1. RECENT GUIDELINES ON MANAGEMENT
OF CHILDHOOD EPILEPSY
 Moderator- Dr. Sharja Phuljhele(M.D.)
Professor and head –Deptt. Of Paediatrics
PRESENTED BY –DR. YAVNIKA JAIN

2. ABOUT EPILEPSY
 A seizure is the manifestation of an abnormal, hypersynchronous discharge of
a population of cortical neurons. It can be a clinical seizure, or it may be
subclinical seizure.
 The current definition of epilepsy is the tendency to have repeated seizures
(at least two) as a consequence of a brain disorder, that is, unprovoked by an
acute systemic or brain insult. This definition stresses that the problem is
one of brain function, and that the patient has the potential for more
seizures. This definition excludes seizures due to exogenous factors.
AMERICAN EPILEPSY SOCIETY 2015

3. ILAE Classification of Seizures
Seizures
Partial Generalized
Simple Partial
Complex Partial
Secondarily
Generalized
Absence
Myoclonic
Atonic
Tonic
Tonic-Clonic
C-Slide 3 ILAE – International League Against Epilepsy

4.  Epileptic discharges that occur in the sensory cortex may produce sensory
seizures that manifest as hallucinations or illusions.
 Motor seizures alter muscle activity. Localized tonic posturing (stiffening) or
clonic movements (twitching, jerking) can occur. Abnormal movements may
be restricted to one body part or involve gradual spread to adjacent areas on
the same side of the body (Jacksonian seizure) or both sides of the body with
loss of consciousness (secondarily generalized seizure).
 The diverse range of simple partial seizures gives rise to diagnostic
challenges.
SIMPLE PARTIAL SEIZURE

5. COMPLEX PARTIAL SEIZURE
 A common misunderstanding is that this requires seizure spread to both sides
of the brain. The majority of complex partial seizures originate in the
temporal lobe and can affect consciousness while still remaining focal.
Automatic movements (automatisms) are common and involve the mouth
(e.g., lip smacking, chewing, swallowing), upper extremities (e.g., fumbling,
picking), vocalization/verbalization (e.g., grunts, repeating a phrase), or
complex acts (e.g., shuffling cards). More dramatic automatisms occasionally
occur (e.g., screaming, running, disrobing, pelvic thrusting).
 After the seizure, postictal confusion is common, usually lasting less than 15
minutes, although other symptoms, such as fatigue, may persist for hours.

6. SECONDARY GENERALIZED EPILEPSY
 Begins focally, with or without focal neurological symptoms. Variable
symmetry, intensity, and duration of tonic (stiffening) and clonic (jerking)
phases . Typical duration 1-3 minutes .Postictal confusion, somnolence, with
or without transient focal deficit.
 following a secondarily generalized seizure, the patient may have focal
weakness (Todd’s paralysis) on the side contralateral to seizure onset.

7. ABSENCE SEIZURE
Brief staring spells (“petit mal”) with impairment of awareness
 3-20 seconds
 Sudden onset and sudden resolution
 Often provoked by hyperventilation
 Onset typically between 4 and 14 years of age
 Often resolve by 18 years of age
 Normal development and intelligence
 EEG: Generalized 3 Hz spike-wave discharges

8. ATYPICAL ABSENCE SEIZURES
Brief staring spells with variably reduced responsiveness
 5-30 seconds
 Gradual (seconds) onset and resolution
 Generally not provoked by hyperventilation
 Onset typically after 6 years of age
 Often in children with global cognitive impairment
 EEG: Generalized slow spike-wave complexes (<2.5 Hz)
 Patients often also have Atonic and Tonic seizures

9. PARTIAL SEIZURE
 Two main types , depending on whether or not consciousness is fully
preserved. Simple partial seizures :consciousness is preserved; the person is
alert, can respond to questions or commands, and cant recall.
 Complex partial seizures: consciousness is altered or lost; the ability to pay
attention or respond to questions or commands is thus impaired or lost.
Often, there is no memory of what happened during all or part of the complex
partial seizure.
 Partial onset seizures may progress to secondarily generalized seizures.
Secondarily generalized seizures ultimately involve motor activity on both
sides of the body.

10. MYOCLONIC SEIZURE
Brief, shock-like jerk of a muscle or group of muscles . Differentiate from
benign, non epileptic myoclonus (e.g., while falling asleep).
EEG: Generalized 4-6 Hz polyspike-wave discharges.
Epileptic myoclonus usually causes bilateral, synchronous jerks most often
affecting the neck, shoulders, upper arms, body, and upper legs.
Consciousness does not usually seem to be impaired, although this is difficult to
verify given the brief duration of <1 second.

