1、Chapter 12. EpilepsyTHE FIRST AFFILIATED HOSPITAL, SUN YET-SEN UNIVERSITYPROF. ZHOU LIE MINlm_KEY WORDS: Epilepsy(癲癇癲癇) Epileptic Seizure (癲癇發作癲癇發作) Electroencephalograph(EEG,腦電圖腦電圖) Epileptiform Discharge(癇性放電癇性放電) Antiepileptic Drugs(抗癲癇藥抗癲癇藥) EPILEPSY The Stress on the Chapter:1. To comprehend th

2、e epileptic definition.2. To understand and have a good command of epilepsy with generalized tonic-clonic seizure, absence seizure, complex partial seizure and Jacksonian epilepsy. West syndrome and Lennox-Gastaut syndrome. 3. To understand and have a good command of epileptic diagnoses 4. To unders

3、tand and have a good command of distinguishing between generalized tonic-clonic seizure and hysteria, absence seizure and syncope.5. To understand and have a good command of definition and treatment principle on status epilepsy with generalized tonic- clonic seizure.6. To comprehend the principle in

4、 using antiepileptic drugs.RonaldoRonaldoVincent van GoghVincent van GoghSeizureEpilepsyEpileptic Seizure Epilepsy is characterized by which sudden recurrent and transient disturbances of mental function or movements and sense of the body are resulted from abnormal neuronal discharges in brain.(used

5、 to)Part 1. DEFINITIONEpilepsy is a disorder of the brain characterized by an enduring predisposition to generate epileptic seizures and by the neurobiologic, cognitive, psychological, and social consequences of this condition. The definition of epilepsy requires the occurrence of at least one epile

6、ptic seizure. (new concept) OUTLINE:the recurrence of epileptic seizures Location: usually arising from the cerebral cortex Pathogenesis: abnormal neuronal discharges Clinical signs: transient disturbances of cerebral functionPart 1. DEFINITIONPart 2. ATIOLOGY AND BASIC MECHANISMS OF EPILEPTOGENESIS

7、, THE INDUCING ELEMENTSSymptomatic . Congenital diseases Structural Lesion of the Brain: Epilepsy . Brain trauma 1. Remarkable decrease in the . Infection of CN synaptic from GABA. . Brain tumor 2.Disorder of blood-brain barrier . Cerebrovascular D Biochemical Aspects: Decrease . Degenrative D in sy

8、nthetic GABA and increase . Toxicosis in excitatory transmitter. . Nutritive and Electrophysiological Aspects: metabolic disturbance Paroxysmal depolarization . The others shift (PDS) discovered Atiology Pathogenesis Primary epilepsy Inheritance Mutation in Ion Channel (Idiopathic) (Usually no brain

9、 damage) cryptogenic ep unclear cannot be discoveried Mutation in Ion ChannelADNFLE The Elements of Interfering with EpilepsyInheritance: Be susceptible to epilepsy or epileptic predisposition ( abnormal EEG)Environmental Influences: Fatigue or hunger, Hyperventilation The Others: Age, Endocrinosis,

10、 Sleep Disturbance (Dysfunction) Part 2. ATIOLOGY AND BBASIC MECHANISMS OF EPILEPTOGENESIS, THE INDUCING ELEMENTSPart 2. BBASIC MECHANISMS OF EPILEPTOGENESIS Inheritance or acquired factorsAbnormal gene expressionNeurotransmitter and modulator dysfunction Structure and function disorder of Ion-chann

11、el Neuronal dischargeEpileptic seizure or epilepsy Part 3: Clinical Manifestations of Epileptic SeizuresClassification of Epileptic Seizures(The 1981 International Classification of Epileptic Seizures)I. Partial seizures(seizures beginning locally)II. Generalized Seizures . Unclassified epileptic se

