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Lookup NU author(s): Dr Karen SuetterlinORCiD
This work is licensed under a Creative Commons Attribution 4.0 International License (CC BY 4.0).
AimsThe aim of this study was to evaluate the sensitivity of the long exercise test (LET) in the diagnosis of periodic paralysis (PP) and assess correlations with clinical phenotypes and genotypes.MethodsFrom an unselected cohort of 335 patients who had an LET we analyzed 67 patients with genetic confirmation of PP and/or a positive LET.Results32/45 patients with genetically confirmed PP had a significant decrement after exercise (sensitivity of 71%). Performing the short exercise test before the LET in the same hand confounded results in four patients. Sensitivity was highest in patients with frequent (daily or weekly) attacks (8/8, 100%), intermediate with up to monthly attacks (15/21, 71%) and lowest in those with rare attacks (9/16, 56%) (p = .035, Mann–Whitney U-test). Patients with a positive LET without confirmed PP mutation comprised those with typical PP phenotype and a group with atypical features.DiscussionIn our cohort, the LET is strongly correlated with the frequency of paralytic attacks suggesting a role as a functional marker. A negative test in the context of frequent attacks makes a diagnosis of PP unlikely but it does not rule out the condition in less severely affected patients.
Author(s): Ribeiro A, Suetterlin K, Skorupinska I, Tan SV, Morrow JM, Matthews E, Hanna MG, Fialho D
Publication type: Article
Publication status: Published
Journal: Muscle & Nerve
Year: 2022
Volume: 65
Issue: 5
Pages: 581-585
Print publication date: 01/05/2022
Online publication date: 24/11/2021
Acceptance date: 16/11/2021
Date deposited: 29/06/2023
ISSN (electronic): 1097-4598
Publisher: Wiley
URL: https://doi.org/10.1002/mus.27465
DOI: 10.1002/mus.27465
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