title: A Case-controlled Investigation of Pain Experience and Sensory Function in Neuronal Ceroid Lipofuscinosis.
abstract: OBJECTIVE
  This case-control study explored pain experience and expression among individuals with neuronal ceroid lipofuscinosis (NCL) through parental report, tactile sensory testing, and infrared thermography (IRT).
  
  
  METHODS
  Participants with NCL (n=8; M=14.8 y) and their unaffected siblings (n=8; M=23.5 y) were characterized in terms of pain response to a brief tactile sensory test (light touch, Von Frey monofilament). During sensory testing, behavioral expression was measured using the Battens Observational Pain Scale and infrared thermography (IRT) was used to quantify changes in skin/eye temperature.
  
  
  RESULTS
  Children with NCL experienced pain frequently and from multiple sources that negatively impacted their lives. Children with NCL were reactive to the sensory testing as indexed by significant increased IRT temperature change (P<0.001). Across combined sensory conditions, individuals with NCL were significantly more reactive (Battens Observational Pain Scale total score) to sensory testing compared with siblings (P<0.05). Similarly, IRT difference scores between sensory conditions revealed a significant increase in temperature for individuals with NCL compared with siblings (P<0.001).
  
  
  CONCLUSIONS
  Ongoing reported pain was a problem for the children with NCL in this sample. Increased pain expression during the repeated Von Frey filament suggests that the pathophysiology of the ongoing pain may be centrally mediated.
authors.affiliation: *Gillette Children's Specialty Healthcare, St Paul †Department of Educational Psychology ‡Center for Neurobehavioral Development, University of Minnesota, Minneapolis, MN.
authors.forename: Chantel C
authors.initials: CC
authors.lastname: Barney
authors.forename: John
authors.initials: J
authors.lastname: Hoch
authors.forename: Breanne
authors.initials: B
authors.lastname: Byiers
authors.forename: Adele
authors.initials: A
authors.lastname: Dimian
authors.forename: Frank J
authors.initials: FJ
authors.lastname: Symons
mesh_terms: D000293:Adolescent; D016022:Case-Control Studies; D002648:Child; D005123:Eye / Q000503:physiopathology; D005260:Female; D006801:Humans; D008297:Male; D009472:Neuronal Ceroid-Lipofuscinoses / Q000503:physiopathology*; D010146:Pain / Q000503:physiopathology*; D010147:Pain Measurement; D017288:Pain Threshold / Q000502:physiology; D012677:Sensation / Q000502:physiology*; D035781:Siblings; D012881:Skin Temperature / Q000502:physiology; D013817:Thermography; D055815:Young Adult