Management of EPILEPSY in

Children

Childhood Epilepsy

• Seizure in childhood is common and indicates

cerebral diseases and damage developing brain

• Incidence Febrile 2%, idiopathic 1%

• Clinical feature limited to motor phenomena

• EEG signs are are often variable and nonspecific

and their interpretation difficult

WHAT IS THE ETIOLOGY OF THE EPILEPSY ?WHAT IS THE ETIOLOGY OF THE EPILEPSY ?

INFANCY 1-6MONTHSINFANCY 1-6MONTHS

CONGENITAL MALDEVELOPMENTCONGENITAL MALDEVELOPMENT

BIRTH INJURYBIRTH INJURY

BIRTH ANOXIABIRTH ANOXIA

HYPOCALCEMIAHYPOCALCEMIA

HYPOGLYCEMIAHYPOGLYCEMIA

VIT B6 DEFICIENCYVIT B6 DEFICIENCY

PHENYLKETONURIA ETC.PHENYLKETONURIA ETC.

WHAT IS THE ETIOLOGY OF THE EPILEPSY ?WHAT IS THE ETIOLOGY OF THE EPILEPSY ?

EARLY CHILDHOOD 6MONTHS TO 3YEARSEARLY CHILDHOOD 6MONTHS TO 3YEARS

FEBRILE SEIZUREFEBRILE SEIZURE

BIRTH INJURYBIRTH INJURY

BIRTH ANOXIABIRTH ANOXIA

INFECTIONSINFECTIONS

TRAUMATRAUMA

WHAT IS THE ETIOLOGY OF THE EPILEPSY ?WHAT IS THE ETIOLOGY OF THE EPILEPSY ?

3-10YEARS3-10YEARS

PERINATAL ANOXIAPERINATAL ANOXIA

BIRTH INJURYBIRTH INJURY

INFECTIONSINFECTIONS

THROMBOSIS OF CEREBRAL THROMBOSIS OF CEREBRAL ARTERIES OR VEINSARTERIES OR VEINS

WHAT IS THE ETIOLOGY OF THE EPILEPSY ?WHAT IS THE ETIOLOGY OF THE EPILEPSY ?

10-18YEARS10-18YEARS

IDIOPATHICIDIOPATHIC

GRANULOMAGRANULOMA

TRAUMATRAUMA

ADULTHOODADULTHOOD

Basic Classification

• Primary

– focal

• simple

• complex partial

– generalized

• Secondary– focal– generalized

• Situation related– Febrile– Drug induced

Neonatal seizure

Incidence

• 5-16/1000 live birth

• Upto 23% in

premature infants

• Morbidity 35-75%

• Mortality 16-60%

Clinical

1. Subtle fragmented

2. Tonic seizure

3. Clonic Seizure

4. Unilateral

Causes of neonatal seizure

• Hypoxic Ischemic Encephalopathy

• Neonatal CVA’s• Intracranial infection• Cerebral malformation• Metabolic

– Hypocalcemia– Hypoglycemia– Hyponatremia– Inborn error of metabolism– Bilirubin encephalopathy– Hypomanicemia– Pyridoxin dependency

• Benign and familial syndromes– Benign familial neonatal

convulsion– Benign neonatal seizure– Benign neonatal sleep

myoclonus• Toxic or withdrawal conulsion

– Drugs and toxin– Anticonvulsant

• Specific– Ohtahara’s syndrome– Neonatal myoclonic

encephalopathy– Early infentile epileptic

encephalopathy

Infantile spasm (West’s syndrome

• Rare 0.25-0.42/1000 LB

• Onset – 3 months – 1year (4-6 months)