11. TONIC SEIZURES
• Symmetric, tonic muscle contraction of extremities with tonic flexion of waist
and neck
• Duration - 2-20 seconds.
• EEG – Sudden attenuation with generalized, low-voltage fast activity (most
common) or generalized polyspike-wave.
ATONIC SEIZURES
• Sudden loss of postural tone
• When severe often results in falls
• When milder produces head nods or jaw drops.
• Consciousness usually impaired
• Duration - usually seconds, rarely more than 1 minute
• EEG – sudden diffuse attenuation or generalized polyspike-wave

12. GENERALIZED TONIC CLONIC SEIZURE
 Associated with loss of consciousness and post-ictal confusion/lethargy
 Duration 30-120 seconds
 Tonic phase
 Stiffening and fall
 Often associated with ictal cry
 Clonic Phase
 Rhythmic extremity jerking
 EEG – generalized polyspikes

13. …
EPILEPSY SYNDROME
Grouping of patients that share similar:
 Seizure type(s)
 Age of onset
 Natural history/Prognosis
 EEG patterns
 Genetics
 Response to treatment

14. …
 INTERNATIONAL CLASSIFICATION OF EPILEPSIES AND EPILEPTIC SYNDROMES
 1. Localization-Related (Local, Focal, Partial) Epilepsies and Syndromes
 1.1 Idiopathic (with age-related onset)
 Benign childhood epilepsy with
 centrotemporal spikes (‘rolandic epilepsy’)
 Childhood epilepsy with occipital paroxysms
 1.2 Symptomatic
 Chronic progressive epilepsia partialis continua of childhood
(‘Rasmussen’s encephalitis’)
 Frontal lobe epilepsies
 Occipital lobe epilepsies
 Parietal lobe epilepsies
 Temporal lobe epilepsies
 1.3 Cryptogenic

15. …
 2. Generalized Epilepsies and Syndromes
 2.1 Idiopathic (with age-related onset)
 Benign neonatal familial convulsions
 Benign neonatal convulsions
 Benign myoclonic epilepsy in childhood
 Childhood absence epilepsy (pyknolepsy)
 Juvenile absence epilepsy
 Juvenile myoclonic epilepsy
 2.2 Cryptogenic or Symptomatic
 West syndrome
 Lennox-Gastaut syndrome
 3. Epilepsies and Syndromes Undetermined Whether Focal or Generalized
 4. Special Syndromes

16. Neonatal sizures-
Hypoxic ischaemic encephalopathy
Intracranial haemorrhage
Hypoglycaemia
Infection
Cerebral dysgenesis
Hypocalcaemia
Infancy and childhood
Prenatal or birth injury
Inborn error of metabolism
Congenital malformation
Childhood and adolescence
Idiopathic/genetic syndrome
Trauma
Etiology of Seizures and Epilepsy

17. …
Adolescence and young adult
Head trauma
Drug intoxication and withdrawal*
Older adult
Stroke
Brain tumor
Acute metabolic disturbances*
Neurodegenerative
*causes of acute symptomatic seizures, not epilepsy

18. Idiopathic Partial Epilepsies in Childhood
 1.Benign epilepsy with cento-temporal spikes
(Benign rolandic epilepsy,BECTS)
 This should be considered when a normal school aged child presents with
brief and infrequent, partial, nocturnal, hemi-facial, sensory or motor
seizures.
 An awake-cum- sleep EEG is necessary, as it displays a characteristic pattern
of sleep activated runs of centro-temporal spikes or sharp waves.
 The syndrome has an excellent prognosis with remission in most cases by the
age of 15-16 years.
Guidelines for Diagnosis and Management of Childhood Epilepsy.
EXPERT COMMITTEE ON PEDIATRIC EPILEPSY, INDIAN ACADEMY OF PEDIATRICS
INDIAN PEDIATRICS VOLUME 46__AUGUST 17, 2009

19. …
 2.Panayiotopoulos syndrome (Early-onset childhood epilepsy with occipital
paroxysms, CEOPs)
 CEOP should be considered when a normal preschool (3-5 yrs) child presents
with severe nocturnal vomiting, followed by eye deviation and status
epilepticus, usually hemiclonic. This syndrome has an excellent outcome. A
later-age Gastaut variant of CEOPS is usually characterized by diurnal simple
partial seizures with visual hallucinations or amaurosis and migraine like
headaches. This has a variable prognosis and can persist into adult life.
 Neuroimaging is considered in cases with an abnormal perinatal history or
examination, atypical EEG, or poorly controlled seizures.
 In long term therapy, carbamazepine or valproate are preferred.

