Pediatric epilepsies

Amr Hassan MD,FEBN
Associate Professor of Neurology
Cairo University
Pediatric Epilepsies

Diagnosis Treatment
Tips and tricks in MANAGEMENT of
pediatric epilepsies

• Not epileptic
• Wrong seizure type (semiology)
• Wrong epileptic syndrome
• Wrong interpretation of EEG and imaging
Tips and tricks in DIAGNOSIS of

• When to start a drug?
• Which drug and in what dose?
• When to change the drug?
• When (and how) to add a second drug (and
which one)?
• When to stop the drug(s)?
• When to consider alternative therapies,
including surgery?
Tips and tricks in TREATMENT of

Diagnosis of Pediatric Epilepsy
5
Axis 1: • Is it epileptic seizure or not?  D.D. Of epilepsy
Axis 2:
• Identify type of seizure (seizure semiology) 
International classification of epileptic seizures.
Axis 3:
• Investigations aiming at confirmation of the
diagnosis & searching for an aetiology
Axis 4:
• Identify epileptic syndrome  International
classification of epilepsy & epileptic syndromes

6
Axis 1:
• Is it epileptic seizure or not?  D.D. Of
epilepsy
Axis 2:
Axis 3:
Axis 4:

7
Underdiagnosis
Unusual
presentation
Overdiagnosis
Epilepsy Mimics

• The movement resembles a tremor
• Distinguished clinically from clonic seizures by:
 No associated ocular movements
 No Autonomic phenomena
 Stimulus sensitivity (eg, triggered by stimulation or
easily stopped with passive movement of the limb).
Jitteriness

• Onset 6mth – 10 yrs, gradually better
• Sudden tremulous contraction (shiver)
• Brief, up to 100/day, cluster
• Intervening several weeks of no spells
• Benign phenomenon
• No treatment, gradually disappears
• Specific stereotypy/mannerism
Shuddering Attacks

• Infants/young children, min to hours
• Episodes of genital and self stimulation
• Paroxysmal, Stereotypic, rhythmic
• Tightening of thighs and rocking
• Pressure to pubic/supra-pubic areas
• Irregular breathing, flushing, diaphoresis
• Mimic complex partial seizures or pain
• Reassure and inform parents
Childhood Masturbation

11
Cyanotic Breath Holding Spell
• 6 months – 2 years (up to 5 years)
• Typically confused with tonic seizure
• Always a trigger: fear, injury, frustration
• Cry → breath holding (expiration) → stiff,
• Loss of awareness → clonic jerks
• Patho-physiology not understood
• Correction of anemia, counseling

• Parasympathetic dysregulation, pale and limp, with
asystole
• The most common stimulus is a painful event.
• The child turns pale (as opposed to blue) and loses
consciousness with little if any crying.
• The EEG is also normal, and again there is no post
ictal phase, nor incontinence.
• The child is usually alert within a minute or so.
Pallid type (Reflex asystole)

Breath-holding spells Epileptic seizures
Trigger Crying, injury Spontaneous, fever, sleep deprivation
Occurrence
during sleep
No May occur during sleep
Event Sequence  provocation
—apnea, cyanosis/ pallor,
limpness.
Associated with stiffening and jerking
of extremities
Postictal state Usually brief Maybe prolonged
Epileptiform
abnormalities
on EEG
Absent Usually present
Treatment Parental reassurance Anticonvulsant therapy
D.D. Breath Holding Spells

• Rapid, random, bilateral/asynchronous
• jerking, may be forceful and rhythmic
• Seconds-minutes or even hours in sleep
• All stages of sleep (Quiet sleep/NREM)
• Differential: Seizures and Jitteriness
• Disappear when infant is woken up
• Not seen during alert wakefulness
• Does not stop on passive flexion (jitter stops)
• EEG: Normal baseline and during events
• Mostly disappear by late infancy
Benign Neonatal Sleep Myoclonus