• Development normal prior to onset, but subsequent development retarded

• Neurological deficit 80%

• Spasm – Generalized flexion or rarely extension myoclonus

• EEG – Hypsarrythmias

• Good response to corticosteroid drugs

• Seizure usually remit on therapy or spontaneously

• Long term prognosis poor, with mental retardation and continuing epilepsy

Causes of Infantile spasm

• Disorder of cerebral development

• Neurocutaneous syndromes– Tuberous sclerosis

– Sturge- Weber Syndrome

– Neurofibromatosis

• Metabolic

• Degenerative

• Perinatal and postnatal – Hypoxic anoxic

encephalopathy

– Cerebral infraction

– Intra-cerebral Hemorrhage

– Cerebral trauma

– Cerebral tumor

– Maternal toxemia

– Metabolic and endocrine disorders

• Idiopathic - 40%

Lanox Gastaut Syndrome

• Onset in childhood

• Severe epileptic disorder with multiple seizure type , myoclonic, atypical absence, tonic and tonic clonic

• Seizure precipitated by drowsiness

• Progressive mental retardation

• Status epilepticus common ( especially non convulsive)

• EEG shows 1-2.5hz spike and wave complex, background abnormalities, no photosensitivity

• Primary (25%) and secondary (75%)

• Poor response to AED

Benign Rolandic epilepsy

• Common – 15% of all

childhood epilepsy

• Age – 5-10 years

• Partial seizure involving

face oropharynx and arm,

usually with preserved

consciousness, initially

and commonly secondary

generalization

• Typically occur during sleep

• Seizure usually infrequent• Normal intelligence and

no other neurological abnormalities

• Family history• EEG – High amplitude

centrotemporal spikes• Excellent response to

AED• Remission by mid teenage

Occipital Epilepsy

• Incidence– Rare– Male 7-15 years– Family history– Febrile convulsion

• EEG– Occipital spikes

• No pathology• Excellent prognosis

• Clinical– Amaurosis– Hemianopia– Head deviation and

blinking– Complex partial or

Generalized – Postictal headache

with vomiting – Prolonged seizure

Electric status epilepticus during slow wave sleep (ESES)

• Rare 0.5% of childhood epilepsy

• Age 1-14 years

• Clinical – Seizure

– Mental retardation

– Neurological signs

– Normal intellectual before onset

– Regression with language dysfunction follows

• EEG : – Generalized spike

wave discharge occupying at least 85% of NREM sleep

• Remission by 15 years

Acquired epileptic aphasia• Rare• Male predominance• Age < 6 years• Development normal• Aphasia subacute or gradual • EEG focal spike and slow wave in slow wave

sleep• Overt seizure mild in 70%• Seizure but not EEG controlled by Drugs• Incomplete recovery of speech

Idiopathic Generalized Epilepsies• Common 10% of

epilepsies and 40% of Tonic clonic epilepsies

• Clinical – GTC, myoclonic and or absence seizure

• Marked diurnal pattern• Precipitated by

– Sleep deprivation– Menstruation– Fatigue– Stress– Alcohol– Photic stimulation

• Strong genetic basis with age specific expression

• Absence of other neurological abnormality and normal intelligence

• Generalized EEG changes 3Hz spike and wave, normal background and photosensitivity

• Excellent response to specific antiepileptic drug

Childhood absence epilepsy• Incidence:

