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Status Epilepticus Quo: Electrical Status Epilepticus in Sleep

Status Epilepticus Quo: Electrical Status Epilepticus in Sleep. November 10, 2010 Aaron Lane Alexander Nelson, M.D. M.B.S. Overview:. Introduction Background The Four E’s: Epidemiology Evaluation Etiology Evidence Based Medicine Summary. Introduction:. Penelope Syndrome:

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Status Epilepticus Quo: Electrical Status Epilepticus in Sleep

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  1. Status Epilepticus Quo:Electrical StatusEpilepticus in Sleep November 10, 2010 Aaron Lane Alexander Nelson, M.D. M.B.S.

  2. Overview: • Introduction • Background • The Four E’s: • Epidemiology • Evaluation • Etiology • Evidence Based Medicine • Summary

  3. Introduction: Penelope Syndrome: • Penelope, wife of Odysseus in Homer’s Odyssey. • While staving off suitors she pretends every day to weave a burial shroud for Odysseus’ elderly father Laertes. • Every night for 3 years she undoes the weaving accomplished during the day, so she makes no progress. • “What is weaved during the day will be unraveled during the night.” • “Penelope Unraveling Her Web” • Joseph Wright of DerbyEnglish, 1783 - 1784 Oil on canvas http://www.getty.edu Tassinari CA et al, 2009. Encephaloptahy with status epilepticus during slow sleep: “The Penelope syndrome.” Epilepsia 50(Suppl. 7):4-8.

  4. Introduction: Electrical Status Epilepticus in Sleep (ESES): • Initially described by Patry et al in 1971 in a group of 6 children with nearly continuous activation beginning with sleep onset, continuing throughout the night, and resolving when children awakened. • “Continuous” if 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. • Spike wave index = total number of minutes of all spike and slow-wave abnormalities multiplied by 100 and divided by the total nonrapid eye movement sleep minutes. • Various criteria are used for ESES/CSWS: • An SWI of 25% (Van Hirtum-Das et al, 2006) or 60% (Inutsuka et al, 2006). • An SWI of at least 85% (Tassinari et al, 2000). • An SWI of 90% (Rossi et al, 1999). • An SWI of 50% (Beaumanoir et al, 1995). • Others organizations merely mention a strong or significant activation of epileptiform activity during sleep (CCT of the ILAE, 1989). • Felt by many to be clinically relevant only when SWI is > 85%. Nickels K and E Wirrell, 2008. Electrical Status Epilepticus in Sleep. Semin Pediatr Neurol 15:50-60. Scheltens-de Boer M, 2009. Guidelines for EEG in encephalopathy related to ESES/CSWS in children. Epilepsia 50(Suppl. 7):13-17.

  5. Background: There are multiple epilepsy syndromes associated with ESES: • Continuous spike-wave in slow wave sleep (CSWS). • According to the 1989 Proposal for Revised Classification of Epilepsies and Epileptic Syndromes, the syndrome of continuous spike-waves during slow sleep (CSWS) is included in the group of epileptic syndromes whose focal or generalized origin remains undetermined. • The 2001 International League Against Epilepsy (ILAE) Commission Report considers the syndrome an epileptic encephalopathy in which the epileptiform abnormalities may contribute to progressive dysfunction. Nickels K and E Wirrell, 2008. Electrical Status Epilepticus in Sleep. Semin Pediatr Neurol 15:50-60. Paquier PF et al, 2009. Acquired cognitive dysfunction with focal sleep spiking activity. Epilepsia 50(Suppl. 7):29-32.

