Pediatric neurology lecture

By Dr. Russul Feihan

Seizures in Childhood` Assist professor
Babylon medical college
Epilepsy in children Part one

Epileptic seizure → clinical manifestation of abnormal &

excessive discharge of a set of neurons in the brain
.
Epilepsy is a chronic neurological condition characterized by
recurrent, unprovoked seizures (no fever, no acute cerebral
insult), occurrence of at least 2 unprovoked seizures 24 hours
apart.
Most Seizures are provoked by infection, fever, head trauma,
hypoxia, toxin, fatigue, hyperventilation alkalosis and drugs like
(INH, penicillin, theophylline …etc).
The incidence of epilepsy is 3% and more than 50% of cases
begin in childhood
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.

Classification of epileptic seizures: ILAE Commission 2017.

SEIZURE TYPES
1.Focal-Onset Seizures
Motor Onset
Tonic, Clonic, Atonic, Myoclonic, Hyperkinetic
Epileptic spasms, Automatisms
Non–Motor Onset
Behavior arrest, Sensory, Cognitive, Emotional, Autonomic
Awareness Descriptor
Aware
Impaired awareness
2.Generalized-Onset Seizures
Motor
Tonic-clonic, Tonic, Clonic, Atonic, Myoclonic, Myoclonic-atonic
Myoclonic-tonic-clonic
Epileptic spasms
Non–Motor (Absence)
Typical, Atypical, Myoclonic, Eyelid myoclonia

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3.Unknown-Onset Seizures
Motor: Tonic-clonic, Epileptic spasms
Non–Motor: Behavior arrest
4. Unclassified Seizures

*ILAE 2010 CLASSIFICATION

GENERALISED SEIZURES: -
(Discharge arises from both hemisphere)
Absence seizures
Myoclonic seizure
Tonic
Tonic clonic
Atonic seizures
FOCAL – SEIZURES: -
(Arise from one or part of one hemisphere)
Frontal seizures
Temporal lobe seizures
Occipital seizures
Parietal lobe seizures
*International league against epilepsy

PARTIAL SEIZURE - FOCAL SEIZURE

Focal Seizures with Preserved Awareness These can take the form of
sensory seizures (auras, called focal aware seizures) or brief motor
seizures, the specific nature of which gives clues as to the location of the
seizure focus.
Focal Seizures with Impaired Awareness These seizures usually last 1-
2 min and are often preceded by an aura, Auras can take the form of a
number of sensations, including visual (e.g., flashing lights or seeing
colors or complex visual hallucinations), somatosensory (tingling),
olfactory, auditory, vestibular, depending on the precise localization of
the origin of the seizures.
Begin in a relatively small group of dysfunctional neurons in one of the
cerebral hemispheres.
• May lead to clonic movements → travel proximally→
(Jacksonian) March from face to arm to leg.

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The most common aura experienced by children consists of epigastric
discomfort or pain and a feeling of fear.
Frontal seizure
Nocturnal autosomal dominant frontal lobe epilepsy has been linked to
acetylcholine-receptor and to KCNT1 gene mutations. It manifests with
nocturnal seizures with dystonic posturing, agitation, screaming, and
kicking that respond promptly to carbamazepine.
Temporal lobe seizure
Benign epilepsy syndrome with focal seizures: benign childhood
epilepsy with centrotemporal spikes: (BECTS)
 The most common epileptic syndrome
 This typically starts during childhood (ages 3-10 yr.) with
remission in adolescence.
 The child typically wakes up at night owing to a focal (simple
partial) seizure causing buccal and throat tingling and tonic or
clonic contractions of 1 side of the face, with drooling and
inability to speak but with preserved consciousness and
comprehension.
 Dyscognitive focal (complex partial) and secondary generalized
seizures can also occur.
 EEG shows typical broad-based centrotemporal spikes that are
markedly increased in frequency during drowsiness and sleep.
MRI is normal.
 Patients respond very well to antiepileptic drugs (AEDs) such as
carbamazepine.
 In some patients who only have rare and mild seizures treatment
might not be needed.

Benign epilepsy with occipital spikes

 Can occur in early childhood and manifests with complex partial
seizures with ictal vomiting, or they appear in later childhood
(Gastaut type) with complex partial seizures, visual auras, and
migraine headaches.
 Both are typically resolve in a few years. Manifestations may
include visual hallucinations and postictal headache (epilepsy–
migraine sequence).

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Generalized seizures:
Typical absence seizures: - petit mal

 It usually starts at 5-8 yr. of age and are often, overlooked by

parents for many months even though they can occur up to
hundreds of times per day.
 They do not have an aura, usually last for only a few seconds, and
are accompanied by eye lid flutter or upward rolling of the eyes,
(absence seizures can have simple automatisms like lip-smacking
or picking at clothing and the head can minimally fall forward).
 Absence seizures do not have a postictal period and are
characterized by immediate resumption of what the patient was
doing before the seizure.
 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 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.

