| H01822 | |
| H number | H01822 |
| Name | Epilepsy with myoclonic absence |
| Description | 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 |
| Network | - |
| Gene | - |
| Pathogen | - |
| Env factor | - |
| Carcinogen | - |
| Drug | - |
| Comment | See also H00808 Idiopathic generalized epilepsies. |
| Other DBs | ICD-11: 8A61.23 ICD-10: G40.3 MeSH: D004831 |
| Reference | PMID:15737698 AUTHORS Bureau M, Tassinari CA TITLE Epilepsy with myoclonic absences. JOURNAL Brain Dev 27:178-84 (2005) DOI:10.1016/j.braindev.2004.01.008 PMID:27770719 AUTHORS Zanzmera P, Menon RN, Karkare K, Soni H, Jagtap S, Radhakrishnan A TITLE Epilepsy with myoclonic absences: Electroclinical characteristics in a distinctive pediatric epilepsy phenotype. JOURNAL Epilepsy Behav 64:242-247 (2016) DOI:10.1016/j.yebeh.2016.08.023 |