Quantitative assessment of motor fatigue in amyotrophic lateral sclerosis

Sanjak, Mohammed; Brinkmann, James R.; Belden, Daryn; Roelke, K A; Waclawik, Andrew J.; Neville, Hans E.; Ringel, Steven P.; Murphy, James R.; Brooks, Benjamin Rix

doi:10.1016/s0022-510x(01)00624-4

Cited by 51 publications

(38 citation statements)

References 13 publications

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“…In addition, these mice display preferential denervation of fast-twitch muscles [52][53][54][55]. It has been suggested that both fast muscle and motor units are selectively vulnerable to the disease process in ALS patients [56,57]. This increased sensitivity to muscle denervation in fast muscles has also been demonstrated in aged mice [27,28] and SOD1-/-mice [35,36].…”

Section: Mutant Sod1 Transgenic Mouse Model Of Falsmentioning

confidence: 99%

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Mechanisms of Muscle Denervation in Aging: Insights from a Mouse Model of Amyotrophic Lateral Sclerosis

Park¹

2015

Aging and disease

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Muscle denervation at the neuromuscular junction (NMJ) is thought to be a contributing factor in age-related muscle weakness. Therefore, understanding the mechanisms that modulate NMJ innervation is a key to developing therapies to combat age-related muscle weakness affecting the elderly. Two mouse models, one lacking the Cu/Zn superoxide dismutase (SOD1) gene and another harboring the transgenic mutant human SOD1 gene, display progressive changes at the NMJ, including muscle endplate fragmentation, nerve terminal sprouting, and denervation. These changes at the NMJ share many of the common features observed in the NMJs of aged mice. In this review, research findings demonstrating the effects of PGC-1α, IGF-1, GDNF, MyoD, myogenin, and miR-206 on NMJ innervation patterns in the G93A SOD1 mice will be highlighted in the context of age-related muscle denervation. Key words: Aging, muscle denervation, amyotrophic lateral sclerosis, SOD1-/-mouse, G93A SOD1 mouse, oxidative stress Many retrospective and prospective studies have shown that age-related muscle weakness (dynapenia) is associated with decreased muscle function and increased risk for mortality in the elderly [1][2][3][4]. Dynapenia is a main cause of muscular instability, contributing to falls and subsequent fractures, ultimately impacting quality of life [5,6]. Both diet and exercise are practical interventions demonstrated to delay many devastating and debilitating age-associated pathologies including type 2 diabetes, cardiovascular disease, cerebrovascular disease, cognitive dysfunction, and even some cancers [7][8][9][10][11][12][13][14]. However, physical activity is the most effective means for increasing the quality of life in the elderly [15,16] and gait speed is a strong predictor of survival rate in elderly subjects [17]. Dynapenia represents a dramatic and inevitable decline in skeletal muscle function, which inevitably poses challenges for implementing a physical activity program to benefit the quality of life in the elderly. It is thought that complex degeneration of the neuromuscular system contributes to dynapenia [18][19][20][21][22].It has been proposed that age-associated changes occur in both the nervous system and the muscular system, leading to an overall loss in muscular strength [23]. In humans, a significant decrease in the number of motor units and muscle strength is observed with aging [24]. Studies using rodent models of aging show extensive alteration in NMJ morphology. Findings from the BaliceGordon lab demonstrated significant instability in the NMJ of aged rats using a longitudinal visualization technique for monitoring the NMJ in vivo [25]. Examination of 12-to 20-month-old rats demonstrated a progressive loss of synaptic areas and fragmentation of muscle endplates, leading to significant losses in pre-and post-synaptic structures over time [26]. Subsequent studies in mice have shown similar NMJ denervation with aging [27][28][29].

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Section: Mutant Sod1 Transgenic Mouse Model Of Falsmentioning

confidence: 99%

“…Similarly, it has also been suggested that the fast muscles and motor units in both ALS patients and mouse models are preferentially denervated [52,[54][55][56][57]. It has been shown that expression of either exogenous MyoD or myogenin in skeletal muscle can shift the muscle phenotype.…”

Section: Role Of Myod Myogenin and Mir-206 In Nmj Maintenancementioning

confidence: 99%

Mechanisms of Muscle Denervation in Aging: Insights from a Mouse Model of Amyotrophic Lateral Sclerosis

Park¹

2015

Aging and disease

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“…Some studies have found that motor fatigue does not correlate well with maximum force (MVIC) (5,6). In this context, the isolated measurement of muscle strength is insufficient and may underestimate fatigue.…”

Section: Introductionmentioning

confidence: 99%

Quantitative assessment of motor fatigue: Normative values and comparison with prior‐polio patients

Meldrum

Cahalane

Conroy

et al. 2007

Amyotrophic Lateral Sclerosis

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Motor fatigue is a common complaint of polio survivors and has a negative impact on activities of daily living. The aim of this study was to establish a normative database for hand grip strength and fatigue and to investigate differences between prior-polio subjects and normal controls. Static and dynamic hand grip fatigue and maximum voluntary isometric contraction (MVIC) of hand grip were measured in subjects with a prior history of polio (n = 44) and healthy controls (n = 494). A normative database of fatigue was developed using four indices of analysis. Compared with healthy controls, subjects with prior polio had significantly reduced hand grip strength but developed greater hand grip fatigue in only one fatigue index. Quantitative measurement of fatigue in the prior-polio population may be useful in order to detect change over time and to evaluate treatment strategies.

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“…For ROM, only a trend towards better improvement to active rTMS was found. MVIC represent a robust motor outcome measures that has been used to assess weakness and fatigue in ALS [24]. Isokinetic assessment was used because it is reliable, has good test-retest reliability in neurological conditions and is considered more specific to human physical performance than MVIC [19,20].…”

Section: Discussionmentioning

confidence: 99%

The effect of repetitive transcranial magnetic stimulation on motor performance, fatigue and quality of life in amyotrophic lateral sclerosis

Zanette

Forgione

Manganotti

et al. 2008

Journal of the Neurological Sciences

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Quantitative assessment of motor fatigue in amyotrophic lateral sclerosis

Cited by 51 publications

References 13 publications

Mechanisms of Muscle Denervation in Aging: Insights from a Mouse Model of Amyotrophic Lateral Sclerosis

Mechanisms of Muscle Denervation in Aging: Insights from a Mouse Model of Amyotrophic Lateral Sclerosis

Quantitative assessment of motor fatigue: Normative values and comparison with prior‐polio patients

The effect of repetitive transcranial magnetic stimulation on motor performance, fatigue and quality of life in amyotrophic lateral sclerosis

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