About Epilepsy in Children

1. SEIZURESEPILEPSY

2. DEFINITIONS
Seizure- A transient occurrence of signs and/or symptoms resulting
from abnormal excessive or synchronous neuronal activity in brain
Epilepsy- Two or more unprovoked seizures more than 24 hrs apart –
epileptic tendency OR One Seizure with abnormal Neurological
Examination Or EEG Or MRI
Acute symptomatic seizures - occur secondary to an acute problem
affecting brain excitability such as electrolyte imbalance but can
also signify major structural, inflammatory, or metabolic disorders of
the brain, such as meningitis, encephalitis, acute stroke, or brain tumor.
Unprovoked seizures- is one that is not an acute symptomatic seizure

3. • Remote symptomatic seizure is one that is considered to be
secondary to a distant brain injury, such as an old stroke.
• Reflex seizures are usually precipitated by a sensory stimulus such as
flashing lights
• Seizure disorder is a general term that is usually used to include any 1 of several
disorders, including epilepsy, febrile seizures, and possibly single seizures and
symptomatic seizures secondary to metabolic, infectious or other etiologies.
• An epileptic syndrome is a disorder that manifests 1 or more specific seizure
types and has a specific age of onset and a specific prognosis.
• An epileptic encephalopathy is an epilepsy syndrome in which there is a severe
EEG abnormality which is thought to result in cognitive and other impairments in
the patient

4. • OLD NAMES
• Idiopathic epilepsy- epilepsy syndrome due to genetics
• Symptomatic epilepsy- due to known underlying brain disorder
• Cryptogenic epilepsy(presumed symptomatic epilepsy)- due
underlying brain disorder which is not known- uknown epilepsy
• NOTE; All seizures are not epilepsy

5. EPIDEMIOLOGY
• Seizures affect 4 to 7 % of children. Up to 1% of all Emergency
Dept visits are paediatric seizures.
• Febrile seizures: 1 in 125 visits (0.8%) while Afebrile: 1 in 500 visits
(0.2%) .
• Epileptic seizures affect 1-2% of the population & 4% of children.
• Developing countries have higher prevalence.
• 40% develop epilepsy before the age of 16 yrs.
• Focal epilepsies commoner than generalized.

6. CAUSES
• A) Infections; meningitis, encephalitis, neurocysticercosis, brain abcesses &
shigella Gastroenteritis
• B) metabolic; hpocalcimea,hypomagnesemia,hyponatremia,hypoglycaemia
,hypoxia,hypercarbia,pyridoxine deficiency & inborn errors of metabolism.
• C) Drugs; Cocaine, amphetamine, Alcohol e.t.c
• D) trauma- head injury( accidental or child abuse), VP shunt malfxn
• E) poisons- OPP, lead f)idiopathic- epilepsy
• g) idiopathic- epilepsy h) dev'tal & congenital- ,genetics(inborn errors of
metabolism), anomalies,brain injury during birth, degenerative cerebral
dsses, neurocutaneous syndromes

7. RISK FACTORS
• A family history of epilepsy
•A previous history of epilepsy; whether the child is •Taking
antiepileptic drugs (AEDs)
• Presence of conditions associated with electrolyte (magnesium,
phosphate, or calcium) disturbances, such as diarrhea or rickets;
• Presence of acidosis associated with hypoxia
• Provoking factors, such as sleep deprivation, fevers, illness or
infections, fatigue, decreased physical health, alcohol ingestion,
emotional stress, flashing lights, menstrual cycle, missed meals

8. PATHOPHYSIOLOGY
• Organized into 4 distinct, sequential, mechanistic processes.
a) Underlying etiology- any pathology or pathologic process that can
disrupt neuronal function and connectivity; are diverse and include,
among other entities, brain tumors and malformations, strokes,
scarring, or mutations of specific genes like those encoding for
voltage gated channels(Na+, K+, Ca2+, Cl) and ligand gated channels
like GABAA receptors.
b) Epileptogenesis- due to repititive stimulation by the etiologies,
epilepsy results. This is because of activation of glutamate
receptors------------> influx of calcium ions-----> increased
excitability.

