title: Congenital hemiparesis, unilateral polymicrogyria and epilepsy with or without status epilepticus during sleep: a study of 66 patients with long-term follow-up.
abstract: OBJECTIVE
  We retrospectively analysed the electroclinical features, treatment, and outcome in patients with unilateral polymicrogyria (PMG), focussing on epileptic syndrome with or without encephalopathy, with status epilepticus during sleep (ESES) or continuous spikes and waves during slow sleep (CSWS) syndrome.
  
  
  METHODS
  From June 1990 to December 2012, 39 males and 27 females, aged 5-26 years, were studied. We did not include patients with bilateral PMG or cases with unilateral PMG associated with other cerebral lesions. The mean follow-up period was 12 years (range: 3-22 years).
  
  
  RESULTS
  Mean age at epilepsy onset was 6.5 years. Focal motor seizures occurred in all cases and 25 had secondary generalised seizures. Six patients also had complex focal seizures. Interictal EEG recordings showed focal spikes in all cases. For 43 of 53 patients with epilepsy, aged 2-9.5 years, the electroclinical features changed. An increase in frequency of focal motor seizures was reported in 20 patients, negative myoclonus occurred in 32 patients, atypical absences in 25 patients, and positive myoclonus in 19 patients. All patients had a continuous symmetric or asymmetric pattern of spike-wave activity during slow-wave sleep.
  
  
  CONCLUSIONS
  For patients presenting with congenital hemiparesis, negative or positive myoclonus, and absences and focal motor seizures with ESES/CSWS, unilateral PMG should be considered. Brain MRI is mandatory to confirm this cortical malformation. The most commonly used treatments were clobazam, ethosuximide, and sulthiame, alone or in combination. For refractory cases, high-dose steroids were administered and surgery was performed in two patients. Outcome was relatively benign.
authors.affiliation: Department of Neurology, Hospital Nacional de Pediatría Juan P Garrahan, Buenos Aires, Argentina.
authors.forename: Roberto Horacio
authors.initials: RH
authors.lastname: Caraballo
authors.affiliation: Department of Neurology, Hospital Nacional de Pediatría Juan P Garrahan, Buenos Aires, Argentina.
authors.forename: Ricardo Oscar
authors.initials: RO
authors.lastname: Cersósimo
authors.affiliation: Department of Neurology, Hospital Nacional de Pediatría Juan P Garrahan, Buenos Aires, Argentina.
authors.forename: Pablo Sebastián
authors.initials: PS
authors.lastname: Fortini
authors.affiliation: Department of Neurology, Hospital Nacional de Pediatría Juan P Garrahan, Buenos Aires, Argentina.
authors.forename: Lorena
authors.initials: L
authors.lastname: Ornella
authors.affiliation: Department of Neurology, Hospital Nacional de Pediatría Juan P Garrahan, Buenos Aires, Argentina.
authors.forename: María Celeste
authors.initials: MC
authors.lastname: Buompadre
authors.affiliation: Department of Neurology, Hospital Nacional de Pediatría Juan P Garrahan, Buenos Aires, Argentina.
authors.forename: Carolina
authors.initials: C
authors.lastname: Vilte
authors.affiliation: Department of Neurology, Hospital Nacional de Pediatría Juan P Garrahan, Buenos Aires, Argentina.
authors.forename: Juan Pablo
authors.initials: JP
authors.lastname: Princich
authors.affiliation: Department of Neurology, Hospital Nacional de Pediatría Juan P Garrahan, Buenos Aires, Argentina.
authors.forename: Natalio
authors.initials: N
authors.lastname: Fejerman
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D004569:Electroencephalography / Q000379:methods; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D054220:Malformations of Cortical Development / Q000503:physiopathology*; D010291:Paresis / Q000151:congenital / Q000503:physiopathology*; D012189:Retrospective Studies; D012890:Sleep / Q000502:physiology*; D013226:Status Epilepticus / Q000175:diagnosis / Q000503:physiopathology*; D013997:Time Factors; D055815:Young Adult
keywords: congenital hemiparesis; continuous spikes and waves; epilepsy; negative myoclonus; sleep; unilateral polymicrogyria