Pediatric Seizure Principles Flashcards
Status epilepticus is a medical emergency where…
Any recurrent or continuous seizure activity lasting 30+ minutes where the patient does not regain baseline mental status
Any seizure that does not stop within ____ should be treated as impending status epilepticus
5 minutes
Patients are often given ____ to use at onset of seizures to decrease risk of progression to status epilepticus
On demand benzodiazepines PRN
BZD’s used in pediatrics include…
Injectable midazolam via nasal atomizer
Infants - rectal diazepam
Epilepsy syndromes refer to…
Cluster of features that may occur together
Including seizure type, EEG findings, imaging findings, age-dependent features, specific comorbidities, triggers, + prognosis
Some examples of epilepsy syndromes include…
Childhood absence epilepsy
Juvenile absence/myoclonic epilepsy
Lennox-Gastuat Syndrome
Dravet Syndrome
Ethosuximide is a ____ ASM.
Narrow-spectrum
Ethosuximide is only indicated for…
Absence seizures
NOT FOR ANY OTHER SEIZURE TYPE
Advantages of ethosuximide include…
Lower rates of attention difficulties compared to VPA
Works quickly
Generally well tolerated
Few drug interactions
Disadvantages of ethosuximide is primarily its…
Narrow-spectrum of activity
Only a good choice to use for uncomplicated absence seizures
Potential AE’s with ethosuximide include…
CNS effects (drowsiness, dizziness, behavioural)
GI effects
Rare - blood dyscrasias, skin rashes
Monitor CBC + platelets annually for rare
Infantile epileptic spasm syndrome decribes…
Epileptic spasms that most often occur in “clusters” on awakening, and involve tonic limb flexion/extension
May have distinctive, disordered EEG = hypsarrhythmia
May have psychomotor arrest
Ideally, infantile epileptic spasms syndrome should be treated early + aggressively to prevent…
Long-term sequelae - intellectual delays, refractory seizures
Treatment options for infantile epileptic spasms syndrome include…
Hormonal therapy
Vigabatrin
Other ASM’s are ineffective
MOA of vigabatrin is
Irreversible inhibition of GABA-transaminase, leading to increased GABA concentrations in the CNS to increase neuro inhibition
AE’s with vigabatrin include…
Visual abnormalities, including permanent vision loss
Vomiting, URTI’s
Asymptomatic MRI changes
Lennox-Gastaut Syndrome presents with…
Multiple drug-resistant seizure types (tonic, atonic, atypical absence, tonic-clonic)
Typical EEG pattern
Intellectual disability - comorbid neurodevelopmental and behavioural disorders
Some options for LGS treatment include…
Valproate
Rufinamide
Lamotrigine
Combo treatment for LGS is likely due to…
Multiple seizure types
Rufinamide MOA is…
Unknown - prolongs inactive state of sodium channels
Rufinamide is approved for…
Adjunctive treatment of seizures associated with LGS in patients 4+
Some AE’s with rufinamide include…
CNS - headache, drowsiness, dizziness, fatigue, trmeor
GI - vomiting, nausea
Shortened QT interval
Multiorgan hypersensitivity
Dravet syndrome encompasses…
Drug-resistant developmental and epileptic encephalopathy - seizures of various types + progressive cerebral atrophy
Poor prognosis
80% of dravet syndrome cases are due to ____. This is significant because…
Loss of function mutation in sodium channel gene. Sodium channel blocking ASM’s are not effective
ASM’s that could be used in Dravet Syndrome include…
Valproate 1st line
Fenfluramine, Stiripentol, Clobazam
VPA is contraindicated in children less than 2 because of…
Fatal hepatotoxicity
If absolutely necessary, VPA could be used in children alongside ____ to mitigate risk.
Levocarnitine - transporter molecule to get rid of toxic fatty metabolites
Fenfluramine was withdrawn from market due to pulmonary arterial hypertension and valvulopathy. At anti-seizure doses…
This has not been seen
Stiripentol indication is for…
Combined treatment with clobazam + valproate for refractory GTC seizures that are uncontrolled
Stiripentol AE’s include…
CNS - drowsiness, agitation, ataxia, tremor
GI - decreased appetite, weight loss, vomiting
Serious - delirium, hallucinations
CBD indications for seizures…
Approval for treatment of seizures associated with LGS or Dravet syndrome
Noted a 2-fold reduction in seizure frequency per month, 50% reduction compared to placebo in Dravet syndrome
CBD AE’s that were noted include…
Diarrhea, vomiting, fatigue, pyrexia, somnolence, LFT abnormalities
Significant DI’s can occur with CBD such as…
CYP 2C19 inhibition decreasing metabolism of clobazam
Additive risks of hepatotoxicity with VPA
CBD is usually a ____ agent; NOT first line for any seizure disorder
3rd or 4th line
A ketogenic diet is…
A high fat, low carb, adequate protein diet that mimics state of starvation
May reduce seizure frequency
Ketogenic diet is often hard to follow because of…
Need for strict compliance
Poor toleration
Nutritional supplements may be required to minimize AE’s
A ketogenic diet could be considered if…
The patient has not responded to appropriate ASM therapy
However treatment choice in GLUT1DS
Surgery is an option for some patients with…
Refractory epilepsy (usually focal) - resections/disconnective
Up to ~70% will achieve seizure freedom
Vagus nerve stimulation is a surgical procedure to…
Implant an electrical pulse generator in chest, with electrodes to vagus nerve in neck - stimulates on a regular basis
May reduce seizure frequency - option in refractory focal onset or generalized seizures
