Poorly understood complication of some epilepsies.

Clinically, global regression esp cognitive and behavioural, associated with paroxysmal epileptic activity during sleep, which may of course not be recognized.  The classic example is Benign Rolandic Epilepsy, where continuous spike waves develop druing slow wave sleep (CSWS).  Also seen in Landau Kleffner syndrome.

The problem can be unmasked by anti-epileptic medication, especially carbamazepine.  Appears to be associated with brain pathologies eg polygyria, migrational disorders plus some chromosomal problems eg 8p-.

Diagnosis should be considered when unexpected cognitive impairment (eg memory, temperospatial skills, language) or behaviour changes (eg hyperactivity, aggression, disorientation).  Motor impairments eg ataxia and dystonia have been described.

Investigations

Dramatic increase in EEG abnormalities of any kind during sleep.

Treatment

Traditional plus newer AEDs have been used, but evidence does not point to any one being superior to any other.

Steroids appear to be effective – unclear whether ACTH or hydrocortisone better.

Outcome

Poor prognosis (ie long term neuropsychiatric problems) appear to be associated with longer duration of ESES, plus frontal neuropsychological deficits and frontal EEG anomalies.

[Epilepsy Research and Treatment 2012 http://dx.doi.org/10.1155/2012/642725]