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Epilepsy Semiology diagnosis Treatment M. Scott Perry, M.D. Emory University April 18, 2007 September 18, 2006 QuickTime™ and a DV/DVCPRO - NTSC decompressor are needed to see this picture.

Introduction To Epilepsy Semiology diagnosis Treatment M. Scott Perry, M.D. Emory University April 18, 2007 September 18, 2006 M. Scott Perry, M.D. Emory

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Page 1: Introduction To Epilepsy Semiology diagnosis Treatment M. Scott Perry, M.D. Emory University April 18, 2007 September 18, 2006 M. Scott Perry, M.D. Emory

Introduction To Epilepsy

Semiology diagnosis Treatment

Introduction To Epilepsy

Semiology diagnosis Treatment

M. Scott Perry, M.D.Emory University

April 18, 2007September 18, 2006

M. Scott Perry, M.D.Emory University

April 18, 2007September 18, 2006

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Page 2: Introduction To Epilepsy Semiology diagnosis Treatment M. Scott Perry, M.D. Emory University April 18, 2007 September 18, 2006 M. Scott Perry, M.D. Emory

ObjectivesObjectives•Recognize different types of seizures.

•Discuss workup for new onset seizures

•Learn classification of epilepsy types based on history, seizure type, MRI, and EEG findings

•Review common treatments used in epilepsy

•Learn prognosis based on epilepsy type

•Briefly review some frequently asked questions

•Recognize different types of seizures.

•Discuss workup for new onset seizures

•Learn classification of epilepsy types based on history, seizure type, MRI, and EEG findings

•Review common treatments used in epilepsy

•Learn prognosis based on epilepsy type

•Briefly review some frequently asked questions

Page 3: Introduction To Epilepsy Semiology diagnosis Treatment M. Scott Perry, M.D. Emory University April 18, 2007 September 18, 2006 M. Scott Perry, M.D. Emory

SpellsSpells

Seizure EquivalentsSeizure EquivalentsGERDGERD

Breath HoldingBreath HoldingInfantile MasturbationInfantile Masturbation

SyncopeSyncopeBenign Sleep Benign Sleep

MyoclonusMyoclonus

SeizureSeizure

Recurrent Recurrent (Epilepsy)(Epilepsy)

SymptomaticSymptomaticElectrolytesElectrolytes

TraumaTraumaIngestionIngestion

Page 4: Introduction To Epilepsy Semiology diagnosis Treatment M. Scott Perry, M.D. Emory University April 18, 2007 September 18, 2006 M. Scott Perry, M.D. Emory

Case 1Case 1

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Page 5: Introduction To Epilepsy Semiology diagnosis Treatment M. Scott Perry, M.D. Emory University April 18, 2007 September 18, 2006 M. Scott Perry, M.D. Emory

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Page 6: Introduction To Epilepsy Semiology diagnosis Treatment M. Scott Perry, M.D. Emory University April 18, 2007 September 18, 2006 M. Scott Perry, M.D. Emory

Seizure ImitatorsSeizure Imitators

•Benign Neonatal Sleep Myoclonus

•Myoclonic jerks are focal, multifocal, unilateral or bilateral

•1-5 hz, distal>proximal

•Begins in first weeks, diminishes by 2nd month, generally gone by 6 months

•Episodes may be exacerbated by benzos

•Benign Neonatal Sleep Myoclonus

•Myoclonic jerks are focal, multifocal, unilateral or bilateral

•1-5 hz, distal>proximal

•Begins in first weeks, diminishes by 2nd month, generally gone by 6 months

•Episodes may be exacerbated by benzos

Page 7: Introduction To Epilepsy Semiology diagnosis Treatment M. Scott Perry, M.D. Emory University April 18, 2007 September 18, 2006 M. Scott Perry, M.D. Emory

Seizure ImitatorsBreath Holding Spells

Seizure ImitatorsBreath Holding Spells

• Incidence: 4.6% (population study, N=4980)

• Onset: 6-18 months

• 90% resolve by age 6y

• cyanotic and pallid

• Incidence: 4.6% (population study, N=4980)

• Onset: 6-18 months

• 90% resolve by age 6y

• cyanotic and pallid

Page 8: Introduction To Epilepsy Semiology diagnosis Treatment M. Scott Perry, M.D. Emory University April 18, 2007 September 18, 2006 M. Scott Perry, M.D. Emory

CYANOTIC BREATH-HOLDING SPELLS

CYANOTIC BREATH-HOLDING SPELLS

• 60 % are cyanotic

• stimulus triggered (anger, frustration)

• short cry

• breathing interrupted in expiration

• cyanotic, limp, LOC

• +/- sleep

• 60 % are cyanotic

• stimulus triggered (anger, frustration)

• short cry

• breathing interrupted in expiration

• cyanotic, limp, LOC

• +/- sleep

Page 9: Introduction To Epilepsy Semiology diagnosis Treatment M. Scott Perry, M.D. Emory University April 18, 2007 September 18, 2006 M. Scott Perry, M.D. Emory

COMPLICATED BREATH-HOLDING

SPELLS

COMPLICATED BREATH-HOLDING

SPELLS• Breath-holding spells + seizure-like

activity

• usually more prolonged

• 15% have complicated features

• clonic activity follows LOC

• stiffening

• Breath-holding spells + seizure-like activity

• usually more prolonged

• 15% have complicated features

• clonic activity follows LOC

• stiffening

Page 10: Introduction To Epilepsy Semiology diagnosis Treatment M. Scott Perry, M.D. Emory University April 18, 2007 September 18, 2006 M. Scott Perry, M.D. Emory

Seizures:What information is

useful?

