Thursday, September 22, 2011

ESES Research Articles

February 2009 - Volume 64 - Issue 2 - p 328-337
doi: 10.1227/01.NEU.0000336767.14252.76
Clinical Studies
Epilepsy Surgery in Children With Electrical Status Epilepticus in Sleep
Loddenkemper, Tobias M.D.; Cosmo, Gary R. EEG T.; Kotagal, Prakash M.D.; Haut, Jennifer Ph.D.; Klaas, Patricia Ph.D.; Gupta, Ajay M.D.; Lachhwani, Deepak K. M.D.; Bingaman, William M.D.; Wyllie, Elaine M.D.
OBJECTIVE: Pediatric epilepsy surgery candidates with unilateral congenital or early-acquired brain lesions may present with refractory seizures and generalized electroencephalographic features such as electrical status epilepticus in sleep (ESES). The purpose of our study was to review the clinical presentation, neuroimaging findings, and outcome in a series of children with unilateral brain lesions and ESES undergoing resective surgery for refractory epilepsy.
METHODS: A total of 415 consecutive patients younger than 18 years of age undergoing video electroencephalographic evaluation and epilepsy surgery at Cleveland Clinic were reviewed for ESES, an underlying pathological lesion, and outcome after surgery.
RESULTS: Eight patients were included. All patients presented with medically refractory epilepsy, hemiparesis, and developmental delay. The pathogenesis was perinatal infarction in 7 patients and malformation of cortical development in 1 patient. Preoperative electroencephalography demonstrated generalized interictal spikes, electroencephalographic seizures, and ESES in all cases. Age at the time of surgery ranged from 3 to 14 years. Six patients underwent hemispherectomy, and 2 patients underwent focal resection. Six patients became seizure-free after resection. Two patients with functional hemispherectomy continued to have rare seizures, but were much improved. These patients also had perinatal infarctions in the hemisphere contralateral to the resection, possibly indicating a less beneficial outcome. Postoperative electroencephalography demonstrated resolution of generalized interictal discharges and ESES in all. Formal pre- and postoperative neuropsychological testing showed overall improvement of age-equivalent scores.
CONCLUSION: Children with unilateral brain lesions and seizures may become seizure-free after epilepsy surgery, even if the preoperative electroencephalogram shows generalized ESES. The lesion occurring early in life and the location of the lesion may play a role in the development of ESES. Cognitive impairment may be aggravated by the persistence of ESES. Preliminary developmental data in this small sample suggest that termination of seizures and possibly of ESES by epilepsy surgery may have developmental benefits.

The treatment of epilepsy: principles & practice

 By Elaine Wyllie, Ajay Gupta, Deepak K. Lachhwani 

Children with BECTS have an impaired ability to process verbal information. The deficiency is apparently a result of the pathological electrical discharges that are part of the syndrome

Neuropsychological deterioration. There is a constant and severe deterioration in neuropsychological functions associated with the disorder, and language capacity can be particularly affected. Patients also may show a profound decrease in intellectual level, poor memory, impaired temporospatial orientation, reduced attention span, hyperkinesis, aggressive behavior, and even psychosis (Jayakar and Seshia 1991; Tassinari et al 1992). 

The typical EEG changes appear 1 year to 2 years after the first seizure and are associated with behavioral deterioration. Focal and generalized interictal spikes occur before this time and persist during wakefulness and REM sleep after the appearance of continuous spike waves during slow wave sleep.

The cause of electrical status epilepticus during slow sleep is unknown.


No information is available.

A deterioration of language is observed in cases showing the predominance of paroxysmal abnormalities over one or both temporal regions (Billard et al 1982), whereas a mental deterioration and an autistic behaviour evoking a frontal lobe syndrome has been described in children exhibiting interictal frontal foci or clear cut anterior predominance of the discharges (Roulet Perez et al 1995).

Therefore, although electrical status epilepticus during slow sleep is currently classified among the epilepsies undetermined whether focal or generalized, consistent data support the view that this syndrome is to be included in the domain of localization-related epilepsies, of cryptogenic or symptomatic nature.


Electrical status epilepticus during slow sleep is a rare disorder. One study revealed an incidence of 0.5% among 12,854 children evaluated during a 10-year period (Morikawa et al 1989). There is no obvious gender preponderance (Tassinari et al 1985).

The condition is rare and controlled clinical trials are very difficult to perform, so the reports about efficacy of different drugs are anecdotal.

