Motor Unit Number Estimation in the Assessment of Performance and Function in Motor Neuron Disease

2008 ◽  
Vol 19 (3) ◽  
pp. 509-532 ◽  
Author(s):  
Mark B. Bromberg ◽  
Alexander A. Brownell
Keyword(s):  
Motor Neuron ◽  
Motor Neuron Disease ◽  
Motor Unit ◽  
Assessment Of Performance ◽  
Motor Unit Number Estimation ◽  
And Function

scholarly journals Activity-Driven Synaptic and Axonal Degeneration in Canine Motor Neuron Disease

2004 ◽  
Vol 92 (2) ◽  
pp. 1175-1181 ◽  
Author(s):  
Dario I. Carrasco ◽  
Mark M. Rich ◽  
Qingbo Wang ◽  
Timothy C. Cope ◽  
Martin J. Pinter
Keyword(s):  
Motor Neuron ◽  
Motor Neuron Disease ◽  
Motor Unit ◽  
Neuromuscular Junctions ◽  
Muscular Atrophy ◽  
Motor Axon ◽  
Medial Gastrocnemius ◽  
Neuron Activity ◽  
Functional Changes ◽  
Unit Function

The role of neuronal activity in the pathogenesis of neurodegenerative disease is largely unknown. In this study, we examined the effects of increasing motor neuron activity on the pathogenesis of a canine version of inherited motor neuron disease (hereditary canine spinal muscular atrophy). Activity of motor neurons innervating the ankle extensor muscle medial gastrocnemius (MG) was increased by denervating close synergist muscles. In affected animals, 4 wk of synergist denervation accelerated loss of motor-unit function relative to control muscles and decreased motor axon conduction velocities. Slowing of axon conduction was greatest in the most distal portions of motor axons. Morphological analysis of neuromuscular junctions (NMJs) showed that these functional changes were associated with increased loss of intact innervation and with the appearance of significant motor axon and motor terminal sprouting. These effects were not observed in the MG muscles of age-matched, normal animals with synergist denervation for 5 wk. The results indicate that motor neuron action potential activity is a major contributing factor to the loss of motor-unit function and degeneration in inherited canine motor neuron disease.

Functional motor unit failure precedes neuromuscular degeneration in canine motor neuron disease

2000 ◽  
Vol 47 (5) ◽  
pp. 596-605 ◽  
Author(s):  
Rita J. Balice-Gordon ◽  
Daniel B. J. Smith ◽  
Joshua Goldman ◽  
Linda C. Cork ◽  
Anne Shirley ◽  
...  
Keyword(s):  
Motor Neuron ◽  
Motor Neuron Disease ◽  
Motor Unit

scholarly journals Motor unit behavior in canine motor neuron disease

1995 ◽  
Vol 15 (5) ◽  
pp. 3447-3457 ◽  
Author(s):  
MJ Pinter ◽  
RF Waldeck ◽  
N Wallace ◽  
LC Cork
Keyword(s):  
Motor Neuron ◽  
Motor Neuron Disease ◽  
Motor Unit

scholarly journals Short-term stability of single motor unit recordings in motor neuron disease: a macro EMG study.

1988 ◽  
Vol 51 (5) ◽  
pp. 671-676 ◽  
Author(s):  
R J Guiloff ◽  
H Modarres-Sadeghi ◽  
E Stalberg ◽  
H Rogers
Keyword(s):  
Motor Neuron ◽  
Motor Neuron Disease ◽  
Motor Unit ◽  
Short Term ◽  
Single Motor Unit ◽  
Single Motor ◽  
Term Stability ◽  
Macro Emg

scholarly journals Effects of 4-Aminopyridine on Muscle and Motor Unit Force in Canine Motor Neuron Disease

1997 ◽  
Vol 17 (11) ◽  
pp. 4500-4507 ◽  
Author(s):  
M. J. Pinter ◽  
R. F. Waldeck ◽  
T. C. Cope ◽  
L. C. Cork
Keyword(s):  
Motor Neuron ◽  
Motor Neuron Disease ◽  
Motor Unit ◽  
Unit Force

scholarly journals Reduced Endplate Currents Underlie Motor Unit Dysfunction in Canine Motor Neuron Disease