20. Idiopathic Generalized Epilepsies
 1.Childhood absence epilepsy (Petit-mal, CAE)
 This should be suspected in a normal school age child with frequent absence
seizures often upto a hundred a day.
 An EEG showing a typical pattern characterized by frontally predominant
generalized bursts of 3 Hz spike wave complexes with abrupt onset is
diagnostic.
 There is no role for routine neuroimaging.
 Valproate and ethosuxsimide followed by lamotrigine and the benzodiazepines
are the drugs of choice.
 Response to AEDs should be confirmed by repeat EEG with hyperventilation to
check the disappearance of typical EEG pattern. Treatment is for a minimum
seizure free period of 2 years with a normal EEG at discontinuation.

21. …
 2.Idiopathic generalized epilepsies of adolescent
 When a child presents with absence, myoclonic or after 10 years, a diagnosis
of idiopathic generalized epilepsies of adolescent onset is considered. EEG
shows generalized paroxysms of spike or polyspikes wave discharges.
Photosensitivity is common. Juvenile myoclonic epilepsy (JME), juvenile
absence epilepsy (JAE) and epilepsies with only GTC seizures should also be
considered in Diagnosis.
 JME presents in adolescents with history of early morning predominant upper
limb myoclonic jerks leading to the patient dropping objects. This occurs
often in sleep-deprived individuals, especially if suddenly awakened. JAE is
similar to CAE, though the numbers of absences are much less and the onset
is usually later. GTC seizures typically occur on awakening or in the evening.

22. …
 Sodium valproate is the most effective drug in most cases of idiopathic
generalized epilepsy.
 Carbamazepine and phenytoin may worsen the syndromes. Lifestyle
adjustments include avoiding precipitating factors like sleep deprivation and
alcohol consumption.
 Seizure control is achieved in 80% with monotherapy. Withdrawal of AEDs
results in relapse in more than 90%, especially in JME and JAE, and often need
low dose lifetime medication.

23. INVESTIGATIONS IN EPILEPSY
 Electroencephalography (EEG)-
 normal EEG does not rule out epilepsy.
 In the child with uncontrolled epilepsy, a repeat EEG helps in reclassifying the
syndrome.
 Before AED discontinuation, an EEG aids in predicting risk of recurrence in
most syndromes barring a few e.g. BECTS.
 In children with unexplained cognitive,neurobehavioral or scholastic
deterioration; an EEG may help in diagnosis of specific disorders like SSPE, or
epileptic encephalopathies like electrical status in slow wave sleep (ESES),
and nonconvulsive status epilepticus.

24. How should an EEG be done?
 EEG should be recorded 3-4 days after the last seizure to avoid post-ictal
slowing from interfering with the interpretation.
 A sleep EEG after deprivation should be part of all routine recordings in
children above the age of three years.
 Minimum activation procedures like hyperventilation and photic stimulation
should be used.
 Omission of AED prior to EEG recording is not recommended.
 Simultaneous video-EEG is useful in differentiating non-epileptic events from
true seizures and for pre-surgical evaluation.

25. NEUROIMAGING
 MRI is more sensitive than CT and is the modality of choice. MRI protocol
should be adapted to the age of the child and the type of epilepsy syndrome.
 CT retains a role in detecting calcification and in acute situations like head
trauma, status epilepticus, and epilepsy, where granulomas are a possibility.
Neuroimaging is not recommended in benign epilepsies.
 High resolution MRI with special techniques is recommended for delineating
the epileptogenic zone and the eloquent cortex in pre-surgical evaluation.

26. Catastrophic Epilepsies in Infancy and
Early Childhood
 1. Early myoclonic encephalopathy
 2. ohtahara syndrome of infancy
 3. epilepsy of infancy with migrating
 4. west syndrome
 5. Dravet syndrome
 6. epilepsy with myoclonic atonic seizure(astatic) {Doose syndrome}
 7. Lennox gestaut syndrome
 8. Landau Kleffner syndrome
 9. epileptic encephalopathy with continuous spike and wave during sleep
(CSWS).

27. WEST SYNDROME
 A detailed history of preceding perinatal events (hypoxic-ischemic
encephalopathy, neonatal hypoglycemia, developmental
milestones,examination of the skin for stigmata of tuberous sclerosis, head
circumference measurement and careful neurologic/developmental
examination looking for deficits/delays will help to differentiate cryptogenic
vs. symptomatic spasms.
 An EEG should be done to confirm the diagnosis, though the characteristic
pattern of hypsarrhythmia is not mandatory for diagnosis. Moreover, an
experienced electroencephalographer should be available for interpretation.