  6. Background: There are multiple epilepsy syndromes associated with ESES: • Continuous spike-wave in slow wave sleep (CSWS). • Landau-Kleffner syndrome (LKS). • Landau and Kleffner first described an association between loss of language skills and the appearance of focal interictal abnormalities in the temporoparietal regions, often bilateral and asynchronous (Landau & Kleffner, 1957). • The occurrence of acute or subacute language loss in the presence of profoundly abnormal EEG—often with continuous spikewave abnormalities, particularly during non-REM (rapid eye movement) sleep Nickels K and E Wirrell, 2008. Electrical Status Epilepticus in Sleep. Semin Pediatr Neurol 15:50-60. Paquier PF et al, 2009. Acquired cognitive dysfunction with focal sleep spiking activity. Epilepsia 50(Suppl. 7):29-32. Deonna T and E Roulet-Perez, 2010. Early-onset acquired epileptic aphasia (Landau-Kleffner syndrome, LKS) and regressive autistic disorders with epileptic EEG abnormalities: The continuing debate. Brain & Development 32:746-752.

  7. Background: There are multiple epilepsy syndromes associated with ESES: • Since the third edition of the textbook on epileptic syndromes of infancy and childhood, encephalopathy with status epilepticus during sleep (ESES) and CSWS have been considered as synonymous and LKS as a particular presentation of epilepsy with CSWS in which acquired aphasia is the core symptom. • Considered to be along a spectrum of epilepsy syndromes, as they share major characteristics: • Cognitive and/or behavioral impairment acquired during childhood. • Associated with strong activation of the interictalepileptiform discharges during non–rapid eye movement (NREM) sleep. • Not related to a factor other than the presence of CSWS. Nickels K and E Wirrell, 2008. Buzatu M, 2009. Corticosteroids as treatment of epileptic syndromes with continuous spike-waves during slow-wave sleep. Epilepsia 50(Suppl. 7): 68-72. Scheltens-de Boer M, 2009. Guidelines for EEG in encephalopathy related to ESES/CSWS in children. Epilepsia 50(Suppl. 7):13-17.

  8. Epidemiology: CSWS: • ~44 cases described in the medical literature from 1971-1984. LKS: • ~200 cases were described in medical literature from 1968-1992. A 5-year review of 1,497 overnight vEEGs found 102 cases (~7%) of significant sleep activation defined as a spike-wave index of 25% or higher. • 20% of those children met criteria for LKS. • 32% had autistic spectrum disorder with seizures and developmental language issues. Nickels K and E Wirrell, 2008. Electrical Status Epilepticus in Sleep. Semin Pediatr Neurol 15:50-60. Liukkonen E et al, 2010. Long-term outcome of 32 children with encephalopathy with status epilepticus during sleep, or ESES syndrome. Epilepsia 51(10):2023-2032.

  9. Epidemiology: Nickels K and E Wirrell, 2008. Electrical Status Epilepticus in Sleep. Semin Pediatr Neurol 15:50-60. Liukkonen E et al, 2010. Long-term outcome of 32 children with encephalopathy with status epilepticus during sleep, or ESES syndrome. Epilepsia 51(10):2023-2032.

  10. Evaluation: Clinical Features: CSWS: • Regression of Skills: Global regression. • Loss of language (including expressive aphasia as well as difficulty with lexical and syntactic skills) with comprehension generally spared. • Loss of temporospatial skills, hyperactivity, poor reasoning, short-term memory deficits, and aggressiveness. • Motor deficits resulting in ataxia, dystonia, and dyspraxia, which may be unilateral. • Epilepsy: 80% present with seizures, and 93% have multiple seizures daily. • Most common seizure types include generalized tonic-clonic, typical absence, atypical absence, and simple and complex partial seizures. Atonic seizures with falls can occur, but tonic seizures are not seen. • Age of Onset: 1 to 14 years with mean between 4 and 8 years. • Outcome tends to be poorer in children with earlier age at onset. Nickels K and E Wirrell, 2008. Electrical Status Epilepticus in Sleep. Semin Pediatr Neurol 15:50-60.

