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Objectives: To document the utility of decremental responses in the repetitive nerve stimulation test (RNS) and spontaneous activities in needle electromyography (EMG) in the trapezius muscle for the diagnosis of amyotrophic lateral sclerosis., Methods: Subjects were retrospectively identified from our EMG database. Cervical spondylosis was represented as a disease control group. We investigated the sensitivity and specificity of RNS and EMG in the trapezius muscle and those of diagnostic criteria including the Gold Coast criteria (GCC)., Results: We reviewed 120 patients with amyotrophic lateral sclerosis and 17 patients with cervical spondylosis. "RNS or EMG" achieved the highest sensitivity (85%). The specificity was the highest for RNS (94%). Addition of RNS of the deltoid muscle achieved 98% sensitivity in the upper-limb onset amyotrophic lateral sclerosis. The sensitivity of the GCC was very high (88%)., Conclusions: Neurophysiological parameters investigated in this study having close to 100% specificities or sensitivities are useful as complements to the GCC., Competing Interests: The authors report no conflicts of interest., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Objective: The aim of this study is to establish reference values for single-fibre electromyography (SFEMG) using concentric needles in a prospective, multicentre study., Methods: Voluntary or stimulated SFEMG at the extensor digitorum communis (EDC) or frontalis (FRO) muscles was conducted in 56-63 of a total of 69 normal subjects below the age of 60years at six Japanese institutes. The cut-off values for mean consecutive difference (MCD) of individual potentials were calculated using +2.5 SD or 95% prediction limit (one-tail) of the upper 10th percentile MCD value for individual subjects., Results: The cut-off values for individual MCD (+2.5 SD) were 56.8μs for EDC-V (voluntary SFEMG for EDC), 58.8μs for EDC-S (stimulated SFEMG for EDC), 56.8μs for FRO-V (voluntary SFEMG for FRO) and 51.0μs for FRO-S (stimulated SFEMG for FRO). The false positive rates using these cut-off values were around 2%., Conclusions: The +2.5 SD and 95% prediction limit might be two optimal cut-off values, depending on the clinical question. The obtained reference values were larger than those reported previously using concentric needles, but might better coincide with conventional values., Significance: This is the first multicentre study reporting reference values for SFEMG using concentric needles. The way to determine cut-off values and the statistically correct definition of the percentile were discussed., (Copyright © 2011 International Federation of Clinical Neurophysiology. Published by Elsevier Ireland Ltd. All rights reserved.)

Objective: To report the SFEMG findings in sporadic inclusion body myopathy (S-IBM)., Methods: We have analyzed the SFEMG data in 25 patients (mean age: 63; 16 males) with S-IBM which was diagnosed by the presence of classical rimmed vacuoles in the muscle biopsy together with clinical, laboratory, and electrophysiological findings., Results: All patients had fibrillations, positive sharp waves, and small-amplitude short-duration motor unit potentials (MUPs) in the needle EMG. High-amplitude MUPs were observed in eight (32%) patients, two of whom had long-duration MUPs. SFEMG was abnormal in 17 (68%) cases: mean "mean consecutive difference (MCD)" was increased beyond the age-adjusted normal limit in 16 cases, and more than 10% of potential pairs (PP) had MCD longer than the upper normal limit of an individual MCD in one case. Mean fiber density (FD) was 2.16, with maximum FD being 4.15. Increased FD was noted in 11 (44%) cases. In four cases, more than 10% of PP had blocking, but there was no neurogenic blocking in any PP. As expected, MCD increased linearly (r=0.85) with the percentage of PP beyond the normal upper limit., Conclusions: The SFEMG findings in S-IBM are typical of the classical pattern of myopathy., Significance: Our findings support the consensus that S-IBM is a myopathy.

Objective The positive correlation between fasciculation potentials (FPs) and decremental responses in repetitive nerve stimulation test (RNS) in amyotrophic lateral sclerosis (ALS) patients has been described based on only one past study. We revisited this issue. Methods Subjects consisted of 30 prospectively-enrolled ALS patients on whom both needle EMG and RNS were conducted in the same trapezius muscle. Fasciculation potentials (FPs) were identified off-line from the restored 3-min signal. Firing rate of FPs (FR-FP) per minute was calculated from the total count of FPs of different origins. Correlations between FR-FP, decremental percentage (Decr%) and the amplitude of the initial compound muscle action potential (CMAPamp) in RNS were investigated. Results There was no correlation between FR-FP and Decr% (r = 0.03) or between FR-FP and CMAPamp (r = 0.04). A significant negative correlation was observed between CMAPamp and Decr% (r = −0.56, P < .005). Conclusion FPs are not correlated with the decremental response in RNS. Significance The underlying mechanism for FPs and decremental responses in ALS must be different and unrelated to each other. [ABSTRACT FROM AUTHOR]

• A case report of MG with inflammatory myopathy without elevation of CK levels. • Most cases of MG with inflammatory myopathy are accompanied by elevated CK levels. • In this case, only elevated myoglobin levels were observed. • Recognition of coexistence of inflammatory myopathy in patients with MG is important. Cases of myasthenia gravis with inflammatory myopathy usually show elevated creatine kinase (CK) levels. There are few case reports of myasthenia gravis with inflammatory myopathy without elevated CK levels, and clinical features and useful diagnostic methods for these patients are little known. We describe the case of a 79-year-old man with myasthenia gravis that was complicated with inflammatory myopathy without elevated CK levels and successfully treated with immunological treatment. Initially, he was diagnosed with ocular myasthenia gravis and treated with pyridostigmine, but dysphagia and weakness in the neck and bilateral upper limb without fatigability gradually developed. Needle electromyography revealed myopathic changes, and the results of muscle biopsy were consistent with inflammatory myopathy. Blood tests showed normal CK levels throughout the clinical course and elevated myoglobin levels alone. The possibility of developing inflammatory myopathy in patients with myasthenia gravis cannot be excluded, even if CK levels are normal. [ABSTRACT FROM AUTHOR]

Objective C8-dominant innervation of ulnar-innervated and T1-dominant innervation of median-innervated intrinsic hand muscles have been suggested, although less is known regarding forearm muscles. We aimed to determine myotomal innervation of the forearm muscles based on the clinical and electromyographial findings of patients with C8 or T1 lesions. Methods Medical Research Council scale and EMG findings were retrospectively reviewed in 16 patients with C8 lesions (2 postmedian sternotomy C8 plexopathy and 14 C8 radiculopathy) and 9 patients with T1-dominant lesions (8 true neurogenic thoracic outlet syndrome and 1 T1 radiculopathy). Results Clinical and EMG findings revealed T1-dominant innervation of the flexor digitorum superficialis, flexor digitorum profundus of the index finger, abductor pollicis brevis, and flexor pollicis longus muscles, and C8-dominant innervation of the flexor carpi ulnaris, flexor digitorum profundus of the little finger, and digit extensors innervated by the posterior interosseous nerve. The first dorsal interosseous, and abductor digiti minimi muscles seem to be innervated by both C8 and T1 roots. Conclusions C8-dominant innervation of ulnar-innervated muscles and T1-dominant innervation of median-innervated muscles are also evident for forearm flexor muscles. Significance Such an additional evidence for myotomal innervation will improve localization in clinical as well as electrophysiological diagnoses. [ABSTRACT FROM AUTHOR]