Generalized motor seizures: Grand mal

 The most common generalized motor seizures are generalized
tonic–clonic seizures that can be either primarily generalized
(bilateral) or secondarily generalized from a unilateral focus.
If there is no partial component, then the seizure usually starts
with loss of consciousness and, at times, with a sudden cry,
upward rolling of the eyes, and a generalized tonic contraction
with falling, apnea, and cyanosis.
 In some, a clonic or myoclonic component precedes the tonic
stiffening.

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  The tonic phase is followed by a clonic phase that, as the seizure
progresses, shows slowing of the rhythmic contractions until the
seizure stops usually 1-2 min later.
Tonic phase
The tonic phase begins with flexion of the trunk and elevation and
abduction of the elbows. Subsequent extension of the back and neck is
followed by extension of arms and legs.
Piercing cry may be present due to passage of air through closed
vocal cords.
Autonomic signs are common during this phase and include increase
in pulse rate and blood pressure, profuse sweating
This stage lasts for 10-20 seconds.
Clonic phase
tremor occurs at a rate of 8 tremors per second, which may slow
down to about 4 tremors per second.
The clonic phase lasts for 30 sec. to 1minute.

 Incontinence and a postictal period often follow. The latter

usually lasts for 30 min to several hours with semi coma and
postictal sleepiness, weakness, ataxia, hyper- or hyporeflexia,
and headaches.
 There is a risk of aspiration and injury. First aid measures
include positioning the patient on his or her side, clearing the
mouth if it is open, loosening tight clothes or jewelry, and
gently extending the head and, if possible, insertion of an
airway by a trained professional.
 The mouth should not be forced open with a foreign object (this
could dislodge teeth, causing aspiration) or with a finger in the
mouth (this could result in serious injury to the examiner’s
finger).

Benign myoclonic epilepsy of infancy consists of the onset of

myoclonic and other seizures during the 1st yr of life, with
generalized 3 Hz spike–and–slow-wave discharges.
Often, it is initially difficult to distinguish this type from more-severe
syndromes, but follow-up clarifies the diagnosis.

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Juvenile myoclonic epilepsy (Janz syndrome) is the most common
generalized epilepsy in young adults, accounting for 5% of all epilepsies.
It has been linked to mutations in many genes.
Typically, it starts in early adolescence with 1 or more of the following
manifestations: myoclonic jerks in the morning, often causing the patient
to drop things; generalized tonic–clonic or clonic–tonic–clonic seizures
upon awakening; and juvenile absences.
The EEG usually shows generalized 4-5 Hz polyspike–and–slow wave
discharges. It may respond to Na valproate which is required lifelong.

SEVERE GENERALIZED EPILEPSIES:

West syndrome: Starts between the ages of 2 and 12 mo and consists
of a triad of *Infantile epileptic spasms that usually occur in clusters
(Particularly in drowsiness or upon arousal), **developmental regression,
and a typical EEG picture called ***hypsarrhythmia.
Hypsarrhythmia is a high-voltage, slow, chaotic background with
multifocal spikes.
Patients with cryptogenic (idiopathic) West syndrome have normal
development before onset, while patients with symptomatic West
syndrome have preceding developmental delay owing to perinatal
encephalopathies, malformations, underlying metabolic disorders, or
other etiologies. In boys, West syndrome can also be caused by ARX gene
mutations (often associated with ambiguous genitalia and cortical
migration abnormalities). West syndrome, especially in cryptogenic
cases, is a medical emergency because diagnosis delayed for 3 wk or
longer can affect long-term prognosis.
 ACTH gel
 Vigabatrin: Its principal side effect is its retinal toxicity.
 Valproate, nitrazepam, and clonazepam, pyridoxine, ketogenic
diet, and (IVIG).

Lennox-Gastaut syndrome typically starts between the ages of 2

and 10 yr and consists of a triad of: developmental delay, multiple
seizure types that as a rule include atypical absences, myoclonic,
astatic, and tonic seizures and the third component is the EEG findings 1-
2 Hz spike–and–slow waves, polyspike bursts in sleep, and a slow
background in wakefulness.

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Patients commonly have seizure types that are difficult to control, and
most are left with long-term cognitive impairment and intractable
seizures despite multiple therapies.

Landau-Kleffner syndrome is a rare condition of unknown cause

characterized by loss of language skills attributed to auditory agnosia
in a previously normal child. At least 70% have associated clinical
seizures, but some do not. The seizures when they occur are of several
types.
CT and MRI studies typically yield normal results.
The approach and therapy:
Valproic acid is often the anticonvulsant that is used first to treat the
clinical seizures and may help the aphasia.
Some children respond to clobazam, to the combination of valproic acid
and clobazam, or to levetiracetam.
For therapy of the aphasia, nocturnal diazepam therapy (0.2-0.5 mg/kg
PO at bedtime for several months) is often used as first- or second-line
therapy, as are oral steroids.