  11. Evaluation: Clinical Features: LKS: Regression of Skills: Language regression. Acquired auditory agnosia that may progress over weeks to months. May be unable to understand spoken language or appear deaf (in extreme cases sounds such as sirens or ringing of the telephone may not be perceived correctly.) Also see irritability, hyperkinesia, attention-deficit disorder, and autistic-like behavior. Prior to onset majority of children have had normal language development. Epilepsy: Seizures in 70-80% of cases. Seizures typically are infrequent and easily treated. Seizure types include generalized clonic, partial clonic, and atypical absence seizures. Atonic seizures are not typically seen. Age of Onset: 3 to 8 years with peak at 4 to 5 years. Nickels K and E Wirrell, 2008. Electrical Status Epilepticus in Sleep. Semin Pediatr Neurol 15:50-60.

  12. Evaluation: Clinical Features: Autism: Regression of Skills: Autistic regression with epilepsy in ~30% of autism. Qualitative deficits in social interaction. Qualitative deficits language. Association with repetitive behaviors. Disrupted sleep patterns common in autistic regression. Epilepsy: Both seizures and other epileptiform abnormalities. Epileptiform EEG without seizures is seen in ~20% of autistic regression (reports range from 6%-31%). Epileptiform activity is higher in disintegrative disorder (as high as 77%). The EEG findings in autistic regression are typically NOT ESES. Age of Onset: Regression usually starts before 24 months. Onset often after 3 years of age in disintegrative disorder, later onset also associated with higher likelihood of epilepsy. Tuchman, Roberto, 2009. CSWS-related autistic regression versus autistic regression without CSWS. Epilepsia 50(Suppl. 7):18-20.

  13. Evaluation: EEG Findings in ESES: Awake: Usually abnormal, frequently showing paroxysmal foci in the frontotemporal or centrotemporal regions or brief bursts of more diffuse spike-wave activity. 71% of cases with anterior foci have spike-wave indices of 85 to 100 63% of cases with posteriorly located foci have spike-wave indices of 85 to 100. Asleep: Significant activation of epileptiform discharges in non-REM sleep which become either continuous or nearly-continuous. In REM-sleep the continuous pattern disappears with rare bursts of focal discharges or diffuse spike-wave activity seen similar to waking state. While discharges may be described as generalized, they typically arise focally and rapidly propagate within and between hemispheres. Coherence and phase analysis done to estimate interhemispheric small time differences in spike-wave activity showed consistent leading hemispheres in the spike-wave bursts, in keeping with rapid secondary bilateral synchrony. Nickels K and E Wirrell, 2008. Electrical Status Epilepticus in Sleep. Semin Pediatr Neurol 15:50-60.

  14. EEG Findings: CSWS: The characteristic electroencephalographic feature of the syndrome occurs during non-REM (rapid eye movement) sleep, and takes the form of continuous bilateral and diffuse slow spike-waves (SW) that persist through all the slow sleep stages. An SW index of 85–100% calculated during all night sleep electroencephalography (EEG) recordings is regarded as a key feature for a diagnosis of CSWS. Although the EEG pattern of CSWS is typically described as bilateral, markedly asymmetrical SW activity over the two hemispheres has been observed. In rare instances, CSWS in patients with symptomatic epilepsy can occur unilaterally, resulting in worsening of spontaneous movements in the hemicorpus that is contralateral to the CSWS activity. Evaluation: Nickels K and E Wirrell, 2008. Electrical Status Epilepticus in Sleep. Semin Pediatr Neurol 15:50-60. Paquier PF et al, 2009. Acquired cognitive dysfunction with focal sleep spiking activity. Epilepsia 50(Suppl. 7):29-32.

