Repetitive nerve stimulation as a diagnostic aid for distinguishing cervical spondylotic amyotrophy from amyotrophic lateral sclerosis

Zheng, Chaojun; Jin, Xiang; Zhu, Yu; Lu, Feizhou; Jiang, Jianyuan; Xia, Xinlei

doi:10.1007/s00586-017-5060-4

Cited by 23 publications

(23 citation statements)

References 33 publications

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“…The patients in the positive decrement group had lower ALSFRS-R scores compared with those in the decremental negative group, suggesting that the former were worse. Some studies have shown that the disease progressed more rapidly in ALS patients with a positive decrement (4,8,13,14), but others contradicted this (5,7,(15)(16)(17). If the RNS decrement responses were not related to the disease progression, the interpretation of the mechanism of decrement responses, the early neural reinnervation, may need to be corrected (5).…”

Section: Discussionmentioning

confidence: 99%

Can the Large-Scale Decrement in Repetitive Nerve Stimulation Be Used as an Exclusion Criterion for Amyotrophic Lateral Sclerosis?

Shang

Chu

2020

Front. Neurol.

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Objective: The objectives of this work were to identify the characteristics of repetitive nerve stimulation (RNS) in patients with amyotrophic lateral sclerosis (ALS) and further verify the electrophysiological exclusion criteria of ALS.Methods: A total of 150 patients with ALS who were admitted to were enrolled. Clinical and electrophysiological data of the enrolled patients were collected. The differences in the amplitudes of the compound muscle action potential (CMAP) between the trapezius muscle (Trap) and the abductor digiti minimi (ADM) in low-frequency RNS were compared. Furthermore, we analyzed the associations between decremental responses and gender, onset age, duration of disease, onset site, Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised (ALSFRS-R), disease progression rate, and CMAP amplitude.Results: A significant decrement (≥20%) in at least one muscle was observed in 11.3% of the ALS patients, while decrements (≥10%) in at least one muscle were observed in 41.3%. The decremental percentage in the trapezius muscle was significantly higher than that in the abductor digiti minimi (P < 0.001). The onset age, duration of disease, onset site, and disease progression rate did not affect decremental responses. The decremental responses in RNS were more significant in ALS patients with low ALSFRS-R scores (P = 0.01). Moreover, there was a positive linear correlation between the CMAP amplitude and the decremental percentage of Trap and ADM in ALS patients.Conclusions: CMAP decremental responses in RNS were common in ALS patients, suggesting abnormalities of neuromuscular junctions (NMJs). It is worthy of further discussion whether to consider a decrement >20% in RNS as a diagnostic exclusion criterion for ALS.

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Section: Discussionmentioning

confidence: 99%

Can the Large-Scale Decrement in Repetitive Nerve Stimulation Be Used as an Exclusion Criterion for Amyotrophic Lateral Sclerosis?

Shang

Chu

2020

Front. Neurol.

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“…We divide the ALS patients into RNS positive (RNS+) and RNS negative (RNS-) group by whether percentage decrease of Del or Trap is greater or less than -10% [13]. The diagnostic delay time is statistically shorter and ALS- FRS-r score is statistically lower in RNS+ group than RNS-group (p = 0.002, 0.000).…”

Section: Resultsmentioning

confidence: 99%

“…So we only conducted slow-rate RNS in axillary and accessory nerves. A decremental response exceeding 10% was considered abnormal in this study according to the conventional criterion suggested by the AAEM Quality Assurance Committee [13].…”

Section: Methodsmentioning

confidence: 99%

Decremental Responses to Repetitive Nerve Stimulation in Amyotrophic Lateral Sclerosis