– 3-12 years (4-8 years)– 6-8/ 100000 – Female > Male– Genetic predisposition

• Clinical– 10-100 times a day– < 15 sec– Blank stare and

unresponsiveness, clonic eyelid movements minor orofacial automatism

– Transient loss of postural tone, increase in tone

• Precipitated

– hyperventilation

– Fatigue

– Emotional upset

– boredom

– Inactivity

• Prognosis

– 40% develop GTCS at 5-10

years after the onset

Juvenile absence epilepsy

Incidence– ¼ of CAE– Male = Female – Age = 10 years

Clinical – Frequency less

– Duration more

– Less profound loss of consciousness

– 1/20th has automatism

– 16% has myoclonic seizure

– 80% develop GTC on awakening or rarely in sleep

EEG– background normal– Generalized spike wave

discharge with frontal predominance 3.5-4Hz

– Precipitated by hyperventilation or sleep deprivation

Prognosis– 80% responds to treatment– Both absence and TC

seizure may persist in adulthood

Juvenile myoclonic epilepsy

• Incidence– 5-10% of epilepsy

• Clinical– Typical Absence 10 years

->

– Myoclonic jerks in morning ->

– GTC on awakening

– Precipitated by light and flash

• EEG

– 4-6Hz polyspike and slow

waves generalized

discharges for 20 sc

– Normal background

• Prognosis

– 90% become seizure free

with medication

– Relapses on stopping

medication

Epilepsy with generalized tonic clonic seizure on awakening

• Incidence– 15-40% epilepsy– Male predominance– 12% family history– Age 9-25years (puberty

peak)• Clinical

– Within 2 hours of awakening, sleep deprivation and alcohol

– Absence in 50%– Myoclonic seizure 30%– GTCS without aura may

precedes myoclonic or absence

• EEG– Generalized spike wave

activity 2.5-4Hz– Polyspike and wave activity– Photosensitive and

hyperventilation may increases

• Prognosis– 65% seizure controlled on

medication– 80% relapses off

medication

Other IGE

• Eyelid myoclonia with typical absence

• Epilepsy with myoclonic absence

• Benign myoclonic epilepsy of infancy

• Benign familial myoclonus

• Perioral myoclonia with absence

Childhood myoclonic epilepsy

• Cryptogenic myoclonic epilepsy

• Benign myoclonic epilepsy of infancy

• Syndrome of severe myoclonic epilepsy of infancy

• Syndrome of myoclonic status in non progressive encephalopathy

• Myoclonic astatic epilepsy

Progressive myoclonic epilepsy

• Lafora body disease

• Unverricht Lundenborg disease

• Mitochondrial encephalopathy

• Neonatal ceroid lipofuscinosis

• Dentato- rubro pallido luysian atrophy

Febrile Seizure

Simple Febrile• Common 2-4% of children• Peak age of onset – 2-4

years• Seizure at the onset of

febrile illness• Tonic clonic form• Recurrence in 30-50%• Rule of 5 - <5year age,

<5min duration, < 5/years

Complex febrile

• duration greater than 30 min,

• focal features,

• recur in 24 hours

Poor prognosis– Onset <13months

– Associated neurological disease

– Complex convulsion

Febrile seizure Rx

• No medication for single febrile seizure

• Diazepam orally for recurrent febrile seizure

• Valproate or phenobarb for recurrent febrile

seizure

COMMON DIAGNOSTIC COMMON DIAGNOSTIC PROBLEMSPROBLEMS

GENNERALIZED CONVULSIVE ATTACKGENNERALIZED CONVULSIVE ATTACK LOSS OF AWARENESSLOSS OF AWARENESS DROP ATTACKSDROP ATTACKS TRANSIENT FOCAL NEUROLOGICAL TRANSIENT FOCAL NEUROLOGICAL

DYSFUNCTIONDYSFUNCTION PSYCHIC EXPERIENCESPSYCHIC EXPERIENCES EPISODIC PHENOMENA IN SLEEPEPISODIC PHENOMENA IN SLEEP PROLONGED CONFUSIONAL OR FUGE PROLONGED CONFUSIONAL OR FUGE

