title: Epileptic spasms in epilepsy with myoclonic-atonic seizures (Doose syndrome).
abstract: OBJECTIVE
  To describe the occurrence of epileptic spasms in epilepsy with myoclonic-atonic seizures (EMAS) or Doose syndrome.
  
  
  METHODS
  Case descriptions of patients with EMAS and epileptic spasms. Diagnosis of EMAS was performed according to the following criteria: (1) onset of myoclonic, myoclonic-atonic, or atonic seizures at between 7 months and 6 years of age; (2) normal development before onset of epilepsy; (3) absence of structural cerebral abnormalities on MRI; (4) presence of generalized spike-waves or polyspike-waves on EEG; and (5) exclusion of other myoclonic epilepsies.
  
  
  RESULTS
  Four patients with EMAS were included. For each of them, epileptic spasms were documented by video interpretation, or video-EEG when available.
  
  
  CONCLUSIONS
  Our description of epileptic spasms in four patients with EMAS enlarges the spectrum of seizures that may be observed in this syndrome, as well as the number of epilepsy syndromes which may involve epileptic spasms. This evidence suggests that the presence of epileptic spasms is consistent with a diagnosis of EMAS; epileptic spasms should therefore not be considered a seizure type that excludes diagnosis of this epilepsy syndrome. The prognostic significance of epileptic spasms associated with EMAS remains unknown.
authors: Neurology Department.
authors: Francesca
authors: F
authors: Pittau
authors: Pediatric Neurology Department, University Hospitals, Geneva, Switzerland.
authors: Christian M
authors: CM
authors: Korff
authors: Epilepsy Center, Ann and Robert H. Lurie Children's Hospital of Chicago, Chicago, Illinois, USA.
authors: Douglas R
authors: DR
authors: Nordli
mesh_terms: D000293:Adolescent; D002648:Child; D004569:Electroencephalography; D006801:Humans; D008297:Male; D018908:Muscle Weakness / Q000503:physiopathology*; D009207:Myoclonus / Q000503:physiopathology*; D012640:Seizures / Q000503:physiopathology*; D013577:Syndrome
keywords: Doose syndrome; drop-attacks; epileptic spasms; myoclonic-atonic seizures