title: Paroxysmal alpha activity in Rett syndrome: a case report.
abstract: BACKGROUND
  Rett syndrome is a severe neurodevelopmental disorder that primarily affects females. Classically the disorder is characterized by early normal development, followed by a period of regression and later recovery or stagnation. Typical features include a loss of purposeful hand skills, development of hand stereotypies, loss of spoken language, gait abnormalities, and acquired microcephaly. Epilepsy affects between 70% and 90% of individuals with Rett syndrome. A number of stereotypical electroencephalography findings have been reported in Rett syndrome.
  
  
  METHODS
  We report a 9-year-old girl with Rett syndrome and epilepsy with a unique electroencephalography finding consisting of intermittent paroxysms of alpha activity in both wakefulness and sleep without clinical signs.
  
  
  RESULTS
  This unique electroencephalography signature has not previously been reported in the English literature.
  
  
  CONCLUSIONS
  Knowledge of this unique electroencephalography pattern of diffuse paroxysmal alpha activity represents an additional distinct feature of the electroencephalogram in Rett syndrome and expands the spectrum of electroencephalography abnormalities in Rett syndrome.
authors.affiliation: Division of Pediatric Neurology, Department of Pediatrics, McMaster Children's Hospital, McMaster University, Hamilton, Ontario, Canada. Electronic address: robyn.whitney@medportal.ca.
authors.forename: Robyn
authors.initials: R
authors.lastname: Whitney
authors.affiliation: Division of Neurology, Department of Pediatrics, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.
authors.forename: Mahendranath
authors.initials: M
authors.lastname: Moharir
authors.affiliation: Division of Neurology, Department of Pediatrics, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.
authors.forename: Anita
authors.initials: A
authors.lastname: Allen
authors.affiliation: Division of Neurology, Department of Pediatrics, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.
authors.forename: Miguel A
authors.initials: MA
authors.lastname: Cortez
mesh_terms: D000513:Alpha Rhythm; D001921:Brain / Q000503:physiopathology*; D002648:Child; D004569:Electroencephalography; D005260:Female; D006801:Humans; D015518:Rett Syndrome / Q000503:physiopathology*; D012890:Sleep / Q000502:physiology; D014851:Wakefulness / Q000502:physiology
keywords: Rett syndrome; diagnosis; electroencephalography pattern; epilepsy