Status Epilepticus and Serial seizures

神經科 黃子軒 051007

癲癇的定義癲癇的定義 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

癲癇的定義癲癇的定義 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 wit

h 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.

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)

癲癇發作癲癇發作 ((seizureseizure)) 的分類的分類International Classification of Epileptic SeizureInternational 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

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

Simple Partial Seizure 單純局部發作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: déjà vu, jamai vu, fear, anxiety…

Simple visual

Somatic sensory

Jacksonian SMA

Simple adversive

Complexadversive

VestibularAutonomicGustatoryVisceral

Auditory

Jacksonian seizureJacksonian seizure

Adversive seizureAdversive seizure

Complex Partial Seizure 複雜局部發作 (Psychomotor seizure, temporal epilepsy)Complex Partial Seizure 複雜局部發作 (Psychomotor seizure, temporal epilepsy)

Consciousness impairedLimbic cortex involvedAmnesiaTwo subtypes

Simple partial onset followed by impaired consciousness

Impaired consciousness at onset

Generalized Seizure 全身發作Generalized Seizure 全身發作

Without local onsetBilaterally symmetricLoss of consciousness

Generalized Seizure(Convulsive or nonconvulsive)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

Absence seizure

Onset: 4-10 yr, usually resolve by age 20No prodrome or auraSeizure: seconds to mins, sudden interruption

of consciousness, automatismNo postictal phaseEasily induced by hyperventilation or photic st

imulationIctal EEG: 3 Hz spikes and waves

Atypical Absence seizures

Usually occurs in patients with neurological or developmental abnormalities.

Ictal EEG: <3Hz spikes and waves.

Seizure classification

癲癇的原因Infants and children Adults

No definite cause determined No definite cause determined

Birth and neonatal injuries Vascular lesions

Vascular insults Head trauma

Congenital or metabolic disorders Drug or alcohol abuse

Head injuries Neoplasia

infection Infection

Neoplasia Heredity

Heredity

Etiology of Acute Symptomatic Seizures

Stroke 40-54%

Toxic-metabolic 15-30%

Neoplasm 8-10%

Trauma 4-10%

Alcohol 3-5%

CNS infection 2-3%

癲癇的診斷

病史1 發作型態

預兆或先兆 (aura)臨床發作內容發作後症狀誘發因素

2 過去病史3 家族史4 心理社會史

頻率及叢集發作之型態 發病年齡 症狀改變與發展之經過 對癲癇藥物之反應

癲癇的診斷癲癇的診斷

身體理學檢查1 一般理學檢查2 神經理學檢查

實驗室檢驗1 血液生化 ( 必要時腦脊髓液檢查 )2 腦電圖 (EEG)3 X-ray, CT, MRT, Cerebral angiography

Dormicum

11499091

Baseline

Voltage mapping 61M EPC

11499091

11499091

61M Epilepsia Partialis Continua

11499091

T2WIEEG Map SPECT

Classification of Seizure Based on Ictal Symptoms

Sensorial sphere (“aura”)Autonomic sphere (“autonomic seizure”)Consciousness (“dialeptic seizures”)Motor phenomenon (“motor seizure”)

癲癇症的治療 I ( 原則 )

治療可能的病因 metabolic disorders, infection, tumor etc...

根據癲癇類型選擇藥物儘可能減少藥物種類，單一處方優於複方簡化給藥方式與劑量教導面對癲癇的正確態度家庭、學校與社會的支持

癲癇症的治療 II (case by case)

Give drug ???

First attack : trauma, stroke, idiopathic

Stop drug ???

months, years, forever

Good prognostic factors for stopping AEDs

Idiopathic epilepsyChildhood onsetEasy to control with AEDsNormal neurological exam, normal

intelligenceNormal EEGSeizure free>3year

Antiepileptic drugs

Antiepileptogenesis: prevent kindlingAntiepileptic activity: fully kindled can be trea

ted (seizure control)

--- Block voltage-gated Na+ channels

Block voltage-gated Ca2+ channels

Increasing GABA transmission

Decreasing Glutamate transmission

Drug choice

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.

Dosing intervals should usually be less than one-third to one-half the drug’s half-life to minimize fluctuations between peak and trough blood concentrations.

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

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

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

癲癇重積症的治療 - II

Preference AlternativesIf poor Sz control clinically or electrophysiologically

Propofol (200mg/20ml/amp) ½ 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

癲癇診斷的層次Hierarchy of Epilepsy Diagnosis癲癇診斷的層次Hierarchy of Epilepsy Diagnosis

Descriptive diagnosis Seizure type diagnosis Epileptic syndrome diagnosis

Etiological diagnosis

Thank you……