Classification of Seizures. Generalized Epilepsies. Classification of Seizures. Classification of Seizures. Bassel F. Shneker

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1 Classification of Seizures Generalized Epilepsies Bassel F. Shneker Traditionally divided into grand mal and petit mal seizures ILAE classification of epileptic seizures in 1981 based on clinical observation and EEG findings Seizures were divided into partial and generalized seizures based on loss of consciousness Partial seizures were divided into simple partial and complex partial based on alteration of consciousness Classification of Seizures Yes Generalized Seizures Seizures Loss of Consciousness? No Partial Seizures Alteration of Consciousness? Yes Complex Partial No Simple Partial Partial Seizures Classification of Seizures Complex Partial Seizures (CPS) With automatism Without automatism Simple Partial Seizures (SPS) Motor Sensory owith march owithout march oversive opostural ophonatory osomatosensory oolfactory ovisual oauditory ogustatory Autonomic Psychiatric overtiginous odysphasic odéjà vu or jamais vu ocognitive oaffective oillusions ostructured hallucinations Secondary Generalized Tonic-Clonic Generalized Seizures Tonic-Clonic (primary tonic-clonic) Absence Myoclonic Clonic Tonic Atonic Atypical Absence Infantile Spasm 1

2 Classification of Epilepsy ILAE classification of epilepsy and epileptic seizures in 1989 Depends on 2 distinctions; Location of pathology (Localized or generalized) Know or presumed etiology Idiopathic Symptomatic Cryptogenic Classification of Epilepsy Localization-Related (named by location) Generalized (named by disease) Idiopathic Benign Rolandic epilepsy (Benign childhood epilepsy with centro-temporal spikes) Benign Neonatal Convulsions (+/- familial) Benign myoclonic epilepsy in infancy Benign occipital epilepsy of childhood Childhood absence epilepsy Autosomal dominant nocturnal frontal lobe epilepsy Juvenile absence epilepsy Primary Reading Epilepsy Juvenile myoclonic epilepsy Epilepsy with GTCs on awakening Some reflex epilepsies Symptomatic Temporal lobe Frontal lobe Early myoclonic encephalopathy Early infantile epileptic encephalopathy Parietal lobe with suppression- burst (Ohtahara s Occipital lobe syndrome) (Rasmussen s encephalitis) Cortical abnormalities -malformations (Most Reflex epilepsies) -dysplasias Metabolic abnormalities - amino acidurias - organic acidurias - mitochondrial diseases - progressive encephalopathies of childhood West s Syndrome Lennox-Gastaut Syndrome Cryptogenic (Any occurrence of partial seizures without obvious pathology.) Epilepsy with myoclonic-astatic seizures Epilepsy with myoclonic absences Generalized Epilepsies (GE) Characterized by the presence of generalized IED or generalized ictal discharges EEG in GE Spike and slow wave (> 3 Hz indicate better prognosis Multiple spike and slow wave (poly spike and wave) Electrodecremental response ( attenuation of EEG) Generalized paroxysmal fast activities (GPFA) Generalized Seizures Generalized tonic-clonic Tonic Clonic Absence Atypical absence Myoclonic Atonic Infantile spasm 2

3 Idiopathic Generalized Epilepsies (IGE) Benign Neonatal Familial Convulsions Benign Neonatal Convulsions Benign Myoclonic Epilepsy in Infancy Childhood Absence Epilepsy (CAE) Juvenile Absence Epilepsy (JAE) Juvenile Myoclonic Epilepsy (JME) Epilepsy with GTCs on Awakening Benign Neonatal Familial Convulsions Inherited (AD) Rare Onset: second or third days of life (second day fit) SZs: clonic, apneic EEG: non specific 14% develop epilepsy Benign Neonatal Convulsions Onset: fifth day of life (fifth day fit) SZs: clonic, apneic EEG: alternating sharp theta waves Prognosis : 100 % no recurrence Benign Myoclonic Epilepsy in Infancy Described in 1981 by Dravet and Bureau? Childhood form of JME Rare FH: 1/3 with convulsions or epilepsy Onset: first or second year of life Szs: 1-3 Second. bursts of Generalized myoclonus, rare GTC (adolescence) EEG: generalized bursts of S/W during light sleep Treatment: VPA Prognosis: good 3

