Syndrome of electrical status epilepticus during slow wave sleep ESES

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ESES (continuous spike-wave of slow sleep [CWES]; Table 1.22) refers to an epileptic encephalopathy characterized by the presence of generalized 1-3 Hz spike-wave discharges occupying 85% or more of the EEG of non-REM sleep. Of children with epilepsy, 0.5% show this EEG pattern. The condition is diagnosed during childhood (1-14 years) with a peak age of onset between 3 and 5 years. About 30% of children showing this pattern have identifiable brain pathology such as previous meningitis or brain anoxia, hydrocephalus and developmental lesions, and one pair of affected monozygous twins has also been reported. There are no specific clinical signs during sleep. Overt seizures occur in daytime and at night, and can take various forms, both focal and generalized. Episodes of status are common. The EEG pattern usually occurs in children with severe epilepsy and learning difficulty. Furthermore, many children exhibit the symptoms of the Landau-Kleffner syndrome, and some authorities consider the two conditions to be synonymous. However, ESES is also seen in cases of the Lennox-Gastaut syndrome and in some cases of BECTS. Indeed, whether this is a specific epileptic syndrome or simply a reflection of severe epilepsy is uncertain. ESES is a largely childhood phenomenon, and the EEG pattern usually disappears by the age of 16 years.

The EEG pattern is usually resistant to conventional antiepileptic therapy, and often long-term corticosteroids or ACTH are recommended, albeit without any clear evidence of efficacy. Intravenous immunoglobulin therapy has been used also. Oral antiepileptic drugs are given to control

Table 1.22 Electrical status epilepticus during slow wave sleep (ESES).

Childhood epileptic encephalopathy Age of onset 1-14 years 0.5% of children with epilepsy

EEG shows continuous epileptic activity during non-REM sleep (> 85% of time)

Overt seizures occur during wakefulness and sleep Learning disability present in most cases 30% of children have an identifiable cause Overlap with Landau-Kleffner and BECTS Epilepsy often remits by age of 16 years seizures. Any first-line antiepileptic can be used and therapy follows conventional lines, although carbamazepine can exacerbate the nocturnal EEG disturbance. The EEG disturbance and seizures remit by the mid-teens. Cognition improves but most children do not gain normal functioning, especially in relation to speech and attention.

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