Ssell Pediatrics 02.seizure Part One
Ssell Pediatrics 02.seizure Part One
Ssell Pediatrics 02.seizure Part One
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3.Unknown-Onset Seizures
Motor: Tonic-clonic, Epileptic spasms
Non–Motor: Behavior arrest
4. Unclassified Seizures
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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.
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Generalized seizures:
Typical absence seizures: - petit mal
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.
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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.
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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.
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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.
Diagnosis of seizures
History: - full description of the seizure and the post ictal state including
the timing, duration, precipitating factors, aura,
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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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