4 Childhood Absence Epilepsy (CAE) (Pyknolepsy) Described in 1900s 2-8 % of all epilepsies FH: AD or complex Onset : 4-10 years ( rare <3, > 11) SZs ABS (? Clonic or myoclonic components), GTC (40 %) EEG: 3 cps generalized S/W, OIRDA (interictally) Treatment: - ESM ( ABS only), VPA, LAM % response Prognosis: 70 % resolve by age 14, 30 % persist (more GTC, easy to control) 8 year old with staring spells Juvenile Absence Epilepsy (JAE) 9 yo girl with staring spells and new onset generalized convulsions. Described recently 1980s? Underdiagnosed (? As frequent as JME) FH: AD or complex Onset : years (overlap with ACE ) SZs: ABS ( not as frequent,?less LOC), GTC (80%), MYO (15%) EEG: as CAE but less regular, multiple S/W Treatment: - VPA, LAM - 80 % response Prognosis: persist more than CAE 4

5 Ref = Balanced Ear 26 yo woman with absences and occ myoclonus. No meds. Juvenile Myoclonic Epilepsy (JME) (Syndrome of Janz) 5-11 % of all epilepsies FH: AD or complex Onset: years SZs: MYO (single), GTC (90-95%) (ctc),abs(30%) EEG: 4-6 cps multiple S/W, frequent photosensitivity (30%) Treatment : VPA (80-90% response), LAM, PHT ( GTC), Clonazepam (MYO) Prognosis: persist but controlled 5

6 Epilepsy with GTCs on Awakening Rare FH: frequently positive Onset: years SZs: GTC (90% on awakening), ABS, MYO EEG: generalized epileptiform discharges, frequent photosensitivity Treatment: Tx of GTC Prognosis: variable Symptomatic Generalized Epilepsies (SGE) Early Myoclonic Encephalopathy Early Infantile Epileptic Encephalopathy with Suppression-Burst (Ohtahara s Syndrome) West s Syndrome Lennox Gastaut Syndrome Malformations Proven or Suspected Inborn Erros of Metabolism Early Myoclonic Encephalopathy FH: frequent Onset: < 3 months Etiology: inborn errors of metabolism Szs: fragmentary myoclonus, massive myoclonias, tonic spasm EEG: - Interictal: suppression-burst pattern - Ictal: Generalized multiple spikes, slow S/W Tx: unsatisfactory Prognosis: arrest of development, death during first year Early Infantile Epileptic Encephalopathy with Suppression-Burst (Ohtahara s Syndrome) FH:? Onset: first few months Etiology: cortical malformation Szs: tonic spasm, myoclonus (rare) EEG: - Interictal: suppression-burst pattern - Ictal: electrodecrement Tx: unsatisfactory Prognosis: arrest of development, death early 6

7 West Syndrome (blitz-nick-salaam Krampfe) Triad : infantile spasm, static encephalopathy, hypsarrhythmia Onset: 4-7 months, boys affected more Etiology: Symptomatic, Cryptogenic, Idiopathic EEG: - Interictal: hypsarrhythmia - Ictal: electrodecrement Tx: ACTH (? long term improvement), BZD, VPA, Vigabatrin Prognosis: 1-2% recover, 30% develop LGS 3 month boy with infantile spasms. 9mo male infant with congenital midline defects, developmental delay, and infantile spasms 7

8 Lennox-Gastaut Syndrome(LGS) 3-10% of all childhood epilepsies Triad: mixed seizures, static encephalopathy, slow S/W Onset: 1-8 years Etiology: Symptomatic, Cryptogenic Szs: tonic, atonic, atypical absence, GTC, MYO EEG: - Interictal: slow S/W ( < 3 cps), GPFA during sleep - Ictal: non specific Tx: VPA, FBM, LMT, TPM, Vigabatrine Prognosis: seizures difficult to control Polyspike and atypical spike wave 48 yo woman with Lennox-Gastaut syndrome. Medications = FBM, VPA 8 yo boy with static encephalopathy and generalized seizures. Meds = PB. State = awake. 8

9 Cryptogenic Generalized Epilepsies (CGE) Epilepsy with Myoclonic-Astatic Seizures Epilepsy with Myoclonic Absences West Syndrome Lennox-Gastaut Syndrome Epilepsy with Myoclonic-Astatic Seizures FH: frequent Onset: 6 months-6 years, boys more affected Szs: MYO, astatic, myoclonic-astatic, ABS, tonic, GTC EEG: theta BKG, irregular fast spikes, fast poly S/W Prognosis: variable Epilepsy with Myoclonic Absences Onset: 7 years, boys more affected, mental retardation Szs: ABS accompanied by severe bilateral clonic jerks and tonic contraction EEG: similar to CAE Tx: VPA, LMT Prognosis: less favorable than CAE, may evolve into LGS 9