• Dystonic, abnormal movements of head, Neck, upper trunk
(Sandifer’s syndrome)
• Life-threatening events – apnea with Cyanosis and/or pallor
• Vomiting, failure to thrive
• More common in delayed/hypotonic patients
• Management:
Confirm diagnosis, treatment of reflux
Gastro-esophageal Reflux

Childhood Parasomnias
Features Nightmares Night terrors
Age of onset
Duration
Semiology
Stage sleep
Time
Recall
2-5 years
<1-2minute
Cling, verbalize
REM
early am
usually able
4-8 years
>5 minutes
vary/autonomic
NREM III & IV
first third of night
not able

• Body rocking
• Head rolling
• Other less common muscle movements include:
• Body rolling
• Leg rolling
• Leg banging
• A combination of the aforementioned symptoms
• Head banging
Rhythmic Movement Disorder

• Cataplexy is a sudden and transient episode of muscle
weakness accompanied by full conscious awareness
• Triggers: emotions (laughing, crying, or terror).
• Cardinal symptom of narcolepsy with cataplexy affecting
roughly 70% of people who have narcolepsy
Cataplexy

PseudoSeizures
Non-epileptic seizure Epileptic seizure
Duration Prolonged (several minutes) Usually less than 2-3 minutes
Clinical features • Fluctuating features
• Usually during wakefulness
• Preserved consciousness,
avoidance behavior
• Side to side head movements
• Out of phase extremity
movements
• Forward pelvic thrusting
• Emotional vocalization
• Pupillary reflex retained
• Stereotypic features
• May occur in sleep
• Altered consciousness
• Head unilaterally turned
• In phase extremity movements
• Retropelvic thrusting
• Monotonous vocalization
• Pupillary reflex absent
Incontinence Rare Present
Tongue bite Occasional Common
Postictal
changes
None Usually present
Affect La Belle indifference Concerned

Myoclonus Tics
Onset Any School age
Pattern Patterned, predictable, identical Variable, wax and wane
Movements Jerky, shock like, sudden Blink, grimace, shrug
Rhythm Maybe Rhythmic Rapid, sudden , random
Duration Brief, intermittent Brief, intermittent
Premonitory
urge
No Yes
Trigger No, action, stimulus sensitive Excitement, stress
Suppression No Brief (causes inner tension).
Family History May be positive Frequently positive
Treatment AED Neuroleptics
Tics Vs Myoclonus

New Diagnostic Criteria for Children
Cyclic Vomiting Syndrome
Li BU, Lefevre F, Chelimsky GG, et al. North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition consensus statement on the diagnosis and
management of cyclic vomiting syndrome. J Pediatr Gastroenterol Nutr. 2008;47:379–393.

Non- Epileptic
Paroxysmal
Events
(NPE)

Diagnosis of Epilepsy
24
Axis 1:
epilepsy
Axis 2:
Axis 3:
Axis 4:

25
Axis 1:
epilepsy
Axis 2:
Axis 3:
Axis 4:
Axis 2:

27
III. Unclassified epileptic seizures (caused by
incomplete data)
I. Partial (focal, local) seizures
II. Generalized seizures (convulsive or
nonconvulsive)
International Classification of Epilepsy

Simple Complex
1. w/ motor
signs
2. w/ somato-
sensory
symptoms
3. w/ autonomic
symptoms
4. w/ psychic
symptoms
1. simple partial
--> loss of
consciousness
2. w/ loss of
consciousness
at onset
Secondary
generalized
1. simple partial
--> generalized
2. complex partial
--> generalized
3. simple partial
--> complex partial
--> generalized
Partial seizures

Operational classification of seizure types by the International League Against Epilepsy: Position Paper of the ILAE Commission for
Classification and Terminology
Operational classification of seizure types by the International League Against Epilepsy: Position Paper of the ILAE Commission for Classification and Terminology, Volume:
58, Issue: 4, Pages: 522-530, First published: 08 March 2017, DOI: (10.1111/epi.13670)
ILAE 2017 Classification of seizure type

