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,按一下以編輯母片,第二層,第三層,第四層,第五層,编辑版ppt,*,按一下以編輯母片標題樣式,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,*,编辑版ppt,*,按一下以編輯母片,第二層,第三層,第四層,第五層,按一下以編輯母片標題樣式,编辑版ppt,*,按一下以編輯母片,第二層,第三層,第四層,第五層,按一下以編輯母片標題樣式,编辑版ppt,*,按一下以編輯母片,第二層,第三層,第四層,第五層,按一下以編輯母片標題樣式,编辑版ppt,*,按一下以編輯母片,第二層,第三層,第四層,第五層,按一下以編輯母片標題樣式,编辑版ppt,*,按一下以編輯母片,第二層,第三層,第四層,第五層,按一下以編輯母片標題樣式,编辑版ppt,*,按一下以編輯母片,第二層,第三層,第四層,第五層,按一下以編輯母片標題樣式,编辑版ppt,*,按一下以編輯母片,第二層,第三層,第四層,第五層,按一下以編輯母片標題樣式,编辑版ppt,*,按一下以編輯母片,第二層,第三層,第四層,第五層,按一下以編輯母片標題樣式,编辑版ppt,*,按一下以編輯母片,第二層,第三層,第四層,第五層,按一下以編輯母片標題樣式,编辑版ppt,*,按一下以編輯母片,第二層,第三層,第四層,第五層,按一下以編輯母片標題樣式,编辑版ppt,*,按一下以編輯母片,第二層,第三層,第四層,第五層,按一下以編輯母片標題樣式,编辑版ppt,*,Status Epilepticus and Serial seizures,1,编辑版ppt,Status Epilepticus and Serial,病史,家族史,家族中其他人是否患有癫痫症?,遗传型发作:,1,、常染色体显性遗传夜间额叶癫痫,2,、良性家族性新生儿惊厥,3,、良性家族性婴儿惊厥,4,、部分发作性癫痫(带听觉综合症),5,、常染色体显性遗传进行性额叶癫痫(带智力缺陷),6,、常染色体显性遗传热痉挛,2,编辑版ppt,病史家族史2编辑版ppt,癲癇的定義,1925,英人,J Hughling Jackson:,腦部神經細胞不正常放電,癲癇是由許多先天或後天的因素所引起的慢性腦病,其特徵是由於腦細胞的過度放電,(excessive discharge),所引起的反覆性發作,(epileptic seizure),,伴隨多種類型的臨床表現,Seizure (,癲癇發作,):,腦部異常放電,症狀,每個人都有可能,(alcohol, drug, infection, exciting etc.),Epilepsy(,癲癇症,):,反覆發作,疾病狀態,3,编辑版ppt,癲癇的定義1925, 英人 J Hughling Jacks,病史,家族史,家族中其他人是否患有癫痫症?,遗传型发作:,1,、常染色体显性遗传夜间额叶癫痫,2,、良性家族性新生儿惊厥,3,、良性家族性婴儿惊厥,4,、部分发作性癫痫(带听觉综合症),5,、常染色体显性遗传进行性额叶癫痫(带智力缺陷),6,、常染色体显性遗传热痉挛,4,编辑版ppt,病史家族史4编辑版ppt,病史,家族史,家族中其他人是否患有癫痫症?,遗传型发作:,1,、常染色体显性遗传夜间额叶癫痫,2,、良性家族性新生儿惊厥,3,、良性家族性婴儿惊厥,4,、部分发作性癫痫(带听觉综合症),5,、常染色体显性遗传进行性额叶癫痫(带智力缺陷),6,、常染色体显性遗传热痉挛,5,编辑版ppt,病史家族史5编辑版ppt,癲癇的定義,Aura:,前兆,希臘語,“,颱風前之微風,”,,發作前之警告,,seizure,的一部分,本身就是,simple partial seizure,。,Automatism:,自動症,,automatic behavior during seizure with altered consciousness.,Fit:,發作。,Ictus:,發作。,Ictal: related to seizure itself.,Postictal: immediately after seizure.,Interictal: between attacks of seizure, often symptom free.,6,编辑版ppt,癲癇的定義Aura: 前兆,希臘語 “颱風前之微風”,發作前,癲癇的流行病學,盛行率,0.3 - 0.7% (,平均每兩百人中一個,),發生率,30-70/100000 (,每年新增病例,),每九到十人中,有一人其一生中發生過一次或多次的癲癇發作,(epileptic seizure),7,编辑版ppt,癲癇的流行病學盛行率7编辑版ppt,癲癇發作,(,seizure,),的分類,International Classification of Epileptic Seizure,Partial (local onset),局部發作,Simple partial seizure,單純局部發作,Complex partial seizure,複雜局部發作,Partial seizure with secondary generalization,局部發作演變成次發性全身發作,Generalized (bilaterally symmetric without local onset),全身發作,GTCS, Absence,.etc,8,编辑版ppt,癲癇發作(seizure)的分類International,癲癇,(,epilepsy,),的分類,International Classification of Epilepsies and Epileptic Syndrome,Idiopathic epilepsy syndromes (focal or generalized),Benign neonatal convulsions,Benign childhood epilepsy,Childhood/juvenile absence epilepsy,Juvenile myoclonic epilepsy,unspecified,Symptomatic epilepsy syndromes ( focal or generalized),West syndrome (infantile spasms),Lennox-Gastaut syndrome,Epilepsia partialis continua,Other epilepsy syndromes of uncertain or mixed classification,9,编辑版ppt,癲癇(epilepsy)的分類International,Simple