STATESTATE

COMMON DIAGNOSTIC PROBLEMSCOMMON DIAGNOSTIC PROBLEMS

GENNERALIZED CONVULSIVE ATTACKGENNERALIZED CONVULSIVE ATTACK

NON EPILEPTIC ATTACK NON EPILEPTIC ATTACK DISORDERDISORDER

SYNCOPE WITH SECONDARY SYNCOPE WITH SECONDARY JERKINGJERKING

EPISODIC INVOLUNTRY EPISODIC INVOLUNTRY MEVEMENT DISORDERSMEVEMENT DISORDERS

HYPEREKPLEXIAHYPEREKPLEXIA

NON EPILEPTIC ATTACK DISORDERNON EPILEPTIC ATTACK DISORDER

EPILEPSY NEAD

PRECIPITATING RARE STRESS, EMOTION

ATTACK IN SLEEP COMMON RARE

ONSET SHORT LONG

AURA STEREOTYPED FEAR, PANIC

SPEECH CRY, GRUNT SEMI-VOLUNTARY

MOVEMENTS TYPICAL ATYPICAL

INJURY TONGUE BITE,FALL

DIRECTEDVIOLENCE

CONSCIOUSNESS LOSS COMPLETE VARIABLE

INCONTINENCE COMMON RARE

DURATION SHORT LONG

LOSS OF AWARENESS (BLACKOUT)LOSS OF AWARENESS (BLACKOUT)

SYNCOPESYNCOPE TRANSIENT CEREBRAL ISCHEMIATRANSIENT CEREBRAL ISCHEMIA MICROSLEEPMICROSLEEP PANIC ATTACKPANIC ATTACK HYPOGYCEMIAHYPOGYCEMIA NEUROLOGICAL DISORDERSNEUROLOGICAL DISORDERS

ARNOLD-CHIARI MALFORMATIONARNOLD-CHIARI MALFORMATION THIRD VENTRICULAR TUMORTHIRD VENTRICULAR TUMOR HEAD INJURYHEAD INJURY

NON-EPILEPTIC ATTACK DISORDERSNON-EPILEPTIC ATTACK DISORDERS

SYNCOPESYNCOPE

EVIDENCE OF PRECIPITAING FACTORSEVIDENCE OF PRECIPITAING FACTORS

LIGHHEADEDNESS, DIZZINESS, NAUSEALIGHHEADEDNESS, DIZZINESS, NAUSEA

TINNITUS, BILATERAL LOSS OF VISISONTINNITUS, BILATERAL LOSS OF VISISON

COLLAPSECOLLAPSE

PALLORPALLOR

SWEATING, SUBSEQUENT FLUSHINGSWEATING, SUBSEQUENT FLUSHING

RPID RECOVERY WHEN SUPINERPID RECOVERY WHEN SUPINE

TRANSIENT FOCAL TRANSIENT FOCAL NEUROLOGICAL DYSFUNCTIONNEUROLOGICAL DYSFUNCTION

FOCAL SEIZURESFOCAL SEIZURES VASCULAR DISORDERS - TIA'S *VASCULAR DISORDERS - TIA'S * MIGRAINE, SDH, LARGE ANEURISM MIGRAINE, SDH, LARGE ANEURISM DEMYELINATIONDEMYELINATION TUMOR, RAISED ICTTUMOR, RAISED ICT METABOLIC - HYPOGLYCEMIA, TOXICMETABOLIC - HYPOGLYCEMIA, TOXIC PSYCHOLOGICALPSYCHOLOGICAL OTHER - VERTIGO, HEADACHE, TGA, VOMITING, OTHER - VERTIGO, HEADACHE, TGA, VOMITING,

ABDOMINAL PAINABDOMINAL PAIN

TRANSIENT PSYCHIC DISTURBANCETRANSIENT PSYCHIC DISTURBANCE

• COMPLEXCOMPLEX PARTIAL, FOCAL PSYCHIC,POSTICTALPARTIAL, FOCAL PSYCHIC,POSTICTAL

• BREATH HOLDINGBREATH HOLDING

• HYPERVENTILATION ATTACKHYPERVENTILATION ATTACK

• PANIC ATTACKPANIC ATTACK

• EPISODIC DYSCONTROL SYNDROMEEPISODIC DYSCONTROL SYNDROME

• EMOTIONAL ATTACKSEMOTIONAL ATTACKS

• TANTRUMTANTRUM

• ABREACTION OR SYMBOLIC ATTACKABREACTION OR SYMBOLIC ATTACK

• DELIBERATE SIMULATIONDELIBERATE SIMULATION

• SHUDDERING ATTACKSSHUDDERING ATTACKS

• MALINGERINGMALINGERING

What blood test in epilepsy?