ILAE 2017 Classification of seizure type

International Classification of Epilepsy :SPS

International Classification of Epilepsy :CPS

International Classification of Epilepsy
36
II. Generalized seizures (convulsive or nonconvulsive)
A. Absence seizures
1. Absence seizures
2. Atypical absence seizures
B. Myoclonic seizures
C. Clonic seizures
D. Tonic seizures
E. Tonic-clonic seizures
F. Atonic seizures (astatic seizures)

Gen. Absences CPS
Aura - +/-
Onset Abrupt Gradual or abrupt
Duration <15 sec >30 sec
Termination Abrupt Usually Gradual
Postictal S & S - Most often +
Frequency Many daily Weekly-monthly
PPT by HV Usually Unlikely
Absence Vs CPS

• Male child , 10 years old
• Unreventful past medical history.
• His mother noticed recurrent attacks of head
and eye deviation to the right side associated
with clonic movements involving the right
upper limb that occur frequently and lasted
for few minutes (according to her own words).
Case Vignette

• Neurological examination was unremarkable.
• The patient’s family could not afford the
requested investigations due to financial
issues.
Case Vignette

Which AED would you like to choose?
• CBZ
• VPA
• LEV
• OXC
• TPM
• OTHERS
Case Vignette

Which AED would you like to choose?
• CBZ 200 mg CR bid
• VPA
• LEV
• OXC
• TPM
• OTHERS
Case Vignette

• In the follow up visit, the patient’s mother
reported no improvement as regard the
seizure frequency.
Case Vignette

What do you think?
• Increase dose of CBZ
• Consider Adding another AED
• Consider replacing CBZ with another AED.
Case Vignette

What do you think?
• Increase dose of CBZ 400 CR mg BID
• Consider Adding another AED
Case Vignette

• Again, in the follow up visit, the patient’s
mother reported no improvement as regard
the seizure frequency.
Case Vignette

What do you think?
• Consider adding another AED
Case Vignette

What do you think?
• Consider adding another AED LEV 250 BID
Case Vignette

• In the follow up visit, the patient’s mother
reported partial reduction in the seizure
frequency.
Case Vignette

50
Axis 1:
epilepsy
Axis 2:
Axis 3:
Axis 4:
Axis 2:

51
Axis 1:
epilepsy
Axis 2:
Axis 3:
Axis 4:
Axis 2:

AXIS 3: INVESTIGATIONS
Labs
52
Should be tailored according to the case;
• Drug screen
• Electrolytes
• KFT
• LFT
• CSF examination
• Lactic acid

EEG
53
Conventional Electroencephalography
• Used in establishing the diagnosis and
focality of an epileptic disorder
• Normal EEG doesnot exclude presence
of epilepsy thus the yield of EEG can be
increased by Sleep deprivation
(< 4 h sleep )

EEG
54
Multiple Electroencephalography (EEG) exams
EEG monitoring with video (video-EEG)

NEUROIMAGING
55
A) MRI

59
Axis 1:
epilepsy
Axis 2:
Axis 3:
Axis 4:
Axis 2:
Axis 3:

60
Axis 1:
epilepsy
Axis 2:
Axis 3:
Axis 4:
Axis 2:
Axis 3:
Axis 4:

Neonatal period
Benign familial neonatal
seizures (BFNS)
Early myoclonic
encephalopathy (EME)
Ohtahara syndrome
Infancy
West syndrome
Myoclonic epilepsy in infancy (MEI)
Benign infantile seizures
Benign familial infantile seizures
Dravet syndrome
Myoclonic encephalopathy
Childhood
Febrile seizures plus (FS+) (can start in infancy)
Early onset benign childhood occipital epilepsy (Panayiotopoulos type)
Epilepsy with myoclonic atonic (previously astatic) seizures
Benign epilepsy with centrotemporal spikes (BECTS)
Autosomal-dominant nocturnal frontal lobe epilepsy (ADNFLE)
Late onset childhood occipital epilepsy (Gastaut type)
Epilepsy with myoclonic absences
Lennox-Gastaut syndrome
Epileptic encephalopathy with continuous spike-and-wave during sleep (CSWS)
Landau-Kleffner syndrome (LKS)
Childhood absence epilepsy (CAE)
AXIS 4: IDENTIFY EPILEPTIC SYNDROME
EPILEPTIC SYNDROMES

