management of epilepsy in children
TRANSCRIPT
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