Peds Epilepsy Flashcards
Epilepsy (operational definition) (3)
- 2 or more unprovoked seizures
- single seizure with a likelihood of a second
- known epilepsy syndrome
What type of seizure is more worrisome: Focal or generalized?
Focal
What is HIE? When does it typically cause seizures?
Hypoxic-ischemic encephalopathy
- most common cause of neonatal seizures (50-60%)
- seizures start within 24-72 hrs after birth, and remit after a few days
- asphyxia usually prior to or during birth
4 types of neonatal seizures
- subtle (50%)
- clonic (25%)
- myoclonic (20%)
- tonic (5%)
Define subtle seizure
- any subtle paroxysmal event that interrupts expected behavior of a newborn
- appears stereotypical or repetitive
Tonic seizures:
- preterm or term?
- focal or generalized?
- Differentiate between decerebrate vs decorticate posturing
- Usually preterm, likely due to ICH
- can be focal or generalized
- Decerebrate posturing: sustained extension of upper and/or lower extremities
- Decorticate: sustained flexion of UEs and/or LEs
Clonic seizures:
- preterm or term?
- focal or generalized?
- limb movement features
- type of injury association
- Usually term
- Focal/multifocal
- –Migrates from limb to limb (Jacksonian March)
- clonic limb movements with slow return movement
- Associated with focal cortical injury:
- -infection, hemorrhage, trauma, infarcts (most common)
Myoclonic seizures:
- focal or generalized?
- type of movements
- how to differentiate from benign neonatal sleep myoclonus?
- Rare
- can be focal, multifocal, or generalized
- rapid jerks of extremities (UE>LE)
- -lacks slow return phase seen with clonic movements
- benign sleep myoclonus ONLY occurs during sleep (and normal EEG)
Status epilepticus
continuous seizures lasting 30 minutes
-associated with worse outcomes
What age group has highest incidence of seizures? When are you most likely to see them?
Neonates
-Majority on the 1st day, most in the first 10 days
Main causes of seizure in term infants (7)
- HIE (hypoxic-ischemic encephalopathy)
- Infection
- ICH (intracranial hemorrhage)
- Inborn error of metabolism
- Perinatal stroke
- Malformation syndromes
- Genetic diseases
Define febrile seizure
Seizure occurring in childhood after age 1 month associated with febrile illness NOT caused by an infection of the CNS (and with no previous seizures)
- upper age limit around 6-7 y/o
- not always w/ convulsive activity
- strong genetic component
Complex febrile seizure features
- focal features
- duration >10-15 mins
- recurrent seizures w/in 24 hrs
3 most commonly used seizure medications
- Phenobarbital
- Keppra
- Dilantin
Define hypsarrythmia. What syndrome is it a diagnostic tool for?
Chaotic EEG pattern, very high amplitudes, “spikes” cross over each other.
“If you turn the EEG reading upside down it looks the same”.
-Diagnostic tool for infantile spasms
Infantile spasms features:
- type of movement
- when does it typically occur
- Epileptic spasms: sudden movement forward or backward of neck, trunk, or extremities followed by stiffening of arms/legs/body lasting 2-10 seconds.
- usually occurs at sleep transition
Infantile spasms:
-2 important diagnostic features
EYES WIDEN and Hypsarrythmia on EEG
Infantile spasms:
- prognosis/long term effects
- Txs
- leads to developmental delay or regression. Early diagnosis and treatment= better developmental outcome
- Tx: Vigabatrin (VGB) or ACTH (1st line if idiopathic)
Lennox Gestaut:
- define
- M or F?
- triad
-childhood epileptic encephalopathy
-5x more common in males
Triad:
-multiple types of seizures
-developmental delay or regression
-characteristic EEG: paroxysmal fast activity (PFA) and slow spike-and-wave discharges
Lennox Gestaut prognosis
Poor, significant intellectual disabilities or death
-worse with symptomatic and early onset of seizures
Self limited (benign) Rolandic epilepsy:
- features
- more common in males or females?
- when do seizures usually stop?
- treatment and prognosis
- occurs more frequently during sleep (like sleepwalking)
- -drooling and facial twitching
- more common in males
- stops at puberty
- treatment usually not necessary
- good prognosis typically (unless abnormal EEGs, may be learning disabilities)
Childhood absence epilepsy (pyknolepsy)
- features
- more common in M or F?
- age onset
- Petit mal, look like staring spells
- most common type of childhood onset epilepsy
- frequent and brief seizures (>100x/day and <10 secs)
- more common in females
- onset 4-8 y/o, peak 6-7 y/o
Childhood absence seizures features
- staring spells, abrupt onset, short, unaware, no postictal phase
- Can have myoclonic and GTC seizures as well
- Can reproduce by having child hyperventilate
Childhood absence:
- Tx (1st line)
- Prognosis
Tx: ethosuximide (if no GTCs), most respond well to medication
-good prognosis typically
Juvenile absence
- M or F?