  15. EEG Findings: CSWS: Awake: EEG during wakefulness typically shows epileptiform discharges that are focal, multifocal, or diffuse with frontotemporal or frontocentral predominance. Asleep: During sleep, there is dramatic activation of diffuse epileptiform discharges. LKS: Awake: EEG is variable and may contain focal, multifocal, or generalized epileptic abnormalities (or be normal). Asleep: Marked activation. In 7 children with LKS, long clusters of spike-wave activity were noted maximally over the centrotemporal head regions but spread diffusely making up 40%-90% of the first slow-wave sleep cycle. Evaluation: 9 year-old boy with CSWS (Awake) 6 year-old boy with LKS (Asleep) 6 year-old boy with LKS (Awake) 9 year-old boy with CSWS (Asleep) Nickels K and E Wirrell, 2008. Electrical Status Epilepticus in Sleep. Semin Pediatr Neurol 15:50-60.

  16. EEG Findings: ESES: Evaluation: Holmes, GL et al, 2006. Role of interictal epileptiform abnormalities in cognitive impairment. Epilepsy & Behavior 8:504-515.

  17. EEG Findings: ESES: Evaluation: Van Hirtum-Das et al, 2006. Children with ESES: Variability in the Syndrome. Epilepsy Research 70S:S248-S258.

  18. Evaluation: MEG Findings: Magnetoencephalography determines electrical currents in the brain by measuring associated magnetic fields. Magnetic fields over the scalp are not distorted by impedence between the scalp surface and brain (as opposed to EEG). Often used for further localization prior to surgery. Paetau et al studied 4 right-handed children with LKS and found that the intrasylvian cortex was the likely pacemaker of epileptic discharges. Sobel et al studied 19 children and found perisylvian spikes in 13 children (10 bilateral and 3 unilateral). Of the remaining 6 children, 4 had spikes outside the perisylvian region. 2 had no spikes recorded. Nickels K and E Wirrell, 2008. Electrical Status Epilepticus in Sleep. Semin Pediatr Neurol 15:50-60.

  19. Evaluation: Structural Neuroimaging: CSWS: MRI abnormalities documented in literature include cortical dysplasia, congenital strokes, diffuse atrophy, white-matter changes, abnorma/delayed myelination, tubers, and Chiari II malformations. Abnormalities found in 33 of 56 children with ESES who did not meet criteria for LKS. ~25% of children with CSWS have hemiplegia, typically congenital with associated underlying neuroimaging findings. LKS: Structural imaging is typically normal in children with LKS. Nickels K and E Wirrell, 2008. Electrical Status Epilepticus in Sleep. Semin Pediatr Neurol 15:50-60. Van Hirtum-Das et al, 2006. Children with ESES: Variability in the Syndrome. Epilepsy Research 70S:S248-S258.

  20. Evaluation: Functional Neuroimaging: CSWS: Gaggero et al studied 10 children with CSWS using single photon emission CT scans performed during drowsiness. Reduced blood flow was noted in 6/10. Of those, 4/6 this correllated with the site of the most prevalent EEG discharges. Longer duration CSWS increases likelihood of abnormal studies. De Tiege et al performed PET in 18 children with CSWS with voxel-based analysis. Hypermetabolism may mark an active phase of epilepsy with evolution to focal hypometabolism or normal metabolism occurring over time in recovery. 10/18 childrewn had hypermetabolism in the parietotemporal junction, which was associated with hypometabolism in the frontal region. Frontal lobe inhibitaion secondary to posterior epileptic brain regions could explain ADD as well as other executive dysfunction seen in CSWS. Absence of a hypermetabolic cerebral region was associated with corticosteroid treatment prior to the study. Maquet et al showed unilateral foci (increased in glucose metabolism) in 5/6 patients with active CSWS. In the same patient group 4/6 subsequently showed focal decreased cortical glucose metabolism following resolution with treatment. Nickels K and E Wirrell, 2008. Electrical Status Epilepticus in Sleep. Semin Pediatr Neurol 15:50-60. Paquier PF et al, 2009. Acquired cognitive dysfunction with focal sleep spiking activity. Epilepsia 50(Suppl. 7):29-32.

  21. Evaluation: Functional Neuroimaging: LKS: Stefano S et al, 2009. Neurophysiology of CSWS-associated cognitive dysfunction. Epilepsia 50(Suppl. 7):33-36.