• Complete blood count

• Blood sugar fasting and post pandrial

• Serum creatinine

• S. Calcium , magnesium, and sodium

• SGPT, bilirubin

EEG in Epilepsy

• To confirm the diagnosis

• To classify the type of seizure

• To locate the focus of discharge

• To find out triggering factors

• To find out associated brain disease

• To monitor anticonvulsant

When to do Neuroimaging?

• Focal onset or focal neurological sign

• Features of raised intracranial pressure

• Uncontrolled seizure

• Features of focal lesion in EEG

CT or MRI

• CT

– Calcification

– Acute hemorrhage

– Emergency

• MRI– Tumor– Old hematoma– AVM– Temporal atrophy– Granuloma

AED after first provoked seizure

• Don’t start– idiopathic generalized

tonic-clonic seizure

– no prior acute symptomatic seizure

– no spikes on EEG

– no sibling with epilepsy

– no status epilepticus

– no Todd's paralysis

– benign childhood epilepsy

• Start– remote symptomatic seizure

– partial seizure

– prior acute symptomatic seizure

– spikes on EEG

– sibling with epilepsy

– first seizure was status epilepticus

– Todd's paralysis

– first seizure during sleep

How to start drug treatment?

• Confirm the diagnosis

• Use single anticonvulsant

• Use proper doses

• Loading dose of certain drug in emergency

• Build up dose of others

• Use minimal effective dose

A. Partial seizures (without or with

secondary generalization) • First choice:

– Carbamazepine, phenytoin

• Second choice: – Gabapentin, valproate, lamotrigine, topiramate,

vigabatrin, tiagabine

• Consider: – Phenobarbital, primidone, clonazepam, clobazam,

clorazepate, acetazolamide

B. Generalized tonic-clonic seizures

• First choice:

– Valproate, phenytoin, carbamazepine

• Second choice:

– Phenobarbital, primidone

• Consider:

– Clonazepam, clobazam

C. Childhood absence epilepsy

Before age 10 years:

• First choice: – Ethosuximide,

– valproate

• Second choice: – Lamotrigine,

– methsuximide,

– acetazolamide,

– clonazepam,

– clobazam

After age 10 years:

• First choice: – Valproate

• Consider: – Carbamazepine,

– phenytoin or

– phenobarbital (for generalized tonic-clonic seizures if valproate or lamotrigine not tolerated)

D. Juvenile myoclonic epilepsy

• First choice:– Valproate

• Second choice: – Lamotrigine, – phenobarbital, – primidone, clonazepam

• Consider: – Carbamazepine, – phenytoin, – methsuximide, – acetazolamide

E. Progressive myoclonic epilepsy

• First choice:

– Valproate

• Second choice:

– Valproate + clonazepam,

– clobazam,

– phenobarbital

F. The Lennox-Gastaut and related syndromes

• First choice:

– Valproate

• Second choice:

– Clonazepam,

– phenobarbital,

– lamotrigine,

– ethosuximide

• Consider: – Methsuximide,

– clobazam,

– nitrazepam,

– ACTH or steroids,

– pyridoxine,

– ketogenic diet,

– felbamate

G. Infantile spasms

• First choice: – ACTH or steroids

• Second choice: – Valproate,

– vigabatrin

• Consider: – Clonazepam,

– nitrazepam,

– pyridoxine

H. Benign epilepsy of childhood with centrotemporal spikes

• First choice: – Carbamazepine, – valproate

• Second choice: – Clobazam, – phenytoin

• Consider: – Phenobarbital,– primidone

I. Neonatal seizures

• First choice: – Phenobarbital

• Second choice: – Phenytoin

• Consider: – Clonazepam, – primidone, – valproate, – pyridoxine

What drug to choose?

