Assessment of the reliability of the motor unit size index (MUSIX) in single subject "round-robin" and multi-centre settings.

OBJECTIVE: The motor unit size index (MUSIX) is incorporated into the motor unit number index (MUNIX). Our objective was to assess the intra-/inter-rater reliability of MUSIX in healthy volunteers across single subject "round robin" and multi-centre settings. METHODS: Data were obtained from (i) a round-robin assessment in which 12 raters (6 with prior experience and 6 without) assessed six muscles (abductor pollicis brevis, abductor digiti minimi, biceps brachii, tibialis anterior, extensor digitorum brevis and abductor hallucis) and (ii) a multi-centre study with 6 centres studying the same muscles in 66 healthy volunteers. Intra/inter-rater data were provided by 5 centres, 1 centre provided only intra-rater data. Intra/inter-rater variability was assessed using the coefficient of variation (COV), Bland-Altman plots, bias and 95% limits of agreement. RESULTS: In the round-robin assessment intra-rater COVs for MUSIX ranged from 7.8% to 28.4%. Inter-rater variability was between 7.8% and 16.2%. Prior experience did not impact on MUSIX values. In the multi-centre study MUSIX was more consistent than the MUNIX. Abductor hallucis was the least reliable muscle. CONCLUSIONS: The MUSIX is a reliable neurophysiological biomarker of reinnervation. SIGNIFICANCE: MUSIX could provide insights into the pathophysiology of a range of neuromuscular disorders, providing a quantitative biomarker of reinnervation.

Reference values for jitter recorded by concentric needle electrodes in healthy controls: A multicenter study.

INTRODUCTION: The aim of this study was to create reference values for jitter measured with concentric needle electrodes. METHODS: Operators worldwide contributed recordings from orbicularis oculi (OO), frontalis (FR), and extensor digitorum (ED) muscles in healthy controls. Criteria for acceptable signal quality were agreed upon in advance. Fifteen or 20 recordings of acceptable quality from each muscle were required for voluntary and electrical stimulation recordings, respectively. RESULTS: Recordings from 59 to 92 subjects were obtained for each muscle and activation type. Outlier limits for mean consecutive difference and individual jitter data for voluntary activation were: OO, 31 and 45 µs; FR, 28 and 38 µs; ED, 30 and 43 µs; and for electrical stimulation they were: OO, 27 and 36 µs; FR, 21 and 28 µs; ED, 24 and 35 µs. CONCLUSION: Reference jitter values from concentric needle electrode recordings were developed from signals of defined quality while seeking to avoid creating supernormal values.

Single fiber EMG Fiber density and its relationship to Macro EMG amplitude in reinnervation.

The objective was to elucidate the relation between the Macro EMG parameters fiber density (FD) and Macro amplitude in reinnervation in the purpose to use the FD parameter as a surrogate marker for reinnervation instead of the Macro amplitude. Macro EMG with FD was performed in 278 prior polio patients. The Biceps Brachii and the Tibialis anterior muscles were investigated. FD was more sensitive for detection of signs of reinnervation but showed lesser degree of abnormality than the Macro amplitude. FD and Macro MUP amplitude showed a non-linear relation with a great variation in FD for given Macro amplitude level. The relatively smaller increase in FD compared to Macro amplitude in addition to the non-linear relationship between the FD and the Macro amplitude regarding reinnervation in prior polio can be due to technical reasons and muscle fiber hypertrophy. The FD parameter has a relation to Macro MUP amplitude but cannot alone be used as a quantitative marker of the degree of reinnervation.

The standard concentric needle cannula cannot replace the Macro EMG electrode.