  22. Evaluation: Genetics: There are multiple identified genes associated with other epilepsy syndromes, including BECTS. There is a rarity of familial cases of ESES, but recently two families were characterized with coexistent BECTS and cryptogenic ESES in first-degree relatives suggesting a possible common genetic basis. Deonna T and E Roulet-Perez, 2010. Early-onset acquired epileptic aphasia (Landau-Kleffner syndrome, LKS) and regressive autistic disorders with epileptic EEG abnormalities: The continuing debate. Brain & Development 32:746-752. Tassinari CA et al, 2009. Encephaloptahy with status epilepticus during slow sleep: “The Penelope syndrome.” Epilepsia 50(Suppl. 7):4-8.

  23. Evaluation: Differential Diagnosis: Benign childhood epilepsy with centrotemporal spikes (BCECTS). Lennox-Gastaut syndrome. Pervasive developmental delay. Autistic regression with and without epileptiform EEG. Disintegrative disorder. Other developmental language disorders. Mental retardation. Nickels K and E Wirrell, 2008. Electrical Status Epilepticus in Sleep. Semin Pediatr Neurol 15:50-60.

  24. Etiology: Electrophysiologic Mechanisms: A single primary focus with secondary generalization. Theoretical Etiologies: • Epileptiform discharges initially caused by activation of the reticulo-thalamic-cortical system with secondary bilateral synchronization through the corpus callosum. (De Negri) • Consistent with the significant activation of epileptiform activity during sleep. • Cats with bilateral thalamic lesions have bilateral synchrony when the suprasylvian cortex is stimulated. • Cats with unilateral (or no) thalamic lesions did not. • Aberrant Synaptogenesis: • 4-8 years of age (mean age of onset for both CSWS and LKS) is a time critical for synaptogenesis in the brain. • With abnormal neuronal activity this could theoretically lead to abnormal synapse formation (kindling). • Would account for the observed fact that prognosis for neuropsychological outcome is dependent on the duration of ESES. Nickels K and E Wirrell, 2008. Electrical Status Epilepticus in Sleep. Semin Pediatr Neurol 15:50-60.

  25. Etiology: Electrophysiologic Mechanisms: A single primary focus with secondary generalization. Theoretical Etiologies: • Distant inhibition effects of neurons that display epileptiform discharges might involve tonic hyperpolarization of afferent neurons or low-energy requiring c-aminobutyric acid (GABA) transmission (Bruehl & Witte, 1995, Chattonet al, 2003). • The existence of surrounding and remote inhibition phenomena has been well documented in different types of animal models of focal epilepsy using various functional cerebral imaging methods such as autoradiography or optical imaging (Witte & Bruehl, 1999). De Tiege X, 2009. Insights into the pathophysiology of psychomotor regression in CSWS syndromes from FDG-PET and EEG-fMRI. Epilepsia, 50(Suppl. 7):47-50.

  26. Etiology: Comorbid Conditions: CSWS: While the majority are normal, ~1/3 of children have a preceding neurologic insult such as meningitis or neonatal encephalopathy. Radiologic correllates include atrophy and cortical migrational anomalies. Minimal evidence of specific underlying genetic etiologies. Genetic linkage analyses difficult given low numbers of patients. 1 pair of monozygotic twins with CSWS has been reported. Family history of seizures (febrile seizures) in up to 15% of cases. LKS: Idiopathic LKS often initially presents with focal seizures suggestive of benign rolandic epilepsy before onset of ESES. Both: Idiopathic ESES in some cases may have an immunologic basis as evidenced by some success with modulatory agents including steroids and IVIG. Nickels K and E Wirrell, 2008. Electrical Status Epilepticus in Sleep. Semin Pediatr Neurol 15:50-60.

