癫痫的定义
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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
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
Stroke
Toxic-metabolic
40-54%
15-30%
Neoplasm
Trauma
8-10%
4-10%
Alcohol
CNS infection
3-5%
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
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
Voltage mapping 61M EPC
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61M Epilepsia Partialis Continua
EEG Map T2WI SPECT
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Classification of Seizure Based on Ictal Symptoms
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
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
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
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.
Status Epilepticus and Serial seizures
神經科 黃子軒 051007
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):ห้องสมุดไป่ตู้
Symptomatic epilepsy syndromes ( focal or generalized)
West syndrome (infantile spasms) Lennox-Gastaut syndrome Epilepsia partialis continua
Other epilepsy syndromes of uncertain or mixed classification
Jacksonian Simple adversive Complex adversive
SMA Somatic sensory
Vestibular Autonomic Gustatory Visceral
Auditory
Simple visual
Jacksonian seizure
Adversive seizure
Idiopathic epilepsy syndromes (focal or generalized)
Benign neonatal convulsions Benign childhood epilepsy Childhood/juvenile absence epilepsy Juvenile myoclonic epilepsy unspecified
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
Sensorial sphere (“aura”) Autonomic sphere (“autonomic seizure”) Consciousness (“dialeptic seizures”) Motor phenomenon (“motor seizure”)
癲癇症的治療 I (原則)
Atypical Absence seizures
Usually occurs in patients with neurological or developmental abnormalities. Ictal EEG: <3Hz spikes and waves.
Seizure classification
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(seizure)的分類
International Classification of Epileptic Seizure
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: dé vu, jamai vu, fear, anxiety… jà
Generalized Seizure 全身發作
Without local onset Bilaterally symmetric Loss of consciousness
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
癲癇的原因
Infants and children
No definite cause determined Birth and neonatal injuries Vascular insults
Adults
No definite cause determined Vascular lesions Head trauma
Congenital or metabolic disorders Drug or alcohol abuse Head injuries infection Neoplasia Heredity Neoplasia Infection Heredity
Etiology of Acute Symptomatic Seizures
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
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.
Drug choice
Idiosyncratic reactions are not dose related; rather they arise either from an immunemediated reaction to the drug or from poorly defined individual factors, largely genetic, that convey an unusual sensitivity to the drug.
2 過去病史 3 家族史 4 心理社會史
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
Baseline
Dormicum
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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