H01822 | |
H番号 | H01822 |
名称 | ミオクロニー欠神てんかん |
概要 | 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. |
カテゴリ | 神経系疾患 |
ネットワーク | - |
病因遺伝子 | - |
病原体 | - |
環境要因 | - |
発癌物質 | - |
治療薬 | - |
コメント | See also H00808 Idiopathic generalized epilepsies. |
リンク | ICD-11: 8A61.23 ICD-10: G40.3 MeSH: D004831 |
文献 | PMID:15737698 著者 Bureau M, Tassinari CA タイトル Epilepsy with myoclonic absences. 雑誌 Brain Dev 27:178-84 (2005) DOI:10.1016/j.braindev.2004.01.008 PMID:27770719 著者 Zanzmera P, Menon RN, Karkare K, Soni H, Jagtap S, Radhakrishnan A タイトル Epilepsy with myoclonic absences: Electroclinical characteristics in a distinctive pediatric epilepsy phenotype. 雑誌 Epilepsy Behav 64:242-247 (2016) DOI:10.1016/j.yebeh.2016.08.023 |