12、izuresPart 3: Clinical Manifestations of Epileptic SeizuresI. Partial seizures(seizures beginning locally)A. Simple partial seizures ( consciousness not impaired) With motor symptoms With somatosensory or special sensory symptoms With autonomic symptoms With psychic symptomsClassification of Epilept

13、ic Seizures(The 1981 International Classification of Epileptic Seizures)頂葉頂葉B. Complex partial seizures (with impairment of consciousness) Beginning as simple partial seizures and progressing to impairment of consciousness With no other features With features as in A.1-4 With automatisms impairment

14、of consciousness at onset With no other features With features as in A.1-4 With automatisms C . Partial seizures secondarily generalized seizureClassification of Epileptic SeizuresII. Generalized Seizures (bilaterally symmetrical and without local onset) Absence seizures(Typical or Atypical AS) Myoc

15、lonic seizures Clonic seizures Tonic seizures Tonic-clonic seizures Atonic seizures. Unclassified epileptic seizures ( Inadequate or incomplete data)Classification of Epileptic SeizuresSimple partial seizures With motor symptomsComplex partial seizuresAbsence seizures(Typical absence seizure)General

16、ized Tonic-clonic seizuresWell focus our discussion on-Status Epilepticus with GTCSBRE, L, parietal L, Occipital L, Temporal L Ep , et al et al Classification of Epilepsy and Epileptic Syndrome from ILAE1.Generalized Seizures: Generalized Tonic-Clonic Seizures/ Absence Seizures)1.1 Generalized Tonic

17、-Clonic Seizures They occur at some time in the course of epilepsy in most patients with seizures regardless of the patientss usual clinical pattern. They are divided into two types: Primary Generalized Tonic-Clonic Seizure and Secondarily Generalized Tonic-Clonic Seizure.Second: Clinical Manifestat

18、ions of Epileptic Seizures1.1-1 Primary Generalized Tonic-Clonic Seizure(PGE): This is characterized by complete loss of consciousness and falling, and no aura.1.1-2 Secondarily Generalized Tonic-Clonic Seizure(SGS): This is characterized by a partial seizure evolving to a secondarily generalized to

19、nic- clonic seizure.Second: Clinical Manifestations of Epileptic Seizures1.Generalized Seizures: Pre-Convulsion Phase PGTCS SGTCS Convulsion Phase (Tonic-Clonic Phase) Tonic Phase Clonic Phase Recovery PhaseSecond: Clinical Manifestations of Epileptic Seizures1.Generalized Seizures:1.1-1 Pre-Convuls

20、ion PhasePGTCE is frequently preceded by several myoclonic jerks or a brief clonic seizure in bilateral limbs, and then falling as well as loss of consciousness.SGTCS usually evolve from partial seizures, in which signs beginning with sensory symptoms are called “aura” . Clinical Manifestations of G

21、eneralized Tonic-Clonic Seizures(Three Phases)1.1-2 Convulsion Phase (Tonic-Clonic Phase)Tonic Phase: As the patient falls, the body stiffens because of genera- lized tonic contraction of the axial and limb muscles.Eyes: upward eye deviation and pupillary dilation.Mouth: to be forced closure of the

22、mouth, which sometimes produces oral trauma.“Epileptic cry”: to be caused by forcible expiration against opposed vocal cords, which results from sudden involuntary contraction of the respiratory and laryngeal muscles.Limbs: to be tonic flexion or extension of the axial musculature; the legs usually

23、extend and the arms flex partially. This phase usually last 10-20 seconds and the EEG show 10Hz multiple spikes. Clinical Manifestations of Generalized Tonic-Clonic Seizures(Three Phases)This is epileptic patient with generalized tonic-clonic seizure.Her tougue was bited during a epileptic seizure1.

