Richard K Shields1, Jinhyun Lee1, Aaron Buelow1, Michael Petrie1, Shauna Dudley-Javoroski1, Stephen Cross2, Laurie Gutmann3, Peggy C Nopoulos2. 1. Department of Physical Therapy and Rehabilitation Science, Roy J. and Lucille A. Carver College of Medicine, University of Iowa, Iowa City, Iowa. 2. Department of Psychiatry, Roy J. and Lucille A. Carver College of Medicine, University of Iowa, Iowa City, Iowa. 3. Department of Neurology, Roy J. and Lucille A. Carver College of Medicine, University of Iowa, Iowa City, Iowa.
Abstract
BACKGROUND: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.
BACKGROUND: 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. METHODS: 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. RESULTS:DM1 was associated with decreased H-reflex depression. The efficacy of doublet stimulation was enhanced, yielding an elevated double-single twitch ratio. DM1participants demonstrated greater error during the SLS task. DM1 individuals with the least-robust LLR responses showed the greatest loss of spinal H-reflex depression. CONCLUSIONS: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.
Authors: A Vihola; G Bassez; G Meola; S Zhang; H Haapasalo; A Paetau; E Mancinelli; A Rouche; J Y Hogrel; P Laforêt; T Maisonobe; J F Pellissier; R Krahe; B Eymard; B Udd Journal: Neurology Date: 2003-06-10 Impact factor: 9.910
Authors: Shih-Chiao Tseng; Keith R Cole; Michael A Shaffer; Michael A Petrie; Chu-Ling Yen; Richard K Shields Journal: Gait Posture Date: 2016-12-15 Impact factor: 2.840
Authors: Marianne Goodwin; Apoorva Mohan; Ranjan Batra; Kuang-Yung Lee; Konstantinos Charizanis; Francisco José Fernández Gómez; Sabiha Eddarkaoui; Nicolas Sergeant; Luc Buée; Takashi Kimura; H Brent Clark; Joline Dalton; Kenji Takamura; Sebastien M Weyn-Vanhentenryck; Chaolin Zhang; Tammy Reid; Laura P W Ranum; John W Day; Maurice S Swanson Journal: Cell Rep Date: 2015-08-06 Impact factor: 9.423
Authors: Ellen van der Plas; Laurie Gutmann; Dan Thedens; Richard K Shields; Kathleen Langbehn; Zhihui Guo; Milan Sonka; Peggy Nopoulos Journal: Muscle Nerve Date: 2021-02-05 Impact factor: 3.217
Authors: Timothy R Koscik; Ellen van der Plas; Laurie Gutmann; Sarah A Cumming; Darren G Monckton; Vincent Magnotta; Richard K Shields; Peggy C Nopoulos Journal: Sci Rep Date: 2021-03-01 Impact factor: 4.379