9. c) Resultant epileptic state of increased excitability- every pt has this
step irrespective of the cause. Epileptic neurons usually have
stereotypic paroxysmal depolarisation phase that result in sudden
excitation of neurons, this is supposed to be followed by
after hyperpolarisation phase but a number of these neurons have loss
of k+ and GABA which opens cl- channels that is responsible for
hyperpolarisation and relaxation of neurons. Hence there is increased
excitation and loss of inhibition.
d) Seizure-related neuronal injury as demonstrated by MRI in patients
after prolonged febrile and afebrile status epilepticus. Due to apoptosis
and necrosis of these neurons. Hippocampus mostly affected.

10. CLASSIFICATION based on ILAE 1981
(The International League Against Epilepsy)
• A) Partial/Focal- limited to a part of 1 cerebral hemisphere. Can be
simple( no alteration of conciousness) or complex also called focal
dyscognitive seizures (there's altered conciousness). It can also
transform into generalised seizure and it's known as secondary
generalised seizure.
• B) Generalized- synchronous involvement of all of both hemispheres
associated with loss of conciousness. Subclasses include; Tonic-clonic,
tonic, clonic, Absence, Myoclonic, atonic,

11. 1. focal seizures
• Approximately 40% of seizures in children.
• Simple vs complex. They can each occur in isolation or one can temporally
lead to the other (usually simple to complex), and/or each can progress
into secondary generalized seizures usually tonic-clonic.
A) Simple; These can take the form of either sensory seizures (auras) or brief
motor seizures, the specific nature of which gives clues as to the location
of the seizure focus.
• Brief motor seizures are the most common and include focal tonic, clonic,
or atonic seizures. Often there is a motor (jacksonian) march from face to
arm to leg, versive head and eye movements to the contralateral side, or
postictal (Todd) paralysis that can last minutes or hours, and sometimes
longer.

12. • B) complex- usually last 1-2mins, preceded by Aura most commonly Abd
discomfort, sense of fear, visual hallucinations. It can also be suggestive of
the focal region- micropsia or macropsia (temporal lobe), generalized
difficult-to-characterize sensations (frontal lobe), focal sensations (parietal
lobe), or simple visual experiences (occipital lobe). Subsequent
manifestations consist of decreased responsiveness, staring, looking
around seemingly purposelessly, and automatisms.
• Often there is salivation, dilation of the pupils, and flushing or color
change. The patient might appear to react to some of the stimulation
around him or her but does not later recall the epileptic event.
• After the seizure, the patient can have postictal automatisms, sleepiness,
and/or other transient focal deficits such as weakness (Todd paralysis) or
aphasia

13. • C) Secondarily generalised- Seizures of this type were previously
known as focal seizures with impairment of consciousness evolving to
bilateral convulsive seizures. There is often adversive eye and head
deviation to the side contralateral to the side of the seizure focus
followed by generalized . tonic, clonic, or tonic–clonic activity.
• Tongue biting, urinary and stool incontinence, vomiting with risk of
aspiration, and cyanosis are common. Fractures of the vertebrae or
humerus are rare complications. Most such seizures last 1-2 min.

14. • The EEG for a focal seizure shows focal spikes or sharp waves in the
lobe where the seizure originates. However 15% may be normal.
• MRI and CT scan done for focal seizures- MRI preferred because of it
will show subtle signs.

15. 2. Generalized seizures
• A) absence- Typical absence seizures usually start at 5-8 yr of age and
are often, they can occur up to hundreds of times per day. Have no
Aura and lasts only few seconds. Has no postictal confusion. before
age 5 it isassociated with mental retardation and tendency for future
seizures
• Accompanied by eye lid flutter or upward rolling of the eyes but
typically not by the usually have simple automatisms like lip-smacking
or picking at clothing .

