title: Panayiotopoulos syndrome with convulsive status epilepticus at the onset: a long-term study.
abstract: OBJECTIVE
  To better define the convulsive status epilepticus (CSE) as a possible manifestation at the onset of Panayiotopoulos syndrome (PS) and to assess its prognostic value in these children.
  
  
  METHODS
  Children with CSE and diagnostic criteria of PS were identified, followed clinically and compared with a group of patients with PS without CSE from 1993 to 2012.
  
  
  RESULTS
  We identified 37 patients with CSE at the onset of PS. During the same period we identified 72 children with autonomic symptoms of PS without CSE. The first episode of CSE occurred at a mean age of 6.5 years. Generalized clonic seizures were the most common ictal event and one-third of the patients required admission to Intensive Care Units. Interictal EEGs showed occipital spike activity in 31 (83.7%) subjects. Only 14 (37.8%) patients were treated with valproic acid and for two of them (5.40%) it was necessary to administer other drugs. There were no intractable cases. The overall prognosis was excellent. After the first event, 15 subjects (40.54%) experienced at least another typical PS seizure, but all patients were seizure free at the last follow-up.
  
  
  CONCLUSIONS
  CSE is not uncommon in PS and it may occur at the onset of benign childhood epilepsy, without leading to a poor prognosis.
authors: Department of Pediatrics, University of Perugia, Perugia, Italy. Electronic address: averrott@unich.it.
authors: Alberto
authors: A
authors: Verrotti
authors: Department of Pediatrics, University of Chieti, Chieti, Italy.
authors: Marianna
authors: M
authors: Sebastiani
authors: Pediatric Neuropsychiatric Division, "Spedali Civili", Brescia, Italy.
authors: Lucio
authors: L
authors: Giordano
authors: Pediatric Neurology and Muscular Diseases Unit, Department of Neurosciences, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health, Institute "Gaslini", University of Genova, Genova, Italy.
authors: Pasquale
authors: P
authors: Striano
authors: Neurology Unit, Department of Neuroscience, "Sant'Anna" Hospital, Como, Italy.
authors: Vincenzo
authors: V
authors: Belcastro
authors: Neuropsychiatry Unit, Department of Pediatrics, University of Bologna, Bologna, Italy.
authors: Emilio
authors: E
authors: Franzoni
authors: Child Neurology, Chair of Pediatrics, II Faculty of Medicine, University of Rome, Rome, Italy.
authors: Pasquale
authors: P
authors: Parisi
authors: Epilepsy Unit, Child Neuropsychiatry Department, University Hospital, Cagliari, Italy.
authors: Dario
authors: D
authors: Pruna
authors: Division of Child Neurology, Department of Pediatrics, University "La Sapienza" Rome, Rome, Italy.
authors: Alberto
authors: A
authors: Spalice
authors: Epilepsy Center, San Paolo Hospital, University of Milan, Milan, Italy.
authors: Aglaia
authors: A
authors: Vignoli
authors: Department of Pediatrics, University of Siena, Siena, Italy.
authors: Salvatore
authors: S
authors: Grosso
mesh_terms: D000293:Adolescent; D000927:Anticonvulsants / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D004569:Electroencephalography; D019305:Epilepsy, Rolandic / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy; D005260:Female; D006801:Humans; D008137:Longitudinal Studies; D008297:Male; D010672:Phenytoin / Q000627:therapeutic use; D012189:Retrospective Studies; D013226:Status Epilepticus / Q000150:complications*
chemical_list: D000927:Anticonvulsants; D010672:Phenytoin
keywords: Convulsive status epilepticus; Occipital spikes; Panayiotopoulos syndrome, Generalized clonic seizures