arrest can occur,Automatisms may be bizarre and may be mistaken fro nonepileptic seizures,Sexual automatisms can be seen,May be associated with falls,Adversive head of eye deviation may occur,Frontal complex partial seizures have a tendency to be nocturnal,May occur in clusters,Complex partial status is relatively common,Postictal Todds paralysis frequent,with onset near motor cortex,EEG frequently normal,even ictally,Neuroimaging studies often negative,localization often difficult,Responds less well to surgery than temporal seizures,SMS:speech arrest,movement of eyes,head toward the extended,abducted arm,Jaksonian motor seizures:march of clonic activity corresonding to the spread of discharge along the precentral motor strip.,Seizure Subclassifications of frontal lobe according to anatomy,Supplementary motor seizures patterns,Cingulate seizures patterns,Anterior frontopolar seizure patterns,Orbitofrontal seizure patterns,Dorsalateral seizure patterns,Opercular seizure patterns,Motor cortex(perirolandic)seizure patterns,Features of frontal lobe epilepsy,Areas,Specific,Non-specific features,Supp,motor,fencing posture,complex focal feature,Postural,simple focal tonic,with localization,speech arrest,motor,Simple partial seizure with topographic localization as involved,Lower periorlandic may present speech arrest,vocalization of dysphasia,swallowing or contralateral tonic clonic,movements,Orbito,frontal,olfactory hallucinationsillusions,autonomic signs,Complex partial with initial motor and gestual,automatism,Dorsa,lateral,speech arrecst,Tonic or less clonic with versive,eye and head movement and,Oper,cular,Mastication,salivation,swallowing,laryngeal Sympt,epigastric,aura with fear,vegetative phenomenon,simple partial seizures,particularly partial clonic,facial seizures,secondary sensory changes,numbness in hand.Bilateral upper movement of the extremities,Front,polar,initial loss of contact,axial clonic,jerks and,falls and autonomic signs,versive,movement of head and eyes,common secondary generalization,Cing,ulate,Vegetative signs as changes in mood and affect,Complex partial with complex motor gestural,automatisms at the onset.,Features of frontal lobe complex partial seizures,Frequent,brief seizures occuring,in clusters,Nonspecific aura,Abrupt onset and end with little or no postictal,confusion,Nocturnal preponderance,Complex motor and sexual automatisms,Prolminent vocalization from simple hummuing,screaming,or expletives,Bizarre hysterical appearance,Complex partial status epilepticus,Lateralising features of frontal lobe epilepsy,Sensory symptoms,Focal clonic,motor activity,Unilateral tonic posture,Version before secondarily generalised,tonic clonic,seizures,Beware of other head turning,especially in,postural seizures,Postictal hemiparesis,PET did not show much hypometabolism,PET MRI fusion was not helpful for this case,But MRI is convincing enough to tell possible cortical dysplasia,Epilepsy Surgery(Continued),Corpus Collosotomy,Multiple Subpial Transections,*Benifla,etc.Childs Nervous System Volume 22,Number 8 August,2006,颞叶内侧癫痫,成人癫痫的常见类型,(,约,1/2),致残,不可恢复的行为异常,绝大多数为药物难治性,药物控制无发作率低于,10%,外科手术治疗效果较佳,Wiebe