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Neurological Sciences

, Volume 40, Issue 2, pp 363–369 | Cite as

Analysis of clinical and electrophysiological characteristics of 150 patients with amyotrophic lateral sclerosis in China

  • Jie LiuEmail author
  • Xuehua Zhang
  • Xiaojun Ding
  • Min Song
  • Kexu Sui
Original Article

Abstract

Objective

To explore the relationship between the clinical onset locations and the electrophysiological characteristics of different spinal segments in amyotrophic lateral sclerosis (ALS) patients. To develop a rapid examination method using electromyographs (EMGs) for the diagnosis of ALS.

Methods

The clinical symptoms and electrodiagnostic examination results of 150 patients with definite or probable ALS were retrospectively analyzed. The patients were divided into four groups according to the primary onset locations (arms and legs onset, arms onset, legs onset, and bulbar onset groups). The differences between the onset locations and the electrophysiological characteristics revealed the lower motor neuron dysfunction in EMGs.

Results

The most affected onset location was the lower limbs (36.7%), particularly in the distal muscles. Nerve conduction showed that the sensory system was damaged in 22 patients (14.7%). The positive diagnostic rate of EMGs varied due to different onset locations. EMG abnormalities were seen in approximately 40% of asymptomatic limb muscles. Distal limb muscles showed higher electrodiagnostic sensitivity (78.4%) than proximal limb muscles. Cervical muscles showed the highest electrodiagnostic sensitivity (86.3%).

Conclusions

The sensory system in ALS patients was commonly impaired. Cervical muscles showed the highest electrodiagnostic sensitivity. The highest positive rate was generated from detecting the spinal segment onset and the special distal muscles onset ALS in our optimized test method. Through this improved examination based on the most affected individual muscles, physicians can greatly optimize the test duration and significantly reduce patient discomfort.

Keywords

Electromyography Onset location Amyotrophic lateral sclerosis Spinal segment Lower motor neuron 

Notes

Acknowledgments

I would like to thank my leader and colleagues for helpful discussions.

Compliance with ethical standards

This study was approved by the Ethics Committee of Qilu Hospital of Shandong University (Qingdao), China. Written informed consent was obtained from all patients.

Competing interests

The authors declare that they have no competing interests.