28. …
 Steroids are the drugs of first choice in all cases of West syndrome especially
so in cryptogenic WS, except in tuberous sclerosis.
 ACTH is preferred over oral steroids.
 Oral prednisolone is given in doses of 2-4 mg/kg or natural ACTH in 30-40
units/day (3-6 U/kg) for 2 weeks, with rapid taper over the next 2 weeks.
 Rapid control of the spasms within 1 month of onset is associated with rapid
developmental gains (VPA could be continued after steroids in symptomatic
West syndrome).
 Vigabatrin is preferred in TS as first choice and in steroid failures in the
others (taking into account availability/affordability issues). It should be used
for a period of 3-6 months only, due to the fear of visual field defects.

29. LENNOX GASTAUT SYNDROME
 Any toddler, who has epileptic drop attacks and is delayed or has arrest in
development, should be considered to have LGS. Usually other types of
seizures are also present (like atypical absence and brief tonic seizures in
sleep).
 MRI is essential.
 An EEG should be performed to confirm the diagnosis though the typical slow
spike-wave paroxysms are not mandatory for diagnosis. It is mandatory for
diagnosis of non-convulsive status epilepticus, which often occurs in LGS and
manifests as decreased responsiveness, drooling and regression of milestones
lasting hours to days
 VPA and CLB should be used initially. LTG or TPM should be added in case of
continuing seizures.Ketogenic diet should be used early, if available.It is best
to refer such children to a tertiary epilepsy center to manage these
complicated patients.

30. DRAVET’S SYNDROME (SEVERE MYOCLONIC
EPILEPSY OF INFANCY)
 They often present as refractory febrile or afebrile status epilepticus (SE)
which often lasts several hours.
 Over the next 2-3 years, delayed language development, autistic features
and later, gait difficulties become evident. Myoclonic and absence seizures,
often photosensitive usually become prominent after the first year, though
they are not mandatory for diagnosis.
 VPA, CLB and TPM are drugs most likely to help prevent SE, though a full
remission is unlikely to occur. LTG and CBZ regularly worsen these seizures
and therefore should be avoided in all febrile seizures in infancy, even those
which are clinically focal.

31. REFRACTORY EPILEPSIES IN OLDER CHILDREN
AND ADOLESCENTS
 MESIAL TEMPORAL LOBE EPILEPSY-
 Clinical recognition of mesial temporal lobe epilepsy (MTLE) is important as it
is the most common refractory epilepsy syndrome in the older child/teenager.
It is often caused by hippocampal sclerosis, though other etiologies like
cortical dysplasia, tumors and vascular malformations may also underlie it. It
presents as complex partial seizures with an aura of fear or epigastric
sensation followed by unresponsiveness, automatisms and later secondary
generalization. The patients have often had febrile seizures (often febrile
status epilepticus) in the past. The complex partial seizures present several
years later and over time become refractory.

32. …
 Sleep deprived EEGs with special electrodes are often needed. High quality
MRIs need to diagnose hippocampal sclerosis and where MTLE is suspected.
 In many children, conventional AEDs like CBZ, PHT and newer AEDs like OXZ
and TPM are effective for a while in controlling seizures.
 A large number become resistant to AEDs and there is a progressive cognitive,
behavioural and memory impairment, if the epilepsy remains uncontrolled.
Early surgery in the form of anterior temporal lobectomy is significantly
more effective than best medical treatment in adults.

33. EPILEPSIA PARTIALIS CONTINUA
 Epilepsia partialis continua should be suspected when focal, fairly constant
myoclonic/clonic jerks involve one or more parts of the body (face/limb/
tongue) only unilateraly. In most children, progressive, presumably immune-
mediated encephalitis, Rasmussen’s encephalitis underlies this disorder.
Over time, a progressive hemiplegia with deterioration in cognition and
behaviour is usual, as the epilepsy is resistant to all AEDs.
 Investigation should include a MRI which is initially often normal but shows
progressive hemiatrophy and unilateral signal changes. EEGs are also helpful
though they may be deceptively show absence of abnormalities. Only focal
background disturbances may be seen. Till a hemiplegia develops immune
therapies like steroids and IVIG can be used.
 Treatment is again primarily surgical and a hemispheric
resection/disconnection are the procedure that seems to benefit a large
number.
 The only drawback is that a permanent motor/visual field defect is invariant
after a hemispherectomy. Hence, this procedure becomes difficult in children
who still have good function of the limbs.