16. • Hyperventilation for 3-5 min can precipitate the seizures and the
accompanying 3 Hz spike–and–slow-wave discharges. Early onset
absence seizures (before the age of 4 yr) should trigger evaluation for
glucose transporter defect that is often associated with low CSF
glucose levels and an abnormal sequencing test of the
transporter gene.
• atypical absence- abnormal sequencing test of the transporter
gene. Atypical absence seizures have associated myoclonic
components and tone changes of the head (head drop) and body and
are also usually more difficult to treat. They are precipitated
by drowsiness and are usually accompanied by 1-2 Hz spike–and–
slow-wave discharges.

17. • B) GTCS- the seizure usually starts with loss of consciousness and, at
times, with a sudden cry, upward rolling of the eyes, and a
generalized tonic (stiffness) contraction of whole body mms
with falling, apnea, and cyanosis. The tonic phase is followed by a
clonic phase (jerky movements/twitching) that, as the seizure
progresses, shows slowing of the rhythmic contractions until the
seizure stops usually 1-2 min later.
• Incontinence and a postictal period often follow. The postictal period
usually lasts for 30 min to several hours with semi coma or
obtundation and postictal sleepiness, weakness, ataxia, hyper- or
hyporeflexia, and complaints of headaches after waking up. There is a
risk of aspiration and injury.

18. • C) Tonic- the tone is greatly increased: the body, arms, or legs become
suddenly stiff or tense. Usually at night and last for <20secs. Occur as part
of epileptic syndromes. There's sustained contraction
• D) myoclonic- brief, shock-like jerks of a individual muscle or a group of
muscles that have rapidly alternating contraction and relaxation—jerking
or twitching—of a muscle. Lasts <50 msec & is often repeated. usually it
don't last more than a second or two. There can be just one, but
sometimes many will occur within a short time. Also does occur as part of
epileptic syndromes.
• E) clonic- rhythmic contractions due to fast stiffening and relaxation of a
muscle. There is repeatited jerking which is more regular and sustained
than myoconic.

19. F) atonic- there's sudden loss of motor tone. Child falls to the floor
and May have myoclonic jerks. It's No a clear generalized seizure and
has apparent syncopal episode with no etiology. Part or all of the
body may become limp. The eyelids may droop, the head may nod or
drop forward, and the child may drop things. if standing, the he/she
may often fall to the ground. These seizures typically last less than 15
seconds. It's characterized by flaccidity or lack of movement during a
convulsion.

20. Other generalised epilepsies
Benign
• Petit mal
• Benign myoclonic epilepsy of
infancy
• Febrile seizures with syndromes
• Juvenile myoclonic epilepsy
(Janz syndrome)
• Photoparoxysmal epilepsy
• Reflex epilepsy
severe
• Early myoclonic infantile
encephalopathy
• Early infantile epileptic
encephalopathy (Ohtahara
syndrome)
• Severe myoclonic epilepsy of
infancy (Dravet syndrome)
• West syndrome
• Lennox-Gastaut syndrome

21. EVALUATION OF THE FIRST SEIZURE
• 1. asses adequacy of the child's ABCs
• 2. asses for - BP, Temp, glucose conc
• 3. adequate hx from both the pt and witnesses - look for underlying or
preceding causes and also events that happened before, during and after
the seizure.
• Before; Aura?, triggering factors?............ During; What was happening?
How long? Was there impairement of conciousness?, was there any bowel
or bladder incontinece? Was there any other postictal complaints? If has
had a seizure before was this typical or atypical?...............
• After; For the observer, was the patient postictal? If postictal, how long
was it? If no observer, did patient know where he/she was, what
had happened immediately after episode? Did the patient have any
complaints when she/he became more awake

