Decremental responses to repetitive nerve stimulation (RNS) in motor neuron disease

Iwanami, Tomoko; Sonoo, Masahiro; Hatanaka, Y.; Hokkoku, Keiichi; Oishi, Chizuko; Shimizu, Teruo

doi:10.1016/j.clinph.2011.05.019

Cited by 43 publications

(68 citation statements)

References 23 publications

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“…Abnormal decremental motor responses on RNS are not specific to myasthenia gravis as many studies have demonstrated neuromuscular transmission impairment in patients with ALS (Bernstein & Antel, 1981; Henderson, Baumann, Hutchinson, & McCombe, 2009; Henderson & Daube, 2004; Iwanami et al., 2011; Killian et al., 1994; Kim, Park, Kim, & Sunwoo, 2011; Mulder et al., 1959; Stålberg, 1982; Wang, De Pasqua, Gérard, & Delwaide, 2001; Yamashita et al., 2012). Abnormal decremental responses have been observed more frequently in proximal than distal muscles (Iwanami et al., 2011; Killian et al., 1994).…”

Section: Discussionmentioning

confidence: 99%

“…Abnormal decremental responses have been observed more frequently in proximal than distal muscles (Iwanami et al., 2011; Killian et al., 1994). In one study, greater decrement was observed in a group of rapidly progressive disease (6 patients) than slowly progressive disease (8 patients) (Bernstein & Antel, 1981).…”

Section: Discussionmentioning

confidence: 99%

“…This results in a low safety factor and insecure neuromuscular transmission. This is supported by findings from other studies that showed a more frequent and greater size of decrement in atrophic muscles (Denys & Norris, 1979; Mulder et al., 1959) as well as in muscles with small CMAP (Henderson et al., 2009; Iwanami et al., 2011; Killian et al., 1994; Wang et al., 2001). In a previous study, the size of decrement predicted further drop in CMAP amplitude, indicating a possible role for immature reinnervation and reduced safety factor in the pathogenesis (Wang et al., 2001).…”

Section: Discussionmentioning

confidence: 99%

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Decremental responses in patients with motor neuron disease

2017

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ObjectiveInvolvement of the neuromuscular junction (NMJ) in amyotrophic lateral sclerosis (ALS) has been reported and is increasingly recognized as an important pathophysiological aspect. The relationship between decrement and clinical measures for possible application as a biomarker has not been comprehensively explored.MethodsWe performed routine repetitive nerve stimulation (RNS) of three nerves on patients with ALS. We captured measures of muscle strength, grip strength, fatigability, and calculated slow vital capacity (SVC) rates of change assessing for associations.ResultsIn 42 subjects, 210 muscles were studied. Negative correlation was found between the percentage of decrement and compound muscle action potential (CMAP) amplitude. Approximately half of the patients with hand weakness did not have decrement. There was no significant correlation between decrement and handgrip fatigue, SVC < 80% predicted, or more rapid worsening of SVC over time.ConclusionsAbnormal decremental responses are well described in ALS. We report that the degree of decremental response does not correlate with the degree of weakness. Abnormal decrement is only rarely present in nerve–muscle pairs with normal motor power. Our findings did not support a correlation between abnormal decrement and clinical measures suggesting that RNS may not be useful as a biomarker to monitor ALS progression.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Decremental responses in patients with motor neuron disease

2017

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“…CMAP amplitude was determined by the first stimulus, and CMAP decrement was evaluated by the percentage decrease in the peak-to-peak amplitude of the CMAP from the first to the fifth response. Based on the previous study, they found that the decremental phenomenon was more frequent observed in proximal muscle for ALS patients [12]. So we only conducted slow-rate RNS in axillary and accessory nerves.…”

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, immunological and neurological examinations, i.e., tests for serum anti-acetylcholine receptor antibodies and the repetitive nerve stimulation test, did not suggest a diagnosis of myasthenia gravis. Iwanami et al have shown that repetitive nerve stimulation tests are positive in only 47% of generalized myasthenia gravis and ocular myasthenia gravis patients (7). Furthermore, approximately 12% of patients with generalized myasthenia gravis have neither acetylcholine receptor antibodies nor muscle-specific kinase antibodies (8).…”

Section: Discussionmentioning

confidence: 99%

Giant-Cell Myocarditis Complicated by Heparin-induced Thrombocytopenia

et al. 2012

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Giant-cell myocarditis is a fatal autoimmune disorder that is often associated with other autoimmune diseases. We herein describe a case of giant-cell myocarditis complicated by heparin-induced thrombocytopenia (HIT). A 71-year-old woman was admitted to our hospital due to palpitations and ptosis. Echocardiography revealed hypokinesis in the left basal ventricular walls. Heart failure gradually developed, and the condition was complicated by HIT. The patient died of cardiogenic and septic shock caused by agranulocytosis. An autopsy showed giant-cell myocarditis. When severe left ventricular dysfunction due to an unknown cause is complicated by HIT, potential diagnoses of giant-cell and other types of autoimmune myocarditis should thus be investigated.

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Decremental responses to repetitive nerve stimulation (RNS) in motor neuron disease

Cited by 43 publications

References 23 publications

Decremental responses in patients with motor neuron disease

Decremental responses in patients with motor neuron disease

Decremental Responses to Repetitive Nerve Stimulation in Amyotrophic Lateral Sclerosis

Giant-Cell Myocarditis Complicated by Heparin-induced Thrombocytopenia

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