OBJECTIVE: To establish the usefulness of the single use and affordable standard concentric EMG electrode as a substitute for the expensive standard macro electrode. METHODS: Macro EMG performed with macro electrode is compared with recordings from the uninsulated cannula of a standard EMG electrode at two different recording depths in the tibialis anterior muscle. This was performed both in muscles with signs of collateral reinnervation and without. RESULTS: The amplitude of the motor units recorded with the uninsulated concentric needle cannula were lower for the deeply recorded motor units compared to motor unit potential (MUP) amplitudes recorded with the standard macro electrode. The deeply recorded concentric needle (CN) cannula recorded MUPs amplitudes were also lower than superficially recorded CN cannula MUPs. The standard Macro EMG signals show no difference between deeply and superficially recorded motor units. CONCLUSION: The uninsulated cannula of the concentric needle electrode cannot replace the standard Macro EMG electrode due to technical reasons, probably from different effects of shunting of the bare cannula in deep vs. superficially recorded motor units. SIGNIFICANCE: The standard CN electrode could not be used as substitute for the standard Macro EMG needle.

Motor Unit Number Index (MUNIX): a novel neurophysiological marker for neuromuscular disorders; test-retest reliability in healthy volunteers.

OBJECTIVE: To investigate the intra-rater and inter-rater test-retest reliability of the Motor Unit Number Index (MUNIX) in healthy subjects in a multicentre setting. METHODS: Six study centres applied the MUNIX technique in 66 healthy subjects. Five to six muscles (biceps brachii, BB; abductor digiti minimi, ADM; abductor pollicis brevis, APB; tibialis anterior, TA; extensor digitorum brevis, EDB and abductor hallucis, AH) were measured in each volunteer four times by two independent examiners. RESULTS: The method was easy to perform and well tolerated. The intraclass correlation coefficient (ICC) varied between centres and muscles. Intra-rater reliability was greatest for the AH (ICC 0.83) and EDB (ICC 0.81). Inter-rater reliability was greatest for the AH (ICC 0.69) and ADM muscles (ICC 0.69). The most critical muscle was the APB muscle (ICC 0.52, total variability). This was mostly due to variability in the compound muscle action potential (CMAP) measurements. MUNIX values of the APB, ADM and TA fell into the same range as in other motor unit number estimation (MUNE) studies. CONCLUSION: MUNIX measurements in multiple muscles show good inter- and intra-rater reliability in healthy subjects. CMAP amplitude must be controlled to optimize reliability. SIGNIFICANCE: Results suggest that MUNIX could serve as a reliable marker for motor neuron loss in diseases like amyotrophic lateral sclerosis.

Macro electromyography and motor unit number index in the tibialis anterior muscle: differences and similarities in characterizing motor unit properties in prior polio.

Our objective was to establish the usefulness of the noninvasive method of the motor unit number index (MUNIX) in a large muscle and to study how macro electromyography (EMG) and MUNIX complement each other in describing the motor units (MUs) in prior polio. MUNIX and macro EMG were performed in 48 tibialis anterior muscles in 33 prior polio patients. In addition, the reproducibility of MUNIX was investigated. It is shown that MUNIX can be used to characterize MUs with high reproducibility, even in a large muscle. As judged by MUNIX values, the patients had a 25% reduction of motor neurons, whereas the macro EMG indicated a loss of 60% of the neurons. Macro EMG showed more pronounced changes compared with control material than the MUNIX. One of the reasons for this finding may be the difference in MU populations studied with the two methods.

Report of five rare or previously unknown amyloidogenic transthyretin mutations disclosed in Sweden.

