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. 2020 Jan;35(1):42-48.
doi: 10.1177/0883073819875915. Epub 2019 Sep 25.

Preliminary Evidence That Resting State Heart Rate Variability Predicts Reactivity to Tactile Stimuli in Rett Syndrome

Alyssa M Merbler  1 , Breanne J Byiers  1 , John Hoch  1 , Adele C Dimian  1 , Chantel C Barney  1   2 , Timothy J Feyma  2 , Arthur A Beisang  2 , Alessandro Bartolomucci  3 , Frank J Symons  1
Affiliations

Preliminary Evidence That Resting State Heart Rate Variability Predicts Reactivity to Tactile Stimuli in Rett Syndrome

Alyssa M Merbler et al. J Child Neurol. 2020 Jan.

Abstract

Patients with Rett syndrome may manifest altered pain perception/experience and are vulnerable to conditions associated with chronic pain. Pain response is difficult to measure, however, because of severe communicative impairment. There is also documented autonomic dysfunction, including decreased heart rate variability. Given the relation between pain and the autonomic nervous system, we tested the feasibility of using resting heart rate variability to predict nonverbal pain/discomfort behavior during a standardized modified quantitative sensory test in Rett syndrome. All stimulus applications resulted in increased behavioral reactivity compared to baseline, with repeated von Frey significantly greater than all other stimuli. Resting heart rate variability predicted behavioral reactivity to repeated von Frey. These preliminary findings provide feasibility evidence for an integrated autonomic-sensory measurement approach and are consistent at a construct level with preclinical evidence in Rett syndrome. Further work is needed to determine how heart rate variability changes during stimulus application.

Keywords: MECP2; Rett syndrome; heart rate variability; quantitative sensory testing.

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Figures

Figure 1.
Figure 1.
Behavioral reactivity by stimulus. Each point represents one participant’s score. Reactivity scores for each stimulus were significantly higher than baseline.
Figure 2.
Figure 2.
Behavioral reactivity within each stimulus by resting RMSSD. There was a significant interaction for stimulus reactivity and resting RMSSD for repeated von Frey (p < 0.001).
See this image and copyright information in PMC

References

    1. Amir RE, Van den Veyver IB, Wan M, Tran CQ, Francke U, Zoghbi HY. Rett syndrome is caused by mutations in X-linked MECP2, encoding methyl-CpG-binding protein 2. Nat Genet. 1999. October;23(2):185–8. doi: 10.1038/13810. - DOI - PubMed
    1. Martin P Pain in Rett syndrome: peculiarities in pain processing and expression, liability to pain causing disorders and diseases, and specific aspects of pain assessment. Adv Autism. 2017; 3(3):163–82. doi: 10.1108/AIA-02-2017-0003. - DOI
    1. Samaco RC, Fryer JD, Ren J, Fyffe S, Chao HT, Sun Y, et al. A partial loss of function allele of methyl-CpG-binding protein 2 predicts a human neurodevelopmental syndrome. Hum Mol Genet 2008;17(12):1718–27. doi: 10.1093/hmg/ddn062. - DOI - PMC - PubMed
    1. Neul JL, Kaufmann WE, Glaze DG, Christodoulou J, Clarke AJ, Bahi-Buisson N et al. Rett syndrome: revised diagnostic criteria and nomenclature. Ann Neurol. 2010. December;68(6):944–50. doi: 10.1002/ana.22124. - DOI - PMC - PubMed
    1. Hagberg B Clinical manifestations and stages of Rett syndrome. Ment Retard Dev Disabil Res Rev. 2002;8(2):61–5. - PubMed

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