Seizures:What information is

useful?•What was the patient doing when it started? Unresponsive?...are you sure? Asleep or awake?

•Tell us exactly what you saw:

•E.R.B.S.A.O?

•Does it make anatomical sense? Same side, both sides, just arms, etc.

•How long did it last?

•What was the patient doing when it started? Unresponsive?...are you sure? Asleep or awake?

•Tell us exactly what you saw:

•E.R.B.S.A.O?

•Does it make anatomical sense? Same side, both sides, just arms, etc.

•How long did it last?

Page 11: Introduction To Epilepsy Semiology diagnosis Treatment M. Scott Perry, M.D. Emory University April 18, 2007 September 18, 2006 M. Scott Perry, M.D. Emory

Clinical Characteristics of Seizures in Neonates (Scher,

et al 1989)

Clinical Characteristics of Seizures in Neonates (Scher,

et al 1989)•No accepted classification for neonatal seizures

•80 neonates with suspicious movements. Only 8 had electrographic seizures.

•Focal/multifocal clonic: 44% epileptic

•“Subtle seizures”-roving eye movements, arrest of behavior, lip smacking, autonomic-30%

•Tonic(focal or generalized) 8%

•Myoclonic 7%

•No accepted classification for neonatal seizures

•80 neonates with suspicious movements. Only 8 had electrographic seizures.

•Focal/multifocal clonic: 44% epileptic

•“Subtle seizures”-roving eye movements, arrest of behavior, lip smacking, autonomic-30%

•Tonic(focal or generalized) 8%

•Myoclonic 7%

Page 12: Introduction To Epilepsy Semiology diagnosis Treatment M. Scott Perry, M.D. Emory University April 18, 2007 September 18, 2006 M. Scott Perry, M.D. Emory

Clinical Characteristics of Seizures in Neonates (Scher,

et al. 1993)

Clinical Characteristics of Seizures in Neonates (Scher,

et al. 1993)

•92 neonates with electrographic seizures (345 EEG recordings)

•48% had electroclinical event

•Subtle 71%, clonic 41%, myoclonic 20%, tonic 9%

•34% with only electrographic events

•17/90 (19%) of paralyzed neonates had electrographic events

•92 neonates with electrographic seizures (345 EEG recordings)

•48% had electroclinical event

•Subtle 71%, clonic 41%, myoclonic 20%, tonic 9%

•34% with only electrographic events

•17/90 (19%) of paralyzed neonates had electrographic events

Page 13: Introduction To Epilepsy Semiology diagnosis Treatment M. Scott Perry, M.D. Emory University April 18, 2007 September 18, 2006 M. Scott Perry, M.D. Emory

Clinical Seizures in Neonates

Clinical Seizures in Neonates

•Duration- average duration 2.25 minutes. Usually shorter, rarely longer. Intertictal recovery 8 minutes (Clancy and Legido, 1987)

•Status Epilepticus- clinical SE is rare, electrographic may not be

•487 seizures, only 2 SE (Clancy, et al)

•33% FT infants, 9% PT (Scher, et al)

•Duration- average duration 2.25 minutes. Usually shorter, rarely longer. Intertictal recovery 8 minutes (Clancy and Legido, 1987)

•Status Epilepticus- clinical SE is rare, electrographic may not be

•487 seizures, only 2 SE (Clancy, et al)

•33% FT infants, 9% PT (Scher, et al)

•Gen. Tonic Clonic seizures don’t happen in neonates.

•69 infants, 101 seizures, only 4 resembled GTCS, none truly were. (Nordli, et al.)

Page 14: Introduction To Epilepsy Semiology diagnosis Treatment M. Scott Perry, M.D. Emory University April 18, 2007 September 18, 2006 M. Scott Perry, M.D. Emory

Seizure Semiology of Neonates

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Focal/Multifocal ClonicTonic

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Subtle

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Tremors

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Page 15: Introduction To Epilepsy Semiology diagnosis Treatment M. Scott Perry, M.D. Emory University April 18, 2007 September 18, 2006 M. Scott Perry, M.D. Emory

Seizure TypesSeizure Types

PartialPartial GeneralizedGeneralized

Simple Partial Simple Partial Complex PartialComplex Partial

Partial SecondarilyPartial Secondarily GeneralizedGeneralized

Page 16: Introduction To Epilepsy Semiology diagnosis Treatment M. Scott Perry, M.D. Emory University April 18, 2007 September 18, 2006 M. Scott Perry, M.D. Emory

Simple partialSimple partial

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•Preserved consciousnessPreserved consciousness•Isolated motor/sensory Isolated motor/sensory •Complex partial involves loss of consciousnessComplex partial involves loss of consciousness

Page 17: Introduction To Epilepsy Semiology diagnosis Treatment M. Scott Perry, M.D. Emory University April 18, 2007 September 18, 2006 M. Scott Perry, M.D. Emory