The number of amyloidogenic transthyretin (TTR) mutations described in the literature is more than 100. However, for several mutations, the phenotype has been described in a few individuals only; thus, the knowledge of the clinical course and the outcome after therapeutical interventions such as liver transplantation is limited. We describe the phenotype associated with five rare amyloidogenic TTR mutations that lately were discovered in Sweden: ATTR Val30Leu, Ala45Ser, Leu55Gln, Gly57Arg and Tyr69His of which ATTR Gly57Arg is previously unknown. The symptoms at onset differed, but cardiomyopathy and peripheral neuropathy were observed in all except the ATTR Tyr69His mutation. Likewise, carpal tunnel syndrome was found or had been present in all cases except the case with the ATTR Val30Leu mutation. The phenotype of the ATTR Tyr69His mutation was characterised by oculo-meningeal symptoms with seizures and a steadily progressing dementia, symptoms rarely found in ATTR amyloidosis, but similar to those previously described for this mutation, where all cases appear to originate from one Swedish family. Two patients with the ATTR Leu55Gln and Ala45Ser mutations have been subjected to liver transplantation, but echocardiographic examination has revealed an increasing cardiomyopathy after transplantation in both cases, the ATTR Leu55Gln patient succumbed 2 years after transplantation from progressive disease.

Reflexes in prior polio and their relation to weakness and anterior horn cell loss.

The aim of this study was to evaluate the reflex pattern in patients with prior polio and to relate these findings to the degree of anterior horn cell (AHC) involvement and loss of muscle force. Twenty-five prior polio subjects were investigated with electromyography (EMG), force testing and reflex studies, which included the patellar and Achilles reflex, H-reflex, T-response and interlimb reflex (ILR). The clinical reflexes, H/M-ratio and T-response amplitude at rest were positively correlated with force and negatively correlated with the degree of AHC loss. The H/M-ratio was decreased compared with age matched controls. ILR was present in 68% of the prior polio patients but did not exist in controls. The presence of the ILR was not correlated with the degree of AHC loss or force. The reflex studies gave two main findings. The first is reduced excitability of monosynaptic connections in the motor neuron pool, which is related to weakness. The other is the presence of ILR as an indicator of interneuronal hyper-excitability, which is not related to weakness.

How to interpret normal electromyographic findings in patients with an alleged history of polio.

OBJECTIVE: In some patients with a history of polio, the electromyography is normal, not showing the typical neurogenic signs. The aim of this study was to explain the normal findings in electromyography, especially in paralytic polio. DESIGN: Retrospective study. SUBJECTS/METHODS: Concentric needle electromyography, macro electromyography (including single fibre electromyography) and neurography were performed in various combinations in 688 patients with an alleged history of polio. RESULTS: Thirty-five patients with paralytic polio had normal or minimally abnormal neurophysiology. In 6 patients the diagnosis of polio was rejected and was instead found to be other diagnoses. Three patients had a very atypical history. Of the 26 with possible paralytic polio, 17 showed a strong suspicion of previous paralytic polio without any neurophysiological signs of degeneration of the anterior horn cells. CONCLUSION: If neurophysiological findings are normal in patients with a history of polio, the original diagnosis may be incorrect. However, the absence of electromyography changes does not entirely exclude a previous history of polio with transient functional loss without degeneration of anterior horn cells vulnerable for later functional impairment.

Changes in macro electromyography over time in patients with a history of polio: a comparison of 2 muscles.

OBJECTIVE: To investigate whether changes over time are different in a weight-bearing leg muscle than in a less heavily used arm muscle. DESIGN: Prospective study. SETTING: University hospital laboratory. PARTICIPANTS: Twenty-three patients with a history of poliomyelitis. INTERVENTION: Two investigations were performed 5 years apart, using macro electromyography and the patients' own assessments of symptoms in the tibial anterior and the biceps brachii muscles. Test-retest of macro electromyography was performed in controls and in patients with old polio. MAIN OUTCOME MEASURES: Macro motor unit potential (MUP) and symptoms in the tibial anterior and biceps brachii over time. RESULTS: The macro MUP amplitude increased by 24% (P<.05) in the tibial anterior but was unchanged in the biceps brachii muscle. CONCLUSIONS: An increase in the macro MUP amplitude of the tibial anterior muscle, but not of the biceps brachii, most likely indicates a more pronounced ongoing denervation-reinnervation process over time in the tibial anterior. This difference could be activity dependent, but other factors cannot be excluded.