Pediatric Seizures Flashcards
True or false: status epilepticus is a medical emergency
true
What is status epilepticus?
any recurrent or continuous seizure activity lasting > 30 minutes in which the patient does not regain baseline mental status
-or a cluster of seizures that does not return to baseline for > 30 minutes
How should a seizure lasting greater than 5 minutes be treated?
any seizure that does not stop within 5 minutes should be treated as impending SE
-operationally seizures lasting > 5 minutes or repetitive seizures for > 5 mins are treated as SE to prevent consequences and increase response to tx
What are patients given to decrease risk of progression to SE?
patients are often given “on demand” benzodiazepines to use “prn” at the onset of seizures to decrease risk of progression to SE
Which benzodiazepine is often give to reduce risk of progression to SE?
midazolam intranasal or buccal
-injectable: via nasal atomizer for nasal administration
rectal diazepam an option for < 3 months
-not really used b/c its traumatic
What are epilepsy syndromes?
refers to clusters of features that may occur together, including seizure type, EEG findings, imaging findings, age-dependent features (e.g., age at onset or remission), specific comorbidities (e.g. psychiatric illness), triggers and sometimes prognosis
What are examples of epilepsy syndromes?
childhood absence epilepsy
juvenile absence epilepsy
juvenile myoclonic epilepsy
Lennox-Gastaut syndrome
Dravet syndrome
What is the spectrum of ethosuximide?
narrow-spectrum ASM
What is the role of ethosuximide?
1st line for absence seizures
-drug of choice
not for any other seizure type
-do not use as monotherapy if mixed seizure types, even if one of these is absence seizures
What are the advantages of ethosuximide?
lower rates of attention difficulties compared to VPA
works quickly
generally well-tolerated
few drug interactions
What are the disadvantages of ethosuximide?
narrow-spectrum of activity
-only a good choice to use for uncomplicated absence seizures
-does not confer protection for generalized tonic-clonic seizures
What are the adverse effects of ethosuximide?
CNS effects
-drowsiness, dizziness, behavioral changes
GI effects
-dose related –> can divide dose to minimize
rare: blood dyscrasias, skin rashes
What are some monitoring parameters for ethosuximide?
CBC & platelets (annually)
What is infantile epileptic spasms syndrome?
epileptic spasms most often occur in “clusters” on awakening and involve tonic limb (+/- head) flexion or extension
-each spasms lasts less than 3 seconds
-repeat every 5-10 seconds
-for a period of 5-15 minutes
What is distinctive about the EEG of a patient with infantile epileptic spasms syndrome?
hypsarrhythmia
How aggressively should infantile epileptic spasms syndrome be treated?
treat early and aggressively to prevent long-term sequelae (e.g. intellectual delays, refractory seizures)
What are the treatment options for infantile epileptic spasms syndrome?
hormonal therapy (oral prednisolone or IM/SC ACTH)
vigabatrin
other ASMs are ineffective
What is the MOA of vigabatrin?
irreversible inhibition of GABA-transaminase leads to increased GABA in the CNS = increased neuro-inhibition
-structural analog of GABA, but does NOT act on GABA receptors
True or false: vigabatrin is titrated slowly
false
titrated q2-3 days
When should we switch off vigabatrin?
no clinical response in 7-14 days, change therapy
What are the CYP interactions of vigabatrin?
no CYP effects
-insignificant metabolism; mainly excreted unchanged in the urine –> renally adjust
What are the adverse effects of vigabatrin?
visual abnormalities
-including permanent vision loss
-ophtho follow-up
vomiting and upper resp tract infections
-more common in infants than adults
asymptomatic MRI changes
-resolves on d/c (may resolve with continued use)
What is Lennox-Gastaut syndrome?
developmental and epileptic encephalopathy with:
1. multiple drug-resistant seizure types. including but not limited to:
-tonic
-atonic (“drop attacks”)
-atypical absence
-generalized tonic-clonic
2. typical EEG pattern
-generalized slow spike-and-wave
-generalized paroxysmal fast activity
3. intellectual disability
children may be medically complex with comorbid neurodevelopmental and behavioral disorders, sleep disorders, mobility requirements, feeding tubes
What is 1st line for Lennox-Gastaut syndrome?
NICE 2022: valproate
Ontario 2020: rufinamide, valproate