Partial Secondarily Generalized

Partial Secondarily Generalized

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•Starts partial, rapidly spreadsStarts partial, rapidly spreads•You have to ask the questions to get the You have to ask the questions to get the answersanswers

Page 18: Introduction To Epilepsy Semiology diagnosis Treatment M. Scott Perry, M.D. Emory University April 18, 2007 September 18, 2006 M. Scott Perry, M.D. Emory

Partial Seizure CluesPartial Seizure Clues

•Contralateral

•Head Deviation, Eye Deviation, Dystonic Posturing, Unilateral Clonic Activity, Postictal Paralysis

•Ipsilateral

•Automatisms, Eye Blinking, Nose Wiping

•Contralateral

•Head Deviation, Eye Deviation, Dystonic Posturing, Unilateral Clonic Activity, Postictal Paralysis

•Ipsilateral

•Automatisms, Eye Blinking, Nose Wiping

Page 19: Introduction To Epilepsy Semiology diagnosis Treatment M. Scott Perry, M.D. Emory University April 18, 2007 September 18, 2006 M. Scott Perry, M.D. Emory

Differentiating Seizure Types - Semiology

Differentiating Seizure Types - Semiology

Partial SeizuresPartial Seizures

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Head and Eye Head and Eye DeviationDeviation

Page 20: Introduction To Epilepsy Semiology diagnosis Treatment M. Scott Perry, M.D. Emory University April 18, 2007 September 18, 2006 M. Scott Perry, M.D. Emory

What do you see?What do you see?

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1. Head 1. Head DeviationDeviation2. Automatism2. Automatism3. Eye Deviation3. Eye Deviation4. Unilateral 4. Unilateral Dystonic/Clonic Dystonic/Clonic ActivityActivity

Page 21: Introduction To Epilepsy Semiology diagnosis Treatment M. Scott Perry, M.D. Emory University April 18, 2007 September 18, 2006 M. Scott Perry, M.D. Emory

Seizure Types

PartialPartial GeneralizedGeneralized

Simple Partial Simple Partial Complex PartialComplex Partial

Partial SecondarilyPartial Secondarily GeneralizedGeneralized

Generalized Tonic ClonicGeneralized Tonic ClonicTonicTonicClonicClonicAtonicAtonic

MyoclonicMyoclonicAbsenceAbsence

Page 22: Introduction To Epilepsy Semiology diagnosis Treatment M. Scott Perry, M.D. Emory University April 18, 2007 September 18, 2006 M. Scott Perry, M.D. Emory

Generalized Seizure Generalized Seizure MyoclonicMyoclonic

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• Characterized by quick, Characterized by quick, arrhythmic, and arrhythmic, and symmetric/asymmetric movementssymmetric/asymmetric movements•Often not reported by patients.Often not reported by patients.•Ask about sudden falls or dropping Ask about sudden falls or dropping objectsobjects

Page 23: Introduction To Epilepsy Semiology diagnosis Treatment M. Scott Perry, M.D. Emory University April 18, 2007 September 18, 2006 M. Scott Perry, M.D. Emory

See the Difference?See the Difference?

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Myoclonic-fast, Myoclonic-fast, jerking motionjerking motion

Clonic-rhythmicClonic-rhythmic

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Page 24: Introduction To Epilepsy Semiology diagnosis Treatment M. Scott Perry, M.D. Emory University April 18, 2007 September 18, 2006 M. Scott Perry, M.D. Emory

SemiologySemiologyTypical Absence SeizuresTypical Absence Seizures

•Characterized by brief, abrupt impairment of consciousness associated with EEG demonstrating 3 Hz spike and slow wave complexes with normal interictal background•May also demonstrate:•mild clonic, tonic, or atonic components•automatisms•autonomic components

Panayiotopoulos “The Epilepsies: Seizures,

Syndromes, and Manangement

Page 25: Introduction To Epilepsy Semiology diagnosis Treatment M. Scott Perry, M.D. Emory University April 18, 2007 September 18, 2006 M. Scott Perry, M.D. Emory

Generalized Seizure Semiology

Generalized Seizure SemiologyInfantile SpasmsInfantile Spasms

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Page 26: Introduction To Epilepsy Semiology diagnosis Treatment M. Scott Perry, M.D. Emory University April 18, 2007 September 18, 2006 M. Scott Perry, M.D. Emory

Review So FarReview So Far

•Common seizure imitators in pediatrics

•Seizures come in two basic types. You have to ask the right questions to distinguish them

•Now...how do you diagnose epilepsy (i.e. when is EEG/MRI necessary) and why do we care?

•Common seizure imitators in pediatrics

•Seizures come in two basic types. You have to ask the right questions to distinguish them

•Now...how do you diagnose epilepsy (i.e. when is EEG/MRI necessary) and why do we care?