EPILEPTIC SYNDROMES
63
EPILEPTIC SYNDROME IS DEFINED BY
1. Type or types of seizure e.g. Absence ,GTC.
2. Age of seizure onset
3. Aetiology
4. Degree of neurologic and intellectual deficit
5. Clinical evolution of the epilepsy
6. EEG pattern
7. Neuroimaging abnormality

Unknown 15.5%
Infection 2.5%
Degenerative 3.5%
Cancer 4.1%
Head Injury 5.5%
Congenital
Malformations 8.0%
Stroke 10.9%
Idiopathic (Genetic) Epilepsies
(~50%)
Symptomatic (Acquired) Epilepsies
(~50%)
Hauser
Etiologies of the epilepsies

Important
epileptic
syndromes
65

EPILEPTIC SYNDROMES
66
Benign epilepsy of childhood with centrotemporal
spikes
Common characteristic features includes :
• Unilateral somatosensory involvement.
• Speech arrest.
• Preservation of consciousness in most cases.
• Pooling of saliva &Tonic or tonic-clonic spread to face.

EPILEPTIC SYNDROMES
67
• The typical interictal EEG shows centrotemporal
spikes or SW, which are either unifocal or bifocal.
• Carbamazepine is often the first medication to be
tried, and seizures usually are well controlled.
• Excellent prognosis.
Benign epilepsy of childhood with centrotemporal
spikes

EPILEPTIC SYNDROMES
69
Childhood epilepsy with occipital paroxysms:
• Early onset type :
• Young childern 5 ys
• Ictal vomiting , deviation of the eye ,impairment
of the consciousness sometimes progress into
GTCs
• Two thirds of seizures occur during sleep

EPILEPTIC SYNDROMES
70
Panayiotopoulos syndrome
• Previously called early-benign childhood seizures with
occipital spikes
• Childhood onset (peak 5 years).
• Focal autonomic seizures or autonomic status epilepticus,
frequently with emesis.
• Interictal EEG with shifting or multifocal high amplitude
spikes, often with occipital predominance.

71
• Favorable outcome with remission in 1–2 years and normal
development.
• EEG spikes occur most commonly in the posterior areas of
the brain including the occipital lobe
• 30% of patients show only extraoccipital discharge or
normal EEGs

EPILEPTIC SYNDROMES

EPILEPTIC SYNDROMES
73
Childhood epilepsy with occipital paroxysms:
• Late onset (Gastaut) type :
• Older childern 9 ys
• Formed visual hallucinations or amaurosis
followed by hemiclonic convulsions with post-
ictal migrain
• EEG: occipital spikes
• Excellent prognosis

EPILEPTIC SYNDROMES
74
Childhood absence epilepsy
• Seizure semiology:
• Absence:Typical absence
• High frequency hundreds / day
• + Myoclonus
• + GTCS
• 3-12 years
• EEG changes: 3-Hz spike and wave
• Treatment : VPA, EXM
• Favorable outcome( long term remission)

EPILEPTIC SYNDROMES
76
West syndrome (infantile spasms):
• Triad of :
1. Seizures
2. Psychomotor retardation or regression
3. Specific EEG changes : hypsarrythmia.
• 1st year of life
• Related to prenatal ,natal or postnatal insult