34. …
 It is important to recognize rare but important syndromes like the Landau-
Kleffner syndrome (LKS) and Continuous Spike-Wave in Slow-Wave Sleep
(CSWS). LKS presents as predominant language deterioration in a previously
normal child who may or may not have clinical seizures. Initially the child
may stop responding when called and may appear to be deaf; there may be
school performance as well as behavior deterioration.
 CSWS is a more global disturbance with a frank dementia and autism. Both
these syndromes are presumably causally associated with a continuous
epileptic discharges in non-REM sleep – electrical status in slow wave sleep
(ESES). Hence, any child with or without epilepsy, who has cognitive,
language and behavioural deterioration should have an awake and more
importantly sleep EEG to establish the diagnosis. Evaluation and management
of these complex syndromes need referral to specialized epilepsy centers.

35. STATUS EPILEPTICUS
 Status epilepticus (SE): A seizure lasting more than 30 minutes or recurrent
seizures for more than 30 minutes during which the patient does not regain
consciousness .
*Operational definition: Generalized, convulsive status epilepticus in adults
and older children (>5 years old) refers to >5 min of (i) continuous seizures or
(ii) two or more discrete seizures between which there is incomplete recovery
of consciousness.
Refractory SE: Seizures persist despite the administration of two appropriate
anticonvulsants at acceptable doses, with a minimum duration of status of 60
minutes (by history or on observation).
Super-refractory SE: SE that continues 24 hours or more after the onset of
anesthesia, including those cases in which the status epilepticus recurs on the
reduction or withdrawal of anesthesia.
Multi-disciplinary Consensus Development Workshop on
Management of Status Epilepticus in Children in India’ 2013

37. REFRACTORY STATUS EPILEPTICUS
 EEG monitoring, if available, is important both to monitor for electroclinical
seizures or non-convulsive electrographic seizures, and to titrate therapy and
the depth of anesthesia. Additional investigations include a high resolution (3
Tesla) MRI to look for cortical dysplasias, metabolic work-up (blood Tandem
mass spectrophotometry and/or blood/urine Gas chromato-graphy mass
spectrophotometry) in young children, and work-up for autoimmune
encephalitis in patients with de novo status epilepticus associated with fever.

38. TREATMENT
 1. benzodiazipines
 2. other anti-epileptic drugs-use wisely
 3. topiramate – newer drug
 4. midazolam infusion/PROPOFOL???  Doses up to 36µg/kg/min have been
used in previous studies, and may be tried provided it is being used in an ICU
setting and appropriate monitoring and management facilities are available.
Tapering should be started 24-48 hours after seizure stops at the rate of 1
µg/kg/min, every 3-4 hours.
 5. Thiopental sodium with bolus of 3 mg/kg, repeated after 2 min, followed
by maintenance (1-5 mg/kg/hr) to control seizures and/or to achieve
"suppression-burst" EEG activity (increasing 1 mg/kg/hr every 2 minutes
 6. Thiopental usually causes respiratory depression. It can also induce
hypotension and heart failure, and inotropic support is frequently needed.

39. SUPER-REFRACTORY STATUS EPILEPTICUS
 Around 15% of all those presenting to hospital in SE develop super-refractory
status epilepticus and the mortality is 30-50%.
 Therapies for this entity have not been well studied. Treatment modalities
depend on the availability of resources, and experience and familiarity of the
treating physicians with the various modalities. Other than the previously
mentioned drugs; the agents and modalities that have been tried in super-
refractory status epilepticus include ketamine, inhalational halogenated
anesthetics, magnesium infusion, steroids and immunotherapy,
ketogenic diet, hypothermia, electrical and magnetic stimulation
therapies, electroconvulsive therapy, and CSF drainage. Emergency
neurosurgery may be considered in children in whom a lesion has been
detected as the cause of status epilepticus, e.g. cortical dysplasia.

40. FEBRILE SEIZURE
 Any prophylaxis of febrile seizures reduces the recurrence of seizures but
does not reduce the risk of future epilepsy. Intermittent prophylaxis with oral
clobazam in a dose of 0.75 mg/kg for 2-3 days in 2 divided doses during fever
is useful to prevent recurrence. Febrile status, complex and recurrent febrile
seizures (>6/year in spite of intermittent prophylaxis) may need EEG,
neuroimaging and continuous prophylaxis with AED. Phenobarbitone and
valproate may be used in infants and older children respectively, for 1-2
years. Carbamazepine and phenytoin are not useful.

