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Myotonic dystrophy type 1 alters muscle twitch properties, spinal reflexes, and perturbation-induced trans-cortical reflexes
Journal article   Open access   Peer reviewed

Myotonic dystrophy type 1 alters muscle twitch properties, spinal reflexes, and perturbation-induced trans-cortical reflexes

Richard K Shields, Jinhyun Lee, Aaron Buelow, Michael Petrie, Shauna Dudley-Javoroski, Stephen Cross, Laurie Gutmann and Peggy C Nopoulos
Muscle & nerve, Vol.61(2), pp.205-212
02/2020
DOI: 10.1002/mus.26767
PMCID: PMC7711310
PMID: 31773755
url
https://www.ncbi.nlm.nih.gov/pmc/articles/7711310View
Open Access

Abstract

Neurophysiologic biomarkers are needed for clinical trials of therapies for myotonic dystrophy (DM1). We characterized muscle properties, spinal reflexes (H-reflexes), and trans-cortical long-latency reflexes (LLRs) in a cohort with mild/moderate DM1. Twenty-four people with DM1 and 25 matched controls underwent assessment of tibial nerve H-reflexes and soleus muscle twitch properties. Quadriceps LLRs were elicited by delivering an unexpected perturbation during a single-limb squat (SLS) visuomotor tracking task. DM1 was associated with decreased H-reflex depression. The efficacy of doublet stimulation was enhanced, yielding an elevated double-single twitch ratio. DM1 participants demonstrated greater error during the SLS task. DM1 individuals with the least-robust LLR responses showed the greatest loss of spinal H-reflex depression. DM1 is associated with abnormalities of muscle twitch properties. Co-occurring alterations of spinal and trans-cortical reflex properties underscore the central nervous system manifestations of this disorder and may assist in gauging efficacy during clinical trials.
Electromyography Electric Stimulation Humans Middle Aged H-Reflex Spine - physiopathology Male Muscle, Skeletal - innervation Electrodiagnosis Myotonic Dystrophy - physiopathology Muscle Contraction Reflex, Abnormal Muscle, Skeletal - physiopathology Adult Female Cohort Studies

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