- Age onset
- different features from childhood absence
- Tx
M=F
- btwn 7-17 y/o. most common in 10-12 y/o
- less common than childhood absence
- fewer but longer seizures than childhood absence
- same tx as childhood, ethosuximide
Status epilepticus (SE)
- what age groups have highest incidence
- most common type of seizures to cause it
- highest in children and elderly
- 80% are generalized tonic clonic
Status epilepticus:
Define T1
T1: time at which tx should be considered or started
- time until seizure mechanisms are difficult to stop
- Typically > 5 min of continuous activity (for generalized convulsions and >5 y/o)
Status epilepticus:
Define T2
T2: time at which to evaluate how aggressively to tx SE
-time to permanent cerebral damage
> 30 minutes (for GTC)
Seizure tx: 1st line (seizure > 5 minutes)
Benzos (diazepam, alprazolam, Versed)
Seizure tx: 2nd line (seizure > 20 minutes)
- Phenytoin
- Keppra
- Valproate
- Phenobarbital
Seizure tx:
3rd line/ Refractory status tx (seizure > 30 minutes)
- Versed drip
- Propofol
- Ketamine
- Pentobarbital (most effective but highest morbidity)
Why should we perform genetic tests on epileptic patients?
- correctly diagnose epilepsy type
- targeted therapy/ disease-specific tx
- avoid unnecessary tests
- family counseling
- prognosis
Breathholding spells (nonepileptic)
- age group affected
- causes
- Most common in 6 month - 5 y/o
- family hx
- autonomic disturbance
- *Always precipitated by an event
Scenario 1:
A 6 month old male is being seen for an urgent visit in your office. Mother states that he was fine until 1 week ago. At that time he started having jerking movements. These involved flexion of his arms, legs and waist. Originally he would just have a single movement, but soon he would combine several together and would cry afterward.
Dx? Testing? Treatment?
Dx: Infantile spasms
Testing: EEG, look for hypsarrythmia
Tx: VGB or ACTH
Scenario 2:
An 18 year old female comes to your office. She had just entered college and had stayed up late two nights ago studying for an exam. Last night, after the exam, she partied late and had 6 beers. This morning her roommate was woken up by her bed shaking and noted a 2 minute generalized tonic-clonic seizure. She also reports occasional morning jerking of her limbs.
Dx? Testing? Treatment?
Dx: Juvenile myoclonic epilepsy
Testing: EEG
Tx: lamicatal, folic acid, calcium/vit D supplementation
Scenario 3:
An 8 year old right handed male is brought to your office by his mother. She says that yesterday night after having been put to sleep he came into their room. He was drooling, had some jerking movements of his right arm and had slurred speech. This lasted about 1.5 minutes and he was tired afterward and went to sleep. He woke up in the morning in his usual state of good health.
Dx? Testing? Treatment?
Dx: Benign Rolandic seizures
Testing: EEG (maybe)
Tx: no tx necessary
Scenario 4:
A 1 year old is seen in your office. He is noted to have a temperature of 102 degrees Fahrenheit. Last night his parents took him to the emergency room because he had a single 1.5 minute generalized seizure. In the emergency room, his temperature was 101 and he had a second two minute generalized seizure. He had a normal lumbar puncture. He did well the following A.M.
Dx? Testing? Treatment?
Dx: Febrile seizure (complex, focal)
Testing: neuro exam, test for meningeal signs, labs, LP, EEG, possibly imaging
Tx: phenobarbital or valproate prophylaxis
Scenario 5:
A 1.5 year old is seen in your office. The mother states that he was playing normally when he tripped and fell on a toy. He cried and then turned blue and stopped breathing. He then fell to the ground, passed out and had some jerking movement. This type of episode had happened once before when a toy was taken away from him.
Dx? Testing? Treatment?
Dx: Breathholding spell
Testing: check for iron deficiency, EEG if tonic clonic activity for a long time
Tx: none
Scenario 6:
An 8 year old female comes to your office because her teachers have been noting staring spells. These typically last 5-10 seconds and even when prodded she doesn’t seem to respond. She is totally normal after the event. She has not been doing as well in school recently. You ask her to blow into a pinwheel and you are able to reproduce an event.
Dx? Testing? Treatment?
Dx: Childhood absence seizure
Testing: EEG
Tx: ethosuximide
Scenario 7:
A child who has known seizures and who is on valproic acid is noted to have abnormal movements of his hands. Because of recent seizures, his dose had been increased last week. They appear to have a suppressible high frequency movement. He also notes some trouble with his vision and his mother notes that he has been very tired for the last several days.
Dx?
Dx: Tremors as a side effect from valproic acid
