#18 Pediatric Seizures & Epilepsy: Shaking Things Up!

February 3, 2021 | By Justin Berk

Summary

In this episode, we cover the full spectrum from febrile seizures all the way to status epilepticus with Dr. John Gaitanis, a pediatric neurologist who specializes in care for children with epilepsy. Dr. Gaitanis teaches us what to look out for on neurologic exam, how to counsel families about safety precautions, and the differential diagnosis for seizure-like activity in infants and children. We also discuss the exciting future of epilepsy treatment.

Credits

• Writer, Producer, Infographic, CME Questions: Clara Mao
• Cover Art: Chris Chiu MD 
• Hosts: Justin Berk MD, Christopher Chiu MD
• Editor: Justin Berk MD; Clair Morgan of nodderly.com
• Guest: John Gaitanis MD

Pediatric Seizures & Epilepsy Pearls

1. Simple febrile seizures in children who have a normal neurologic exam are largely a benign process and do NOT require lumbar puncture, EEG, neuroimaging, or treatment with AEDs (Subcommittee on Febrile Seizures, 2008 and 2011).
2. Be on the lookout for infantile spasms, as these can be associated with serious developmental delay and increased risk of developing severe epilepsy.
3. Antiepileptic drugs should be started after two unprovoked seizures (febrile seizures don’t count) and continued until at least two years seizure free.
4. If you can’t get IV access during status epilepticus, consider temporary alternatives like rectal diazepam, IM/intranasal midazolam, or an intraosseous line.
5. Don’t overlook alternatives like the ketogenic diet, surgery, and cannabidiol to help manage refractory seizures in certain types of epilepsy.

Pediatric Seizures & Epilepsy Notes 

Febrile Seizures 

Defined as seizures that occur in the setting of a fever (temperature >100.4ºF or 38ºC) in children aged 6 months to 5 years old without a known history of epilepsy. They are seen in 2-5% of all children (Subcommittee on Febrile Seizures, 2008). Thought to be a response to fever and cytokines in the setting of genetic predisposition towards seizure activity. Febrile seizures generally occur early in the course of illness and may be the presenting sign. 

Types of Febrile Seizures

The distinction between simple and complex febrile seizures is based on focality, duration, and recurrence. 

Simple febrile seizures are generalized, last less than 15 minutes, and only occur once within 24 hours. 

Complex febrile seizures are focal, prolonged (>15 minutes), and recur multiple times within 24 hours.

Tips for History and Exam

• Ask about family history of seizures and epilepsy, including parental history of febrile seizures which can be reassuring
• Concerning findings include focal neurologic defects or global delay (ex. extreme hypotonia, poor visual function)
• Patients with simple febrile seizures should return to baseline quicker than children with epilepsy, with the postictal state resolving well before the 1 hour mark

Workup & Treatment – Simple Febrile Seizures

• Lumbar puncture: NOT recommended…unless there are signs and symptoms of meningitis
• EEG: NOT recommended
• Neuroimaging: NOT recommended 
• Anti-epileptic drugs: NOT recommended even if multiple febrile seizures
• Antipyretics: OK for comfort but do not prevent further febrile seizures

Recommendations from: AAP Subcommittee on Febrile Seizures (2008 and 2011)

What can we tell parents and caregivers about the prognosis?

• Simple febrile seizures have a favorable prognosis but do recur in ⅓ of patients. Children with febrile seizures have a 1-2% risk of developing epilepsy later in life, which is only slightly higher than the general population risk of 0.5-1% (Subcommittee on Febrile Seizures, 2011). 
• Acknowledge that this can be a frightening experience and reassure them that the brain is resistant to injury, especially if their child is back to baseline. 
• On the other hand, the presence of obvious abnormalities on exam, seizures lasting greater than 30 minutes, and family history of epilepsy do raise the risk of developing epilepsy.