22. Other important aspects of hx;
• Hx of illnesses like infections, diarrhoea, trauma & child abuse,
poisoning, non compliance w AEDs if has had epilepsy before
• Birth hx & dev't hx
• Fmshx; family hx of epilepsies, febrile seizures, neurological dsses &
social hx of drug abuse

23. 4. do thorough P/E - both general and neurological. Check at the vitals,
anthropometry, do eye examination to r/o signs like papilledema, look for
cutaneous lesions or pigmentations to r/o neurocutaneous disorders that might be
causing the seizure e.g., facial nevus. Examine for focal deficits that were subtle e.g
subtle hemiparesis, subtle sensory or motor dysfxn
5. investigate- metabolic profile( UECs, Ca, Mg, toxicology screens, RBS, drug levels
if pt on AEDs). infection profile- CBC with differentials, consider lumbar puncture if
having signs of meningism, suspected encephalitis, in children without brain
swelling or papilledema, and in children in whom a history of intracranial
bleeding is suspected without evidence of such on head CT.(on CSF exam check for
xanthocromia if suspecting hemorrhage). Holter monitoring and/or ECG because
seizures are assoc with long QT syndrome and there's risk of dyarrythmias.
6. EEG- done for all pts with afebrile sezures and in all patients with paroxysmal
event.

24. EEG
USES
• To diff seizures from non-
seizures
• To classify the seizure- focal or
generalised
• Decides treatment
• Predicts seizure recurrence
• 3-4 days after the episode to r/o
risk of postictal slowing
• Sleep deprived EEG increases
the yield.
• Photic stimulation & hyper-
ventilation helps
• No need to stop AEDs before
EEG

25. • 7. head imaging- CT or MRI. Emergency head CT in a child presenting
with a first unprovoked nonfebrile seizure is often useful for
acute management of the patient. MRI> CT & CT can be used if
there's suspected mass lesion, hemorrhage, or large stroke. Also used
if MRI is contraindicated.
• Indications for neuro- imaging : Seizures in early infancy, Focal
seizures (not indicated in benign partial seizure) and Developmental
delay
• Not Indicated in : Non-epileptic events, Febrile seizures , Absence
seizures

26. NOTE
• Patients with recurrent seizures, specifically with 2 seizures spaced apart
by longer than 24 hr, warrant further work-up directed at the underlying
etiology. In patients with drug-resistant epilepsy, or in infants with new-
onset epilepsy in whom the initial testing did not reveal an underlying
etiology, a full metabolic work-up, and CSF studies, is needed. Genetic
testing also recommended.
• However Most patients do not require extensive testing. The pace and
extent of the work-up must depend critically upon the clinical epileptic and
nonepileptic features, the family and antecedent personal history of the
patient, the medication responsiveness of the seizures, the likelihood of
identifying a treatable condition, and the wishes and need of the family to
assign a specific diagnosis to the child’s illness.

27. MANAGEMENT

28. Principles of management
• Long term therapy not considered if first seizure and there's normal
neurological exam and imaging but if abnormal exam and imaging
studies or there's a positive family history of epilepsy, then the risk is
higher and often long term therapy considered.
• Educating the family and the child about the disease, its management
and the limitations it might impose I.e any activity where seizure
might cause dangerous fall and how to deal with them E.g To
participate in athletics, there should be a proper medical
management, good seizure control, and proper supervision are crucial
to avoid significant risks. Parents should also know that poor seizure
control and poor meds compliance increases risk of
sudden unexpected death in epilepsy.

29. • Always start monotherapy at low dose & titrate slowly until seizure
remit or adverse effects emerge. Then if not controlled, choose
alternate monotherapy & gradually withdraw 1st drug. if still not
controlled, refer to child neurologist. May require polytherapy,
ketogenic diet or surgery.
• Remember before labelling drug failure, always check compliance;
rule out progressive neurological disorder
• First Line of management are Approved Anti-Epileptic Drugs (AEDs)
• Second Line (intractable epilepsy) are Epilepsy Surgery , Vagus Nerve
Stimulation Therapy, Experimental Therapy with AEDs and Implanted
Devices.