  27. Etiology: Differential Distinctions: Regression in CSWS is more global versus language regression in LKS. Language regression in CSWS consists of an expressive aphasia with difficulties in lexical and syntactic skills; in LKS issues is primarily an auditory agnosia. EEG findings differ in location. In CSWS EEG focal abnormalities are more likely to be frontal to frontotemporal while in LKS they are more likely to be temporal to posterotemporal (on SPECT as well as EEG) which would be consistent with the more primary language involvement. Is the clinical difference mostly due to location? Nickels K and E Wirrell, 2008. Electrical Status Epilepticus in Sleep. Semin Pediatr Neurol 15:50-60. Van Hirtum-Das et al, 2006. Children with ESES: Variability in the Syndrome. Epilepsy Research 70S:S248-S258.

  28. Evidence Based Medicine: Treatment: Standard Antiepileptic Management: • Recommended Medications: • Valproic acid, ethosuximide, and benzodiazepines have been reportedly beneficial in multiple case series. • Levetiracetam reduced amplitude and spread of EEG discharges in 7 of 12 children (58%) with ESES (though spike-wave index reduction was seen in only 25%) and correlated with improved neuropsychological function. • Sulthiame, very efficacious in benign rolandic epilepsy of childhood, has been reportedly helpful in ESES with reports of individual cases of marked improvement or resolution of epileptiform discharges. • Contraindicated Medications: • Phenytoin, carbamazepine, and barbiturates all can reduce seizures in the short term but are contraindicated in ESES due to worse neuropsychological outcomes. • Carbamazepine has actually precipitated CSWS in documented/reported cases and can maintain abnormal EEG patterns. • Polypharmacy should be avoided. In a study of 88 patients with ESES, reduction of polytherapy coincided with an improvement in the syndrome. Nickels K and E Wirrell, 2008. Electrical Status Epilepticus in Sleep. Semin Pediatr Neurol 15:50-60.

  29. Evidence Based Medicine: Treatment: High-Dose Benzodiazepines: A single dose of rectal diazepam (1mg/kg) was initially given to 16 children with CSWS or Landau-Kleffner syndrome with all showing significant improvement on EEG. Subsequently treated with 3-4 weeks of diazepam (0.50-0.75mg/kg/day) with a positive response in 14 (88%). 5 (31%) ultimately relapsed and required further cycles of diazepam. When neuropsychological testing was done at 6 months, 70% had improving or resolved symptoms, 10% had no change, and 20% had worsening neuropsychiatric symptoms. In some cases children may display problematic disinhibition. Nickels K and E Wirrell, 2008. Electrical Status Epilepticus in Sleep. Semin Pediatr Neurol 15:50-60.

  30. Evidence Based Medicine: Treatment: Ketogenic Diet: In a study of 5 children with CSWS there was no significant improvement either clinically or on EEG. In 3/3 children with LKS refractory to traditional therapy Bergqvistet al reported improvement in language, behavior, and seizures. Vagal Nerve Stimulator: Only a single retrospective with minimal EEG/neuropsychological data exists which reports improvement in ~50% of treated children at 6 months with improved seizure frequency and “quality of life”. Nickels K and E Wirrell, 2008. Electrical Status Epilepticus in Sleep. Semin Pediatr Neurol 15:50-60. Nikanorova M, 2009. Ketogenic diet in the treatment of refractory continuous spikes and waves during slow sleep. Epilepsia 50(5):1127-1131.