41. WITHDRAWL OF ANTI-EPILEPTICS ??
 New-onset SE: Further treatment decisions should be similar to that for a First
seizure.
Acute symptomatic seizures: Further treatment depends on the control of the
precipitating event.
SE in known epilepsy:
• After control of SE for 24 hours, tapering of drugs should be started with
‘last in, first out’ as the guiding principle.
• All the AEDs should preferably be stopped during hospital stay and the child
discharged on:
– Augmented dose of the previous AED/s (if levels were sub-therapeutic or
prescribed dose was less than maximum dose); and
– Introduction of another appropriate AED (either replacement or addition),
if previously receiving maximum doses of AED/s.

42. AEDs-
 Phenobarbitone –
 Enhances GABA a receptor mediated synaptic inhibition and antiglutamate
action.
 can be used as a first line AED in neonatal seizures, in the first two years of
life for partial/GTC seizures and in neonatal and early infantile status
epilepticus(SE). Since deleterious cognitive and behavioral side effects remain
a concern, it should be avoided In schoolgoing children.
 Phenytoin-
 Prolongs inactive state of sodium channel + pro-gaba + anti glutamate action.
 Though effective, should not be preferred as a primary AED in newly
diagnosed epilepsy, especially in infancy, as levels fluctuate frequently in
infants, making monitoring of drug levels imperative, and in adolescent girls
as cosmetic side effects may be unacceptable. Maintenance dosages in older
children are between 5-6 mg/kg given in one or two divided doses, but infants
may need upto 15-18 mg/ kg in 3-4 divided doses.

43. …
 Valproate-
 Pro GABA ,anti glutamate , prolongs inactive state of sodium channels and weak attenuation
of Ca mediated T current.
can be the drug of choice for most children with newly diagnosed epilepsy, like idiopathic
generalized epilepsy (CAE, JAE, BMEI, and JME), epilepsies with prominent myoclonic
seizures or with multiple seizure types, and photosensitive epilepsies.
In adolescent girls or obese patients, one may not use it as first line agent. Hair loss may
be reduced by use of supplemental biotin. It could be used in partial epilepsies in infants
where carbamazepine might precipitate generalized seizures and in refractory status
epilepticus.
The dose averages between 10-40 mg/kg/day. Twice-a-day dosing is preferred with
extended release preparations, except in syrup (3 times a day). Parents should be
counseled regarding danger symptoms and signs of hepatitis, like nausea, vomiting,
drowsiness etc, especially in children below the age of 2 years, those on polytherapy and
those with associated IEM, necessitating routine monitoring of LFT. Enzyme elevation upto
twice normal or borderline elevation of ammonia can be disregarded when asymptomatic.
The drug must be stopped immediately in all symptomatic patients irrespective of enzyme
levels. In case the cause of the hepatitis becomes clear e.g. hepatitis A confirmed by
serology, then valproate could be restarted after the hepatitis has resolved. In cryptogenic
hepatitis it is best avoided. Carnitine supplements are not routinely recommended.

44. …
 Carbamazepine-
 Prolongs inactive state of sodium channels, modifies maximal electroshock seizures
raises threshold.
 It is the drug of first choice for all newly diagnosed partial epilepsies, after the age of 2
years. The dose varies between 10-30 mg/kg in the form of twice day dosing and
preferably given as slow release preparations, if syrups are used they should be given
three times a day. Carbamazepine may induce or exacerbate generalized seizures like
infantile spasms, myoclonic, tonic and absence seizures in the younger child.
Paradoxically, it may exacerbate partial seizures as well, in benign partial epilepsies. It
may worsen the EEG with deterioration in cognition, behavior and language & can
rarely precipitate electrical status in slow wave sleep (ESES). Parents should be
informed about the common side effects like appearance of new seizures, deterioration
of school performance or appearance of rash (Steven’s) Johnson’s and Drug Rash
Eosinophilia and Systemic Symptoms- DRESS syndrome shared with phenytoin and
pheno-barbitone) which should be reported immediately. A routine hematological
monitoring is not recommended.

45. …
 Oxcarbazepine- converts to glucoronide conjugated product and not
oxidized.
 Can be used as monotherapy in newly diagnosed partial epilepsy for children
above 4 years of age, if affordable and available. It can be used for add-on
therapy in refractory partial and secondary generalized epilepsy. Start with a
dose of 10 mg/kg; titrated upwards weekly, guided by seizure control, to a
maximum of 40 mg/kg. Though once daily preparations are marketed, it is
prudent to give this drug in two divided doses. An abrupt switchover from CBZ
to OXZ can be done in a dose ratio of 2:3. No routine monitoring of drug
levels, blood counts or sodium is recommended, unless symptomatic
(vomiting, drowsiness or increased seizures)

