Altered activation of the tibialis anterior in individuals with Pompe disease: Implications for motor unit dysfunction. Issue 6 (24th April 2015)

• Record Type: Journal Article
• Authors: Corti, Manuela; Smith, Barbara K.; Falk, Darin J.; Lawson, Lee Ann; Fuller, David D.; Subramony, S.H.; Byrne, Barry J.; Christou, Evangelos A.
• Is Part Of: Muscle & nerve. Volume 51:Issue 6(2015:Jun.)
• Page Start: 877
• Page End: 883
• Publication Date: 2015-04-24
• Subjects: Neuromuscular diseases -- Periodicals; Muscles -- Periodicals; Nerves -- Periodicals; 616.74
• DOI: 10.1002/mus.24444
• Languages: English
• ISSNs: 0148-639X

Abstract

<abstract abstract-type="main"> <title>ABSTRACT</title> <sec id="mus24444-sec-0001" sec-type="section"> <title>Introduction</title> <p>Pompe disease is a progressive disease that affects skeletal muscles and leads to loss of ambulation. We investigated the activation of the tibialis anterior (TA) in late‐onset Pompe disease (LOPD) individuals during maximal voluntary contraction (MVC) and evoked involuntary responses.</p> </sec> <sec id="mus24444-sec-0002" sec-type="section"> <title>Methods</title> <p>Four LOPD patients and matched control subjects performed MVC of the TA using dorsiflexion and TA evoked responses. Activation of the TA was recorded with surface electromyography.</p> </sec> <sec id="mus24444-sec-0003" sec-type="section"> <title>Results</title> <p>The Pompe patients exhibited greater power at frequencies below 60 H<sc>z</sc> and reduced power above 100 H<sc>z</sc>. They also exhibited a reduced increase in M‐wave and prolonged M‐wave latency and duration in response to stimulation.</p> </sec> <sec id="mus24444-sec-0004" sec-type="section"> <title>Conclusions</title> <p>These results provide evidence that LOPD individuals have an altered activation pattern of the TA during maximal contractions. The observed activation pattern may reflect impairments in voluntary command, neuromuscular junction pathology, or compensatory drive due to a reduced number of functional motoneurons. <italic>Muscle Nerve</italic> 51: 877–883, 2015</p> </sec> </abstract>