Page 27: Introduction To Epilepsy Semiology diagnosis Treatment M. Scott Perry, M.D. Emory University April 18, 2007 September 18, 2006 M. Scott Perry, M.D. Emory

Epilepsy Types

Partial Generalized

IdiopathicBenign Rolandic

EpilepsyBenign Occipital

Epilepsy

Symptomatic

Idiopathic Symptomatic

West SyndromeLennox-Gastaut

Childhood AbsenceJuvenile Absence

Juvenile MyoclonicGrand Mal Upon

Awakening

•Primary = Idiopathic = presumed genetic•Secondary = Symptomatic=underlying cerebral cause (i.e. injury, dyplasia, etc.)

Cryptogenic

Cryptogenic

Page 28: Introduction To Epilepsy Semiology diagnosis Treatment M. Scott Perry, M.D. Emory University April 18, 2007 September 18, 2006 M. Scott Perry, M.D. Emory

Idiopathic Partial EpilepsyBenign Rolandic Epilepsy

(Benign Childhood Epilepsy with Centro-Temporal Spikes)

•Onset 1-14 years, 75% between 7-10 years of age

•Prevalence is 15% of children with seizures

•Characterized by infrequent, often single, focal seizures consisting of unilateral facial sensorimotor symptoms, oropharyngolaryngeal manifestations, speech arrest, or hypersalivation lasting 1-2 minutes

•1/3 -2/3 will have secondarily generalized seizures

•75% are nocturnal

•MRI normal

•Typical EEG

Page 29: Introduction To Epilepsy Semiology diagnosis Treatment M. Scott Perry, M.D. Emory University April 18, 2007 September 18, 2006 M. Scott Perry, M.D. Emory
Page 30: Introduction To Epilepsy Semiology diagnosis Treatment M. Scott Perry, M.D. Emory University April 18, 2007 September 18, 2006 M. Scott Perry, M.D. Emory

Benign Rolandic EpilepsyPrognosis/Treatment

•2-3% school age children have CT spikes with <10% having BRE•Remission usually within 2-4 years from onset and before the age of 16 years•Less than 2% will develop infrequent generalized seizures in adulthood•Treat or not to treat

Page 31: Introduction To Epilepsy Semiology diagnosis Treatment M. Scott Perry, M.D. Emory University April 18, 2007 September 18, 2006 M. Scott Perry, M.D. Emory

Benign Occipital EpilepsyGastaut Type

•Onset 3-15 years•Manifest as visual hallucinations, blindness, or both-lasting seconds to <3 minutes•Rarely terminate with hemiconvulsions or generalized convulsion•50% have postictal headache•Similar manifestation to seizures from occipital lesions - MRI needed•Typical EEG

Page 32: Introduction To Epilepsy Semiology diagnosis Treatment M. Scott Perry, M.D. Emory University April 18, 2007 September 18, 2006 M. Scott Perry, M.D. Emory

Benign Occipital EpilepsyFixation-Off EEG

Page 33: Introduction To Epilepsy Semiology diagnosis Treatment M. Scott Perry, M.D. Emory University April 18, 2007 September 18, 2006 M. Scott Perry, M.D. Emory

Benign Occipital EpilepsyPrognosis/Treatment

•Remission occurs 2-4 years from onset for 50-60% of patients•Dramatic response to carbamazepine in >90%•15% association with celiac disease

Page 34: Introduction To Epilepsy Semiology diagnosis Treatment M. Scott Perry, M.D. Emory University April 18, 2007 September 18, 2006 M. Scott Perry, M.D. Emory

Symptomatic Partial Epilepsy

Symptomatic Partial Epilepsy

•Abnormal MRI (stroke, dyplasia, etc.) or abnormal EEG without classic pattern

•History not consistent with primary partial epilepsy

•Prognosis varies

•Abnormal MRI (stroke, dyplasia, etc.) or abnormal EEG without classic pattern

•History not consistent with primary partial epilepsy

•Prognosis varies

Page 35: Introduction To Epilepsy Semiology diagnosis Treatment M. Scott Perry, M.D. Emory University April 18, 2007 September 18, 2006 M. Scott Perry, M.D. Emory

Secondary Partial Epilepsy - MRI

Secondary Partial Epilepsy - MRI

HeterotopiaHeterotopia Mesial Temporal SclerosisMesial Temporal Sclerosis

Page 36: Introduction To Epilepsy Semiology diagnosis Treatment M. Scott Perry, M.D. Emory University April 18, 2007 September 18, 2006 M. Scott Perry, M.D. Emory

Idiopathic Generalized Epilepsy

Childhood Absence Epilepsy

•Onset 2-10 years, peak 5-6 years•2/3 are females•Abrupt cessation of activity or speech last 4-20 seconds followed by return to baseline •Normal MRI•Typical EEG with 3Hz SW often provoked with HV

Page 37: Introduction To Epilepsy Semiology diagnosis Treatment M. Scott Perry, M.D. Emory University April 18, 2007 September 18, 2006 M. Scott Perry, M.D. Emory

Idiopathic Generalized Epilepsy

Childhood Absence Epilepsy

Page 38: Introduction To Epilepsy Semiology diagnosis Treatment M. Scott Perry, M.D. Emory University April 18, 2007 September 18, 2006 M. Scott Perry, M.D. Emory

Idiopathic Generalized Epilepsy

Childhood Absence Epilepsy

Prognosis/Treatment•Remission often occurs before 12 years of age•Less than 10% develop infrequent generalized tonic clonic seizures in adolescence or adult life•Rarely will patients continue to have absence seizures as adults•Treatment with valproic acid, ethosuximide, or lamotrigine will control absences in >80%•Possible role for topiramate and levetiracetam

