Case 1 History: 4-year-old male brought to the ER with continuous generalized tonic- clonic seizures lasting 25 minutes. Previously healthy, with a history of febrile seizures at age 2. He had a fever (39°C) for the past 12 hours before the seizure episode. Family History: Mother had febrile seizures as a child. Examination: Vitals: HR 150 bpm, BP 110/70 mmHg, RR 28 breaths/min, Temp 39.2°C. Neurological Exam: Unresponsive, continuous generalized tonic- clonic movements.
Diagnosis: Clinical Diagnosis: Convulsive status epilepticus secondary to febrile illness. Laboratory Tests: CBC, electrolytes, blood glucose (all within normal limits), CSF analysis (pending). Imaging: CT head (normal). Management: Acute Management: Initial: Administered lorazepam 0.1 mg/kg IV (stopped the seizures). Further Treatment: Given a loading dose of fosphenytoin 20 mg PE/kg IV. Supportive Care: IV fluids, antipyretics, monitoring in PICU. Post-Seizure Care: Empiric antibiotics (pending CSF results), seizure precautions, scheduled follow-up with a pediatric neurologist.
Case 2 History: 8-year-old female with a 2-day history of confusion, altered behavior, and intermittent staring spells. Background: History of absence epilepsy, poorly controlled on valproate. Family History: No significant history of epilepsy or other neurological conditions. Examination: Vitals: HR 90 bpm, BP 100/60 mmHg, RR 20 breaths/min, Temp 36.8°C. Neurological Exam: Altered mental status, frequent staring episodes lasting 30-60 seconds, no convulsive movements.
Management: Acute Management: Initial: Administered lorazepam 0.1 mg/kg IV (stopped the seizures temporarily). Further Treatment: Adjusted valproate dosage and added levetiracetam 20 mg/kg IV. Supportive Care: Monitoring in PICU, addressing underlying causes (e.g., medication non-compliance). Post-Seizure Care: Regular follow-up with a neurologist, education on medication adherence, and seizure action plan.
Case 3 History: Presentation: 10-year-old male with continuous focal seizures evolving to generalized seizures, lasting over 60 minutes, unresponsive to initial treatment. Background: History of Lennox- Gastaut syndrome with multiple seizure types, on multiple antiepileptic drugs (valproate, clobazam , topiramate ). Family History: No relevant history. Examination: Vitals: HR 130 bpm, BP 120/75 mmHg, RR 25 breaths/min, Temp 37.2°C. Neurological Exam: Unresponsive, ongoing focal seizures with right arm and leg twitching.
Diagnosis: Clinical Diagnosis: Refractory status epilepticus. Laboratory Tests: CBC, electrolytes, liver function tests (all normal), antiepileptic drug levels (within therapeutic range). Imaging: MRI brain (showed prior cortical dysplasia).
Management: Acute Management: Initial: Administered lorazepam 0.1 mg/kg IV, followed by fosphenytoin 20 mg PE/kg IV (seizures persisted). Further Treatment: Administered midazolam infusion 0.1 mg/kg/ hr IV, increased to 0.2 mg/kg/hr. Refractory Treatment: Added phenobarbital 20 mg/kg IV and initiated ketogenic diet. Supportive Care: Continuous EEG monitoring, mechanical ventilation due to sedative use, PICU care. Post-Seizure Care: Ongoing care with a multidisciplinary team, including neurology, dietitian (for ketogenic diet), and critical care.
Introduction Definition : Status epilepticus (SE) is defined as a condition in which a seizure lasts longer than 30 minutes or when seizures occur close together and the person doesn’t recover between seizures. Operational Definition : SE is typically defined as continuous seizure activity lasting more than 5 to 10 minutes (depending on type of seizure) or recurrent seizures without return to baseline mental status between seizures. The traditional definition of 30 minutes was revised to facilitate earlier intervention.
Epidemiology : SE is more common in children, with an annual incidence of 17-23 per 100,000 children. Early childhood and infancy are the most common periods. Significance : SE is a neurological emergency with significant morbidity and mortality, requiring immediate intervention to prevent permanent neurological damage and systemic complications.
Chronic Causes : Epilepsy : Known seizure disorder with breakthrough seizures. Neurodegenerative Disorders : Conditions like Batten disease, mitochondrial disorders. Congenital Brain Malformations : Cortical dysplasia, lissencephaly. Idiopathic : Often seen in previously healthy children with no identifiable cause.
Pathophysiology Early Phase : Excitotoxicity : Excessive release of excitatory neurotransmitters (e.g., glutamate) leading to sustained neuronal depolarization. Calcium Influx : Increased intracellular calcium causes neuronal injury. Established Phase : Inflammation : Activation of inflammatory pathways exacerbates neuronal damage. Blood-Brain Barrier Disruption : Allows harmful substances to enter the brain, worsening edema and injury.
Refractory Phase : Altered Receptor Function : Changes in GABA and NMDA receptors make seizures more resistant to treatment. Neuronal Death : Persistent seizures lead to apoptosis and necrosis of neurons.
Classification Convulsive SE : Characterized by prolonged tonic- clonic activity. Generalized Convulsive SE (GCSE) : Includes tonic- clonic seizures involving the entire body. Focal SE with Secondary Generalization : Begins as focal and progresses to generalized. Focal motor SE (simple partial SE)
Non-convulsive SE : Includes subtle motor symptoms and altered consciousness. Absence SE : Prolonged periods of impaired awareness without convulsions. Complex Partial SE : Prolonged focal seizures with impaired consciousness.