30. AEDs
• Anticonvulsant drugs/ Antiepileptic drugs(AEDs) are used- they reduce
the excitation and enhance inhibition. The therapy should be based on the
type of seizure and the epilepsy syndrome, Drug safety & tolerability, Age &
gender, Ease of administration, Lifestyle, pre- morbid conditions.
• Indications of AED after 1 st seizure are; Focal, Myoclonic & absence
seizures, 1 st episode of status epilepticus, presence of Underlying
structural lesion and also Severe parental anxiety.
• Duration of AED : – Generally given for minimum of 2 yrs seizure free
period in most of idiopathic generalized tonic epilepsies. – AED is required
for longer duration in symptomatic epilepsy, epileptic syndromes & if there
are multiple risk factors for recurrence.

31. • How to taper AED : – Always individualized. Slow taper over 3-6
months after consultation with parents & explaining risk of
recurrence. Most epilepsies remit, relapse in 1-2 yrs after drug
discontinuation. If you Restart previous AED, most patients will remit
again.
• Risk factors for relapses are symptomatic epilepsies, structural brain
lesions, abnormal neurological signs & EEG
• Therapeutic drug monitoring(tdm) indicated if there's Suspicion of
non-compliance, Intractable epilepsy, signs of toxicity and patients on
poly therapy.

32. ALGORITHM FOR AEDs in MXT of Epilepsy
• Most appropriate is low dose First line monotherapy. If uncontrolled
Gradually increase the dose. If seizure recurs, increase to maximum
tolerated dose while looking for side effects and Check compliance.
• Re-evaluate and Rule out progressive neurological disorder.
• Add alternate 2 nd drug and taper & stop the 1 st drug
• If Seizure persists Timely referral/ polytherapy

33. CHOICE OF
AEDS
1st choice drugs;
Focal-
Carbamazepine,
Absence-
ethosuximide, GTCS-
levetiracetam,
carbamazepine, pheno
barb, lamotrigine.

34. SIDE EFFECTS OF AEDs
• Phenobarbital-sedation
• Valproate-weight gain, liver toxicity, decreased platelets, pancreatitis
• Lamotrigine-Steven’s Johnson syndrome
•Topiramate-weight loss, language dysfunction, kidney stones,
glaucoma

35. Intractable epilepsy
• An epilepsy that is not responding well to medical treatment and
experts say it is if it involves 2 AEDs and still not responding.
• 25-35% of all epilepsies are intractable
• However continue the drugs and explore other options;
1. Surgery
2. Vagus N stimulation
3. Experiemental therapy with AEDs and implanted devices like
responsive neurostimulator.
4. Ketogenic diet

36. INFANTILE SPASMS
• A type of severe generalised seizure that's part of a syndrome called
West syndrome that also consists of developmental regression and a
typical EEG picture called hypsarrhythmia.
• starts between the ages of 2 and 12 mo
• Drug used to rx; Adrenocorticotropin Hormone (ACTH) 20 U/day (IM)
for 2 wk, and if no response occurs, the dosage is increased to 30 and
then 40 U/day IM for an additional 4 wk. Though there's no uniform
dose.
• Prednisone is an alternative -2 mg/kg/24 hr for 2 wk with addditional
4weeks if seizure persists

37. STATUS EPILEPTICUS
• is a medical emergency defined as continuous seizure activity or
recurrent seizure activity without regaining of consciousness lasting
for more than 5 min. Previously was '> 30mins'.
• 10-60/100,000 in whole population. >100/100,000 in children <5yrs.
• 30% of these patients are having their first seizure, 40% of these
later develop epilepsy.
• Febrile status epilepticus is the most common type of
status epilepticus in children
• 14% risk of new neurological deficit and 4-5% Mortality rate

38. MECHANISMS
• The mechanisms leading to the establishment of sustained seizure
activity seen in status epilepticus appear to involve;
1. failure of desensitization of glutamate receptors, thus persistence of
increased excitability.
2. Intracellular internalization of GABA receptors causing reduction of
GABA-mediated inhibition
Hypoxic brain injury can result in status epilepticus if prolonged for
30mins or more because of increased brain metabolism that outweighs
the demand more than the supply.