24、1-2 Convulsion Phase (Tonic-Clonic Phase)Tonic Phase: As the patient falls, the body stiffens because of genera- lized tonic contraction of the axial and limb muscles.Eyes: upward eye deviation and pupillary dilation.Mouth: to be forced closure of the mouth, which sometimes produces oral trauma.“Epi

25、leptic cry”: to be caused by forcible expiration against opposed vocal cords, which results from sudden involuntary contraction of the respiratory and laryngeal muscles.Limbs: to be tonic flexion or extension of the axial musculature; the legs usually extend and the arms flex partially. This phase u

26、sually last 10-20 seconds and the EEG show 10Hz multiple spikes. Clinical Manifestations of Generalized Tonic-Clonic Seizures(Three Phases) 尖波尖波 棘波棘波Clonic Phase: The transition from the tonic to the clonic phase is gradual, not sharply demarcated. After the tonic stage, jerking or clonic ( interrup

27、ted tonic ) movements occur in all limbs for less than a minute. This phase usually last 30 seconds to 1 minute and the EEG show multiple spikes-wave complexes and spike-wave complex. Clinical Manifestations of Generalized Tonic-Clonic Seizures(Three Phases)1.1-2 Convulsion Phase (Tonic-Clonic Phase

28、) Clinical Manifestations of Generalized Tonic-Clonic Seizures(Three Phases) Associated With Signs in the Tonic-Clonic Phase : Autonomic Disorders: Heart rate and blood pressure may be increased more than double, increased salivation and breathing may cause frothing at the mouth, urinary incontinenc

29、e may occur, pupillary dilatation with loss of reactivity to light may be seen Respiration System: Respiration stops and pallor or cyanosis may be seen. Reflex: physiological reflex disappear and pathological reflex may be seen. Clinical Manifestations of Generalized Tonic-Clonic Seizures(Three Phas

30、es)1.1-3 Immediate Postictal Phase (Recovery Phase): Recovery of respiration function recovery of autonomic function recovery of consciousness (sleep-confusion-normal) This phase usually last 4-9 minutes and the EEG show low-voltage slow waves, and then recovery of normal background waves1.1-4 Statu

31、s Epilepticus with GTCS Definition: Retitive tonic-clonic convulsions, in which the patient does not recover normal alert state between attacks, is status epilepticus with GTCS. These patients are usually associated with high fever, dehydration, aspiration pneumonitis, loss of acid - base balance, w

32、ater and electrolytic disorder, brain edema, and so on.Clinical ManifestationsWe have to emphasize it is one of the emergencies and severe caseThis seizures are short interruptions of consciousnessthat last from 3 to 15 seconds , less than 30 seconds each, and begin and end abruptly as well as recur

33、 from a few to several hundred times per day. The patients may show ongoing behavior stopping, inconspicuous flickering of the eyelids or eyebrows at about times/second, partial atony, partial muscle stiffness and autonomic signs. EEG of this type show bilaterally synchronous 3 Hz spike- wave comple

34、x.1.2 Absence Seizures1.Generalized Seizures:Fp1-F3Fp2-F4F3-C 3F4-C 4C 3-P3C 4-P4P3-O 1P4-O 2Fp1-F7Fp2-F8F7-T3F8-T4T3-T5T4-T6T5-O 1T6-O 2EEG of the patient with AS show 3 Hz spike-wave complex.2.1 Simple Partial Seizure (SPS) They are characterized by which I. symptoms often indicated the area of th

35、e brain, II. the clinical sign are only partial dysfunction, III. consciousness is not impairment. They are divided into four types: partial motor attacks, somatosensory attacks 、 and special sensory attacks，autonomic attacks， as well as attacks with psychic symptoms.Second: Clinical Manifestations

36、of Epileptic Seizures2. Partial Seizure2.1-1 Partial Motor Seizure:Involve motor activity from any area of the body. Usually, they involve the limbs, face, or head and sometimes cause speech arrest. Jacksonian Seizure: If a PMS progresses with sequential involvement of parts of the body that are rep

37、resented by contiguous cortical areas, it is known as a _.Todd Paralysis: Localized paralysis or weakness that may last for minutes or days after a partial motor seizure is called _.Second: Clinical Manifestations of Epileptic Seizures2.1-1 Partial Motor Seizure:Involve motor activity from any area