Epilepsy

Types of Seizures

Generalized:

• Absence – brief staring episodes
• Tonic-Clonic
  • Tonic phase – stiffening of extremity 
  • Clonic phase – rhythmic relaxation of tonic phase
• Myoclonic – brief jerks of body or limb
• Atonic – collapse from loss of body tone

Focal:

• Motor vs Non-Motor
  • Motor – involving only one side of body
  • Non-motor – ex. foul odor in temporal lobe epilepsy
• Aware vs Impaired Awareness

EEGs

A routine office EEG is 30-45 minutes long and has approximately 50% sensitivity (Baldwin et al, 2014). A normal EEG is not entirely reassuring but an abnormal EEG is concerning.

Neuroimaging

CT is generally avoided in children to limit total radiation exposure and because MRI provides better quality visualization. Administer gadolinium contrast only if the initial read suggests a structural abnormality to look for breakdown of the blood-brain barrier (Gaillard et al, 2009). 

Expert Opinion: If the child has a reassuring exam, consider deferring to outpatient imaging. In the hospital it can be difficult to keep young patients NPO for sedation when there is no exact time scheduled.

Counseling Parents on Safety

Biking and swimming have their own inherent risks, and patients with seizures should take the same precautions as any other child (ex. never swim unsupervised, never bike without a helmet). It is important to recognize that while many children may outgrow their seizures, the effects of limiting their participation in sports and activities with friends can have long-term effects.

Most states have their own policies on driving, but most require that a patient be 6 months seizure-free to drive. Make sure to counsel patients that they should avoid driving if they miss a dose of seizure medication or are sleep deprived.

Seizure Differential Diagnosis

Important to consider the age of the patient. Ask if parents/caregivers have videos of the episodes and show them examples if not.

• Benign neonatal sleep myoclonus (1 week old): Myoclonic jerks during NREM sleep. Resolves within a few months.

• • Infantile spasms (4-6 month old): Brief episodes of head flexion + arm extension that last 1-2 seconds in duration and often cluster. May also involve eye deviation. Do not want to miss this because infantile spasms can evolve into Lennox-Gastaut syndrome and serious developmental delays (Sidhu et al, 2013)

• Breath-holding spells (18 month old): Last 1-2 minutes and cause the child to turn blue or lose consciousness. Often precipitated by emotion or injury. May trigger real seizures.

• Sandifer Syndrome: Posturing and stiffening related to GERD from feeding.

• Psychogenic Nonepileptic Seizures (PNES): Can be difficult to distinguish from real seizures, especially because some patients do have a history of epilepsy. Gold standard of evaluation is continuous EEG. Important to remember that this is not malingering. 

• Syncope: Fainting spells can appear like convulsions.

Antiepileptic Drugs

Initiation of AEDs is recommended after two unprovoked seizures (febrile seizures do not count), as the risk of having another seizure is close to 80%. AEDs should be continued until the patient has been seizure free for at least two years (Sidhu et al, 2013). Check an EEG and taper slowly. Options for AEDs include:

• Levetiracetam – Can be used for focal and generalized seizures. Great AED for all clinicians to be comfortable with – think of it like sertraline for depression or ceftriaxone for meningitis (expert opinion).
• Oxcarbazepine – Acts as a sodium-channel blocker and treats focal seizures. Be careful because it may worsen generalized seizures (Gelisse et al, 2004).

Status Epilepticus

Defined as seizure activity lasting greater than 30 minutes or multiple seizures with no return to consciousness. Should be treated urgently to reduce risk of brain damage (Lowenstein et al, 1999). 

The FEBSTAT study found that 10% of participants with febrile status epilepticus lasting >30 minutes had abnormalities of the hippocampus on follow-up imaging with MRI (Lewis et al, 2014). The concern is that hippocampal sclerosis caused by prolonged seizure activity could increase risk of developing temporal lobe epilepsy later in life.

Treatment (Sidhu et al, 2013)

1st line: IV lorazepam 0.1mg/kg, can give a second dose if seizure continues after 5-10 minutes

• Note – Don’t waste time if unable to get IV access. Other initial options include rectal diazepam and intranasal or intramuscular midazolam. As a last resort, can consider placing an intraosseous line.

