1: Peds Epilepsy Flashcards
Epilepsy syndrome found in neonates that has recurrent seizures and usually ends in 1 to 4 months.
Benign familial neonatal seizures
Treatment for juvenile myoclonic epilepsy.
Well controlled with broad-spectrum: Valproic acid Lamotrigine Levetiracetam Clonazepam Life-long disorder that must be treated indefinitely. Good prognosis of normal cognition.
Describe the spasms in West’s syndrome.
Sudden, rapid, tonic contraction of trunk and limbs. Intensity can vary from subtle head nodding to powerful body contraction. Can occur in clusters of several dozen separated by a few seconds. Often occurs during sleep/wake transition.
What are the general treatment options for pediatric epilepsy (3)?
Antiseizure meds
Surgery
Ketogenic diet
This infantile epilepsy is a severe myoclonic form with febrile seizures. It can have status epilepticus and lead to developmental delay.
Dravet syndrome
Family history of seizures (from any etiology) increases risk for febrile seizures following _____ vaccination.
Family history of seizures (from any etiology) increases risk for febrile seizures following MMRV vaccination.
What is the triad in Aicardi’s syndrome?
Infantile spasms
Agenesis of corpus callosum
Retinal malformation
What is the concern with repeated or prolonged febrile seizures?
Serious underlying cause like bacterial meningitis or encephalitis.
Criteria for febrile seizures (5).
Seizure + temperature greater than 38°C
Younger than 6
No central nervous system infection or inflammation
No acute systemic metabolic abnormality that may produce convulsions
No previous history of afebrile seizures
Ohtahara syndrome can develop into what?
West’s syndrome
Lennox-Gestaut syndrome
What is the difference between an anatomical and a functional hemispherectomy?
Anatomical: complete removal of one hemisphere.
Functional: disconnection of hemisphere. Some tissue left intact.
How long should the ketogenic diet be followed?
Not more than two years
Patient is typically a healthy young teenager with one or more of the following seizures:
Absence seizures
Myoclonic jerks
Generalized convulsions
Juvenile myoclonic epilepsy
Those with West’s syndrome have a poor prognosis. Idiopathic do better than symptomatic. What can develop in those with symptomatic disease (3)?
Severe mental retardation (70%)
Lennox-Gastaut syndrome (18-50%)
Autism spectrum disorders
What is the treatment for reflex epilepsy?
Avoidance of the trigger
T/F Viral/bacterial illness may precipitate fever and cause seizure.
True.
What two medications should be prescribed with a ketogenic diet?
Calcium
Vitamin D
Signs to look for: eye fluttering, eyes rolling upward, lip smacking, and autonomic symptoms (e.g., flushing or tachycardia).
Childhood absence epilepsy
This epilepsy syndrome occurs in juveniles and adults. It is triggered by a specific stimuli, such as emotions or sounds.
Reflex epilepsy
This epilepsy has acquired aphasia (word deafness).
Landau-Kleffner syndrome
What is the peak incidence of childhood absence epilepsy?
6 to 7 years old
Early myoclonic encephalopathy in neonates can become what after several months?
West’s syndrome
Treatment for Lennox-Gastaut syndrome (4).
- No drug has been very effective. Rufinamide (Banzel) and Clobazam (Onfi) used. Can use Valproic acid and Lamotrigine.
- Ketogenic diet
- Corpus callosotomy
- Vagal nerve stimulator
West’s syndrome is often symptomatic. Name examples.
The structural underlying cause can be a prenatal, perinatal, or postnatal disorder. Such as microcephaly, severe hydrocephalus, or encephalitis.
A continuous multifocal spike and slow waves with high amplitude.
Hypsarrhythmia
Surgery for atonic seizures.
Corpus callosotomy. Involves disconnecting the anterior 2/3 of corpus callosum. Works better on children than adults.
The earliest onset age-related epileptic encephalopathy in neonates. Onset is often within the first 10 days to three months of life.
Ohtahara syndrome
2 types of febrile seizures.
Simple
Complex
What can worsen drop attacks in Lennox-Gastaut syndrome?