References

  1. 1.
    Rowland LP, Shneider NA (2001) Amyotrophic lateral sclerosis. N Engl J Med 344(22):1688–1700.  https://doi.org/10.1056/nejm200105313442207 CrossRefPubMedGoogle Scholar
  2. 2.
    Brooks BR (1994) El Escorial world Federation of Neurology criteria for the diagnosis of amyotrophic lateral sclerosis. Subcommittee on motor neuron diseases/amyotrophic lateral sclerosis of the world Federation of Neurology Research Group on neuromuscular diseases and the El Escorial “clinical limits of amyotrophic lateral sclerosis” workshop contributors. J Neurol Sci 124(Suppl):96–107CrossRefGoogle Scholar
  3. 3.
    de Carvalho M, Dengler R, Eisen A, England JD, Kaji R, Kimura J, Mills K, Mitsumoto H, Nodera H, Shefner J, Swash M (2008) Electrodiagnostic criteria for diagnosis of ALS. Clin Neurophysiol 119(3):497–503.  https://doi.org/10.1016/j.clinph.2007.09.143 CrossRefPubMedGoogle Scholar
  4. 4.
    Cedarbaum JM, Stambler N, Malta E, Fuller C, Hilt D, Thurmond B, Nakanishi A (1999) The ALSFRS-R: a revised ALS functional rating scale that incorporates assessments of respiratory function. BDNF ALS study group (phase III). J Neurol Sci 169(1–2):13–21CrossRefGoogle Scholar
  5. 5.
    Moschovos C, Ghika A, Karandreas N, Kyrozis A (2018) A strong linear relationship between turns/amplitude peak ratio and ratio at maximal effort. J Electromyogr Kinesiol 39:26–34.  https://doi.org/10.1016/j.jelekin.2018.01.005 CrossRefPubMedGoogle Scholar
  6. 6.
    Preston DC and Shapiro BE (2013) Detailed Nerve conduction studies Electromyography and Neuromuscular Disorders: Clinical-Electrophysiologic CorrelationsGoogle Scholar
  7. 7.
    Kiernan MC, Vucic S, Cheah BC, Turner MR, Eisen A, Hardiman O, Burrell JR, Zoing MC (2011) Amyotrophic lateral sclerosis. Lancet (London, England) 377(9769):942–955.  https://doi.org/10.1016/s0140-6736(10)61156-7 CrossRefGoogle Scholar
  8. 8.
    Karam C, Scelsa SN, Macgowan DJ (2010) The clinical course of progressive bulbar palsy. Amyotroph Lateral Scler 11(4):364–368.  https://doi.org/10.3109/17482960903513159 CrossRefPubMedGoogle Scholar
  9. 9.
    Eisen A, Swash M (2001) Clinical neurophysiology of ALS. Clin Neurophysiol 112(12):2190–2201CrossRefGoogle Scholar
  10. 10.
    de Carvalho M, Swash M (2000) Nerve conduction studies in amyotrophic lateral sclerosis. Muscle Nerve 23(3):344–352CrossRefGoogle Scholar
  11. 11.
    Mauro Mondelli MD, Alessandro Rossi MD, Stefano Passero MD, Guazzi GC (1993) Involvement of peripheral sensory fibers in amyotrophic lateral sclerosis: electro-physiological study of 64 cases. Muscle Nerve 16(2):166–172CrossRefGoogle Scholar
  12. 12.
    Heads T, Pollock M, Robertson A, Sutherland WH, Allpress S (1991) Sensory nerve pathology in amyotrophic lateral sclerosis. Acta Neuropathol 82(4):316–320CrossRefGoogle Scholar
  13. 13.
    Hammad M, Silva A, Glass J, Sladky JT, Benatar M (2007) Clinical, electrophysiologic, and pathologic evidence for sensory abnormalities in ALS. Neurology 69(24):2236–2242.  https://doi.org/10.1212/01.wnl.0000286948.99150.16 CrossRefPubMedGoogle Scholar
  14. 14.
    Guo YS, Wu DX, Wu HR, Wu SY, Yang C, Li B, Bu H, Zhang YS, Li CY (2009) Sensory involvement in the SOD1-G93A mouse model of amyotrophic lateral sclerosis. Exp Mol Med 41(3):140–150.  https://doi.org/10.3858/emm.2009.41.3.017 CrossRefPubMedPubMedCentralGoogle Scholar
  15. 15.
    Isaacs JD, Dean AF, Shaw CE, Al-Chalabi A, Mills KR, Leigh PN (2007) Amyotrophic lateral sclerosis with sensory neuropathy: part of a multisystem disorder? J Neurol Neurosurg Psychiatry 78(7):750–753.  https://doi.org/10.1136/jnnp.2006.098798 CrossRefPubMedPubMedCentralGoogle Scholar
  16. 16.
    Luigetti M, Conte A, Del Grande A, Bisogni G, Romano A, Sabatelli M (2012) Sural nerve pathology in ALS patients: a single-centre experience. Neurol Sci 33(5):1095–1099.  https://doi.org/10.1007/s10072-011-0909-5 CrossRefPubMedGoogle Scholar
  17. 17.
    Argyriou AA, Polychronopoulos P, Talelli P, Chroni E (2006) F wave study in amyotrophic lateral sclerosis: assessment of balance between upper and lower motor neuron involvement. Clin Neurophysiol 117(6):1260–1265.  https://doi.org/10.1016/j.clinph.2006.03.002 CrossRefPubMedGoogle Scholar
  18. 18.
    de Carvalho M, Scotto M, Lopes A, Swash M (2002) F-waves and the corticospinal lesion in amyotrophic lateral sclerosis. Amyotroph Lateral Scler Other Motor Neuron Disord 3(3):131–136.  https://doi.org/10.1080/146608202760834139 CrossRefPubMedGoogle Scholar
  19. 19.
    Kyuno K, Ito H, Saito T, Kowa H, Tachibana S (1996) Needle electromyography in the thoracic paraspinal muscles of motor neuron disease. No to shinkei = Brain and nerve 48(7):637–642PubMedGoogle Scholar
  20. 20.
    Preston DC, Shapiro BE, Raynor EM, Kothari MJ (1997) The relative value of facial, glossal, and masticatory muscles in the electrodiagnosis of amyotrophic lateral sclerosis. Muscle Nerve 20(3):370–372.  https://doi.org/10.1002/(sici)1097-4598(199703)20:3<370::aid-mus18>3.0.co;2-4 CrossRefPubMedGoogle Scholar
  21. 21.
    Cappellari A, Brioschi A, Barbieri S, Braga M, Scarlato G, Silani V (1999) A tentative interpretation of electromyographic regional differences in bulbar- and limb-onset ALS. Neurology 52(3):644–646CrossRefGoogle Scholar

Copyright information

© Springer-Verlag Italia S.r.l., part of Springer Nature 2018

Authors and Affiliations

  1. 1.Department of NeurologyQilu Hospital of Shandong University (Qingdao)QingdaoChina

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