Focal/GTCS CBZ, PHY, VALPHB,

Absence VAL, EHT, BNZ

Myoclonic VAL, CLN

Drug level monitoring

• To maintain

minimum dose

• Uncontrolled

epilepsy

• Noncompliance

• Polytherapy

• Drug interaction

• Toxicity

• Hepatic diseases

• Pregnancy

What is the chance of remission?

• 50% Remission off treatment for 20

years

• 20% Remission on treatment

• 30% Seizure on treatment

Good prognostic signs• Granuloma• Early posttraumatic epilepsy• Mild infrequent seizure• Secondary systemic or toxic

seizure• Benign rolandic epilepsy• Primary generalized epilepsy• Absence seizure• Early treatment

Bad prognostic signs

• Diffuse cerebral disease

• Late posttraumatic epilepsy

• Multiple seizure types

• Complex partial seizure

• Long untreated seizure

• History of Status in the past

When to stop treatment

• Primary generalized seizure with normal

EEG for 2-3 seizure free years

• Taper slowly

• Severe brain damaged needs life long

treatment

• Short course following medical disorder

Intractable seizures

• 20-30% of epilepsy

• Poor compliance

• Inadequate drug doses

• Improper choice of drug

• Inappropriate combination of drugs

• Misdiagnosis of seizure or seizure type

New antiepileptic drugs

1. Clobazam

2. Gabapantin

3. Lamotrigine

4. Topiramate

5. Vigabatrin

6. Falbamate

Clobazam

• Benzodiazepine, anxiolytic

• Weak antiepileptic

• For add on therapy

• Less side effect

• Can be used in children with primay and febrile seizure

• Dose: 0.1-0.5mg/Kg/dayBD

Gabapantine

• First pass metabolism

• No interaction

• Drug level monitoring not required

• Can be use in high doses

• Renal and hepatic failure and transplant patient

Lamotrigine

• Broad spectrum antiepileptic

• Skin rash common, no other significant toxicity

• Can be used in all age as primary and secondary drug

• Dose: 0.5-10mg/kg in two divided dose

Topiramate

• GABArgic

• Efficacy: Partial seizure

• Side effects: fatigue, nervousness, difficulty with concentration, tremor, weight loss, renal stone

• Dose: 50-400mg in two divided doses

Status epilepticus causes

Drug withdrawal 25

Alcohol withdraw 25

Cerebrovascular: 22

Metabolic: 10

Systemic infection 12

Trauma 15

Drug toxicity 15

CNS infection 12

Tumor 8

Congenital lesion 8

Prior Epilepsy 33

Idiopathic 30

Status epilepticus management

• ABCD

• Blood: Electrolytes, CBC, Calcium, Magnesium, BUN, Liver function Anticonvulsant level, Alcohol, Toxicology screen

• If hypoglycemia suspected, give 50% glucose

• Give Thiamine 100 mg iv

• Lorazepam 0.1 mg/kg iv

• Phenytoin 20 mg/kg iv, 50 mg/min

Status management cont.

If seizure persists:

• Phenobarbital 20 mg/kg iv at 50 to 100 mg/min

• Review lab result and correct any abnormality

• CT/MRI: bleed, infection, AV malformations, neoplasm

• Lumbar puncture: if CNS infection suspected

• Blood cultures: Sepsis

For refractory seizure:• Intubation, EEG

monitoring and Pentobarbital 5-15 mg/kg loading over 3 minutes, 0.5 to 5 mg/kg/hr drip or

• Midazolam (Versed) 0.15-0.20 mg/kg loading, then 0.06-1.1 mg/kg/hr drip

• Propofol 1-2 mg/kg loading, then 3-10 mg/kg/h

Surgical Procedures

• Resection of epileptic focus

– cortical resection

– temporal lobectomy

– Amygdylohippocampectomy

• Corpus callosotomy

• Hemispherictomy