Page 39: Introduction To Epilepsy Semiology diagnosis Treatment M. Scott Perry, M.D. Emory University April 18, 2007 September 18, 2006 M. Scott Perry, M.D. Emory

Idiopathic Generalized Epilepsy

Juvenile Absence Epilepsy•Age of onset 9-13 years

•80% suffer from GTCS and 15-25% have Myoclonic seizures with onset 1-10 years after absences•Frequent/severe absences•Absence status in 20%•Prognosis: 70-80% will be controlled, though this is a lifelong disorder•20% may have intractable absences and GTCS

Page 40: Introduction To Epilepsy Semiology diagnosis Treatment M. Scott Perry, M.D. Emory University April 18, 2007 September 18, 2006 M. Scott Perry, M.D. Emory

Idiopathic Generalized Epilepsy

Juvenile Myoclonic Epilepsy

•Characterized by myoclonic jerks upon awakening starting in adolescence•GTCS (>90%) may begin a few months later, occasionally earlier•Absence seizures (33%), if present, begin between 5-16 years•M:F equal•Seizure precipitants: Sleep deprivation, alcohol, stress, video games

Page 41: Introduction To Epilepsy Semiology diagnosis Treatment M. Scott Perry, M.D. Emory University April 18, 2007 September 18, 2006 M. Scott Perry, M.D. Emory

Idiopathic Generalized Epilepsy

Juvenile Myoclonic Epilepsy•EEG: irregular generalized 3-6hz spike/polyspike-slow

wave discharges and generalized fragments. 33% have photoparoxysmal responses

Page 42: Introduction To Epilepsy Semiology diagnosis Treatment M. Scott Perry, M.D. Emory University April 18, 2007 September 18, 2006 M. Scott Perry, M.D. Emory

Idiopathic Generalized Epilepsy

Juvenile Myoclonic Epilepsy

Treatment/Prognosis•Valproic Acid, levetiracetam most commonly used monotherapy treatment•Lamotrigine, clonazepam•Prognosis: Seizures well controlled in up to 90% of patients. Treatment is lifelong, as 80% relapse after drug withdrawal•Carbamazepine, oxcarbazepine, phenytoin, gabapentin, tiagabine, and vigabatrin are contraindicated•Lifestyle management with regards to alcohol use, sleep deprivation, etc.

Page 43: Introduction To Epilepsy Semiology diagnosis Treatment M. Scott Perry, M.D. Emory University April 18, 2007 September 18, 2006 M. Scott Perry, M.D. Emory

Symptomatic Generalized Epilepsy

Infantile SpasmsWest Syndrome

“...these bobbings...they come on whether sitting or lying; just before they come on he is

all alive and in motion...and then all of a sudden down goes his head and upwards his knees; he then appears frightened and screams out. --

W.J. West (1841)

Page 44: Introduction To Epilepsy Semiology diagnosis Treatment M. Scott Perry, M.D. Emory University April 18, 2007 September 18, 2006 M. Scott Perry, M.D. Emory

Symptomatic Generalized Epilepsy

Infantile SpasmsWest Syndrome•Onset between 3-12 months, peak at 5

months•Incidence: 3-5/10,000•Spasms are flexor, extensor, or combined•Clusters with 20-150 seizures per day, occurring most often on awakening or prior to sleep•Developmental delay preceeds spasms in 2/3•Classified as symptomatic, probably symptomatic, and cryptogenic

Page 45: Introduction To Epilepsy Semiology diagnosis Treatment M. Scott Perry, M.D. Emory University April 18, 2007 September 18, 2006 M. Scott Perry, M.D. Emory

Symptomatic Generalized Epilepsy

Infantile SpasmsWest Syndrome•80% symptomatic with most caused

by pre-, peri-, or post-natal insults (i.e. HIE, ICH, dysplasias, trauma)•50% of patients with TS have spasms•3% of patients with Trisomy 21•Aicardi’s syndrome (spasms, agenesis of the corpus callosum, and retinal lacunes•EEG demonstrates hypsarrhythmia•High voltage, chaotic, arrhythmic and asynchronous which becomes more synchronous in NREM sleep•Multifocal independent spike wave discharges•Periods of electrodecrement

Page 46: Introduction To Epilepsy Semiology diagnosis Treatment M. Scott Perry, M.D. Emory University April 18, 2007 September 18, 2006 M. Scott Perry, M.D. Emory

Symptomatic Generalized Epilepsy

Infantile SpasmsWest Syndrome

Page 47: Introduction To Epilepsy Semiology diagnosis Treatment M. Scott Perry, M.D. Emory University April 18, 2007 September 18, 2006 M. Scott Perry, M.D. Emory

Symptomatic Generalized Epilepsy

Infantile SpasmsPrognosis

•Spasms typically will remit, even without treatment, by 18 months of age•60% of patients develop other seizure types, CPS and Lennox-Gastaut syndrome are most common•90% of patients have developmental delay, 66% are severely cognitively impaired

Page 48: Introduction To Epilepsy Semiology diagnosis Treatment M. Scott Perry, M.D. Emory University April 18, 2007 September 18, 2006 M. Scott Perry, M.D. Emory

Symptomatic Generalized Epilepsy

Infantile SpasmsTreatment•ACTH - 50% remission, all or none.

