title: Detecting autonomic response to pain in Rett syndrome.
abstract: OBJECTIVE
  To quantify pain response in girls affected by Rett syndrome (RTT) using electrodermal activity (EDA), a measure of skin conductance, reflecting sympathetic activity known to be modulated by physical and environmental stress.
  
  
  METHODS
  EDA increase, heart rate (HR) increase and Face Legs Activity Cry Consolability (FLACC) values calculated during venipuncture (invasive) and vital signs collection (non-invasive) events were compared with values calculated during a prior baseline and a RTT clinical severity score (CSS).
  
  
  RESULTS
  EDA and HR increase were significantly higher than baseline during venipuncture only and not significantly correlated with FLACC or CSS. EDA increase was the most sensitive measure of pain response.
  
  
  CONCLUSIONS
  These preliminary findings revealed that motor impairment might bias non-verbal pain scales, underscore the importance of using autonomic measures when assessing pain and warrant further investigation into the utility of using EDA to objectively quantify RTT pain response to inform future RTT pain management.
authors.affiliation: a Department of Neurology , Boston Children's Hospital and Harvard Medical School , Boston , MA , USA.
authors.forename: Heather M
authors.initials: HM
authors.lastname: O'Leary
authors.affiliation: b Institute of Physiology, Graz Medical University , Graz , Austria.|c Department of Women's and Children's Health , Center for Neurodevelopmental Disorders (KIND), Karolinska Institute , Stockholm , Sweden.
authors.forename: Peter B
authors.initials: PB
authors.lastname: Marschik
authors.affiliation: d Roche Pharma Research and Early Development, Roche Innovation Center, F. Hoffmann-La Roche , Basel , Switzerland.
authors.forename: Omar S
authors.initials: OS
authors.lastname: Khwaja
authors.affiliation: e Department of Neurology , Children's Hospital Los Angeles , Los Angeles , CA , USA , and.
authors.forename: Eugenia
authors.initials: E
authors.lastname: Ho
authors.affiliation: a Department of Neurology , Boston Children's Hospital and Harvard Medical School , Boston , MA , USA.
authors.forename: Katherine V
authors.initials: KV
authors.lastname: Barnes
authors.affiliation: f Division of Developmental Medicine , Boston Children's Hospital and Harvard Medical School , Boston , MA , USA.
authors.forename: Tessa W
authors.initials: TW
authors.lastname: Clarkson
authors.affiliation: a Department of Neurology , Boston Children's Hospital and Harvard Medical School , Boston , MA , USA.
authors.forename: Natalie M
authors.initials: NM
authors.lastname: Bruck
authors.affiliation: a Department of Neurology , Boston Children's Hospital and Harvard Medical School , Boston , MA , USA.
authors.forename: Walter E
authors.initials: WE
authors.lastname: Kaufmann
mesh_terms: D001341:Autonomic Nervous System / Q000503:physiopathology*; D002648:Child; D002675:Child, Preschool; D005260:Female; D005712:Galvanic Skin Response; D006339:Heart Rate; D006801:Humans; D010147:Pain Measurement / Q000295:instrumentation / Q000379:methods*; D015518:Rett Syndrome / Q000175:diagnosis / Q000503:physiopathology*
keywords: Electrodermal activity; FLACC; Rett syndrome; heart rate; pain; skin conductance