Peripheral nerve conduction studies and neuromuscular junction testing

Kimura, Junko

doi:10.1016/s1567-4231(04)04012-2

Cited by 4 publications

(4 citation statements)

References 92 publications

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“…In all patients, motor and sensory NCS were performed on ipsilateral median/ulnar nerves and contralateral median nerve with surface stimulation at standard sites including the axilla. 8 Compound muscle action potentials were recorded following supramaximal stimulation from abductor pollicis brevis and abductor digiti minimi muscle. Sensory nerve action potentials were obtained antidromically over second, third, and fifth digit.…”

Section: Methodsmentioning

confidence: 99%

Anterior interosseous nerve syndrome

et al. 2014

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Objective:We sought to determine lesion sites and spatial lesion patterns in spontaneous anterior interosseous nerve syndrome (AINS) with high-resolution magnetic resonance neurography (MRN).Methods:In 20 patients with AINS and 20 age- and sex-matched controls, MRN of median nerve fascicles was performed at 3T with large longitudinal anatomical coverage (upper arm/elbow/forearm): 135 contiguous axial slices (T2-weighted: echo time/repetition time 52/7,020 ms, time of acquisition: 15 minutes 48 seconds, in-plane resolution: 0.25 × 0.25 mm). Lesion classification was performed by visual inspection and by quantitative analysis of normalized T2 signal after segmentation of median nerve voxels.Results:In all patients and no controls, T2 lesions of individual fascicles were observed within upper arm median nerve trunk and strictly followed a somatotopic/internal topography: affected were those motor fascicles that will form the anterior interosseous nerve further distally while other fascicles were spared. Predominant lesion focus was at a mean distance of 14.6 ± 5.4 cm proximal to the humeroradial joint. Discriminative power of quantitative T2 signal analysis and of qualitative lesion rating was high, with 100% sensitivity and 100% specificity (p < 0.0001). Fascicular T2 lesion patterns were rated as multifocal (n = 17), monofocal (n = 2), or indeterminate (n = 1) by 2 independent observers with strong agreement (kappa = 0.83).Conclusion:It has been difficult to prove the existence of fascicular/partial nerve lesions in spontaneous neuropathies using clinical and electrophysiologic findings. With MRN, fascicular lesions with strict somatotopic organization were observed in upper arm median nerve trunks of patients with AINS. Our data strongly support that AINS in the majority of cases is not a surgically treatable entrapment neuropathy but a multifocal mononeuropathy selectively involving, within the main trunk of the median nerve, the motor fascicles that continue distally to form the anterior interosseous nerve.

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Section: Methodsmentioning

confidence: 99%

Anterior interosseous nerve syndrome

et al. 2014

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“…Together with the tests discussed above, a series of other functional measures have been widely used to assess the electrophysiological activity of motor neurons, the axon number and reinnervation potential, and the structural changes in spinal cord and muscles. For example, electromyography (EMG), compound muscle action potential (CMAP), and motor neuron number index (MUNIX) have been classically used in patients affected with neuromuscular disorders to assess the functional status of the motor unit [ 41 , 42 , 43 , 44 ]. The EMG and CMAP methods have been used for SMA diagnosis [ 43 , 45 , 46 ] and the CMAP has also been demonstrated to be a good prognostic marker [ 47 ].…”

Section: Evaluation Of Functional Outcomes In Adult Patientsmentioning

confidence: 99%

The Identification of Novel Biomarkers Is Required to Improve Adult SMA Patient Stratification, Diagnosis and Treatment

Smeriglio

Langard

Querin

et al. 2020

JPM

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Spinal muscular atrophy (SMA) is currently classified into five different subtypes, from the most severe (type 0) to the mildest (type 4) depending on age at onset, best motor function achieved, and copy number of the SMN2 gene. The two recent approved treatments for SMA patients revolutionized their life quality and perspectives. However, upon treatment with Nusinersen, the most widely administered therapy up to date, a high degree of variability in therapeutic response was observed in adult SMA patients. These data, together with the lack of natural history information and the wide spectrum of disease phenotypes, suggest that further efforts are needed to develop precision medicine approaches for all SMA patients. Here, we compile the current methods for functional evaluation of adult SMA patients treated with Nusinersen. We also present an overview of the known molecular changes underpinning disease heterogeneity. We finally highlight the need for novel techniques, i.e., -omics approaches, to capture phenotypic differences and to understand the biological signature in order to revise the disease classification and device personalized treatments.

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“…A compound muscle action potential (CMAP) is obtained by providing a supramaximal stimulus to a motor nerve. The muscle response recorded is an indirect measure of the number of intact and available motor neurons, representing total functional status of the motor unit (70, 71). Electromyography (EMG) fibrillation potentials and positive sharp waves are spontaneous action potentials recorded from a relaxed muscle and generated by denervated muscle fibers (72).…”

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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Peripheral nerve conduction studies and neuromuscular junction testing

Cited by 4 publications

References 92 publications

Anterior interosseous nerve syndrome

Anterior interosseous nerve syndrome

The Identification of Novel Biomarkers Is Required to Improve Adult SMA Patient Stratification, Diagnosis and Treatment

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

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