Refractory SE : Seizures persist despite initial treatment with a benzodiazepine and another antiepileptic drug. Super-refractory SE : Seizures continue for 24 hours or more despite treatment, including general anesthesia.
Clinical Manifestations Convulsive SE : Tonic- Clonic Movements : Sustained muscle contractions followed by rhythmic jerking. Altered Consciousness : Ranges from confusion to deep coma. Autonomic Dysfunction : Tachycardia, hypertension, hyperthermia, excessive salivation, and incontinence.
Non-convulsive SE : Subtle Motor Activity : Minor twitching, nystagmus, facial myoclonus. Cognitive and Behavioral Changes : Confusion, agitation, hallucinations. Diagnosis : Often requires EEG due to subtlety of clinical signs.
Diagnosis History : Seizure Characteristics : Onset, duration, type of seizure activity. Precipitating Factors : Fever, recent illness, head trauma, ingestion of toxins. Past Medical History : Previous seizures, developmental history, family history of epilepsy.
Physical Examination : Neurological Examination : Assess level of consciousness, focal deficits, signs of increased intracranial pressure. Systemic Examination : Look for signs of infection, metabolic disorders, or trauma.
Laboratory Workup : Metabolic Panel : Glucose, electrolytes, calcium, magnesium, renal and liver function tests. Complete Blood Count : To check for signs of infection or anemia. Toxicology Screen : To rule out ingestion of toxic substances. Lumbar Puncture : If CNS infection is suspected.
Imaging: CT Scan : For immediate evaluation of acute structural abnormalities. MRI: For detailed imaging of brain structures. EEG : Essential for diagnosing non-convulsive SE and guiding management in refractory cases.
Diagnostic Criteria Continuous Seizure Activity : Lasting more than 5 minutes. Recurrent Seizures : Without full recovery between episodes. EEG Findings : Continuous epileptiform activity, even in the absence of overt clinical seizures.
Management Immediate Management : Airway Management : Ensure airway patency, consider intubation if necessary. Breathing and Circulation : Provide oxygen, establish IV access, monitor vital signs.
First-Line Treatment : Administer a benzodiazepine (e.g., lorazepam 0.1 mg/kg IV, diazepam 0.2 mg/kg IV, or midazolam 0.2 mg/kg IM/IN). Second-Line Treatment: IV Antiepileptic Drugs: Fosphenytoin (20 mg PE/kg), valproate (20-40 mg/kg), or levetiracetam (20-60 mg/kg).
Refractory SE: Continuous Infusion: Midazolam (0.2 mg/kg bolus followed by 0.1-2 mg/kg/hour), or propofol (1-2 mg/kg bolus followed by 2-10 mg/kg/hour), or pentobarbital (5-15 mg/kg bolus followed by 0.5-5 mg/kg/hour). Monitoring: Continuous EEG monitoring, frequent reassessment of neurological status.
Supportive Care : Metabolic Management : Correct electrolyte imbalances, manage hypoglycemia. Treatment of Underlying Cause : Antibiotics for infections, surgery for structural lesions if indicated. ICU Care : Close monitoring in an intensive care setting, support for systemic complications (e.g., respiratory support, hemodynamic stabilization).
Complications of Status Epilepticus in Pediatrics Neurological Damage : Cognitive Impairment : Prolonged seizures can lead to long-term cognitive deficits, affecting memory, attention, and learning abilities. Epilepsy : Increased risk of developing chronic epilepsy after an episode of status epilepticus. Developmental Regression : In severe cases, children may experience a regression in developmental milestones.
Systemic Complications : Hypoxia : Due to prolonged seizures, there may be insufficient oxygen delivery to the brain, leading to hypoxic-ischemic injury. Metabolic Derangements : Includes acidosis, hyperkalemia, hypoglycemia, and hyponatremia, which can occur due to prolonged convulsive activity. Rhabdomyolysis : Muscle breakdown from prolonged convulsions can lead to myoglobin release into the bloodstream, potentially causing kidney damage.
Cardiovascular Complications : Arrhythmias: Prolonged seizures can lead to cardiac arrhythmias, which may be life-threatening. Hypotension or Hypertension: Seizures can cause fluctuations in blood pressure, leading to potential cardiovascular instability.
Respiratory Complications : Aspiration Pneumonia : Risk of aspiration of saliva or gastric contents due to impaired swallowing and protective airway reflexes. Respiratory Failure : Prolonged seizures can lead to respiratory muscle fatigue and respiratory failure.
Renal Complications : Acute Kidney Injury (AKI): Resulting from rhabdomyolysis and systemic hypoperfusion during prolonged seizures. Psychiatric Complications: Behavioral Changes: Postictal behavioral changes such as agitation, aggression, or depression. Post-Traumatic Stress Disorder (PTSD): In some cases, children and their families may experience PTSD following a severe episode.
Status epilepticus, especially when not promptly treated, can lead to a significant increase in mortality risk, particularly in children with underlying neurological disorders.
Summary Pediatric status epilepticus is a medical emergency that requires prompt recognition and intervention. Management involves initial stabilization, administration of benzodiazepines, and escalation to second-line antiepileptic drugs if necessary. Refractory cases require continuous infusion of anesthetic agents and intensive care monitoring. Identifying and treating the underlying cause is essential for effective management and preventing recurrence.
References UpToDate . (2024). "Management of convulsive status epilepticus in children.“ Nelson Textbook of Pediatrics, 21st Edition. TASH Pediatric emergency management protocol .