EPILEPTIC SYNDROMES
77
West syndrome (infantile spasms):
• Seizures
1. Flexor spasms
2. Extensor spasms
3. Mixed flexor & extensor spasms
• Resistant to treatment
• Poor prognosis

EPILEPTIC SYNDROMES
78
Lennox Gastaut syndrome
• Mixed seizure disorder:
• (Tonic, TC, Myoclonic, Atypical absence & drop attacks )
• Mental retradation
EEG:
 Slow spike & wave
 Sharp & slow wave complex
 Slow abnormal background
Treatment : commonly need Polytherapy
Poor prognosis & cure rarely achieved

79
Axis 1:
epilepsy
Axis 2:
Axis 3:
Axis 4:
Axis 2:
Axis 3:
Axis 4:

Treatment pitfalls
• When to start a drug?
• Which drug and in what dose?
• When to change the drug?
• When (and how) to add a second drug (and
which one)?
• When to stop the drug(s)?
• When to consider alternative therapies,
including surgery?

1840 1860 1880 1900 1920 1940 1960 1980 2000
0
5
10
15
20
Bromide
Phenobarbital
Phenytoin Primidone
Ethosuximide
Sodium Valproate
Benzodiazepines
Carbamazepine
Zonisamide
Felbamate
Gabapentin
Topiramate Fosphenytoin
OxcarbazepineTiagabine
Levetiracetam
Rufinamide
Lacosamide Pregabalin
Calendar Year
NumberofLicensedAntiepilepticDrugs
Lamotrigine
Vigabatrin
Perampanel
Retigabine
Levetiracetam
eslicarbazepine
Antiepileptic Drugs (AEDs)

Seizure type
Epilepsy syndrome
Efficacy
Cost
Pharmacokinetic profile
Adverse effects
American Epilepsy Society 2010
Considerations in Choosing AED in Ped Epilepsy

AED
Na+
channels
Ca+
channels
GABA Glutamate Others
Clinical efficacy (type of
seizures/syndromes)
CBZ + FS, GTCS
CLB GABAA receptors Broad spectrum
CZP GABAA receptors Broad spectrum
ETS + Absence
PB + GABAA receptors FS, GTCS, myoclonic
PHT + FS, GTCS
VPA GABAA receptors
NMDA
receptors
Broad spectrum
FBM + GABAA receptors
NMDA
receptors
Atonic, tonic, atypical
absence in LGS
GPT + GABAB receptors FS

AED
Na+
channels
Ca+
channels
seizures/syndromes)
GVG
GABA
transaminase
FS, infantile spasms
LEV SV2A FS, GTCS, myoclonic
LTG + +
FS, GTCS, absence,
infantile spasms
OXC + FS, GTCS
PGB + FS
TGB GABA transporters FS
TPM + GABAA receptors
Carbonic
anhydrase
FS, GTCS, myoclonic,
infantile spasms
ZNS + +
Carbonic
anhydrase
FS
ESL + FS

AED
Na+
channels
Ca+
channels
seizures/syndromes)
LCS + FS
PER
AMPA
receptors
FS
RTG KCNQ channels FS
RUF +
FS, atonic, tonic in
LGS
STM
Carbonic
anhydrase
FS
STP GABAA receptors Dravet syndrome
Anna Rosati et al., 2015

RICHARD E. APPLETON and J. HELEN CROSS, Drug treatment of paediatric epilepsy, chapter 30

Broad-Spectrum Agents
Valproate
Felbamate
Lamotrigine
Topiramate
Zonisamide
Levetiracetam
Rufinamide*
Vigabatrin
Narrow-Spectrum Agents
Partial onset seizures
Phenytoin
Carbamazepine
Oxcarbazepine
Gabapentin
Pregabalin
Tiagabine
Lacosamide
Eslicarbazepine
Absence
Ethosuximide

Seizure type
Efficacy
Cost
Pharmacokinetic profile
Adverse effects
Considerations in Choosing AED in ped epilepsy