Jin

et al. 2018

Eur Neurol

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Introduction: To illuminate the mechanism of neuromuscular junction involvement by analyzing the features of a slow-rate repetitive nerve simulation (RNS) and EMG in amyotrophic lateral sclerosis (ALS) patients. Methods: We retrospectively analyzed relationship between clinical features and the decremental response in RNS in 184 sporadic ALS patients. Results: (1) 44.3 and 43.5% of compound muscle action potentials (CMAP) decrement were more than –10% in deltoid muscle (Del) and Trap; (2) ALS patients were divided into RNS positive (RNS+) and RNS negative (RNS–) group according to decremental percentage of RNS greater or less than –10%. The diagnostic delay time was shorter and ALSFRS-r score was lower in RNS+ than RNS– group (p < 0.05), progression rate had no difference. The incidence of RNS+ decrement was higher in cervical enlargement onset and definite diagnostic degrees (p < 0.05); (3) In EMG+ group, the CMAP amplitude was lower (Axillary: 3.1 ± 1.91 vs. 5.92 ± 2.896, p = 0.000; Accessory: 2.68 ± 1.349 vs. 3.65 ± 1.53, p = 0.002), decremental percentage of RNS was higher (Axillary: –10.85 ± 7.508 vs. –5.43 ± 8.425, p = 0.000; Accessory: –13.11 ± 7.539 vs. –8.03 ± 5.999, p = 0.000) compared with needle EMG– group whether in Del or Trap; (4) Decremental response of RNS was positively correlated with the CMAP amplitude in Axillary and Accessory nerves (R = 0.201, p < 0.0001; R = 0.103, p < 0.0001). Conclusions: Our clinical results support the mechanism of decremental phenomenon of RNS is immature sprouts and unstable conduction by the degenerating axons in ALS patients. The more serious the axon damage, the more significant the RNS decremental response. But decremental response dose not effect disease progression.

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“…However, the pathological mechanism and the clinical implications of this phenomenon remain elusive. [ 4 5 6 7 8 9 10 ] Immature sprout, due to denervation and innervation after MN loss, is typically considered a possible mechanism. The animal experiments demonstrated the possibility of NMJs defection occurring before any loss of MNs, termed the “dying back” theory.…”

Section: Introductionmentioning

confidence: 99%

Repetitive Nerve Stimulation in Amyotrophic Lateral Sclerosis

Sun

Liu

Chen

et al. 2018

Chinese Medical Journal

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Background:Nowadays, it is widely known that decremental responses in low-frequency repetitive nerve stimulation (LF-RNS) are frequently observed in patients with amyotrophic lateral sclerosis (ALS). The pathological mechanism of this phenomenon remains unknown. This study aimed to illuminate the features of RNS in Chinese patients with ALS.Methods:Clinical and electrophysiological data of 146 probable and definite ALS patients who underwent RNS were retrospectively enrolled and analyzed. LF-RNS (3 Hz) was performed in trapezius, deltoid, abductor digiti minimi (ADM), quadriceps femoris, and tibialis anterior. High-frequency RNS (HF-RNS, 10 Hz) was performed only in ADM. The two-sample t-test and Chi-squared test were used for statistical analysis.Results:Decremental responses to LF-RNS (≥10%) in at least one muscle were detected in 83 (56.8%) of the cases and were most commonly seen in trapezius and deltoid. The incidence of decremental response was higher in patients with upper limb onset. Incremental responses to HF-RNS (≥60%) in ADM were observed in 6 (5.6%) of the cases. In 106 muscles with decremental response, 62 (57.4%) muscles had a continuous decremental pattern, more than a U-shape pattern (37 cases, 34.3%). Nineteen cases showed definite decrements in LF-RNS tests in trapezius, while no abnormalities were found in the electromyography and neurological examination of the sternocleidomastoid muscle, supplied by the accessory nerve as well.Conclusions:Decremental responses in the RNS are commonly observed in ALS patients. The findings regarding the trapezius indicated that some ALS onsets could be initiated by a “dying back” process, with destruction of neuromuscular junctions (NMJs) before motor neurons. Incremental responses in the ADM implied damage of the NMJs involved both the post and presynaptic membranes.

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Repetitive nerve stimulation as a diagnostic aid for distinguishing cervical spondylotic amyotrophy from amyotrophic lateral sclerosis

Cited by 23 publications

References 33 publications

Can the Large-Scale Decrement in Repetitive Nerve Stimulation Be Used as an Exclusion Criterion for Amyotrophic Lateral Sclerosis?

Can the Large-Scale Decrement in Repetitive Nerve Stimulation Be Used as an Exclusion Criterion for Amyotrophic Lateral Sclerosis?

Decremental Responses to Repetitive Nerve Stimulation in Amyotrophic Lateral Sclerosis

Repetitive Nerve Stimulation in Amyotrophic Lateral Sclerosis

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