46. …
 Lamotrigine –
 broader spectrum , CBZ like action+prolongs sodium inactive state,directly
inhibiting sodium channels(preventing release of aspartate and glutamate).
 Monotherapy in newly diagnosed generalized epilepsy (absence and
myoclonic) and in other partial/generalized epilepsies, and in specific
epilepsy syndromes like idiopathic generalized epilepsy in teenage years,
especially girls (as first choice). Occasionally, myoclonic jerks maybe
paradoxically worsened by lamotrigine, especially in JME. Add-on in
refractory generalized epilepsies like absence, tonic and tonic-clonic and
syndromes like LGS and in refractory partial epilepsies.
 The dose should initially be 0.5 mg/kg (alone), 0.2 mg/kg (with VPA), and 0.6
mg/kg (with phenobarbitone, phenytoin, carbamazepine); it should be
doubled every 2 weeks to a maximum of 15mg/kg (alone) and 5mg/kg/day
(with VPA) and higher when used with enzyme inducers. LTG has to be
titrated slowly to prevent rashes and Stevens Johnson syndrome.

47. …
 Topiramate-
 Hyperpolarizes the membrane,phenytoin like prolongation of inactive state of
sodium channels, phenobarb like pro GABA action and antagonist of
glutamate.
 It can be used as a second line add-on agent in refractory partial and
generalized epilepsies as well as Lennox Gastaut syndrome. It maybe
particularly useful in certain syndromes like infantile spasms and Dravet’s
syndrome. At present, its use as first line monotherapy in newly diagnosed
epilepsy is not recommended because of a significant adverse effect profile.It
should be started at a dose of 0.5-1 mg/kg in bid doses, escalated weekly or
biweekly, upto maximum of 5-10mg/kg; Higher doses (10-30 mg/kg) and rapid
escalation (every 3 days) are considered in special situations (infantile
spasms, status epilepticus); however, there could be a higher incidence of
adverse events with high doses.
 Clinical monitoring for adverse effects like weight loss, eye symptoms like
blurring, redness,watering and eye pain (glaucoma/myopia), metabolic
acidosis and oligohydrosis is necessary in all cases. Decreased appetite and
weight loss are expected and should be communicated to the caregivers.
Cognitive adverse effects can be minimized by converting to topiramate
monotherapy, if possible. Hydration should be maintained and calcium
supplements should be avoided to minimize risk of renal stones.

48. …
 Levatiracetam-
 It should be used only as an add-on drug to refractory partial and some
generalized epilepsies like,refractory absence or progressive myoclonic
epilepsies. It is not recommended to be used as a first-line agent in newly
diagnosed epilepsies,though recent data support a role in the idiopathic
generalized epilepsies of adolescents (JME etc). Behavioral adverse effects
like aggression are the most common adverse effects, rarely a paradoxical
increase in seizure frequency may occur and this should be monitored
carefully. The usual effective dose is between 20-60 mg kg /day. One can
start at 20 mg/kg in two doses and increase every 1-2 weeks till 60
mg/kg/day.

49. …
 Tiagabine-
 Potentiates GABA mediated neuronal inhibition.
 As add-on in refractory partial seizures. It is not recommended as
monotherapy in children with newly diagnosed epilepsy. NCSE (non convulsive
status epilepticus) can occur in about 8% of patients and should be carefully
excluded in children whose seizures/mental status deteriorate on treatment.
It is used in an initial daily dose 0.1 mg/kg TID; increased weekly by 0.1
mg/kg; maximum daily dose 0.4 and 0.7 mg/kg (uninduced and induced,
respectively). In children over 12 years, it can be initiated at 4 mg/day; total
daily dose increased by 4 mg in week 2 (divided doses); then increased by 4 to
8 mg/day (divided doses) each week until clinical response is achieved or to a
maximum daily dose of 32 mg/d is reached.

50. LATEST DRUGS-
 ZONISAMIDE-
Focal seizures with or without GTCS. Used as adjunct in LGS and JME.
Started at 1-2 mg /kg/day to maintenance of 4-8 mg/kg/day.
S/E Nephrolithiasis and oligohydrosis.
 LACOSAMIDE-
Paediatric experience is limited .
Used in children >16 yrs with refractory focal seizures.
S/E  dizziness, headache,diplopia.
 RUFINAMIDE-
Add-on therapy for LGS .
Start with 200mg /day in children <30 kg , upto maximum of 1000mg/day.( if
used with valproate then 400 mg/day)
Not use in those with short QTc syndrome.
 STIRIPENTOL-
Used in GTCS with Dravets syndrome

51. …
Guidelines for Diagnosis and Management of Childhood Epilepsy.
Neurology chapter of IAP-2014.

