Motor unit number estimates and quantitative motor unit analysis in healthy subjects and patients with amyotrophic lateral sclerosis

Objective To investigate whether GSTA1, GSTO2, and GSTZ1 are relevant to an increased risk of amyotrophic lateral sclerosis (ALS) in a Chinese population. Methods In this study, 143 sporadic ALS (sALS) patients (83 men, 60 women) and 210 age- and sex-matched healthy subjects were enrolled. Blood samples were collected by venipuncture. Genomic DNA was isolated by polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP) according to the manufacturer’s instructions. The potential associations between ALS and GSTA1, GSTO2, and GSTZ1 polymorphisms were estimated using chi-squared analysis and unconditional logistic regression. Results The D allele and genotype frequencies of GSTO2 were increased in sALS patients compared with healthy subjects, indicating that the GSTO2 DD genotype was associated with an increased risk of sALS (odds ratio [OR] = 3.294, 95% confidence interval [CI] = 1.039–10.448). However, a significant association between the DD genotype and the risk of sALS was evident in men only (OR = 7.167, 95% CI = 1.381–37.202). Conclusion This study revealed that the D allele and genotype frequencies of GSTO2 were increased in sALS patients. The GSTO2 DD genotype was associated with an increased risk of sALS in men in a Chinese population.

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O25 Motor unit number estimation methods compared to quantitative motor unit potential analysis in amyotrophic lateral sclerosis

Clinical Neurophysiology ◽

10.1016/j.clinph.2017.07.036 ◽

2017 ◽

Vol 128 (9) ◽

pp. e188

Author(s):

Rikke Soegaard Kristensen ◽

Anna Bystrup Jacobsen ◽

Anders Fuglsang-Frederiksen ◽

Hatice Tankisi

Keyword(s):

Amyotrophic Lateral Sclerosis ◽

Motor Unit ◽

Estimation Methods ◽

Motor Unit Potential ◽

Potential Analysis ◽

Motor Unit Number Estimation ◽

Motor Unit Potential Analysis ◽

Lateral Sclerosis

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Motor Unit Number Estimation in Neuromuscular Disease

Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques ◽

10.1017/s0317167100008568 ◽

2008 ◽

Vol 35 (2) ◽

pp. 153-159 ◽

Cited By ~ 22

Author(s):

Omid Rashidipour ◽

K. Ming Chan

Keyword(s):

Amyotrophic Lateral Sclerosis ◽

Motor Unit ◽

Neuromuscular Disease ◽

Muscular Atrophy ◽

Neuromuscular Diseases ◽

Neuronal Loss ◽

Response To Treatment ◽

Motor Unit Number Estimation ◽

Electrophysiological Method ◽

Lateral Sclerosis

Motor unit number estimation (MUNE) is an electrophysiological method designed to quantify motor unit loss in target muscles of interest. Most of the techniques are noninvasive and are therefore well suited for longitudinal monitoring. In this brief review, we describe the more commonly used techniques and their applications in amyotrophic lateral sclerosis, poliomyelitis, spinal muscular atrophy and hereditary sensorimotor neuropathies. Findings in some of these studies offer important pathophysiological insights. Since conventional electrophysiologic methods are not sensible measures of motor neuronal loss, MUNE could play a potentially important role in the diagnosis, monitoring of disease progression and response to treatment in neuromuscular diseases in which motor unit loss is a major feature.

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EMG and motor unit estimation in amyotrophic lateral sclerosis

Electroencephalography and Clinical Neurophysiology ◽

10.1016/0013-4694(96)80274-4 ◽

1996 ◽

Vol 98 (3) ◽

pp. P12-P13

Author(s):

J.H. Petajan

Keyword(s):

Amyotrophic Lateral Sclerosis ◽

Motor Unit ◽

Lateral Sclerosis

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Monitoring respiratory muscle strength assists in early diagnosis of respiratory dysfunction as opposed to the isolated use of pulmonary function evaluation in amyotrophic lateral sclerosis

Arquivos de Neuro-Psiquiatria ◽

10.1590/s0004-282x2013000300004 ◽

2013 ◽

Vol 71 (3) ◽

pp. 146-152 ◽

Cited By ~ 6

Author(s):

Guilherme Fregonezi ◽

Palomma Russelly Saldanha Araújo ◽

Tathiana Lindemberg Ferreira Macêdo ◽

Mario Emilio Dourado Junior ◽

Vanessa Regiane Resqueti ◽

...

Keyword(s):

Amyotrophic Lateral Sclerosis ◽

Muscle Strength ◽

Early Diagnosis ◽

Pulmonary Function ◽

Healthy Subjects ◽

Respiratory Muscle ◽

Function Evaluation ◽

Respiratory Muscle Strength ◽

Respiratory Dysfunction ◽

Lateral Sclerosis

ObjectiveIt was study the relationship between respiratory muscle strength and forced vital capacity (FVC) in patients with amyotrophic lateral sclerosis (ALS) versus healthy subjects.MethodsPulmonary function and respiratory muscle strength [maximal inspiratory (PImax), maximal expiratory (PEmax) and sniff nasal inspiratory pressure (SNIP)] were assessed in patients with ALS and healthy subjects, matched using cutoffs established in the literature for impaired pulmonary function and respiratory muscle weakness.ResultsTwenty-eight ALS patients and 28 healthy subjects were studied. We found sensitivity and specificity for PImax, PEmax and SNIP of 75/58%, 81/67% and 75/67%. The Receiver Operating Characteristic curve (ROC curve) indicated that the variables PImax, PEmax and SNIP can identify differences in respiratory muscle strength between ALS and healthy individuals at 0.89, 0.9 and 0.82, respectively. A positive correlation was recorded between FVC (%) versus SNIP, PImax and PEmax.ConclusionIn ALS, monitoring respiratory muscle strength assists in early diagnosis of respiratory dysfunction as opposed to the isolated use of FVC.

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