Case Vignettes 3 yo child is rushed to the emergency department because of an episode of fits associated with uprolling of eyes and high fever O/E Kernig’s sign is negative No post-ictal neurological deficits There is a h/o a similar episode when the child had a fever due to gastroenteritis. What is the diagnosis?
Definition Febrile seizures are seizures that are associated with fever (> 38°C ( 100.4°F )) in the absence of CNS infection, metabolic abnormalities, or a history of afebrile seizures
Epidemiology Peak incidence : 2 nd year of life ; most commonly between 6 months to 5 years of age Prevalence : ∼ 4% of all children
Pathophysiology: Recent advances Inherited predisposition Hippocampal atrophy- FEBSTAT study Genetic susceptibility ( SCN1A , HCN ) Fever and seizure induction 2-hit hypothesis Immune pathway activation Sawires R, Buttery J and Fahey M(2022) A Review of Febrile Seizures:Recent Advances in Understanding of Febrile Seizure Pathophysiology and Commonly Implicated Viral Triggers. Front. Pediatr . 9:801321.doi : 10.3389/ fped.2021.801321
“2-hit hypothesis” Recruitment of astrocytes and microglia Increased excitability of pyramidal and dentate granule cells Hyperthermia Ectopic granule cells
Immune pathway activation Do antipyretics have a role in preventing febrile seizure recurrences?
Vaccines and febrile seizures “ THBT vaccines are implicated in the causation of febrile seizures”
Clinical features ILAE classification of febrile seizures Simple febrile seizures (~75%) Complex febrile seizures (~25%) Clinical features Generalised , usually tonic clonic seizures (clonic seizures are also possible) Symmetrical No other apparent neurologic disorders Focal onset Pronounced on one side of the body Transient hemiparesis and speech impairement Duration and frequency <15 min Maximum of one seizure within 24h >15 min >1 seizure within 24 h Age 6 months to 5 years More commonly outside the typical range of 6 months to 5 years Postical phase Typically a quick return to normal Confusion and drowsiness may be present for a short period of time Prolonged drowsiness or deviated eyes may be a sign of other etiology (e.g. meningitis ) or of ongoing seizure activity
Diagnostics Approach Simple febrile seizures do not require specific diagnostic workup Diagnostic examination focuses on identifying the cause of fever Complex febrile seizures always require specific investigative tests (e.g., EEG and imaging)
Diagnostics Determine the cause of fever Physical examination Blood investigations ( CBC , CRP, electrolytes, blood glucose) Urinalysis and urine cultures Consider imaging to locate the source of infection (ultrasound, X-ray)
Diagnostics Additional diagnostic steps : for complex febrile seizures and/or abnormal neurological examination Lumbar puncture ? Indications EEG Imaging (e.g., CT vs M RI)
Lumbar Puncture < 6 months 6-12 months > 12 months Should be done in all infants Unknown immunisation status Hib and pneumococcal vaccines not administered Features suggestive of intracranial infection Signs and symptoms of meningitis
Genetic epilepsy syndromes GFES+ Dravet syndrome From precision diagnosis to precision treatment
Treatment Uncomplicated seizures: self-resolving Consider abortive therapy if seizures ≥ 5 min or in complex seizures Abortive therapy Treatment of choice : IV lorazepam ( 0.1 mg/kg) Alternative: IV diazepam ( 0.1mg /kg) , buccal/intranasal midazolam ( 0.2mg /kg; max- 10 mg) If necessary, repeat medication after 5 min Additional measures are needed with status epilepticus
Febrile status epilepticus If seizure continues administer sequentially consider
Febrile status epilepticus consider IV phenobarbitone 20 mg/kg infusion Rpeat , if needed, up to twice, at 10 mg/kg IV midazolam at 1-15 mcg/kg/min infusion Coma induced by phenobarbitone infusion IV Ketamine and oral topiramate Immunotherapy dietary therapy Vagal nerve stimulation Epilesy surgery
Reassure caregivers and provide information After a febrile seizure , initiate antipyretic therapy ( NSAIDs and acetaminophen ) at an early stage Treatment ? Role of long-term or intermittent anticonvulsant prophylaxis
Prognosis Risk factor Risk for developing epilepsy Simple febrile seizures 1 Recurrent febrile seizures 4 Complex seizure 6 Fever <1 hr before febrile seizure 11 Family history of epilepsy 18 Focal complex febrile seizures 29 Neurodevelopmental abnormalities 33 ? What are the chances of recurrence ? What is the risk of developing epilepsy ? Are there any long-term effects
QUIZ
Q1) 3 yo child is rushed to the emergency department because of an episode of fits associated with uprolling of eyes and high fever O/E Kernig’s sign is negative No post-ictal neurological deficits There is a h/o a similar episode when the child had a fever due to gastroenteritis. a) What is the diagnosis? Why?