52. …
DOSES AND SIDE EFFECTS OF COMMON ANTIEPILEPTIC DRUGS-
 Phenobarbitone 3-8 mg/kg Hyperactivity, academic deterioration, reversal of sleep
cycles,megaloblastic anaemia,osteomalacia
 Phenytoin 5-15 mg/kg Poor seizure control due to fluctuating drug levels, cosmetic
side effects,hirsutism, ataxia
 Valparin 10-60 mg/kg Nausea, vomiting, loss of appetite, weight gain, irregular
menstruation, alopecia, hepatotoxicity,somnolence,teratogenicity
 Carbamazepine 10-30 mg/kg Drug rash, worsening seizures,dizziness, vertigo,
rarely worsening school performance
 Oxcarbazepine 20-45 mg/kg Somnolence, vomiting (hyponatremia), seizure
exacerbation
 Lamotrigine 0.2- 15 mg/kg Drug rash, Steven-Johnson syndrome
 Clobazam 0.4-1.2 mg/kg Behaviour changes, aggression, sleep disturbances,
constipation, weight gain
 Topiramate 3-9 mg/kg Cognitive/language deterioration, fever, acidosis in infancy
 Levateracetam 15-45 mg/kg Behaviour changes
 Tiagabine 0.5-2 mg/kg Somnolence, Seizure exacerbation

53. DIET
 Ketogenic Diet in Epilepsy - The ketogenic diet (KD) is a stringently controlled high
fat and low protein/carbohydrate diet given with/without a restricted fluid intake
to maintain ketosis on a long term basis. It has been shown that it is more
efficacious than newer AEDs in controlling refractory seizures and is more cost
effective. It can be used with both non-vegetarian and vegetarian diets at any age
and for all types of Seizures. It has significant improvements in hyperactivity and
aggression in almost all patients. Hence, it should be tried in all children above the
age of 1 year with drug-resistant epilepsy, especially those who are not a surgical
candidates or where surgery cannot be performed due to availability/affordability
issues. Referral to centers providing the KD should be considered once adequate
trials of three AEDs have failed, suggestive of pharmaco-resistant epilepsy. Adverse
effects include GI disturbances, acidosis, increased susceptibility to infections,
drowsiness, weight loss, nutritional deficiencies and rarely, renal calculi and
pancreatitis. Most of these occur early in the diet and should be carefully
monitored. The diet should be considered a failure if there is no benefit in 3-6
months and it should be discontinued after this time. In responders, it should be
continued for 2-3 year after which it is gradually tapered.
 The ketogenic diet for the treatment of childhood epilepsy: a randomised controlled trial Lancet
Neurol 2008; 7: 500–06 Published Online May 3, 2008
Elizabeth G Neal, Hannah Chaff e, Ruby H Schwartz, Margaret S Lawson, Nicole Edwards,
Geogianna Fitzsimmons, Andrea Whitney,

54. SURGERY
 Ideal surgically remediable syndromes
 include:
 • Hemispheric epilepsies with pre-existing contralateral hemiplegias/visual
field defects caused by large unilateral gliotic lesions/atrophy, Rasmussen’s
encephalitis, hemispheric dysplasias etc, where
hemispherectomy/hemispherotomies could offer a possible surgical cure.
 Discrete lesions without involvement of functional motor, visual and language
cortex,where a lesionectomy will often result in a complete cure. Common
lesions would include developmental tumors, cortical dysplasias, AVMs etc.
Sometimes lesions like large dysplasias/infarcts may need
lobectomies/multilobar resections.
 • Mesial temporal lobe epilepsy caused often by hippocampal sclerosis is not
uncommon in teenagers and is amenable to an anterior temporal lobectomy.
 • Drop attacks with injuries respond well to corpus callosotomy and should be
offered as a palliative procedure.

55. REFERENCES-
 1 .Guidelines for Diagnosis and Management of Childhood Epilepsy.
EXPERT COMMITTEE ON PEDIATRIC EPILEPSY, INDIAN ACADEMY OF PEDIATRICS
INDIAN PEDIATRICS VOLUME 46__AUGUST 17, 2009
 2. Guidelines for Diagnosis and Management of Childhood Epilepsy.
Neurology chapter of IAP-2014.
 3. Multi-disciplinary Consensus Development Workshop on Management of Status
Epilepticus in Children in India’ 2013.
 4. The ketogenic diet for the treatment of childhood epilepsy: a randomised controlled
trial Lancet Neurol 2008; 7: 500–06 Published Online May 3, 2008
Elizabeth G Neal, Hannah Chaff e, Ruby H Schwartz, Margaret S Lawson, Nicole Edwards,
Geogianna Fitzsimmons, Andrea Whitney,