Seizure type
Efficacy
Cost
Pharmacokinetics
Adverse effects

AED Dosage (oral) (mg/kg/day) AEDs interactions
CBZ 10–20 PB, VPA, LTG, ESL, LCS, PER, STP, CZP
CLB 0.5–1 (maximum 30 mg/day) FBM, GVG, PGB, STP
CZP 3–6 CBZ, PB, PHT, VPA
ETS 20–30 VPA
PB 3–5 < 5 years; 2–3 > 5 years CBZ, PHT, VPA, ESL, LCS, PER, RUF, STP, CZP
PHT 8–10 < 3 years; 4–7 > 3 years
PB, VPA, GVG, OXC, TPM, ESL, LCS, PER, RUF, STP,
CZP

VPA 15–40 CBZ, ETS, PB, PHT, LTG, TPM, CZP
FBM 15–45 CLB
GPT 25–35
GVG
20–80; 100–150 for infantile
spasms
CLB, PHT, RUF
LEV 20–40
LTG
5–15 (add-on enzyme inducers); 1–
3 (add-on VPA); 1–5 (add-on
VPA + inducers)
CBZ, VPA, OXC, RUF, RTG

TGB 0.5–2 PGB
TPM 4–6 PHT, VPA, ZNS, ESL
ZNS 4–12 TPM
ESL 800–1200 mg/day (adults) CBZ, PB, PHT, TPM
LCS 200–400 mg/day (adults) CBZ, PB, PHT
PER 8–12 mg/day (>12 years) CBZ, PHT, PB, OXC

RUF 30–40 PB, PHT, GVG, LTG
STM 5 CLB
STP 50 CBZ, CLB, PB, PHT

AED Common AEs Severe AEs
CBZ Ataxia, diplopia, rash
Aplastic anaemia, agranulocytosis,
liver toxicity, SJS/TEN, pancreatitis,
SLE
CLB Sedation No
CZP Sedation No
ETS
Gastric discomfort, hiccups, rash, blurred
vision, headache
SJS/TEN, liver toxicity, SLE
PB
Behavioural problems, drowsiness, rash,
cognitive impairment
Agranulocytosis, SJS/TEN, liver
toxicity, SLE
PHT
Ataxia, diplopia, nystagmus, acne, gum
hypertrophy, hirsutism, cognitive and sedative
affects, peripheral neuropathy
Megaloblastic anaemia, lymphoma,
agranulocytosis, SJS/TEN, liver
toxicity, SLE, encephalopathy,
choreoathetosis

VPA
Nausea, epigastric pain, tremor, alopecia,
weight gain, hyperammonaemia
SJS/TEN, liver toxicity, SLE,
pancreatitis, encephalopathy
FBM
Insomnia, somnolence, behavioural problems,
movement disorders, vomiting, anorexia,
urolithiasis.
SJS/TEN, liver toxicity, pancreatitis,
SLE
GPT
Asthenia, somnolence, depression,
behavioural problems, ataxia, diplopia, rash,
urinary incontinence.
SJS/TEN, liver toxicity, behavioural
problems
VGB
Sedation, behavioural problems,
hallucinations, blurred vision, nausea,
anorexia, weight gain, MRI abnormalities.
Liver toxicity, pancreatitis, psychosis,
visual field defects, encephalopathy
.
LEV
Asthenia, somnolence, behavioural problems,
hallucinations, headache, vomiting, infections.
Psychotic events, liver toxicity,
pancreatitis .
LTG
Behavioural problems, ataxia, tremor,
dizziness, diplopia, dyskinesia, tics, nausea,
Aplastic anaemia, SJS/TEN, liver
toxicity, pancreatitis. Lyell’s