Acquired epileptic aphasia. The distinction between electrical status epilepticus during slow sleep and the Landau-Kleffner syndrome or acquired epileptic aphasia is disputed. All-night EEGs reveal that many children diagnosed with the Landau-Kleffner disorder actually have the electrical status epilepticus during slow sleep. In Landau-Kleffner syndrome, however, acquired aphasia is the most predominant neuropsychological symptom; bilateral temporal EEG spikes are present but epileptic seizures do not always occur. In our opinion, electrical status epilepticus during slow sleep and Landau Kleffner syndrome are two facets of a same entity, in which the type of neuropsychological dysfunction depends on the location (frontal in continuous spike and waves during slow wave sleep and temporal in Landau Kleffner syndrome) of interictal foci (Tassinari 1995; De Negri 1997).


The seizures in electrical status epilepticus during slow sleep are self-limited and disappear in the mid-teens. The good seizure outcome is independent of the etiology and is observed also in cases with cortical malformations such as multilobar polymicrogyria (Guerrini et al 1998). The characteristic EEG patterns during slow wave sleep also disappear at approximately the same time, but focal interictal spikes may persist (Morikawa et al 1989; Bureau 1995). Improvement in language dysfunction, mental retardation, and psychiatric disturbances generally occurs but is variable and individualized. The majority of affected children never return to normal levels, particularly in the verbal area and attention (Roulet Perez et al 1993; Morikawa et al 1985).

Neuropsychological development. Normal neuropsychological development at the beginning of the disease is the norm in 60% to 75% of cases (Dalla Bernardina et al 1989; Morikawa et al 1989; 1995; Tassinari et al 1992b).

  Rapid cognitive and behavioral deterioration is followed after the appearance of electrical status epilepticus during slow sleep, with deterioration of IQ, language, cognitive functions, and behavior. Patients can show a profound decrease in intellectual level, poor memory, impaired temporospatial orientation, reduced attention span, hyperkinesis, aggressive behavior, and even psychosis (Jayakar and Seshia 1991; Tassinari et al 1992b). Mental and behavioral deterioration evoking a frontal lobe syndrome has been described in children with spikes or spike-waves discharges in the frontal lobe (Roulet Perez et al 1993) that can even persist after resolution of electrical status epilepticus during slow sleep in the EEG (Veggiotti et al 2001). A comprehensive study of language development in 10 right-handed children and adolescents with continuous spike-and-wave sleep syndrome was reported. Severe language impairments in lexical and syntactic skills were found. Their language profile was different from that observed in Landau Kleffner syndrome (Debiais et al 2007). Longer duration of electrical status epilepticus during sleep appears to be the major predictor of poor outcome, and this might be explained by aberrant synapse formation as a result of abnormal neuronal activity during a critical period for synaptogenesis (Nickels and Wirrell 2008).

Seizure outcome after epilepsy surgery in children and adolescents

1.     Dr. Elaine Wyllie MD1,*,

2.     Youssef G. Comair MD2,

3.     Prakash Kotagal MD1,

4.     Juan Bulacio MD1,

5.     William Bingaman MD2,

6.     Paul Ruggieri MD3

Article first published online: 8 OCT 2004

DOI: 10.1002/ana.410440507

Few epilepsy surgery outcome data are available from series of pediatric patients. We studied seizure outcome in 136 pediatric patients who had surgery for intractable epilepsy at The Cleveland Clinic between January 1990 and June 1996, with a postoperative follow-up of 1 to 7.5 years (mean, 3.6 years). Sixty-two children (3 months to 12 years old at time of surgery) were compared with 74 adolescents (13–20 years old). Extratemporal or multilobar resections and hemispherectomies were similarly frequent among children (50%) and adolescents (44%), but these procedures strongly predominated in infancy (90% of patients 0–2 years of age). The remaining patients had temporal resection. Cortical dysplasia and low-grade tumor were the most common causes and hippocampal sclerosis was rare. Seizure-free outcome was achieved for 69% of adolescents, 68% of children, and 60% of the infant subgroup, overall; for 23 (74%) of 31 children and 33 (80%) of 41 adolescents after temporal resection; for 11 (58%) of 19 children and 15 (52%) of 29 adolescents after extratemporal or multilobar resection; and for 8 (67%) of 12 children and 3 (75%) of 4 adolescents after functional hemispherectomy. Seizure-free outcome was more frequent after temporal resection (56 of 72, 78%) than after extratemporal or multilobar resection (26 of 48, 54%; 41 of 48 with a focal lesion on magnetic resonance imaging), and among patients with tumor (36 of 44, 82%) versus cortical dysplasia (16 of 31, 52%). The frequency of seizure-free outcome after epilepsy surgery was similar for infants, children, and adolescents, and comparable with results from adult series. Most patients in each age, surgery type, and causal group were free from seizures after surgery. These results suggest that children should be considered for surgical evaluation at whatever age they manifest with severe, intractable, disabling localization-related epilepsy.

1 comment:

TherExtras said...

Slightly daunting reading, Amanda, but worth the attempt.