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. 2015 Nov;31(11):998-1003.
doi: 10.1097/AJP.0000000000000192.

A Case-controlled Investigation of Pain Experience and Sensory Function in Neuronal Ceroid Lipofuscinosis

Chantel C Barney  1 , John Hoch, Breanne Byiers, Adele Dimian, Frank J Symons
Affiliations

A Case-controlled Investigation of Pain Experience and Sensory Function in Neuronal Ceroid Lipofuscinosis

Chantel C Barney et al. Clin J Pain. 2015 Nov.

Abstract

Objectives: 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).

Materials and 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).

Discussion: 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.

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Figures

Figure 1
Figure 1
Mean BOPS scores depicting pain expression in each of the two sensory testing conditions (light touch [LT], repeated Von Frey [RVF]) are shown for individuals with NCL, and the sibling comparison group. Across combined conditions (light touch, repeated Von Frey), the NCL group was significantly more reactive (BOPS total score) to sensory testing (M= 9.25, SD= 9.17) compared to the sibling comparison group (M=1.25, SD=1.49; t(7.37)= 2.43, p< .05).
Figure 2
Figure 2
Mean IRT temperatures for each IRT site during each of the two sensory testing conditions (light touch [LT], repeated Von Frey [RVF]) are shown for children with NCL, and the sibling comparison group. The difference scores between the two conditions for all IRT sites were calculated for the NCL group (m = −2.00, SD = 0.97) and sibling group (m = 0.13, SD = 0.26) and were significantly different (t(14)= −5.98, p<.001). Total body temperature (measured with a standard ear thermometer) of NCL group (m = 97.6, SD = 0.93) did not differ from the sibling comparison group (m = 97.8, SD = 0.99; t(14)= −0.36, p= .72).
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References

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