39. THERAPY
1. Secure ABCs first
2. Continuous monitoring of vital signs and also ECG
3. Management of the underlying etiology (e.g., hypoglycemia) if known.
4. Laboratory studies, including glucose, sodium, calcium, magnesium,
complete blood count, UECs for all patients.
5. CT & Continuous EEG needed for all patients.
6. Lumbar puncture spinal fluid cultures, toxic screens, and tests for inborn
errors of metabolism are often needed as per the clinical suspicion.
7. Neuroimaging done- MRI depending on clinical suspicion. Done only
after the child has been stabilised

40. EEG in STATUS EPILEPTICUS
• Advs; 1. R/O pseudo–status epilepticus and other mov't disorders
2. identifying the type of status epilepticus (generalized vs focal)
3. guides the further tests to confirm underlying etiology and
also treatment
4. distinguish between postictal depression and later stages of
status epilepticus in which the clinical manifestations are subtle
or absent
5. monitoring the therapy, particularly in patients who are
paralyzed and intubated.

41. • Initial emergent therapy for status epilepticus; BENZODIAZEPINES
IV Lorazepam – 0.05–0.1 mg/kg IV administered slowly at 1-2 mg/min
up to 10 mg. Can also be given Per Rectal or sublingual, same dose.
IV Diazepam - 0.1–0.3 mg/kg IV at 2 mg/min up to 20 mg. Can also be
given Per Rectal at 0.2–0.5 mg/kg.
• Midazolam - 2.5-15 mg IV or 0.15–0.3 mg/kg IV/IM. Very short acting.
Buccal or nasal route (0.5 mg/kg) is another option when IV access is
not available
NOTE; BE PREPARED TO INTUBATE because benzodiazepines cause
resp depression. Ideal place for mxt is ICU.

42. • Emergent therapy; Intravenous lorazepam (0.1 mg/kg per dose) usually is
administered first in treating status epilepticus.
• If the seizures do not break, a second dose of intravenous lorazepam (0.1
mg./kg per dose) then;
• Urgent therapy with fosphenytoin (20 mg/kg per dose).
• Next, a loading dose of phenobarbital (20 mg/kg per dose for neonates and
5-10 for older children) is considered if seizures continue . With control of
the seizures, phenobarb maintenance dose of 3–5 mg/kg/24 hr given
divided into two equal doses
• Intubation is a consideration if respiratory depression is observed with
either benzodiazepines or phenobarbital.

43. • Propofol and phenobarbital are acceptable options for treating refractory
seizures in ICU setting.
• Get help from a neurologist if you are in the ICU.
• If the status epilepticus is not controlled by the preceding strategy,
consider elective intubation and assisted ventilation and also consider these
strategies:
A) diazepam infusion or
B) constant midazolam infusion (0.20 mg/kg bolus, 20–400 μg/kg/hr infusion) or
propofol (1–2 mg/kg, 2–10 mg/kg/hr infusion)
c) barbiturate coma;The initial IV loading dose of thiopental is 2–4 mg/kg and is
then titrated to achieve a burst suppression EEG pattern, continued for at least 48
hr, followed by cessation of thiopental until the serum phenobarbital level falls to
the therapeutic range.

44. • Barbiturate coma requires careful monitoring because hypotension
due to myocardial depression often requires pressor therap
• d) paraldehyde,
• e) general anesthesia- probably acts by reversing cerebral anoxia and
the concomitant metabolic abnormalities, allowing the previously
administered anticonvulsants to exert their effect