2nd line: IV fosphenytoin (20mg/kg) OR IV levetiracetam (40mg/kg)

3rd line: IV phenobarbital (20mg/kg)

4th line: Induction of medical coma with a phenobarbital, midazolam, or propofol drip.

• Continuous EEG must be used to monitor for subclinical seizures and confirm suppression of all epileptiform activity.

Be sure to assess respiratory status and need for intubation throughout the process. May miss high CO2 in the setting of normal oxygen saturation maintained with supplemental oxygen. 

Future of Epilepsy Treatment

Important to consider for patients with refractory seizures despite treatment with multiple AEDs.

• Ketogenic diet: Used more widely since the 1980s (Meira et al, 2019)
• Surgery: Standard of care was previously surgical resection but newer options include laser ablation and electrical stimulators.

Case Study: Dravet Syndrome & Cannabidiol

Dravet syndrome is a rare form of severe epilepsy caused by mutations in the sodium channel SCN1A gene. Patients develop frequent seizures of multiple types that are refractory to multiple lines of therapy. They also experience neurodevelopmental regression and are at greater risk for sudden unexpected death in epilepsy (SUDEP). This condition has been central to FDA approval of cannabidiol for treatment of certain seizure types (Devinsky et al, 2017).

Goal

Listeners will develop a framework for the diagnosis and management of pediatric seizures and epilepsy.

Learning objectives

After listening to this episode listeners will…

1. Explain the difference between seizures and epilepsy.
2. Discuss the differential diagnosis of seizure-like activity.
3. Recognize concerning signs of febrile seizures that require further evaluation.
4. Select appropriate workup for new-onset seizures in children.
5. Choose evidence-based antiepileptic drugs for treatment of pediatric epilepsy.
6. Describe the management of status epilepticus in a child.
7. Educate families and have meaningful discussions about management of childhood seizures.

Disclosures

Dr. Gaitanis reports no relevant financial disclosures. The Cribsiders report no relevant financial disclosures. 

Citation

Gaitanis J, Mao C, Chiu C, Berk J. “#18 Shaking Things Up: Pediatric Seizures & Epilepsy with Dr. John Gaitanis”. The Cribsiders Pediatric Podcast. https:/www.thecribsiders.com. February 3, 2020.

References

1. Baldwin et al. Yield of epileptiform electroencephalogram abnormalities in incident unprovoked seizures: a population-based study. Epilepsia. 2014. [https://pubmed.ncbi.nlm.nih.gov/25041095/]
2. Devinsky et al. Trial of cannabidiol for drug-resistant seizures in the Dravet syndrome. NEJM. 2017. [https://pubmed.ncbi.nlm.nih.gov/28813226/]
3. Gaillard et al. Guidelines for imaging infants and children with recent-onset epilepsy. Epilepsia. 2009. [https://pubmed.ncbi.nlm.nih.gov/19389145/]
4. Gelisse et al. Worsening of seizures by oxcarbazepine in juvenile idiopathic generalized epilepsies. Epilepsia. [https://pubmed.ncbi.nlm.nih.gov/15461683/]
5. Lewis et al. Hippocampal sclerosis after febrile status epilepticus: the FEBSTAT study. Ann Neurol. 2014. [https://pubmed.ncbi.nlm.nih.gov/24318290/]
6. Meira et al. Ketogenic Diet and Epilepsy: What We Know So Far. Front Neurosci. 2019. [https://pubmed.ncbi.nlm.nih.gov/30760973/]
7. Sidhu et al. Pediatric Seizures. Pediatrics in Review. 2013. [https://pubmed.ncbi.nlm.nih.gov/23908360/]
8. Subcommittee on Febrile Seizures. Clinical Practice Guideline – Febrile Seizures: Guideline for the Neurodiagnostic Evaluation of the Child With Simple Febrile Seizure. Pediatrics. 2011. [https://pubmed.ncbi.nlm.nih.gov/21285335/]

Subcommittee on Febrile Seizures. Febrile Seizures: Clinical Practice Guideline for the Long-term Management of the Child With Simple Febrile Seizures. Pediatrics. 2008. [https://pubmed.ncbi.nlm.nih.gov/18519501/]