Partial Seizure,單純局部發作,Consciousness not impaired,意識清楚,With,Motor symptoms,:,simple clonic convulsion, Jacksnoian marching, adversion,Somatosensory or special sensory symptoms,:,paresthesia, auditory or visual hallucination,Autonomic symptoms,:,abdominal cramp, vomiting, vertigo,Psychic symptoms,:,dj vu, jamai vu, fear, anxiety,10,编辑版ppt,Simple Partial Seizure 單純局部發作C,Simple visual,Somatic sensory,Jacksonian,SMA,Simple,adversive,Complex,adversive,Vestibular,Autonomic,Gustatory,Visceral,Auditory,11,编辑版ppt,Simple visualSomatic sensoryJa,病史,家族史,家族中其他人是否患有癫痫症?,遗传型发作:,1,、常染色体显性遗传夜间额叶癫痫,2,、良性家族性新生儿惊厥,3,、良性家族性婴儿惊厥,4,、部分发作性癫痫(带听觉综合症),5,、常染色体显性遗传进行性额叶癫痫(带智力缺陷),6,、常染色体显性遗传热痉挛,12,编辑版ppt,病史家族史12编辑版ppt,Jacksonian seizure,13,编辑版ppt,Jacksonian seizure13编辑版ppt,病史,家族史,家族中其他人是否患有癫痫症?,遗传型发作:,1,、常染色体显性遗传夜间额叶癫痫,2,、良性家族性新生儿惊厥,3,、良性家族性婴儿惊厥,4,、部分发作性癫痫(带听觉综合症),5,、常染色体显性遗传进行性额叶癫痫(带智力缺陷),6,、常染色体显性遗传热痉挛,14,编辑版ppt,病史家族史14编辑版ppt,Adversive seizure,15,编辑版ppt,Adversive seizure15编辑版ppt,Complex Partial Seizure,複雜局部發作,(Psychomotor seizure, temporal epilepsy),Consciousness impaired,Limbic cortex involved,Amnesia,Two subtypes,Simple partial onset followed by impaired consciousness,Impaired consciousness at onset,16,编辑版ppt,Complex Partial Seizure 複雜局部發作,Generalized Seizure,全身發作,Without local onset,Bilaterally symmetric,Loss of consciousness,17,编辑版ppt,Generalized Seizure 全身發作Withou,Generalized Seizure(Convulsive or nonconvulsive),Absence seizure (petit mal),失神發作,Myoclonic seizure,肌抽躍發作,Clonic seizure,間代發作,Tonic seizure,強直發作,Tonic-clonic seizure (grand mal),強直,-,間代發作,Atonic seizure,失張發作,18,编辑版ppt,Generalized Seizure(Convulsiv,病史,家族史,家族中其他人是否患有癫痫症?,遗传型发作:,1,、常染色体显性遗传夜间额叶癫痫,2,、良性家族性新生儿惊厥,3,、良性家族性婴儿惊厥,4,、部分发作性癫痫(带听觉综合症),5,、常染色体显性遗传进行性额叶癫痫(带智力缺陷),6,、常染色体显性遗传热痉挛,19,编辑版ppt,病史家族史19编辑版ppt,Absence seizure,Onset: 4-10 yr, usually resolve by age 20,No prodrome or aura,Seizure: seconds to mins, sudden interruption of consciousness, automatism,No postictal phase,Easily induced by hyperventilation or photic stimulation,Ictal EEG: 3 Hz spikes and waves,20,编辑版ppt,Absence seizureO,Atypical Absence seizures,Usually occurs in patients with neurological or developmental abnormalities.,Ictal EEG: 3year,37,编辑版ppt,Good prognostic factors for st,Antiepileptic drugs,Antiepileptogenesis: prevent kindling,Antiepileptic activity: fully kindled can be treated (seizure control),- Block voltage-gated Na+ channels,Block voltage-gated Ca2+ channels,Increasing GABA transmission,Decreasing Glutamate transmission,38,编辑版ppt,Antiepileptic drugsAntiepilept,Drug choice,39,编辑版ppt,Drug choice39编辑版ppt,40,编辑版ppt,40编辑版ppt,41,编辑版ppt,41编辑版ppt,Idiosyncratic reactions are,not dose related,; rather they arise either from an,immune-mediated reaction,to the drug or from poorly defined,individual factors,largely genetic, that convey an,unusual sensitivity to the drug,.,42,编辑版ppt,Idiosyncratic