Treatment of Electrical Status Epilepticus of Sleep (ESES) in Pediatric Patients
The most effective treatment approach for ESES in pediatric patients is levetiracetam as first-line therapy, followed by benzodiazepines (particularly clobazam) and corticosteroids for refractory cases. 1, 2
First-Line Pharmacological Therapy
Levetiracetam
- Dosing: 40-50 mg/kg/day divided into two doses (maximum 2.5g)
- Efficacy: Shown to be effective in 41% of ESES cases 1
- Advantages:
- Favorable safety profile with minimal adverse effects
- No significant drug interactions
- Available in both oral and IV formulations
- Recommended as an anticonvulsant in pediatric patients 3
Alternative First-Line Options
- Valproate: 20-30 mg/kg/day divided into two doses
Second-Line Therapy (For Inadequate Response)
Benzodiazepines
- Clobazam: Start at 0.2-0.5 mg/kg/day, can increase up to 40 mg daily for patients >30kg 5
- Effective in 31% of ESES cases 1
- Lower sedation profile compared to other benzodiazepines
- High-dose diazepam: Effective in 37% of cases, but responses are typically temporary 1
Corticosteroids
- Efficacy: 65% response rate in ESES 1
- Regimen options:
- Oral prednisolone: 2 mg/kg/day for 1 month with gradual taper over 2 months
- Pulse methylprednisolone: 20-30 mg/kg/day (max 1g) for 3 days, followed by oral prednisolone taper
- Important consideration: High relapse rate (89%) after steroid discontinuation 2
Third-Line Options
Sulthiame
- Effective in 17% of ESES cases 1
- Less commonly used in the US but may be beneficial in select cases
Intravenous Immunoglobulin (IVIG)
- Efficacy rate of 33% 1
- Consider for cases refractory to anticonvulsants and steroids
- Typical dose: 2 g/kg divided over 2-5 days
EEG Monitoring and Treatment Goals
- Definition of ESES: Spike-wave index (SWI) ≥85% during non-REM sleep 2
- Treatment target: Reduction of SWI to <50% 2
- Monitoring frequency:
- Baseline EEG with sleep recording at diagnosis
- Follow-up EEG every 3-6 months to assess treatment response
- More frequent monitoring if clinical deterioration occurs
Duration of Treatment
- Critical factor: Duration of ESES correlates significantly with residual intellectual deficit 1
- Treatment continuation: Maintain effective therapy until:
- Resolution of ESES pattern on EEG (SWI <50%)
- At least 2 years of seizure freedom if clinical seizures are present
- Weaning: Gradual withdrawal over 6+ weeks after sustained EEG improvement 4
Special Considerations
Etiology-Based Approach
Benign partial epilepsies of childhood (37% of ESES cases) 1
- More favorable prognosis
- May respond better to standard anticonvulsants
Structural brain abnormalities (40% of ESES cases including cerebral palsy, hydrocephalus) 1
- More resistant to treatment
- Earlier and more aggressive therapy may be warranted
Cognitive and Behavioral Monitoring
- Regular neurocognitive assessment is essential
- 57% of children with ESES develop cognitive deterioration 1
- Early intervention for cognitive/behavioral issues improves outcomes
Treatment Algorithm
- Initial therapy: Start levetiracetam at 40-50 mg/kg/day
- If inadequate response after 2-4 weeks:
- Add clobazam OR
- Switch to valproate (if not contraindicated)
- If still inadequate after additional 2-4 weeks:
- Initiate steroid therapy
- For refractory cases:
- Consider IVIG or sulthiame
- Evaluate for underlying structural abnormalities if not already done
Pitfalls and Caveats
- Underdiagnosis: ESES requires sleep EEG for diagnosis; maintain high clinical suspicion
- Delayed treatment: Longer ESES duration correlates with worse cognitive outcomes 1
- Overreliance on seizure control: Treatment success should be measured by EEG improvement, not just seizure reduction
- Medication discontinuation: High relapse rates, particularly with steroids (89%) and benzodiazepines (60%) 2
- Inadequate follow-up: Regular EEG monitoring is essential to confirm treatment efficacy
Early recognition and effective therapy are crucial for improving long-term neuropsychological outcomes in children with ESES.