38、of the body. Usually, they involve the limbs, face, or head and sometimes cause speech arrest. Jacksonian Seizure: If a PMS progresses with sequential involvement of parts of the body that are represented by contiguous cortical areas, it is known as a _.Todd Paralysis: Localized paralysis or weaknes

39、s that may last for minutes or days after a partial motor seizure is called _.Second: Clinical Manifestations of Epileptic Seizures (used to be called psychomotor seizures) Characteristically, complex partial seizure begin with emotional, psychic, illusory, hallucinatory, or special sensory symptoms

40、. These are followed by clouding or unconsciousness with automatic behavior and amnesia(遺忘）遺忘）. Complex partial seizure usually begin in the temporal lobe, but may originate from the frontal, parietal, or occipital areas.2.2 Complex Partial SeizuresSecond: Clinical Manifestations of Epileptic Seizur

41、esClinical Manifestation: Unconsciousness include confusion or somnolism or Somnambulance, twilight state朦朧狀態朦朧狀態, and is not coma. Automatisms are apparently purposeless and mechanical activity, such as picking at clothes, examining near by objects, simply walking about, chewing, swallowing, lickin

42、g and et al. Complex partial seizure may begin with simple partial seizure, such as emotional, psychic, illusory, hallucinatory, or special sensory symptoms.2.2 Complex Partial SeizuresBRE, L, parietal L, Occipital L, Temporal L Ep , et al et al Classification of Epilepsy and Epileptic Syndrome from

43、 ILAE The infantile spasms (West syndrome) denotes a unique, age-specific form of generalized epilepsy that may be either idiopathic (15%) or symptomatic (85%). Symptomatic cases result from diverse conditions, including cerebral dysgenesis(發育不全）發育不全）, tuberous sclerosis, phenylketonuria苯丙酮尿, intrau

44、terine infections, 宮內感染宮內感染 or hypoxic-ischemic injury.3. Infantile spasms (West syndrome- refractory epilepsy) Seizures are characterized by sudden flexor or extensor spasms that involve the head, trunk, and limbs simultaneously. The attacks usually begin before 6 months of age. The EEG is generali

45、zed abnormal, showing chaotich雜亂雜亂, high-voltage slow activity with multifocal spikes, a pattern termed hypsarrhythmia. 3. Infantile spasms (West syndrome- refractory epilepsy)The EEG Feature in Patients with WS is HypesarrhythmiaFp1-F3Fp2-F4F3-C3F4-C4C3-P3C4-P4P3-O1P4-O2Fp1-F7Fp2-F8F7-T3F8-T4T3-T5T

46、4-T6T5-O1T6-O2 This term is applied to a heterogeneous group of childhood epileptic encephalopathies that are characterized by mental retardation, uncontrolled seizures, which consist of GTCS, TS, CS and Atonic seizure, and a distinctive EEG pattern. 4. Lennox-Gastaut Syndrome- refractory epilepsy T

47、he syndrome is not only a pathologic entity, because clinical and EEG manifestations result from brain malformations, perinatal asphyxia, severe head injury, CNS infection, or, rarely, a progressive degenerative or metabolic syndrome. A presumptive cause can be identified in 65 to 70% of affected ch

48、ildren. Seizures usually begin before age 4 years, and about 25% of children have a history of infantile spasms. No treatment is consistently effective, and 80% of children continue to have seizures as adults. 4. Lennox-Gastaut Syndrome-refractory epilepsyFp 1 -F3Fp 2 -F4F3 -C3F4 -C4C3 -P3C4 -P4P3 -

49、O 1P4 -O 2Fp 1 -F7Fp 2 -F8F7 -T3F8 -T4T3 -T5T4 -T6T5 -O 1T6 -O 2The EEG Sign in Patients With L-GS is 2.5-4Hz Spike-Wave Complex It is important for symptomatic epilepsy to be completely examine in nerve system.EEG test is necessary for patients with epilepsy, but only 80-95% of the patients may be