reactions are no,病史,家族史,家族中其他人是否患有癫痫症?,遗传型发作:,1,、常染色体显性遗传夜间额叶癫痫,2,、良性家族性新生儿惊厥,3,、良性家族性婴儿惊厥,4,、部分发作性癫痫(带听觉综合症),5,、常染色体显性遗传进行性额叶癫痫(带智力缺陷),6,、常染色体显性遗传热痉挛,43,编辑版ppt,病史家族史43编辑版ppt,Dosing intervals should usually be less than one-third to one-half the drugs half-life,to minimize fluctuations between peak and trough blood concentrations.,44,编辑版ppt,Dosing intervals should usuall,癲癇重積症;持續性癲癇狀態,(Status epilepticus),連續發作超過,10,分鐘,反復發作間隔病人意識沒有清醒過來,(N Eng J Med. 1998),高危險,高死亡率,(8-20%),原因:,cessation of drug,alcohol withdrawal,infection,metabolic disorders,cerebrovascular disease,45,编辑版ppt,癲癇重積症;持續性癲癇狀態(Status epilepti,Consequences of status epilepticus,Organs,Damage,Acid-base,Acidosis lactic, respiratory,Heart,Arrhythmia,Lung,Pulmonary edema, aspiration,Bone,Shoulder dislocation, rib fracture,Muscle,Rhabdomyolysis, myoglobinuria,46,编辑版ppt,Consequences of status epilept,癲癇重積症的治療,- I,Preference,Alternatives,0 min,(record the exact time of ativan IV),Lorazepam,(2) 2 amp slow IV push (may repeat after 10 mins),Diazepam,(10) 1-2 amp slow IV push; may repeat after 10 min,3 min,Valproate,(400) 2amp (15mg/kg) slow IV push, then 1.5 amp IVF q8h,Phenytoin,(100) 7 amp,(15-18 mg/kg, 50 mg/min) IVF in 20 min, then 1 amp IV q8h,If GSz recur,( in 10-40 mins),Lorazepam,(2) 2 amp slow IV push,Diazepam,(10) 1-2 amp slow IV push,If GSz recur,(in 40-60 mins),Midazolam,(5) 2 amp slow IV push, then 0.05-0.4 mg/kg/hr,Midazolam,(5) 2 amp slow IV push, then 0.05-0.4 mg/kg/hr,47,编辑版ppt,癲癇重積症的治療- IPreferenceAlternati,癲癇重積症的治療,- II,Preference,Alternatives,If poor Sz control clinically or electrophysiologically,Propofol,(200mg/20ml/amp) amp (2mg/kg) slow IV push, then 5-10 mg/kg/hr,Thiopentone,100-250 mg slow IV push, then 3-5 mg/kg/hr,Phenobarbitone,5-20 mg/kg, then 0.5-3 mg/kg/hr,Ending of therapy,(good clinical and EEG control for 24 hrs),Stop midazolam or propofol or other alternatives,acutely,and close observe clinical Sz and EEG, succeed or failure,Failure of stopping midazolan or propofol or other alternatives,Restore previous regimen for another 24 hrs, and add another major oral AED,Try ending again,Stop midazolam or propofol or other alternatives,acutely,and close observe clinical Sz and EEG, succeed or failure,48,编辑版ppt,癲癇重積症的治療- IIPreferenceAlternat,癲癇診斷的層次,Hierarchy of Epilepsy Diagnosis,Descriptive diagnosis,Seizure type diagnosis,Epileptic syndrome diagnosis,Etiological diagnosis,49,编辑版ppt,癲癇診斷的層次Hierarchy of Epilepsy,Thank you,50,编辑版ppt,Thank you50编辑版ppt,病史,家族史,家族中其他人是否患有癫痫症?,遗传型发作:,1,、常染色体显性遗传夜间额叶癫痫,2,、良性家族性新生儿惊厥,3,、良性家族性婴儿惊厥,4,、部分发作性癫痫(带听觉综合症),5,、常染色体显性遗传进行性额叶癫痫(带智力缺陷),6,、常染色体显性遗传热痉挛,51,编辑版ppt,病史家族史51编辑版ppt,病史,家族史,家族中其他人是否患有癫痫症?,遗传型发作:,1,、常染色体显性遗传夜间额叶癫痫,2,、良性家族性新生儿惊厥,3,、良性家族性婴儿惊厥,4,、部分发作性癫痫(带听觉综合症),5,、常染色体显性遗传进行性额叶癫痫(带智力缺陷),6,、常染色体显性遗传热痉挛,52,编辑版ppt,病史家族史52编辑版ppt,
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