Carbamazepine
If a child has a family hx of any type of seizure, how should they receive the MMRV vaccine?
They should receive the MMR and the varicella, not the combination MMRV.
T/F Febrile seizures should be treated with a short course of antiseizure meds.
False. Only slightly increased risk for epilepsy later in life.
What is the treatment for childhood absence epilepsy?
Valproic acid
Lamotrigine
Ethosuximide (older peds patients)
This neonatal form of epilepsy has focal seizures with EEG changes. Etiology is unknown and it has a poor prognosis. It often leads to severe epilepsy.
Migrating partial seizures of infancy
Causes of Lennox-Gastaut syndrome (5).
Genetic disorders Neurocutaneous syndromes (tuberous sclerosis) Encephalopathies Meningitis Head injuries
Which hemispherectomy has the risk of complications from hydrocephalus?
Anatomical and functional
When do seizures disappear in West’s syndrome?
Usually by 4 to 5 years old
This epilepsy is often misdiagnosed as ADHD.
Childhood absence epilepsy
This medication is more commonly used in pediatrics than adults. There is a 30% risk of irreversible visual field constriction. Patients need visual field testing q six months.
Vigabatrin
T/F About 58% of children are seizure-free 5 years after hemispherectomy.
True
Simple febrile seizures are more common. Describe them.
Duration less than 15 minutes.
No focal features.
If in a series, shorter than 30 minutes.
How fast is the clinical response to ketogenic diet?
Usually within a few weeks.
Cortical resection has a higher success rate when?
Removing a single lesion
What is unilateral mesial temporal sclerosis?
Shriveling/hardening of the hippocampus d/t prolonged seizures.
T/F Antipyretics can help prevent febrile seizures.
False
The most common type of epilepsy in children.
Benign rolandic epilepsy
What are the complications of a hemispherectomy?
Aseptic meningitis
Hydrocephalus
Blood loss
Hemorrhage
What age do febrile seizures occur?
6 months - 5 years
Peak incidence of 19-20 months
What is the triad in West’s syndrome?
Infantile spasms
Interictal electroencephalogram (EEG) pattern termed hypsarrhythmia
Mental retardation
What is the ketogenic diet?
4:1
Fat:Protein/Carbs
Persistant ketosis has antiseizure effect.
This epilepsy occurs at ages 3 to 7 and can be gradual or sudden onset. It affects Broca’s and Wernicke’s areas of the brain.
Landau-Kleffner syndrome
Side effects of the ketogenic diet (6).
Dehydration Nausea Vomiting Diarrhea/Constipation Metabolic Acidosis Osteopenia
T/F Childhood absence epilepsy occurs more frequently in girls and is usually in developmentally normal children.
True
When does West’s syndrome usually appear?
First year of life
Children may become seizure-free (33–80%).
Some go on to develop generalized tonic-clonic (GTC) seizures (40%).
Childhood absence epilepsy
When might a patient need a hemispherectomy?
When the hemisphere is severely diseased. Patients are often having 30 to 50 seizures a day.
What is the treatment for West’s syndrome?
Goal is to improve quality of life.
Common medications:ACTH, Vigabatrin, Vitamin B6, Prednisone
Ketogenic diet
Potential surgery depending on cause
Describe complex febrile seizures.
Duration longer than 15 minutes.
Can have focal signs or postictal paresis.
If in a series, longer than 30 minutes.
An ill-defined syndrome that is associated with severe seizures in childhood. Can be tonic-clonic, absence, or myoclonic. Mental retardation or regression. Neurodevelopment normal until first seizure.
Lennox-Gastaut syndrome
What are the age brackets for benign rolandic epilepsy?
Usually starts between ages 3 to 13.
Peak ages 8 to 9.
Usually ends ages 14 to 18.
What type of epilepsy responds best to surgery?
Localized
T/F Immunizations do not cause febrile seizures.
False. Rarely they can.
EEG can be normal, but may have interictal spike (typically at 3–4 Hz) and wave discharges
Childhood absence epilepsy