  31. Treatment: Steroids/ACTH: In a trial with corticosteroids to treat CSWS 41 patients complete control of seizures was achieved in 33 patients. Positive response to steroids was found during the 3 months of treatment in 34/44 patients (77%). Normalization of EEG seen in 21 patients (48%). Relapse occurred in 14/44 (32%). 20 patients (45%) were therefore long-term responders after a single but prolonged corticosteroid trial. Follow-up studies showed both steroids or ACTH may also be effective in improving both EEG abnormalities in neuropsychologicl function in LKS. Prednisone typically dosed at 2-5mg/kg/day. Methylprednisolone typically dosed at 20 mg/kg/day for 3 days. ACTH is given at 80IU/day with a subsequent 3-month taper. IVIG: Isolated case reports suggest IVIG may be beneficial in ESES (Mikatiet al). 5 children with newly diagnosed LKS given IVIG 2g/kg over 4 days with 2/5 (40%) responding with resolution of severe language and EEG abnormalities. Evidence Based Medicine: Nickels K and E Wirrell, 2008. Electrical Status Epilepticus in Sleep. Semin Pediatr Neurol 15:50-60. Arts WFM et al, 2009. Landau-Kleffner syndrome and CSWS syndrome: Treatment with intravenous immunoglobulins. Epilepsia 50(Suppl. 7):55-58. Buzatu M, 2009. Corticosteroids as treatment of epileptic syndromes with continuous spike-waves during slow-wave sleep. Epilepsia 50(Suppl. 7): 68-72.

  32. Evidence Based Medicine: Treatment: Surgery: Difficult in patients with LKS due to eloquent areas. Subpial transections may be an option in refractory situations. Techingue involved parallel slices through the cortex to disrupt horizontal cortical synchronizing networks while leaving subcortical-cortical input intact. In a series of 14 LKS patients (refractory to AEDs and steroids) by Morrell et al 7 (50%) regained age-appropriatespeech, and 4 more (29%) showed significant improvement; however in another series of 4 patients by Blount et al only 1 patient (25% showed improvement). A series of 5 ESES patients in the UK showed all 5 patients had significant clinical improvement following surgery. Those with longer duration of seizures prior to surgical intervention tend to have a worse likelihood of good outcome. Metanalysis of 211 patients who underwent multiple subpial transections with or without resection for intractable partial epilepsy found no new language or sensory deficits after the procedure; in one series specifically in the language cortex postoperative dysphasia occurred in 6/10 (60%) but resolved in all by 6 weeks post-operatively. 8 year-old girl with LKS pre- and post-operatively. Nickels K and E Wirrell, 2008. Electrical Status Epilepticus in Sleep. Semin Pediatr Neurol 15:50-60. Van Hirtum-Das et al, 2006. Children with ESES: Variability in the Syndrome. Epilepsy Research 70S:S248-S258.

  33. Evidence Based Medicine: Treatment: Surgery: Difficult in patients with LKS due to eloquent areas. More feasible in patients with other focal causes of ESES: Loddenkemper T, 2009. Epilepsy surgery in children with electrical status epilepticus in sleep. Neurosurgery 62:328-337.

  34. Evidence Based Medicine: Treatment: Behavior: Poor attention and hyperactivity commonly seen. Case series of 5 children with ESES meeting criteria for ADD by Finck et al were proscribed methylphenidate, mostly successfully: 3/5 (60%) were treated during active continuous spike-waves while 2/5 (40%) were treated after resolution of continuous spike-waves. In 3/5 (60%) methylphenidate was very effective. 1/5 (20%) showed improved hyperactivity and attention but less efficacy for impulsivity. 1/5 (20%) with attention deficit without hyperactivity worsened showing apathy, depression, and insomnia. Don’t forget to treat behavioral symptoms while working to control the underlying etiology! Nickels K and E Wirrell, 2008. Electrical Status Epilepticus in Sleep. Semin Pediatr Neurol 15:50-60.

