title: [The syndrome of malignant migrating partial seizures in infancy or Coppola-Dulac syndrome (19 cases)].
abstract: Malignant migrating partial seizures in infancy (MMPSI) or Coppola-Dulac syndrome is a rare epilepsy syndrome with the onset in the first 6 months of life, characterized by multiple continuous electroencephalographic and electroclinical focal ictal patterns due to the involvement of different independent areas of both hemispheres with the arrest of psychomotor development. This article is based on the personal observations of 19 cases. Four subtypes of the syndrome were determined in our population of infants with MMPSI (n=19): 1) a "classic" form with pharmacoresistant migrated status epilepticus (SE) of migrating multifocal seizures, and with absolutely poor prognosis (n=7); 2) a severe pharmacoresistant mixed form (MMPSI + EME) with the combination of electroclinical characteristics of MMPSI with migrating multifocal SE and early myoclonic encephalopathy (n=5); 3) a "mild" variant (n=5); 4) a "subtle" form (n=2). In basic therapy, drugs of choice are antiepileptic drugs (AEDs) with a wide range of action (valproates, in the combination with barbiturates, benzodiazepines and levetiracetam) and also very old drugs as bromides. The preferred drugs are the valproate forms available for patients with disturbances of swallowing (depakine chronosphere or depakine syrup).
authors.forename: A A
authors.initials: AA
authors.lastname: Kholin
mesh_terms: D000927:Anticonvulsants / Q000008:administration & dosage*; D018450:Disease Progression; D004569:Electroencephalography; D004828:Epilepsies, Partial / Q000175:diagnosis* / Q000188:drug therapy / Q000503:physiopathology; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007275:Injections, Intravenous; D008297:Male; D011379:Prognosis; D012640:Seizures; D013577:Syndrome; D014635:Valproic Acid / Q000008:administration & dosage*
chemical_list: D000927:Anticonvulsants; D014635:Valproic Acid