No proven dosing regimen, no clear reason why it works•Topiramate - similar efficacy usually in high doses (25-30mg/kg/d)•Vigabatrin - especially useful in TS (90%), beware of irreversible visual field defects•Pyridoxine, valproate, zonisamide, levetiracetam, lamotrigine, felbatol, keto diet•Surgery

Page 49: Introduction To Epilepsy Semiology diagnosis Treatment M. Scott Perry, M.D. Emory University April 18, 2007 September 18, 2006 M. Scott Perry, M.D. Emory

Symptomatic Generalized Epilepsy

Lennox-Gastaut Syndrome•Three criteria•Multiple intractable seizures including tonic (80-100%), atypical absence (66%), and atonic (50%)•cognitive and behavioral abnormalities•Slow (<2.5 Hz) generalized spike wave•Onset 1-7 years, peak 3-5•10-30% develop from West syndrome or other epileptic encephalopathies

Page 50: Introduction To Epilepsy Semiology diagnosis Treatment M. Scott Perry, M.D. Emory University April 18, 2007 September 18, 2006 M. Scott Perry, M.D. Emory

Symptomatic Generalized Epilepsy

Lennox-Gastaut Syndrome

Page 51: Introduction To Epilepsy Semiology diagnosis Treatment M. Scott Perry, M.D. Emory University April 18, 2007 September 18, 2006 M. Scott Perry, M.D. Emory

Symptomatic Generalized Epilepsy

Lennox-Gastaut SyndromePrognosis/Treatment•5% die, 80-90% have seizures as

adults, and approximately 90% have severely impaired cognition and behavior•Treatment includes almost every AED with polypharmacy common.•Ketogenic diet, VNS, corpus callosotomy

Page 52: Introduction To Epilepsy Semiology diagnosis Treatment M. Scott Perry, M.D. Emory University April 18, 2007 September 18, 2006 M. Scott Perry, M.D. Emory

Choosing an AEDChoosing an AED

•Type of epilepsy•Type of epilepsy

Page 53: Introduction To Epilepsy Semiology diagnosis Treatment M. Scott Perry, M.D. Emory University April 18, 2007 September 18, 2006 M. Scott Perry, M.D. Emory

Treatment of Epilepsy: AEDs

Treatment of Epilepsy: AEDs

PartialPartial GeneralizedGeneralizedPhenytoinPhenytoin

PhenobarbitalPhenobarbitalValproic AcidValproic Acid

CarbamazepineCarbamazepineOxcarbazepineOxcarbazepine

GabatrilGabatrilGabapentinGabapentinTopiramateTopiramateLamotrigineLamotrigineZonisamideZonisamide

LevetiracetamLevetiracetam

Valproic AcidValproic AcidTopiramateTopiramateZonisamideZonisamideLamotrigineLamotrigine

LevetiracetamLevetiracetamEthosuximideEthosuximide

FelbamateFelbamate

Page 54: Introduction To Epilepsy Semiology diagnosis Treatment M. Scott Perry, M.D. Emory University April 18, 2007 September 18, 2006 M. Scott Perry, M.D. Emory

Choosing an AED

•Type of epilepsy

•Type of formulation (IV, capsule, sprinkle, etc.)

Page 55: Introduction To Epilepsy Semiology diagnosis Treatment M. Scott Perry, M.D. Emory University April 18, 2007 September 18, 2006 M. Scott Perry, M.D. Emory

Choosing an AEDFormulation

Choosing an AEDFormulation

•IV: Benzos, phenytoin, phenobarbital, valproic acid, levetiracetam

•Sprinkles: valproate, topiramate

•Liquids: carbazepine, oxcarb, levetiracetam, valproate, dilantin. zonegran,lamictal,topiramate will dissolve in H20

•Extended release: valproate, carbamazepine

•IV: Benzos, phenytoin, phenobarbital, valproic acid, levetiracetam

•Sprinkles: valproate, topiramate

•Liquids: carbazepine, oxcarb, levetiracetam, valproate, dilantin. zonegran,lamictal,topiramate will dissolve in H20

•Extended release: valproate, carbamazepine

Page 56: Introduction To Epilepsy Semiology diagnosis Treatment M. Scott Perry, M.D. Emory University April 18, 2007 September 18, 2006 M. Scott Perry, M.D. Emory

Choosing an AED

•Type of epilepsy

•Type of formulation (IV, capsule, sprinkle, etc.)

•Time to onset

Page 57: Introduction To Epilepsy Semiology diagnosis Treatment M. Scott Perry, M.D. Emory University April 18, 2007 September 18, 2006 M. Scott Perry, M.D. Emory

Choosing an AEDTime To Onset

Choosing an AEDTime To Onset

•Rapid onset: Any IV form

•Onset in 24 hours: Levetiracetam

•Onset in Days: carbamazepine, oxcarb, dilantin, valproate, zarontin.