  35. Evidence Based Medicine: Prognosis with Treatment: EEG/Seizures: CSWS: • Exacerbation of both partial and multiple types of generalized seizures occurs at the time of ESES. • In addition to focal motor or generalized tonic-clonic seizures, absences, and atonic seizures, which often lead to falls are seen. • A series of 26 cases of ESES done by Tassinari et al showed initially severe epilepsy markedly decreasing or resolving by puberty, even in cases with symptomatic causes. • 31% experienced resolution of seizures before resolution of ESES. • 31% had resolution of seizures and ESES simultaneously. • 38% had rare seizures persisting after ESES resolved. • A series of 9 patients with ESES and multilobar polymicrogyria done by Guerrini et al, showed resolution of all seizures by age 12. LKS: • Seizures in LKS are usually infrequent and nocturnal ESES: • ESES is self-limited and typically resolves by puberty. • Electroencephalographic improvement occurs progressively over time with initial reduction in frequency and spread of discharges in sleep followed by normalization of the awake recording and ultimately normalization of the sleep recording. Nickels K and E Wirrell, 2008. Electrical Status Epilepticus in Sleep. Semin Pediatr Neurol 15:50-60.

  36. Evidence Based Medicine: Prognosis with Treatment: Neuropsychiatric Outcome: Nickels K and E Wirrell, 2008. Electrical Status Epilepticus in Sleep. Semin Pediatr Neurol 15:50-60.

  37. Evidence Based Medicine: Prognosis with Treatment: Cognitive Outcome: Liukkonen E et al, 2010. Long-term outcome of 32 children with encephalopathy with status epilepticus during sleep, or ESES syndrome. Epilepsia 51(10):2023-2032.

  38. Summary: • Introduction • Background • The Four E’s: • Epidemiology • Evaluation • Etiology • Evidence Based Medicine • Summary

  39. Sources Thanks! • Arts WFM et al, 2009. Landau-Kleffner syndrome and CSWS syndrome: Treatment with intravenous immunoglobulins. Epilepsia 50(Suppl. 7):55-58. • Buzatu M, 2009. Corticosteroids as treatment of epileptic syndromes with continuous spike-waves during slow-wave sleep. Epilepsia 50(Suppl. 7): 68-72. • De Tiege X, 2009. Insights into the pathophysiology of psychomotor regression in CSWS syndromes from FDG-PET and EEG-fMRI. Epilepsia 50(Suppl. 7):47-50. • Deonna T and E Roulet-Perez, 2010. Early-onset acquired epileptic aphasia (Landau-Kleffner syndrome, LKS) and regressive autistic disorders with epileptic EEG abnormalities: The continuing debate. Brain & Development 32:746-752. • Holmes, GL et al, 2006. Role of interictal epileptiform abnormalities in cognitive impairment. Epilepsy & Behavior 8:504-515. • Liukkonen E et al, 2010. Long-term outcome of 32 children with encephalopathy with status epilepticus during sleep, or ESES syndrome. Epilepsia 51(10):2023-2032. • Loddenkemper T, 2009. Epilepsy surgery in children with electrical status epilepticus in sleep. Neurosurgery 62:328-337. • Nickels K and E Wirrell, 2008. Electrical Status Epilepticus in Sleep. Semin Pediatr Neurol 15:50-60. • Nikanorova M, 2009. Ketogenic diet in the treatment of refractory continuous spikes and waves during slow sleep. Epilepsia 50(5):1127-1131. • Paquier PF et al, 2009. Acquired cognitive dysfunction with focal sleep spiking activity. Epilepsia 50(Suppl. 7):29-32. • Scheltens-de Boer M, 2009. Guidelines for EEG in encephalopathy related to ESES/CSWS in children. Epilepsia 50(Suppl. 7):13-17. • Stefano S et al, 2009. Neurophysiology of CSWS-associated cognitive dysfunction. Epilepsia 50(Suppl. 7):33-36. • Tassinari CA et al, 2009. Encephaloptahy with status epilepticus during slow sleep: “The Penelope syndrome.” Epilepsia 50(Suppl. 7):4-8. • Tuchman, Roberto, 2009. CSWS-related autistic regression versus autistic regression without CSWS. Epilepsia 50(Suppl. 7):18-20. • Van Hirtum-Das et al, 2006. Children with ESES: Variability in the Syndrome. Epilepsy Research 70S:S248-S258. Questions?

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