Spontaneous activity in electromyography may differentiate certain benign lower motor neuron disease forms from amyotrophic lateral sclerosis

Jokela, Manu; Jääskeläinen, Satu K.; Sandell, Satu; Palmio, Johanna; Penttilä, Sini; Saukkonen, Anna-Maija; Soikkeli, Raija; Udd, Bjarne

doi:10.1016/j.jns.2015.06.002

Cited by 6 publications

(3 citation statements)

References 28 publications

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“…Previous studies have demonstrated differences between SBMA and ALS in sensory action potential amplitudes, needle electromyography manifestations, cortical excitability testing, serum creatine kinase levels, and clinical presentations ( Ferrante and Wilbourn, 1997 ; Hirota et al, 2000 ; Vucic and Kiernan, 2008 ; Chahin and Sorenson, 2009 ; Hama et al, 2012 ; Jokela et al, 2015 ). It has been suggested that the frequencies of giant F-waves and repeater F-waves are significantly increased in patients with anterior horn cell disorders compared with the healthy subjects ( Petajan, 1985 ; Ibrahim and el-Abd, 1997 ).…”

Section: Introductionmentioning

confidence: 98%

Differences in F-Wave Characteristics between Spinobulbar Muscular Atrophy and Amyotrophic Lateral Sclerosis

Jia

Liu

Guan

et al. 2016

Front. Aging Neurosci.

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There is limited data on the differences in F-wave characteristics between spinobulbar muscular atrophy (SBMA) and lower motor neuron dominant (LMND) amyotrophic lateral sclerosis (ALS). We compared the parameters of F-waves recorded bilaterally from the median, ulnar, tibial, and deep peroneal nerves in 32 SBMA patients, 37 patients with LMND ALS, and 30 normal controls. The maximum F-wave amplitudes, frequencies of giant F-waves, and frequencies of patients with giant F-waves in all nerves examined were significantly higher in the SBMA patients than in the ALS patients and the normal controls. The mean F-wave amplitude, maximum F-wave amplitude, frequency of giant F-waves, and frequency of patients with giant F-waves in the median and deep peroneal nerves were comparable between the ALS patients and normal controls. Giant F-waves were detected in multiple nerves and were often symmetrical in the SBMA patients compared with the ALS patients. The number of nerves with giant F-waves seems to be the most robust variable for differentiation of SBMA from ALS, with an area under the curve of 0.908 (95% CI: 0.835–0.982). A cut-off value of the number of nerves with giant F-waves (≥3) for diagnosing SBMA showed high sensitivity and specificity: 85% sensitivity and 81% specificity vs. ALS patients. No significant correlations were found between the pooled frequency of giant F-waves and disease duration in the SBMA (r = 0.162, P = 0.418) or ALS groups (r = 0.107, P = 0.529). Our findings suggested that F-waves might be used to discriminate SBMA from ALS, even at early stages of disease.

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Section: Introductionmentioning

confidence: 98%

Differences in F-Wave Characteristics between Spinobulbar Muscular Atrophy and Amyotrophic Lateral Sclerosis

Jia

Liu

Guan

et al. 2016

Front. Aging Neurosci.

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“…This probably also explains why the standardized (per the 2013 European Standard Population) incidence of Frontotemporal Lobar Degeneration (FTD) is so high in northern Savo (7.01/100,000) and lower (4.16/100,000), although still relatively high (the estimated annual incidence rate in Europe being 2.36/100,000), in northern Ostrobothnia [ 63 ]. Overlapping just north of the ALS hotspot, north Karelia ( Figure 3 ) is an eastern high-incidence area for another motor neuron disorder that must be considered in the differential diagnosis of ALS, namely Spinal muscular atrophy, Jokela type (SMAJ, previously called Late-Onset Spinal Motor Neuronopathy or LOSMoN) [ 64 , 65 , 66 ]. SMAJ is caused by a dominant mutation in CHCHD10, and before it was identified, these cases were probably diagnosed as slowly progressing ALS and confounded epidemiological studies.…”

Section: High Risk and Even Higher Riskmentioning

confidence: 99%

Adult-Onset Neuroepidemiology in Finland: Lessons to Learn and Work to Do

Sipilä

2023

JCM

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Finland is a relatively small genetic isolate with a genetically non-homogenous population. Available Finnish data on neuroepidemiology of adult-onset disorders are limited, and this paper describes the conclusions that can be drawn and their implications. Apparently, Finnish people have a (relatively) high risk of developing Unverricht-Lundborg disease (EPM1), Multiple Sclerosis (MS), Amyotrophic Lateral Sclerosis (ALS), Spinal muscular atrophy, Jokela type (SMAJ) and adult-onset dystonia. On the other hand, some disorders, such as Friedreich’s ataxia (FRDA) and Wilson’s disease (WD), are almost absent or completely absent in the population. Valid and timely data concerning even many common disorders, such as stroke, migraine, neuropathy, Alzheimer’s disease and Parkinson’s disease, are unavailable, and there are virtually no data on many less-common neurological disorders, such as neurosarcoidosis or autoimmune encephalitides. There also appear to be marked regional differences in the incidence and prevalence of many diseases, suggesting that non-granular nationwide data may be misleading in many cases. Concentrated efforts to advance neuroepidemiological research in the country would be of clinical, administrative and scientific benefit, but currently, all progress is blocked by administrative and financial obstacles.

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“…Em seguida, a sequência utilizada para a exposição dos dados. Quando se trabalha com eletromiografia de superfície, diferentes padrões de anormalidades em distintas desordens dos neurônios motores podem ser descobertos, sendo um importante mecanismo de prognóstico e ajuda na realização de outros exames ou testes genéticos (JOKELA et al, 2015). A combinação dos exames de eletromiografia de superfície e ultrassonografia torna-se importante para avaliação, detecção e diagnóstico de doenças neurofisiológicas, do que se fossem utilizados separadamente (GRIMM et al, 2015).…”

Section: Resultsunclassified

Efeito da esclerose lateral amiotrófica na atuação do sistema estomatognático - análises eletromiográfica, ultrassonográfica, força de mordida e eficiência mastigatória

Gonçalves¹

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Spontaneous activity in electromyography may differentiate certain benign lower motor neuron disease forms from amyotrophic lateral sclerosis

Cited by 6 publications

References 28 publications

Differences in F-Wave Characteristics between Spinobulbar Muscular Atrophy and Amyotrophic Lateral Sclerosis

Differences in F-Wave Characteristics between Spinobulbar Muscular Atrophy and Amyotrophic Lateral Sclerosis

Adult-Onset Neuroepidemiology in Finland: Lessons to Learn and Work to Do

Efeito da esclerose lateral amiotrófica na atuação do sistema estomatognático - análises eletromiográfica, ultrassonográfica, força de mordida e eficiência mastigatória

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