Epilepsy with myoclonic absences (EMA) is characterized clinically by rhythmic and bilateral jerks of the limbs of severe intensity, often associated with a progressive tonic contraction. The diagnosis is based on clinical observation and ictal polygraphic and video-EEG recording. The ictal EEG shows bilateral, synchronous and symmetrical spike and wave discharges repeated at 3 Hz. The age of onset of seizures is usually between 3 and 12 years, with a peak at around 5 to 7 years of age. The mental development of children with EMA is usually slow, and the long-term prognosis is poor.
Category
Nervous system disease
Brite
Human diseases in ICD-11 classification [BR:br08403]
08 Diseases of the nervous system
Epilepsy or seizures
8A61 Genetic or presumed genetic syndromes primarily expressed as epilepsy
H01822 Epilepsy with myoclonic absence
Comment
See also H00808 Idiopathic generalized epilepsies.