2002 ◽  
Vol 88 (6) ◽  
pp. 3293-3304 ◽  
Author(s):  
Mark M. Rich ◽  
Robert. F. Waldeck ◽  
Linda C. Cork ◽  
Rita J. Balice-Gordon ◽  
Robert E. W. Fyffe ◽  
...  
Keyword(s):  
Motor Neuron ◽  
Motor Neuron Disease ◽  
Motor Unit ◽  
Conduction Velocity ◽  
Motor Neurons ◽  
Muscle Fiber ◽  
Muscular Atrophy ◽  
Motor Units ◽  
Postnatal Maturation ◽  
Axonal Conduction

Hereditary canine spinal muscular atrophy (HCSMA) is an autosomal dominant degenerative disorder of motor neurons. In homozygous animals, motor units produce decreased force output and fail during repetitive activity. Previous studies suggest that decreased efficacy of neuromuscular transmission underlies these abnormalities. To examine this, we recorded muscle fiber endplate currents (EPCs) and found reduced amplitudes and increased failures during nerve stimulation in homozygotes compared with wild-type controls. Comparison of EPC amplitudes with muscle fiber current thresholds indicate that many EPCs from homozygotes fall below threshold for activating muscle fibers but can be raised above threshold following potentiation. To determine whether axonal abnormalities might play a role in causing motor unit dysfunction, we examined the postnatal maturation of axonal conduction velocity in relation to the appearance of tetanic failure. We also examined intracellularly labeled motor neurons for evidence of axonal neurofilament accumulations, which are found in many instances of motor neuron disease including HCSMA. Despite the appearance of tetanic failure between 90 and 120 days, average motor axon conduction velocity increased with age in homozygotes and achieved adult levels. Normal correlations between motor neuron properties (including conduction velocity) and motor unit properties were also observed. Labeled proximal motor axons of several motor neurons that supplied failing motor units exhibited little or no evidence of axonal swellings. We conclude that decreased release of transmitter from motor terminals underlies motor unit dysfunction in HCSMA and that the mechanisms determining the maturation of axonal conduction velocity and the pattern of correlation between motor neuron and motor unit properties do not contribute to the appearance or evolution of motor unit dysfunction.

Following disease progression in motor neuron disorders with 3 motor unit number estimation methods

2018 ◽  
Vol 59 (1) ◽  
pp. 82-87 ◽  
Author(s):  
Anna Bystrup Jacobsen ◽  
Hugh Bostock ◽  
Hatice Tankisi
Keyword(s):  
Motor Neuron ◽  
Disease Progression ◽  
Motor Unit ◽  
Estimation Methods ◽  
Motor Unit Number Estimation

Outcomes of Physical Therapy, Speech Pathology, and Occupational Therapy for People with Motor Neuron Disease: A Systematic Review

2006 ◽  
Vol 20 (3) ◽  
pp. 424-434 ◽  
Author(s):  
Meg E. Morris ◽  
Alison Perry ◽  
Belinda Bilney ◽  
Andrea Curran ◽  
Karen Dodd ◽  
...  
Keyword(s):  
Systematic Review ◽  
Physical Therapy ◽  
Occupational Therapy ◽  
Motor Neuron ◽  
Motor Neuron Disease ◽  
Data Base ◽  
Critical Evaluation ◽  
International Classification Of Functioning ◽  
Speech Pathology ◽  
And Function

This article describes a systematic review and critical evaluation of the international literature on the effects of physical therapy, speech pathology, and occupational therapy for people with motor neuron disease (PwMND). The results were interpreted using the framework of the International Classification of Functioning, Disability and Health. This enabled us to summarize therapy outcomes at the level of body structure and function, activity limitations, participation restrictions, and quality of life. Databases searched included MEDLINE, PUBMED, CINAHL, PSYCInfo, Data base of Abstracts of Reviews of Effectiveness (DARE), The Physiotherapy Evidence data base (PEDro), Evidence Based Medicine Reviews (EMBASE), the Cochrane database of systematic reviews, and the Cochrane Controlled Trials Register. Evidence was graded according to the Harbour and Miller classification. Most of the evidence was found to be at the level of “clinical opinion” rather than of controlled clinical trials. Several nonrandomized small group and “observational studies” provided low-level evidence to support physical therapy for improving muscle strength and pulmonary function. There was also some evidence to support the effectiveness of speech pathology interventions for dysarthria. The search identified a small number of studies on occupational therapy for PwMND, which were small, noncontrolled pre-post-designs or clinical reports.

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