title: Altered activation of the tibialis anterior in individuals with Pompe disease: Implications for motor unit dysfunction.
abstract: BACKGROUND
  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.
  
  
  METHODS
  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.
  
  
  RESULTS
  The Pompe patients exhibited greater power at frequencies below 60 Hz and reduced power above 100 Hz. They also exhibited a reduced increase in M-wave and prolonged M-wave latency and duration in response to stimulation.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Pediatrics, College of Medicine, University of Florida, Gainesville, Florida, USA.|Department of Neurology, College of Medicine, University of Florida, Gainesville, Florida, USA.|Department of Applied Physiology and Kinesiology, College of Health & Human Performance, University of Florida, Gainesville, Florida, USA.
authors.forename: Manuela
authors.initials: M
authors.lastname: Corti
authors.affiliation: Department of Pediatrics, College of Medicine, University of Florida, Gainesville, Florida, USA.|Department of Physical Therapy, College of Public Health & Health Profession, University of Florida, P.O. Box 100296, Gainesville, Florida, 32610, USA.
authors.forename: Barbara K
authors.initials: BK
authors.lastname: Smith
authors.affiliation: Department of Pediatrics, College of Medicine, University of Florida, Gainesville, Florida, USA.
authors.forename: Darin J
authors.initials: DJ
authors.lastname: Falk
authors.affiliation: Department of Pediatrics, College of Medicine, University of Florida, Gainesville, Florida, USA.
authors.forename: Lee Ann
authors.initials: LA
authors.lastname: Lawson
authors.affiliation: Department of Physical Therapy, College of Public Health & Health Profession, University of Florida, P.O. Box 100296, Gainesville, Florida, 32610, USA.
authors.forename: David D
authors.initials: DD
authors.lastname: Fuller
authors.affiliation: Department of Neurology, College of Medicine, University of Florida, Gainesville, Florida, USA.
authors.forename: S H
authors.initials: SH
authors.lastname: Subramony
authors.affiliation: Department of Pediatrics, College of Medicine, University of Florida, Gainesville, Florida, USA.|Department of Neurology, College of Medicine, University of Florida, Gainesville, Florida, USA.
authors.forename: Barry J
authors.initials: BJ
authors.lastname: Byrne
authors.affiliation: Department of Physical Therapy, College of Public Health & Health Profession, University of Florida, P.O. Box 100296, Gainesville, Florida, 32610, USA.|Department of Applied Physiology and Kinesiology, College of Health & Human Performance, University of Florida, Gainesville, Florida, USA.
authors.forename: Evangelos A
authors.initials: EA
authors.lastname: Christou
mesh_terms: D000293:Adolescent; D000328:Adult; D004558:Electric Stimulation; D019054:Evoked Potentials, Motor / Q000502:physiology; D005260:Female; D006009:Glycogen Storage Disease Type II / Q000150:complications*; D006801:Humans; D007537:Isometric Contraction / Q000502:physiology*; D008297:Male; D009069:Movement Disorders / Q000209:etiology*; D018482:Muscle, Skeletal / Q000503:physiopathology*; D055815:Young Adult
keywords: Pompe disease; glycogen-storage disease; modulation; motoneuron; skeletal muscle