•Slow titration: Topiramate, zonisamide

•Really slow: Lamictal

•Rapid onset: Any IV form

•Onset in 24 hours: Levetiracetam

•Onset in Days: carbamazepine, oxcarb, dilantin, valproate, zarontin.

•Slow titration: Topiramate, zonisamide

•Really slow: Lamictal

Page 58: Introduction To Epilepsy Semiology diagnosis Treatment M. Scott Perry, M.D. Emory University April 18, 2007 September 18, 2006 M. Scott Perry, M.D. Emory

Choosing an AED

•Type of epilepsy

•Type of formulation (IV, capsule, sprinkle, etc.)

•Time to onset

•Side Effects

Page 59: Introduction To Epilepsy Semiology diagnosis Treatment M. Scott Perry, M.D. Emory University April 18, 2007 September 18, 2006 M. Scott Perry, M.D. Emory

Choosing An AEDSide Effects

Choosing An AEDSide Effects

SomnolenceSomnolence•AllAll

RashRash•LamictalLamictal•PhenytoinPhenytoin•PhenobarbPhenobarb

Renal StonesRenal Stones•TopiramateTopiramate•ZonisamideZonisamide

HyponatremiaHyponatremia•CarbamazepineCarbamazepine•OxcarbazepineOxcarbazepine

ParasthesiaParasthesia•TopiramateTopiramate•ZonisamideZonisamide

CognitiveCognitive•PhenobarbPhenobarb•TopiramateTopiramate

BehaviorBehavior•LevetiracetamLevetiracetam

Labs drawsLabs draws•CarbamazepineCarbamazepine•Valproic AcidValproic Acid•PhenytoinPhenytoin

LevelsLevels•AllAll

Page 60: Introduction To Epilepsy Semiology diagnosis Treatment M. Scott Perry, M.D. Emory University April 18, 2007 September 18, 2006 M. Scott Perry, M.D. Emory

Choosing an AED

•Type of epilepsy

•Type of formulation (IV, capsule, sprinkle, etc.)

•Time to onset

•Side Effects

•Dosing Schedule

Page 61: Introduction To Epilepsy Semiology diagnosis Treatment M. Scott Perry, M.D. Emory University April 18, 2007 September 18, 2006 M. Scott Perry, M.D. Emory

Choosing An AEDDosing ScheduleChoosing An AEDDosing Schedule

•QD: Depakote ER, Zonisamide

•TID: Depakene, Neurontin, Tegretol, Phenytoin (neonates)

•BID: Everything else

•QD: Depakote ER, Zonisamide

•TID: Depakene, Neurontin, Tegretol, Phenytoin (neonates)

•BID: Everything else

Page 62: Introduction To Epilepsy Semiology diagnosis Treatment M. Scott Perry, M.D. Emory University April 18, 2007 September 18, 2006 M. Scott Perry, M.D. Emory

Febrile SeizureFebrile Seizure•3 types (simple, complex, status)

• NIH consensus: Febrile seizure is an event in infancy or childhood, usually 3m-5 years, associated with fever but without evidence of intracranial infection or defined cause. Seizures with fever in children who have suffered a previous nonfebrile seizure are excluded.

• incidence- 4%: absolute risk increased with family hx (1 relative 10%, 2-32%), daycare (7%), dev delay (10%)

•Risk of recurrence: 1 in 24

• risk of future epilepsy: 2-10%

•workup - MRI/EEG does not predict recurrence

•treatment

•3 types (simple, complex, status)

• NIH consensus: Febrile seizure is an event in infancy or childhood, usually 3m-5 years, associated with fever but without evidence of intracranial infection or defined cause. Seizures with fever in children who have suffered a previous nonfebrile seizure are excluded.

• incidence- 4%: absolute risk increased with family hx (1 relative 10%, 2-32%), daycare (7%), dev delay (10%)

•Risk of recurrence: 1 in 24

• risk of future epilepsy: 2-10%

•workup - MRI/EEG does not predict recurrence

•treatment

Page 63: Introduction To Epilepsy Semiology diagnosis Treatment M. Scott Perry, M.D. Emory University April 18, 2007 September 18, 2006 M. Scott Perry, M.D. Emory

Practice ParameterFebrile Seizures

Practice ParameterFebrile Seizures

• Current Recommendations AAP [Pediatrics 97(5), May 1996, 769-71.]

• Age 6-12 months with febrile seizure should strongly consider LP

• Age 12-18 months should consider

• >18 months may use physical exam, associated symptoms to drive need

• Based recommendations on 4 studies reporting 13-15% of children will present with seizures as the initial manifestation of seizures with 30-35% having no meningeal signs.

• More recent reviews have suggested the presence of meningitis in the absence of associated signs is rare (1/200), with a large percentage of such patients with normal CSF at presentation. The introduction of the H.Flu vaccine has significantly altered the epidemiology of infantile bacterial meningitis making present treatment different from that 30 years ago (which the AAP based their recommendations).

• Most physicians would agree that LP in children outside the range of febrile convulsions is necessary, as well as children within the range with sign or symptoms of CNS infection, such as nuchal rigidity, altered mental status, etc.

• Current Recommendations AAP [Pediatrics 97(5), May 1996, 769-71.]

