Classification of

• Traditionally divided into “ grand mal” and “petit mal” seizures Generalized • ILAE classification of epileptic seizures in 1981 based on clinical observation and EEG findings • Seizures were divided into partial and generalized Bassel F. Shneker seizures based on loss of consciousness • Partial seizures were divided into simple partial and complex partial based on alteration of consciousness

Classification of Seizures Classification of Seizures Partial Seizures Generalized Seizures Complex Partial Seizures (CPS) Tonic-Clonic (primary tonic-clonic) –With automatism Absence –Without automatism Myoclonic Simple Partial Seizures (SPS) Clonic –Motor Tonic Seizures oWith march oWithout march Atonic oVersive Atypical Absence Loss of Consciousness? oPostural oPhonatory Infantile Spasm –Sensory Yes No oSomatosensory oOlfactory Generalized oVisual Partial Seizures oAuditory Seizures oGustatory oVertiginous Alteration of Consciousness? –Autonomic –Psychiatric oDysphasic Yes No oDéjà vu or jamais vu oCognitive oAffective oIllusions Complex Partial Simple Partial oStructured hallucinations Secondary Generalized Tonic-Clonic

1 Classification of Classification of Epilepsy Localization-Related (named by location) Generalized (named by disease) Idiopathic Benign (Benign childhood epilepsy with Benign Neonatal (+/- familial) centro-temporal spikes) Benign in infancy Benign occipital epilepsy of childhood Childhood absence epilepsy • ILAE classification of epilepsy and Autosomal dominant nocturnal Juvenile absence epilepsy Primary Reading Epilepsy Juvenile myoclonic epilepsy epileptic seizures in 1989 Epilepsy with GTCs on awakening Some reflex epilepsies • Depends on 2 distinctions; Symptomatic Temporal lobe Early myoclonic encephalopathy Frontal lobe Early infantile epileptic encephalopathy – Location of pathology (Localized or Parietal lobe with suppression- burst (Ohtahara’s Occipital lobe syndrome) generalized) Cortical abnormalities (Rasmussen’s encephalitis) -malformations (Most Reflex epilepsies) -dysplasias – Know or presumed etiology Metabolic abnormalities • Idiopathic - amino acidurias - organic acidurias • Symptomatic - mitochondrial diseases - progressive encephalopathies of childhood • Cryptogenic 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) Generalized Seizures • Generalized tonic-clonic • Characterized by the presence of generalized IED • Tonic or generalized ictal discharges • Clonic • EEG in GE – Spike and slow wave (> 3 Hz indicate better prognosis • Absence – Multiple spike and slow wave (poly spike and wave) • Atypical absence – Electrodecremental response ( attenuation of EEG) • Myoclonic – Generalized paroxysmal fast activities (GPFA) • Atonic • Infantile spasm

2 Idiopathic Generalized Epilepsies (IGE) Benign Neonatal Familial Convulsions

• Benign Neonatal Familial Convulsions • Inherited (AD) • Benign Neonatal Convulsions • Rare • Benign Myoclonic Epilepsy in Infancy • Onset: second or third days of life (second day fit) • Childhood Absence Epilepsy (CAE) • SZs: clonic, apneic • Juvenile Absence Epilepsy (JAE) • EEG: non specific • Juvenile Myoclonic Epilepsy (JME) • 14% develop epilepsy • Epilepsy with GTCs on Awakening

Benign Neonatal Convulsions Benign Myoclonic Epilepsy in Infancy

• Described in 1981 by Dravet and Bureau • Onset: fifth day of life (fifth day fit) • ? Childhood form of JME • SZs: clonic, apneic • Rare • EEG: alternating sharp theta waves • FH: 1/3 with convulsions or epilepsy • Onset: first or second year of life • Prognosis : 100 % no recurrence • 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 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 - 80-95 % response • Prognosis: 70 % resolve by age 14, 30 % persist (more GTC, easy to control) • 8 year old with staring spells

Juvenile Absence Epilepsy (JAE)

• Described recently 1980s • ? Underdiagnosed (? As frequent as JME) • FH: AD or complex • Onset : 10-17 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 • 9 yo girl with staring spells and new onset generalized convulsions.

4 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: 12-18 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 Symptomatic Generalized Epilepsies Epilepsy with GTCs on Awakening (SGE)

• Rare • Early Myoclonic Encephalopathy • FH: frequently positive • Early Infantile Epileptic Encephalopathy with • Onset: 10-20 years Suppression-Burst (Ohtahara’s Syndrome) • SZs: GTC (90% on awakening), ABS, MYO • West’s Syndrome • EEG: generalized epileptiform discharges, frequent • Lennox – Gastaut Syndrome photosensitivity • Malformations • Treatment: Tx of GTC • Proven or Suspected Inborn Erros of Metabolism • Prognosis: variable

Early Infantile Epileptic Encephalopathy with Early Myoclonic Encephalopathy Suppression-Burst (Ohtahara’s Syndrome)

• FH: frequent • FH: ? • Onset: < 3 months • Onset: first few months • Etiology: inborn errors of metabolism • Etiology: cortical malformation • Szs: fragmentary myoclonus, massive myoclonias, tonic • Szs: tonic spasm, myoclonus (rare) spasm • EEG: - Interictal: suppression-burst pattern • EEG: - Interictal: suppression-burst pattern - Ictal: electrodecrement - Ictal: Generalized multiple spikes, slow S/W • Tx: unsatisfactory • Tx: unsatisfactory • Prognosis: arrest of development, death early • Prognosis: arrest of development, death during first year

6 West Syndrome (blitz-Nick-Salaam Krampfe)

• Triad : infantile spasm, static encephalopathy, • 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 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 = • 8 yo boy with static encephalopathy and generalized seizures. FBM, VPA Meds = PB. State = awake.

8 Cryptogenic Generalized Epilepsies Epilepsy with Myoclonic-Astatic Seizures (CGE)

• Epilepsy with Myoclonic-Astatic Seizures • FH: frequent • Epilepsy with Myoclonic Absences • Onset: 6 months-6 years, boys more affected • West Syndrome • Szs: MYO, astatic, myoclonic-astatic, ABS, tonic, GTC • Lennox-Gastaut Syndrome • 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

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