doose syndrome
Pronunciation: DOH-zeh
An epilepsy syndrome characterized by myoclonic-astatic seizures in previously normal children with genetic predisposition.
Full Definition
Doose syndrome, also known as myoclonic-astatic epilepsy (MAE), is a genetic generalized epilepsy syndrome that typically begins between 7 months and 6 years of age in previously normal children. The syndrome is characterized by myoclonic-astatic seizures (drop attacks), often preceded by myoclonic jerks, and may include other seizure types such as absence and tonic-clonic seizures. There is typically a strong family history of epilepsy or febrile seizures. The prognosis varies widely, with some children achieving seizure freedom while others develop cognitive impairment and refractory epilepsy.
Usage
Usage note: Named after Hermann Doose; capitalize when referring to the eponymous syndrome.
In Context
- "The combination of myoclonic-astatic seizures and family history suggested Doose syndrome." — Epilepsy clinic assessment
- "Genetic testing was ordered to confirm the suspected diagnosis of Doose syndrome." — Clinical genetics referral