• Age 6-12 months with febrile seizure should strongly consider LP

• Age 12-18 months should consider

• >18 months may use physical exam, associated symptoms to drive need

• Based recommendations on 4 studies reporting 13-15% of children will present with seizures as the initial manifestation of seizures with 30-35% having no meningeal signs.

• More recent reviews have suggested the presence of meningitis in the absence of associated signs is rare (1/200), with a large percentage of such patients with normal CSF at presentation. The introduction of the H.Flu vaccine has significantly altered the epidemiology of infantile bacterial meningitis making present treatment different from that 30 years ago (which the AAP based their recommendations).

• Most physicians would agree that LP in children outside the range of febrile convulsions is necessary, as well as children within the range with sign or symptoms of CNS infection, such as nuchal rigidity, altered mental status, etc.

Page 64: Introduction To Epilepsy Semiology diagnosis Treatment M. Scott Perry, M.D. Emory University April 18, 2007 September 18, 2006 M. Scott Perry, M.D. Emory

Practice ParametersFirst Unprovoked Seizure

Practice ParametersFirst Unprovoked Seizure• Laboratory investigations (CBC, CMP, tox screens)

should be considered based on historic and clinical findings

• LP is of limited value in first unprovoked afebrile seizure

• EEG is recommended to dx epilepsy syndromes and provide for prognosis

• MRI is preferred modality and should be considered in children with cognitive/motor impairment that is unexplained, focal onset seizures, or in children < 1y.

• Emergenat imaging should be performed in children with prolonged todd’s, or prolonged (several hours) postictal state.

• Treatment: 46% have recurrence in 10years, 19% > 4 seizures, and 10%>10 seizures

• Laboratory investigations (CBC, CMP, tox screens) should be considered based on historic and clinical findings

• LP is of limited value in first unprovoked afebrile seizure

• EEG is recommended to dx epilepsy syndromes and provide for prognosis

• MRI is preferred modality and should be considered in children with cognitive/motor impairment that is unexplained, focal onset seizures, or in children < 1y.

• Emergenat imaging should be performed in children with prolonged todd’s, or prolonged (several hours) postictal state.

• Treatment: 46% have recurrence in 10years, 19% > 4 seizures, and 10%>10 seizures

Page 65: Introduction To Epilepsy Semiology diagnosis Treatment M. Scott Perry, M.D. Emory University April 18, 2007 September 18, 2006 M. Scott Perry, M.D. Emory

FAQ (the ED)FAQ (the ED)•I have a 14 month old with a febrile seizure

and a “raging otitis,” do I need to do a LP?

•(3a.m.) Hey, how are you? I have a kid here with known epilepsy that had a breakthrough seizure (like he does once every 3 months or so), do you want to increase his medicine?

•Do I need to CT this kid?

•I have a patient of Dr. Flamini’s here, how do you want to treat him?

•I have a 14 month old with a febrile seizure and a “raging otitis,” do I need to do a LP?

•(3a.m.) Hey, how are you? I have a kid here with known epilepsy that had a breakthrough seizure (like he does once every 3 months or so), do you want to increase his medicine?

•Do I need to CT this kid?

•I have a patient of Dr. Flamini’s here, how do you want to treat him?

Page 66: Introduction To Epilepsy Semiology diagnosis Treatment M. Scott Perry, M.D. Emory University April 18, 2007 September 18, 2006 M. Scott Perry, M.D. Emory

FAQ (the parents)FAQ (the parents)

•Why does my child have seizures?

•Will my child be stupid?

•How long does my child need treatment?

•What do I do when my child has a seizure?

•Why does my child have seizures?

•Will my child be stupid?

•How long does my child need treatment?

•What do I do when my child has a seizure?

Page 67: Introduction To Epilepsy Semiology diagnosis Treatment M. Scott Perry, M.D. Emory University April 18, 2007 September 18, 2006 M. Scott Perry, M.D. Emory

CaseCase•16 year old female with first unprovoked

seizure, described as generalized tonic clonic•16 year old female with first unprovoked

seizure, described as generalized tonic clonic

•Focal signs at onset?Focal signs at onset?•Myoclonic or absence type episodes?Myoclonic or absence type episodes?•Time of day?Time of day?•Previous workup?Previous workup?

•Treatment choices: Depakote, Keppra, Treatment choices: Depakote, Keppra, TrileptalTrileptal

•How long will she need medicine?How long will she need medicine?

Page 68: Introduction To Epilepsy Semiology diagnosis Treatment M. Scott Perry, M.D. Emory University April 18, 2007 September 18, 2006 M. Scott Perry, M.D. Emory

CaseCase

•8 y/o with frequent episodes of staring, at times associated with lip smacking

•8 y/o with frequent episodes of staring, at times associated with lip smacking

•Focal signs at onset? Can they be stopped?Focal signs at onset? Can they be stopped?•Myoclonic or GTC episodes?Myoclonic or GTC episodes?•Duration?Duration?•Time of day?Time of day?•Previous workup?Previous workup?•Treatment choices: Depakote, Keppra, Treatment choices: Depakote, Keppra, TrileptalTrileptal

Page 69: Introduction To Epilepsy Semiology diagnosis Treatment M. Scott Perry, M.D. Emory University April 18, 2007 September 18, 2006 M. Scott Perry, M.D. Emory