AUTHOR=Kersebaum Dilara , Sendel Manon , Lassen Josephine , Fabig Sophie-Charlotte , Forstenpointner Julia , Reimer Maren , Canaan-Kühl Sima , Gaedeke Jens , Rehm Stefanie , Gierthmühlen Janne , Baron Ralf , Hüllemann Philipp 

TITLE=Cold-evoked potentials in Fabry disease and polyneuropathy

JOURNAL=Frontiers in Pain Research

VOLUME=Volume 5 - 2024

YEAR=2024

URL=https://www.frontiersin.org/journals/pain-research/articles/10.3389/fpain.2024.1352711

DOI=10.3389/fpain.2024.1352711

ISSN=2673-561X

ABSTRACT=Background: Fabry disease (FD) causes cold-evoked pain and impaired cold perception through small-fiber damage, which also occurs in polyneuropathies (PNP) of other origins. The integrity of thinly myelinated fibers and the spinothalamic tract is assessable by cold-evoked potentials (CEPs). We aimed to assess the clinical value of CEP by investigating associations of CEP with pain, autonomic measures, sensory loss or neuropathic signs. Methods: CEPs were examined at the hand and foot dorsum of FD (n=16) and PNP (n=21) patients and healthy controls (n=23). Sensory phenotyping was performed by quantitative sensory testing (QST). The painDETECT questionnaire (PDQ), FabryScan and measures for the autonomic nervous system were applied. Group comparisons and correlation analyses were performed.Results: CEPs of 87.5% of the FD and 85.7% of the PNP patients were eligible for statistical analysis. In all patients combined, CEP data correlated significantly with cold-detection-loss, PDQ-items, pain and autonomic measures. Abnormal CEP-latency in FD patients was associated with an abnormal heart frequency variability item (r=-0.684; adjusted p=0.04). In PNP patients, CEP-latency correlated significantly with PDQ-items, and CEP-amplitude with autonomic measures (r=0.688; adjusted p=0.008 and r=0.619; adjusted p=0.024). Mechanical pain thresholds differed significantly between FD (gain range) and PNP patients (loss range) (p=0.01).Conclusions: Abnormal CEPs were associated with current pain, neuropathic signs and symptoms as well as an abnormal function of the autonomic nervous system. The latter has not been mirrored by QST-parameters. Therefore, CEPs appear to deliver a wider spectrum of information on the sensory nervous system than QST alone.