The value of <scp>MUNIX</scp> as an objective electrophysiological biomarker of disease progression in chronic inflammatory demyelinating polyneuropathy

Okhovat, Ali Asghar; Advani, Soroor; Ziaadini, Bentolhoda; Panahi, Akram; Salehizadeh, Saeideh; Nafissi, Shahriar; Ashtiani, Bahram Haghi; Rajabally, Yusuf A.; Fatehi, Farzad

doi:10.1002/mus.27498

Cited by 4 publications

(1 citation statement)

References 35 publications

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“…Demonstration of axonal loss of sensory and motor fibers was proved using sural nerve biopsy [ 32 ] and neurophysiological tolls, such as motor unit number estimation (MUNE) and motor unit number index (MUNIX), that were both decreased in CIDP patients [ 13 , 33 , 34 ]. Despite clinical and neurophysiological improvement, residual alterations of nerve conduction parameters after ScIg treatment for 24 months (increased DML and reduced CMAP amplitude and area), in the absence of demyelinating features (conduction blocks and temporal dispersion), suggest an axonal degeneration process [ 35 ].…”

Section: Discussionmentioning

confidence: 99%

Neurophysiological Hallmarks of Axonal Degeneration in CIDP Patients: A Pilot Analysis

et al. 2022

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In this work, we aim to identify sensitive neurophysiological biomarkers of axonal degeneration in CIDP patients. A total of 16 CIDP patients, fulfilling the clinical and neurophysiological criteria for typical CIDP, treated with subcutaneous immunoglobulin (ScIg) (0.4 g/kg/week) were evaluated at baseline (before ScIg treatment) and after long-term treatment with ScIg (24 months) by clinical assessment scales, nerve conduction studies (NCS) and electromyography (EMG). Conventional and non-conventional neurophysiological parameters: motor unit potential (MUP) analysis, MUP thickness and size index (SI)] and interference pattern (IP) features were evaluated after long-term treatment (24 months) and compared with a population of 16 healthy controls (HC). An increase of distal motor latency (DML) and reduced compound motor action potential (CMAP) amplitude and area in CIDP patients suggest axonal damage of motor fibers, together with a significant increase of MUP amplitude, duration and area. Analysis of non-conventional MUP parameters shows no difference for MUP thickness; however, in CIDP patients, SI is increased and IP area and amplitude values are lower than HC. Despite clinical and neurophysiological improvement after ScIg treatment, neurophysiological analysis revealed axonal degeneration of motor fibers and motor unit remodeling. Correlation analysis shows that the axonal degeneration process is related to the diagnostic and therapeutic delay. MUP area and SI parameters can detect early signs of axonal degeneration, and their introduction in clinical practice may help to identify patients with the worst outcome.

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Section: Discussionmentioning

confidence: 99%

Neurophysiological Hallmarks of Axonal Degeneration in CIDP Patients: A Pilot Analysis

et al. 2022

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Contemporary challenges in the diagnosis and management of chronic inflammatory demyelinating polyneuropathy

Rajabally

2022

Expert Review of Neurotherapeutics

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Value of MUNE versus compound muscle action potential in assessing motor unit loss in patients with carpal tunnel syndrome

Abdul-Muneem,

Kaddoori

2024

Egypt J Neurol Psychiatry Neurosurg

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Background The most prevalent nerve entrapment disorder, known as carpal tunnel syndrome (CTS), is brought on by wrist-based median nerve compression. Focal demyelination progresses to axonal dysfunction as the condition worsens. In order to detect motor unit (MU) loos, this study compares two motor unit number estimation (MUNE) techniques with compound muscle action potential (CMAP) amplitude. The CMAP amplitude and MUNE of the median nerve in 137 hands of 70 neurophysiologically approved CTS patients, aged 40.27 ± 10.06 years were examined. Another 90 hands from 56 healthy volunteers who are age- and gender-matched serve the control group. Results In contrast to 192.5 and 248.5 in controls, the median nerve values of incremental and adapted multipoint stimulation (aMPS) MUNE in CTS patients were, respectively, 111 and 133 (p < 0.0001). Patients with severe CTS compared to those with mild CTS using both methods had significantly lower MUNE. MUNE values are the same regardless of gender or hand dominance. In comparison to MUNE methods (cutoff values of 106.5 and 203, respectively), CMAP amplitude had a sensitivity and specificity of more than 60% in detecting MU loss (cutoff value of 6.85 mV). The CTS grading had no effect on the CMAP amplitude. MUNE values had positive with CMAP amplitude and negative with CTS grading and Phalen test positivity. Conclusions When identifying motor nerve involvement in CTS patients, the MUNE technique is more accurate than a standard motor nerve conduction study (NCS). It was emphasized that MUNE evaluation in determining MU loss in the early stages of CTS may be helpful in diagnosis and treatment. There was no correlation between handedness and the number of MUs as determined by MUNE techniques. Both methods almost equally identify MU loss and have the same sensitivity and specificity.

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The value of MUNIX as an objective electrophysiological biomarker of disease progression in chronic inflammatory demyelinating polyneuropathy

Cited by 4 publications

References 35 publications

Neurophysiological Hallmarks of Axonal Degeneration in CIDP Patients: A Pilot Analysis

Neurophysiological Hallmarks of Axonal Degeneration in CIDP Patients: A Pilot Analysis

Contemporary challenges in the diagnosis and management of chronic inflammatory demyelinating polyneuropathy

Value of MUNE versus compound muscle action potential in assessing motor unit loss in patients with carpal tunnel syndrome

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