Electrical status epilepticus in sleep (ESES) is an electroencephalographic pattern showing significant epileptiform discharges during sleep. Two clinical syndromes associated with ESES are continuous spike wave in slow-wave sleep (CSWS) and Landau-Kleffner syndrome (LKS). CSWS often presents with global regression and loss of language or cognitive skills, while LKS primarily involves language regression. Treatment of ESES focuses on antiepileptic drugs and corticosteroids, with the goal of resolving seizures and discharges to improve long-term neuropsychological outcomes.

1. Electrical status epilepticus in sleep
Wafaa AL Shehhi
R3 Pediatric Neurology Resident
January 2013

2. Roadmap
 Definitions.
 Etiology.
 Pathophysiology.
 CSWS.
 LKS.
 Electrophysiology and imaging.
 Differential Diagnosis.
 Treatment.
 Prognosis.

3. Definitions
 Electrical status epilepticus in sleep (ESES): an
electroencephalographic pattern showing significant
activation of epileptiform discharges in sleep.
 Continuous spike wave in slow-wave sleep (CSWS) and
Landau-Kleffner syndrome (LKS) describe the clinical
epileptic syndromes seen with ESES.

4. Definitions
 In Party’s original article, “continuous” was used only if
the EEG showed a spike wave index of 85% to 100% and
if these abnormalities persisted on 3 or more records over
a 1- month period of time.
 The spike wave index defined as: total number of minutes
of all spike and slow-wave abnormalities multiplied by 100
and devided by the total nonrapid eye movement sleep
minutes.

5. Etiology
 Underlying structural abnormalities.
 CNS infection.
 Underlying immune disorder.
 Idiopathic.

6. Pathophysiology
 ESES: Activation of the reticulo-thalamic-cortical system
with seconday bilateral synchronization through the
corpus callosum.

7. CSWS
• Children with CSWS often presented with:
 global regression.
 Loss of language expressive aphasia.
 Loss of temporospatial skills.
 Hyperactivity.
 Short-term memory deficits.
 Aggressiveness.
 Motor deficits ataxia, dystonia, dyspraxia.

8. CSWS
 Epilepsy:
 Seizures are the presenting symptom in 80% of children
with CSWS.
 The most common seizure types including GTC, typical
absence and simple acnd complex partial seizures.

9. CSWS
 Age of onset:
 Ranging from 1-14 years.
 A mean between 4-8 years.
 Outcome: poorer in children with earlier age at onset.

10. LKS
 The primary clinical manifestation : language regression.
 An acquired auditory agnosia.
 Other clinical symptoms:
 Irritability.
 Hyperkinesia.
 Attention-deficit disorder.
 Autistic- like behavior.

11. LKS
 Epilepsy:
 Seizures present in 70-80% and are typically infrequent
and easily treated.
 Age of onset:
 3-8 years.
 A peak at 4-5 years.

12. Electrophysiology and imaging
 EEG:
 In CSWS: EEG during wakefulness typically shows
epileptiform discharges that are focal, multifocal, or
diffuse, often with frontotemporal or frontocentral
predominance.
 During sleep, there is dramatic activation of diffuse
epileptiform discharges.

13. Electrophysiology and imaging
 In LKS: the awake EEG is variable; it may contain focal,
multifocal or generalized epileptic abnormalities, or it
may be normal.
 The focal epileptiform activity is often posterotemporal.
 During sleep marked activation.

14. Electrophysiology and imaging
 Magnetoencephalography:
 Determination of electrical currents in the brain by
measuring their associated magnetic field.
 Structural neuroimaging:
 In children with LKS usually normal.
 In CSWS: cortical dysplasia, congenital stroke, diffuse
atrophy, white matter changes, abnormal or delayed
myelination, tubers and chiri II malformation.

16. Differential Diagnosis
 BCECTS (benign childhood epilepsy with centrotemporal
spikes).
 Lennox Gastaut Syndrome.
 Pervasive Developmental Disorders.
 Developmental Language Disorders.

17. Treatment
 Antiepileptic agents:
 Valproic acid, ethosuximide ,benzodiazepines and
keppra.
 Contraindicated: phenytoin, carbamazepine and
barbiturates.
 High –Dose Benzodiazepines (1mg/kg).

18. Treatment
 Steroids and Adrenocorticotropic Hormone:
 Prednisolone (2-5 mg/kg/d).
 Methylprednisolone (20mg/kg/d x 3 days).
 ACTH (80 IU/d with a 3 months taper).
 Intravenous Gamma-Globulin:
 IVIG (2 g/kg over 4 days).

19. Treatment
 Multiple Subpial Transections.
 Other therapies:
 Ketogenic diet.
 Vagal nerve stimulation.
 Treatment of attention deficit in ESES:
 Methylphenidate.

20. Prognosis
 Seizures:
 Seizures ultimately resolve or markedly decrease in
frequency by puberty.
 Electroencephalographic discharge:
 Improvement occures progressively over time, with initial
reduction in frequency and spread of discharge in
sleepfollowed by normalization of the awake recording
and normalization of the sleep recording.

21. Prognosis
 Neuropsychological outcome:
 10-44% normal language and intelligence.
 One of the most predictive factors for poor long-term
neuropsychological outcome is the duration of ESES.

23. Summary
 CSWS and LKS are uncommon but probably
underrecognized epileptic syndromes that are
associated with ESES.
 If clinically suspected, an adequate sleep EEG with
recording of slow- wave sleepmust be obtained to
confirm this diagnosis.
 Early treatment with effective therapy to ameliorate the
ESES pattern is essential for improvement in
neuropsychological outcome.

24. References