S,et al.New Engl J Med 2001;345:311-318,Free from Complex Partial&Generalized Seizures,University of Western Ontario&London Health Sciences Centre,Ontario,CANADA,*,Wiebe,S,et.al.,New Engl,J Med.2001;345:311-318,生活质量改善情况,(QOL-89),Months,Higher scores=Better QOL,P 0.001,University of Western Ontario&London Health Sciences Centre,Ontario,CANADA,*,Wiebe,S,et.al.,New Engl,J Med.2001;345:311-318,就业及教育情况,University of Western Ontario&London Health Sciences Centre,Ontario,CANADA,Wiebe S,et.al.,New Engl,J Med.2001;345:311-318,*,Recommendations for Surgery,1.Patients with disabling complex partial seizures,with or without secondary generalized seizures,who have failed appropriate trials of first-line antileptic,drugs should be considered for referral to an epilepsy surgery center,although criteria for failure of drug treatment have not been definitely established.,Engel,Jr.,et al.Neurology 2003;60:538-547,Recommendations,2.Patient referred to an epilepsy surgery center for the reasons stated above who meet established criteria for an anteromesial temporal resection and who accept the riskes and benefits of this procedure,as opposed to continuing pharmacotherapy,should be offered surgical treatment.,Recommendations,3.There is insufficient evidence at this time to make a definitive recommendation as to whether patients with a localized neocortical epileptogenic region will benefit or not from surgical resection.,Reasons surgery was considered the last resort for epilepsy,Fear of surgery,Lack of information,Epilepsy surgery is expensive,颞叶癫痫分型标准,颞叶内侧型癫痫诊断标准,具有典型颞叶内侧癫痫发作的临床表现，如上腹部感觉异常、恐惧等先兆，口咽及运动自动症等；,脑电图显示前或前中颞癫痫样放电。,MRI,显示颞叶内侧病灶,HS,正常,颞叶外侧型癫痫诊断标准,具有典型颞叶外侧癫痫发作的临床表现，如听觉、前庭或复杂视幻觉；,脑电图显示后或中后颞癫痫样放电。,MRI,显示颞叶外侧病灶,患者的人口学资料、病史及家族史,组别,(n=278),年龄段,（岁）,男,女,比例,发病年龄,（岁）,病程,（年）,FC,史,家族史,例,%,颞叶,内侧型,(n=252),473,1.51,1.567,(20.2,12.3),0.243,（,10.0,8.2,）,66(26.2),14(5.6),颞叶,外侧型,(n=15),859,0.71,1650,(19.8,15.4),0.3 16,（,5.8,5.2,）,1(6.7),0(0),未明,确分型,(n=11),972,1.21,944,(28.4,14.1),0.234,（,7.8,15.1,）,0(0),0(0),颞叶癫痫,临床特点,:,1)SPS:,自主神经,/,精神症状,/,特殊感觉,2)CPS:,停止运动,/,口,-,咽自动症,/,其他自动症,3)GTCS,MTLE,特征,:,上腹部不适,/,自主神经症,LTLE,特征,:,听幻觉,/,错觉,/,梦样状态,临床表现,颞叶内侧型癫痫组：,最常见的发作类型,-CPS,复杂部分性发作的特点之一,-,自动症,152,例（,60.3%,）,口咽自动症（,130,例次）,手及上肢自动症（,35,例次）,其他动作自动症（,5,例次）,18,例患者同时出现两种形式的自动症,临床表现,颞叶内侧型癫痫组：,先兆,SPS,胃气上升感、恶心等上腹部的异样感（,87,例）,情感异常,-,恐惧感（,12,例）、欣快感（,5,例）、忧伤感（,3,例）等,知觉异常,-,似曾相识感、陌生感飘感、脑子空白感等（,17,例）,幻嗅（,5,例）,临床表现,颞叶外侧型癫痫组,SPS,幻听（,8,例）,幻视（,6,例）,梦样状态（,1,例）；,86.7%,出现继发全面强直阵挛发作,临床表现,未明确分型组,全面强直阵挛发作,:100%,复杂部分性发作,3,例,脑电图特点,发作间期,EEG,1),无异常,2),背景活动轻度减弱或不对称,3),颞叶棘波,/,尖波,/,慢波,蝶骨电极,脑电图特点,发作期,EEG,EEG,对,MTLE,定位准确率为,76%85%,，是术前定位的主要手段。,脑电图,颞叶内侧型癫痫组,发作间期脑电图：,95.2%,通过常规脑电图，,4.8%,通过长程脑电图监测到前或前中颞区局灶性尖波棘波、尖慢波棘慢波、慢波，偶发或频繁出现；,脑电图双侧异常者,31%,，单侧异常者,69%,；,248,例行蝶骨电极监测，,95.2%,显示前颞尖波针锋相对和（或）慢波位相倒置，其中,26,例普通电极监测未见异常。,脑电图,颞叶外侧型癫痫组,发作间期脑电图：,100%,常规脑电图监测显示后或中后颞区局灶性尖波棘波、尖慢波棘慢波、慢波，偶发或频繁出现；,40%,双侧异常者,6,例，,60%,单侧异常,12,例行蝶骨电极监测，结果同背景,脑电图,未明确分型组,发作间期脑电图：,100%,常规脑电图监测显示颞区局灶性尖波棘波、尖慢波棘慢波、慢波，偶发或频繁出现；,双侧异常者,4,例，单侧异常者,7,例；,8,例行蝶骨电极监测，结果同背景。,病因,病灶性,肿瘤,血管病,颅内感染,围产期损伤,外伤,皮质发育异常,非病灶性,海马硬化,病因,颞叶内侧型癫痫组：,病灶性,-65,例（,25.8%),颅内感染,2