Status Epilepticus and Serial seizures

1. Status Epilepticus and Serial seizures 神經科 黃子軒 051007

2. 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. 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.

4. 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)

5. 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

6. 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

7. 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…

8. Jacksonian SMA Simple adversive Somatic sensory Complex adversive Vestibular Autonomic Gustatory Visceral Simple visual Auditory

9. Jacksonian seizure

10. Adversive seizure

11. 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

12. Generalized Seizure 全身發作 • Without local onset • Bilaterally symmetric • Loss of consciousness

13. 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

14. 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

15. Atypical Absence seizures • Usually occurs in patients with neurological or developmental abnormalities. • Ictal EEG: <3Hz spikes and waves.

16. Seizure classification

17. 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 癲癇的原因

18. Etiology of Acute Symptomatic Seizures

19. 病史 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 過去病史 家族史 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

20. 癲癇的診斷 • 身體理學檢查 • 一般理學檢查 • 神經理學檢查 • 實驗室檢驗 • 血液生化(必要時腦脊髓液檢查) • 腦電圖(EEG) • X-ray, CT, MRT, Cerebral angiography

21. Dormicum Baseline 11499091

22. Voltage mapping 61M EPC 11499091

23. 11499091

24. 61M Epilepsia Partialis Continua EEG Map T2WI SPECT 11499091

25. Classification of Seizure Based on Ictal Symptoms • Sensorial sphere (“aura”) • Autonomic sphere (“autonomic seizure”) • Consciousness (“dialeptic seizures”) • Motor phenomenon (“motor seizure”)

26. 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

27. 癲癇症的治療 II (case by case) • Give drug ??? First attack : trauma, stroke, idiopathic • Stop drug ??? months, years, forever

28. Good prognostic factors for stopping AEDs • Idiopathic epilepsy • Childhood onset • Easy to control with AEDs • Normal neurological exam, normal intelligence • Normal EEG • Seizure free>3year

29. 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

30. Drug choice

33. 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.

34. 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.

35. 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

36. Consequences of status epilepticus

37. 癲癇重積症的治療- I

38. 癲癇重積症的治療- II

39. 癲癇診斷的層次Hierarchy of Epilepsy Diagnosis • Descriptive diagnosis • Seizure type diagnosis • Epileptic syndrome diagnosis • Etiological diagnosis

40. Thank you……