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Febrile Seizures
Definition
A febrile seizure is a seizure occurring in children between 6 months and 6 years of age (peak incidence 9-20 months), associated with fever (temperature ≥38°C / 100.4°F), in the absence of CNS infection, metabolic disturbance, or prior afebrile seizures. Despite involving epileptic activity, febrile seizures are not classified as epilepsy.
Epidemiology
- Affects 2-5% of children (up to 8% in Japanese children)
- The most common seizure disorder in the pediatric age group
- Age range: 6 months to 6 years; rare before 3 months or after 6 years
- Strong familial tendency - polygenic inheritance in most cases; autosomal dominant in some families
Classification
Simple Febrile Seizures
All of the following must be true:
- Generalized tonic-clonic semiology
- Duration <15 minutes
- Only one seizure in a 24-hour period
- No focal features
- Child aged 6 months to 6 years
- Full recovery to neurological baseline
Complex Febrile Seizures
One or more of:
- Duration >15 minutes
- Focal features (focal ictus or lateralized postictal weakness such as Todd's paralysis)
- More than one seizure in 24 hours or within the same febrile illness
- Age <6 months or >6 years
A subset - febrile status epilepticus - is defined as a febrile seizure lasting >30 minutes.
Genetics
- Polygenic in isolated cases; single-gene mutations more often found in familial recurrent cases
- GEFS+ (Generalized Epilepsy with Febrile Seizures Plus): Autosomal dominant, heterogeneous expression. Some family members have typical self-limiting febrile seizures; others have febrile seizures persisting beyond age 6 or overlapping with other seizure types.
- Dravet syndrome (Severe Myoclonic Epilepsy of Infancy): Starts with prolonged, often asymmetrical febrile seizures, but progresses to afebrile seizures and cognitive decline. Caused by truncating mutations in SCN1A (sodium channel subunit) or GABRG2 (GABA-A receptor subunit). This is NOT a benign condition.
- Digenic inheritance has been described (two mutations required for expression).
Pathophysiology
- Rising or peak body temperature lowers the seizure threshold in the immature brain
- Herpesvirus 6 (HHV-6) is a common precipitant - it causes high fever and may have direct neurotropic effects
- Any viral or bacterial illness, or rarely immunization, can trigger fever and thus a febrile seizure
- Febrile status epilepticus can injure the hippocampus: ~11.5% of affected children show unilateral increased hippocampal T2 signal on MRI acutely, which may evolve into hippocampal sclerosis
Workup
Simple Febrile Seizure
| Investigation | Recommendation |
|---|
| Blood/urine tests | Only as needed to find the source of fever |
| Lumbar puncture | Strongly consider if signs/symptoms of meningitis; consider in children <12 months or unimmunized (H. influenzae type b, S. pneumoniae) |
| EEG | Not required |
| Neuroimaging | Not required |
| Hospital admission | Not required if child returns to baseline |
Per the AAP guidelines, routine blood studies, neuroimaging, and EEG are not indicated for simple febrile seizures.
Complex Febrile Seizure
- Routine LP, labs, and imaging are still not automatically indicated in the absence of other signs of serious infection
- Exception: children with seizure >30 minutes who appear ill warrant evaluation for serious bacterial infection (including CSF)
- Do not delay antibiotics in a sick-appearing child pending workup
- Bacterial meningitis incidence in complex febrile seizures without clinical signs of meningitis is low (~2 cases in 526 patients in one study), but is significantly higher (15-18%) when seizure duration exceeds 30 minutes
Treatment
Acute Seizure
- Most self-terminate; supportive care is sufficient
- For prolonged seizures: rectal or IV diazepam is the agent of choice
- For febrile status epilepticus: follow the standard status epilepticus protocol (benzodiazepines → second-line agents - see AES guideline)
Prophylaxis
- Prophylactic daily anticonvulsants are NOT recommended - side effects outweigh the minor risk of recurrence
- Antipyretics (paracetamol, ibuprofen) are indicated for comfort but have no proven efficacy in preventing febrile seizures
- Intermittent diazepam (oral or rectal) at the time of fever may be used in children with frequent recurrent febrile seizures - though side effects (sedation, ataxia) limit this approach
Prognosis and Risk of Recurrence
- ~30-40% of children will have at least one recurrence; multiple recurrences are uncommon
- Risk factors for recurrence:
- Age at onset <1 year
- Family history of febrile seizures or epilepsy
- Attendance at daycare (increased exposure to febrile illness)
Risk of Later Epilepsy
| Population | Epilepsy risk by age 25 |
|---|
| General population | ~1% |
| Simple febrile seizure | ~2-2.4% (slightly above baseline) |
| One complex feature | 6-8% |
| Two complex features | 17-22% |
| All three complex features | 49% |
Complex features (prolonged, focal, multiple) predict focal epilepsy; large number of febrile seizures + family history of epilepsy predict generalized epilepsy. The link between childhood febrile status epilepticus and later temporal lobe epilepsy (via hippocampal sclerosis) is well-established - in one series, 70% of patients with medial temporal lobe epilepsy had a history of complex febrile seizures in the first 5 years.
Parent Counseling
- Febrile seizures are not dangerous in the vast majority of cases
- Having a febrile seizure does not mean the child will develop epilepsy
- Teach parents: keep the child safe during a seizure (lateral position, nothing in the mouth), time the seizure, call emergency services if >5 minutes
- Anticipatory guidance for emergency rectal/intranasal diazepam if recurrence is likely
- Reassure that normal intellect and development are expected
Sources:
- Bradley and Daroff's Neurology in Clinical Practice, p. 2255-2256
- Adams and Victor's Principles of Neurology, 12th ed., p. 342-343
- Tintinalli's Emergency Medicine, p. 936
- Rosen's Emergency Medicine, p. 3287