Probable case of FEBRILE SEIZURES due to: High fever No post-ictal deficits Negative Kernig’s sign (ruling out meningitis) Febrile seizures are seizures that- Occur between the age of 6 months and 5 years Occur at a temperature of 38°C ( 100.4°F ) or higher Are not due to any CNS infection or any metabolic imbalance Occur without a history of prior afebrile seizures Occur with no post-ictal neurological deficit
b) In the previous question, which of the following is false regarding the type (febrile seizures) of seizures? Likelihood of developing epilepsy is low Associated with temperature of 38°C or higher Antipyretics reduce the risk of febrile convulsions Absence of having a developmental delay
3) Antipyretics reduce the risk of febrile convulsions Antipyretics do not reduce the risk of recurrent febrile seizure, the seizure often occurs as the temperature is rising or falling abruptly.
Q2) CBC, CRP should be considered in- Choose one or more appropriate responses: A) Febrile status epilepticus B) Simple febrile seizure C) Hypoglycemic seizure D) Complex febrile seizure
A) Febrile status epilepticus D) Complex febrile seizures
Q3) Infant with febrile seizure brought convulsing to hospital (age < 1year). Select investigation(s) that may be considered- CBC for anemia serum magnesium blood sugar serum potassium serum calcium serum sodium
Q4) Your neighbour frantically knocks on your door to seek help as her toddler had suddenly started convulsing with uprolling of eyes. The child had a high fever and this was his 3 rd episode of seizures in 6 months. Post-recovery, the mother is still anxious and wishes to know the risk factors for such recurrences of febrile seizures. You would explain all of the following except- Duration of fever<24 hr Late age of onset Family history of epilepsy Lower serum sodium
B) Late age of onset Early age of onset (age <1 year) is a risk factor for recurrence of febrile seizures . The other risk factors for such recurrences are as follows- Major : Age <1 year Duration of fever < 24 hours Fever 38-39°C Minor : Family history of febrile seizures Family history of epilepsy Complex febrile seizure Male gender Lower serum sodium at the time of presentation
Q5) An 8 year old boy was playing at his school when he suddenly developed rapid jerky movements of both upper and lower limbs and was rushed to the emergency department. It had been ongoing for the past 10 minutes and the boy had been unresponsive throughout. Which of the following is not useful in the management of this condition? Lorazepam Phenytoin Phenobarbitone Carbamazepine
D) Carbamazepine The clinical scenario is that of status epilepticus. This is due to rapid jerky movements causing continuous seizure activity for more than 5 minutes. Carbamazepine is not commonly used in the treatment of status epilepticus.
Q6) In case of seizures with fever, Lumbar puncture is advised for following conditions. Select one or more correct statements All children with complex febrile seizures Age > 12 months who have received antibiotics Routine CSF viral or bacterial panel NOT indicated for all patients with febrile seizures; It is indicated only if routine CSF analysis is indicative of meningitis Age < 12 months, first episode who have not received pneumococcal immunization Children aged 12-18 months who have not received a full course of Hib and pneumococcal vaccination and there are no clinical features of meningitis
(B) Age > 12 months who have received antibiotics (C) Routine CSF viral or bacterial panel NOT indicated for all patients with febrile seizures; It is indicated only if routine CSF analysis is indicative of meningitis (D) Age < 12 months, first episode who have not received pneumococcal immunization
Q7) In management of children with febrile seizures; Select one or more correct statements PCM (15 mg/kg/dose) administered round the clock for duration of fever does NOT prevent occurrence or recurrence of seizure but will make the child comfortable Continuous prophylaxis is NOT recommended in simple febrile seizures Empirical supplementation with oral iron, zinc or vitamin D Intranasal Midazolam (0.2 mg/kg) is the recommended as abortive medication for domiciliary management of acute seizures
PCM (15 mg/kg/dose) administered round the clock for duration of fever does NOT prevent occurrence or recurrence of seizure but will make the child comfortable Continuous prophylaxis is NOT recommended in simple febrile seizures (D) Intranasal Midazolam (0.2 mg/kg) is the recommended as abortive medication for domiciliary management of acute seizures