OXC
ataxia, dizziness, diplopia, headache, abdominal
pain, nausea, vomiting, rash, infections, fever
SJS/TEN, liver toxicity
PGB
Somnolence, behavioural problems, dizziness,
weight gain, ataxia
TGB
hallucinations, dizziness
SJS/TEN, non convulsive status
epilepticus
TPM
Asthenia, somnolence, aphasia, dysarthria,
depression, behavioural problems,
hallucinations, ataxia, dizziness, paresthesia,
headache, diarrhoea, nausea, anorexia,
hypohidrosis, urolithiasis, infections, fever
SJS/TEN, liver toxicity, pancreatitis
ZNS
headache, constipation, diarrhoea, abdominal
pain, nausea, vomiting, anorexia, rash,
urolithiasis, infections, fever
No

LCS
Insomnia, somnolence, depression, suicidal ideation,
behavioural problems, blurred vision, tics, nausea,
haematological abnormalities.
No
PER
Dizziness, nausea, somnolence, fatigue, irritability,
headachea
No
RTG
Dizziness, somnolence, headache, fatigue, confusional
state, dysarthria, ataxia, blurred vision, tremor, nausea,
urinary tract infectionsa [95]
Suicidal ideationa [95]
RUF
Ashenia, somnolence, dizziness, diplopia, headache,
nausea, vomiting, anorexia, rash
No
STM Somnolence, vomiting, restlessness, anorexia No
STP
Insomnia, somnolence, aphasia, dysarthria, behavioural
problems, ataxia, tremor, diplopia, headache, abdominal No

schooling

Typically dose-related:
Dizziness , Fatigue , Ataxia, Diplopia
 all AEDs
Irritability
 levetiracetam
Word-finding difficulty, scholastic achievement
 topiramate
Weight loss/anorexia
 topiramate, zonisamide, felbamate
Weight gain
 valproate (also associated with polycystic ovarian syndrome )
 carbamazepine, gabapentin, pregabalin

Typically Idiosyncratic:
Renal stones
 topiramate, zonisamide
Anhydrosis, heat stroke
 topiramate
Acute closed-angle glaucoma
 topiramate
Hyponatremia
 carbamazepine, oxcarbazepine

Serious AE
Typically Idiosyncratic:
Aplastic anemia
 felbamate, zonisamide, valproate, carbamazepine
Hepatic Failure
 valproate, felbamate, lamotrigine, phenobarbital
Peripheral vision loss
 vigabatrin
Rash
 phenytoin, lamotrigine, zonisamide, carbamazepine

Ketogenic deit
 Main experience with children, especially with multiple
seizure types.
 Likely anti-seizure effect of ketosis (beta hydroxybutyrate), but
other mechanisms also may be responsible for beneficial
effects.
 Low carbohydrate, adequate protein, high fat.
 50% with a >50% seizure reduction, 30% with >90% reduction.
 Side effects include kidney stones, weight loss, acidosis,
dyslipidemia.

 FDA approval 1997
 Patients with intractable epilepsy <
12ys
 35% of patients have a 50% reduction
in seizure frequency
 20% experience a 75% reduction
after 18 months of therapy.
Vagal Nerve stimulation

• Epilepsy syndrome not responsive to medical management
– Unacceptable seizure control despite maximum tolerated
doses of 2-3 appropriate drugs as monotherapy
• Potentially curative
– Resection of epileptogenic region (“focus”) avoiding
significant new neurologic deficit
• Palliative
– Partial resection of epileptogenic region
– Disconnection procedure to prevent seizure spread
• Callosotomy
• Multiple subpial transections
Epileptic surgery

 Seizure freedom for  2 years implies overall >60%
chance of successful withdrawal in some epilepsy
syndromes.
 Favorable factors
 Control achieved easily on one drug at low dose.
 No previous unsuccessful attempts at withdrawal.
 Normal neurologic exam and EEG.
 Primary generalized seizures except JME.
 “Benign” syndrome.
Discontinuing AED

Tips and tricks in MANAGEMENT of

THANK YOU
amrhasanneuro@kasralainy.edu.eg

Pediatric epilepsies

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