50、diagnosed by EEG test at onset phase. EEG may show spike waves, spike-wave complex, sharp waves and sharp-wave complex.CT scan, MRI +MRS, SPECT, PET, DSA, MEG and Cerebrospinal Fluids test are helpful for us to find epileptic cause and locus.Part 4: Physical Examination and Laboratory Test 尖波尖波 棘波棘波

51、Spike waves, spike-wave complex, sharp waves and sharp-wave complex.Part 5: Diagnosis and Differential DiagnosisDiagnosis of Epilepsy-Answer some questions1. Whether the seizure is epilepsy or not2.Which types or syndrome of epilepsy3. Idiopathic or Symptomatic or Cryptogenic Epilepsy 5.1 What is th

52、e basis of diagnosing epilepsy ?Part 5: Diagnosis and Differential DiagnosisDiagnosis of EpilepsyDetailed Medical HistoryEEGCT or MRI+MRSSPECT or PETDSAMEG 5.1 What is the basis of diagnosing epilepsy ?5.1-1. It is important for us to explore the detailed and accurate history, which is provided by t

53、he patients with partial seizure or their relatives with generalized seizure.Part 5: Diagnosis and Differential Diagnosis We should know : where epileptic seizure is, how long the seizure continue, what signs there are during seizure, and we should also know the patientss consciousness during seizur

54、e.Part 5: Diagnosis and Differential Diagnosis5.1-2 Physical Examination: for the symptomatic ep.Part 5: Diagnosis and Differential Diagnosis5.1-3 Laboratory Test:Which one is important for epilepsy in lab test ?Diagnosis of EpilepsyEEGCT or MRI+MRSSPECT or PETDSAMEGPart 5: Diagnosis and Differentia

55、l Diagnosis5.2 Differential Diagnosis 5.2-1 GTCS and Hysteria 5.2-2 Absence Seizure and Syncope5.2-3 Idiopathic and Symptomatic Epilepsy Table 1. General Differentiation of GTCS from Hysteria Characteristic GTCS Hysteria Seizure Triggers Uncommon Emotional disturbance Diurnal pattern Day or night Us

56、ually day, never during sleep Convulsion Stereotypic synchro- Atypical, irregular movements nous Movements Forced eye closure , Crying Consciousness Unconsciousness(coma) Confusion, Twilight state Pupillary and Dilatation Dilatation or normal Reactivity to light Loss Normal Physiological reflex Disa

57、ppear Normal Babinski reflex (+) (-) Enuresis (+) (-) Part 5: Diagnosis and Differential DiagnosisPart 5: Diagnosis and Differential Diagnosis Table 2. General Differentiation of Absence Seizure from SyncopeCharacteristic Absence Seizure SyncopeCauses Unclear(Inheritance) Vasodepressor syncope Ortho

58、static hypotension Cardiac origin Hypoglycemia Aura No Dizziness, Visual blurring or loss Palpitation(心悸心悸), Short of breath Onset Sudden Gradual Facial color Normal Pallor Drop attack No More commonPart 5: Diagnosis and Differential DiagnosisDiagnosis of Epilepsy-Answer Question1. Whether the seizu

59、re is epilepsy or not2.Which types of epilepsy3. Idiopathic or Symptomatic or Cryptogenic EpilepsyPart 5: Diagnosis and Differential DiagnosisDiagnosis of Epilepsy-Answer some question2. Which types of epilepsy? An attempt should be made at the same time to categorise the seizure according to classi

60、fication,because it is greatly important for correct therapy.5.3 The Type of Epileptic Seizure Have to Be Diagnosis for Treatment.Part 5: Diagnosis and Differential DiagnosisPartial seizures(seizures beginning locally) A. Simple partial seizures B. Complex partial seizures C . Partial seizures secon