title: Epilepsy with myoclonic atonic seizures: an electroclinical study of 69 patients.
abstract: Epilepsy with myoclonic-atonic seizures is characterized by myoclonic-atonic, absence, tonic-clonic, and eventually tonic seizures, appearing in previously normal children at ages 18-60 months. We analyzed the electroclinical features, treatment, and outcome of 69 patients with myoclonic-atonic seizures; these patients were followed between 1990 and 2012 at the Juan P. Garrahan Pediatric Hospital, Buenos Aires, Argentina. No structural or metabolic etiology was identified. Based on the electroclinical features and evolution, two groups could be distinguished. The first group of 39 patients with myoclonic and myoclonic-atonic seizures with or without generalized tonic-clonic seizures and absences associated with generalized spike- and polyspike-and-wave paroxysms had excellent prognoses. The second group of 30 patients had myoclonic jerks and myoclonic-atonic seizures associated with other seizure types including tonic seizures; some had myoclonic status epilepticus and cognitive deterioration. The interictal EEG showed frequent generalized spike- and polyspike-and-wave paroxysms. In 16 patients, the seizures remitted within 3.6 years. The two groups were distinguished in retrospect, when enough time had elapsed to evaluate cognitive deterioration and different seizure types. In conclusion, epilepsy with myoclonic atonic seizures is an epileptic syndrome with a broad clinical spectrum and variable prognosis.
authors.affiliation: Servicio de Neurologia, Hospital de Pediatría Prof Dr Juan P Garrahan, Buenos Aires, Argentina. rhcaraballo@arnet.com.ar
authors.forename: Roberto H
authors.initials: RH
authors.lastname: Caraballo
authors.forename: Noelia
authors.initials: N
authors.lastname: Chamorro
authors.forename: Francesca
authors.initials: F
authors.lastname: Darra
authors.forename: Sebastian
authors.initials: S
authors.lastname: Fortini
authors.forename: Hugo
authors.initials: H
authors.lastname: Arroyo
mesh_terms: D017668:Age of Onset; D000927:Anticonvulsants / Q000627:therapeutic use; D002675:Child, Preschool; D055423:Diet, Ketogenic; D004569:Electroencephalography; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D020190:Myoclonic Epilepsy, Juvenile / Q000188:drug therapy / Q000378:metabolism / Q000503:physiopathology*; D059906:Neuroimaging; D009460:Neurologic Examination; D011379:Prognosis; D012189:Retrospective Studies; D012640:Seizures / Q000503:physiopathology*; D003294:Seizures, Febrile / Q000503:physiopathology; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D055536:Vagus Nerve Stimulation
chemical_list: D000927:Anticonvulsants