Motor unit loss estimation by the multipoint incremental MUNE method in children with spinal muscular atrophy – A preliminary study

Gaweł, Malgorzata; Kostera‐Pruszczyk, Anna; Łusakowska, Anna; Jędrzejowska, Maria; Ryniewicz, Barbara; Lipowska, Marta; Gawel, Damian; Kamińska, Anna

doi:10.1016/j.nmd.2014.11.012

Cited by 23 publications

(18 citation statements)

References 22 publications

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“…Furthermore, multipoint incremental MUNE has shown that spinal muscular atrophy (SMA) is associated with a reduction in CMAP and MUNE and increase in measures of unit size with a lower MUNE correlating with a lower function score (Gawel et al, 2015). The lack of correlations between CMAP Scan features and clinical features in MMN patients in the current study may relate to the highly individual and variable course of MMN and the effects of IVIg on the natural history of the disorder.…”

Section: Discussionmentioning

confidence: 58%

Motor unit remodelling in multifocal motor neuropathy: The importance of axonal loss

Garg

Howells

Yiannikas

et al. 2017

Clinical Neurophysiology

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Motor unit remodelling in multifocal motor neuropathy: the importance of axonal loss, Clinical Neurophysiology (2017), doi: http://dx.doi.org/10. 1016/j.clinph.2017.07.414 This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain. Conclusions: MMN is associated with marked axonal loss which may be masked by striking re-innervation resulting in preservation of distal CMAP amplitudes. Significance: Assessment of motor unit properties should be incorporated into assessment of disease progression in MMN, given that nerve conduction studies are insensitive to motor unit remodelling.

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Section: Discussionmentioning

confidence: 58%

Motor unit remodelling in multifocal motor neuropathy: The importance of axonal loss

Garg

Howells

Yiannikas

et al. 2017

Clinical Neurophysiology

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“… 11 Our experience with motor unit number estimation (MUNE) methods started with MUNE in the Shefner's modification. 12 , 13 The method was used to measure the progress of motor neuron loss in physiological aging 14 and in diseases with motor neuron degeneration in the anterior horns of the spinal cord, as well as in spinal muscular atrophy and ALS. 15 , 16 Our studies revealed that the MUNE method was a noninvasive, easy to perform approach, and with a very low variability.…”

Section: Discussionmentioning

confidence: 99%

Does the MUNIX Method Reflect Clinical Dysfunction in Amyotrophic Lateral Sclerosis

Gaweł

Kuźma‐Kozakiewicz

2016

Medicine

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The aim of our study was to assess the usefulness of the MUNIX method in reflecting the clinical dysfunction in patients with amyotrophic lateral sclerosis (ALS), as well as to assess an intra-rater reproducibility of MUNIX. The study group consisted of a total of 15 ALS patients. The mean age of symptoms onset was 55 years, and the mean disease duration was 10 months. The muscle strength and patients’ functional status were assessed according to the Medical Research Council (MRC) and by ALS functional rating scale revised (ALSFRS-R), respectively. The MUNIX was performed in 6 muscles: abductor pollicis brevis (APB), abductor digiti minimi (ADM), biceps brachii (BB), tibial anterior (TA), extensor digitorum brevis (EDB), and abductor hallucis (AH), unilaterally, at a less affected side. Both muscle-specific and global MRC and MUNIX scores were calculated. In 11 patients, the study protocol was repeated at least twice every 3 months. An additional testing of the intra-rater reliability was performed at the first visit.There were no significant differences between MUNIX test and re-test values in the APB, ADM, BB, TA, EDB, and AH muscles (P >0.05). The highest variability of the test–retest values was found in the BB muscle (7.53%). Although there was a significant test–retest difference in the global MUNIX score (P = 0.02), the variability of the results was as low as 1.26%. The MUNIX value correlated with the muscle-specific MRC score in ABP, ADM, TA, EDB and AH (P <0.05), and the global MUNIX values correlated with global MRC scores (P <0.05). There was also a significant correlation between the global MUNIX score and the clinical dysfunction measured by the ALSFRS-R scale (P <0.05). The global MUNIX showed a higher monthly decline (4.3%) as compared with ALFRS-R (0.7%) and the MRC global score (0.5%).This study confirms that the MUNIX method is a sensitive, reliable, and accurate tool reflecting both motor dysfunction and disease progression in ALS. We have found this approach to be more reliable and technically easier in distal muscles with less atrophy and a better strength.

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“…Motor unit number estimation (MUNE) provides an assessment of axon number and the capacity for reinnervation represented by size of the average single motor unit potential (SMUP). These parameters are linked closely to pathophysiology in SMA (42, 68, 83–85). Furthermore, in comparison to clinical measures of strength and function in SMA, MUNE as an objective measure is not limited by effort, fatigue, contractures, or developmental stage.…”

Section: Electrophysiological Biomarkersmentioning

confidence: 99%

Biomarkers and the Development of a Personalized Medicine Approach in Spinal Muscular Atrophy

et al. 2019

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Recent unprecedented advances in treatment for spinal muscular atrophy (SMA) enabled patients to access the first approved disease modifying therapy for the condition. There are however many uncertainties, regarding timing of treatment initiation, response to intervention, treatment effects and long-term outcomes, which are complicated by the evolving phenotypes seen in the post-treatment era for patients with SMA. Biomarkers of disease, with diagnostic, prognostic, predictive, and pharmacodynamic value are thus urgently required, to facilitate a wider understanding in this dynamic landscape. A spectrum of these candidate biomarkers, will be evaluated in this review, including genetic, epigenetic, proteomic, electrophysiological, and imaging measures. Of these, SMN2 appears to be the most significant modifier of phenotype to date, and its use in prognostication shows considerable clinical utility. Longitudinal studies in patients with SMA highlight an emerging role of circulatory markers such as neurofilament, in tracking disease progression and response to treatment. Furthermore, neurophysiological biomarkers such as CMAP and MUNE values show considerable promise in the real word setting, in following the dynamic response and output of the motor unit to therapeutic intervention. The specific value for these possible biomarkers across diagnosis, prognosis, prediction of treatment response, efficacy, and safety will be central to guide future patient-targeted treatments, the design of clinical trials, and understanding of the pathophysiological mechanisms of disease and intervention.

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Motor unit loss estimation by the multipoint incremental MUNE method in children with spinal muscular atrophy – A preliminary study

Cited by 23 publications

References 22 publications

Motor unit remodelling in multifocal motor neuropathy: The importance of axonal loss

Motor unit remodelling in multifocal motor neuropathy: The importance of axonal loss

Does the MUNIX Method Reflect Clinical Dysfunction in Amyotrophic Lateral Sclerosis

Biomarkers and the Development of a Personalized Medicine Approach in Spinal Muscular Atrophy

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