Long-term therapy is often needed irrespective of what the patient

responds to.
If the seizures and aphasia persist after diazepam and steroids trials, then
a course of intravenous immunoglobulins should be considered.

Diagnosis of seizures
History: - full description of the seizure and the post ictal state including
the timing, duration, precipitating factors, aura,

1) personality changes or intellectual deterioration which may suggest

a degenerative disease of CNS where as vomiting and FTT might
indicate a metabolic disorder or a structural lesion.
2) Physical examination: - to search for organic cause, B.P, wt,
length and H.C should be recorded and plotted on a growth chart.
Look for any unusual facial features or associated hepato –
splenomegaly which may indicate a metabolic or storage disease.
Search for cutaneous lesion (neurocutaneous syndromes), eye
examination for (retinal phakoma, papillodema, retinal hemorrhage,

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 chorio retinitis), hyper ventilation for (3-4) min produce absence
seizure.
3) Investigation: - in the first a febrile seizure we must do
a) (FBS, Ca ++, mg++ and electrolyte) estimations.
b) EEG (normal EEG seen in 40% of patients), so we may do
activation procedures. (Hyperventilation, eye closure, photic
stimulation and sleep deprivation), which will ↑the positive
results. Seizures discharges are more likely to be recorded in
infants and children than in the adolescent or adult.
c) CT and MRI: indicated if there is suspicion of *intra –
cranial lesion, *prolonged partial seizure, *focal neurological
deficit, *no response to anticonvulsant and *evidence of ↑
I.C. P.
d) CSF examination: - if there is suspicion of infection, sub-
archnoid hemorrhage or demyelination diseases.
e) Specific metabolic tests.

Treatment of epilepsy
* Be sure that the patient has seizure disorder and not a condition that
mimic epilepsy.
* After a first seizure, and the patient has normal neurodevelopmental
status, EEG, and MRI, then treatment is usually not started.
* If the patient has abnormal EEG, MRI, development, and/or neurologic
exam and/or a positive family history of epilepsy, then the risk is higher
and often treatment is started.
The choice of anti- epileptic drug depends on: (type of seizure and
epilepsy syndrome, potential for paradoxical seizure aggravation, adverse
effects, cost and availability, ease of initiation, drug interactions, the
presence of comorbid conditions, the coexisting seizures, mechanism of
drug actions, ease of use, ability to monitor the medication and adjust the
dose, patient's and family's preferences and the teratogenic profiles).
Initiating and monitoring therapy: In nonemergency situations or when
loading is not necessary, the maintenance dose of the chosen AED is
started. For example, the starting dose of carbamazepine is usually 5-
10 mg/kg/day. Increments of 5 mg/kg/day can be added every 3 days
until a therapeutic level is achieved and a therapeutic response is
established or until unacceptable adverse effects occur.

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Titration: If a therapeutic level needs to be achieved faster, a loading
dose may be used. For valproate it is 25 mg/kg, for phenytoin it is
20 mg/kg, and for phenobarbital it is 10-20 mg/kg.
Only one drug should be used initially and the dose increased until
complete control is achieved or until side effects had appeared. Then
another drug be added and the initial one subsequently tapered.
Monitoring: Before starting treatment, baseline laboratory studies
including CBC, liver enzymes, and possibly kidney function tests and
urinalysis are often obtained and repeated periodically. Allergic hepatitis
and agranulocytosis are more likely to occur in the first 3-6 months of
therapy, so these laboratory studies are checked once or twice during the
first month, then every 3 to 4 months thereafter.
Additional treatment: Steroid, Intravenous gamma globulin (IVIG).
Ketogenic diet and Surgery.
Discontinuation of Therapy: is usually indicated when children are free
of seizures for at least 2 yr. Most children who have not had a seizure for
≥2 yr and who have a normal EEG when withdrawal is initiated had
remained free of seizures after discontinuing medication, and most
relapses occur within the first 6 mo.
Risk factors for seizure relapse:
• Abnormal EEG before medication is discontinued.
• Symptomatic epilepsy.
• Absences seizure.
• Those who treated with valproate.
• Older age of epilepsy onset.
• Longer duration of epilepsy.
• Presence of multiple seizure types.
• Need to use more than one AEDs.
Therapy should be discontinued over a period of 3-6 months because
abrupt discontinuation can result in withdrawal seizures or status
epilepticus.
Withdrawal seizures are especially common with phenobarbital and
benzodiazepines.
Seizures that occur more than 2 - 3 months after AEDs are completely
discontinued indicate relapse, and resumption of treatment is usually
warranted.

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