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Time of symptoms beyond the bulbar region predicts survival in bulbar onset amyotrophic lateral sclerosis

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Abstract

Background

Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease. Spreading pattern and time interval of spreading are getting more and more attention. The aim of present study was to investigate spreading pattern in bulbar onset ALS patients and to explore the relationship between time interval of spreading and survival.

Methods

ALS patients with bulbar onset diagnosed at Chinese PLA General Hospital from January 2015 to December 2018 were recruited. Clinical features including gender, onset age, diagnostic delay, the second involved region (SIR), time of symptoms beyond the bulbar region, forced vital capacity (FVC), ALSFRS-R score, electromyography results, and survival time were retrospectively collected.

Results

A total of 96 bulbar onset ALS patients were collected. Overall patients showed female predominance. Median age at onset was 56 years. Median diagnostic delay was 8.5 months. Median time of symptoms beyond the bulbar region (TBBR) was 7 months. Median ALSFRS-R score at baseline was 40. Fifty-six (58.3%) patients’ SIR were upper limb, 6 (6.3%) patients’ SIR were lower limb, 3 (3.1%) patients’ SIR were upper and lower limbs, and 5 (5.2%) patients’ SIR were thoracic region. Twenty-six (27.1%) patients did not report SIR. The median survival time of patients with TBBR ≥ 7 months was significantly longer than that with TBBR < 7 month. Multivariate Cox regression showed that onset age and TBBR were prognostic factors.

Conclusions

In bulbar onset ALS patients, cervical region is the second most common SIR. TBBR is an independent prognostic factor.

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References

  1. Brooks BR et al (2000) El Escorial revisited: revised criteria for the diagnosis of amyotrophic lateral sclerosis. Amyotroph Lateral Scler Other Motor Neuron Disord 1(5):293–299

    Article  CAS  Google Scholar 

  2. Gromicho M et al (2020) Spreading in ALS: the relative impact of upper and lower motor neuron involvement. Ann Clin Transl Neurol 7(7):1181–1192

    Article  Google Scholar 

  3. Walhout R et al (2018) Patterns of symptom development in patients with motor neuron disease. Amyotroph Lateral Scler Frontotemporal Degener 19(1–2):21–28

    Article  Google Scholar 

  4. McAlary L, Yerbury JJ, Cashman NR (2020) The prion-like nature of amyotrophic lateral sclerosis. Prog Mol Biol Transl Sci 175:261–296

    Article  CAS  Google Scholar 

  5. Frost B, Diamond MI (2010) Prion-like mechanisms in neurodegenerative diseases. Nat Rev Neurosci 11(3):155–159

    Article  CAS  Google Scholar 

  6. Pradat PF, Kabashi E, Desnuelle C (2015) Deciphering spreading mechanisms in amyotrophic lateral sclerosis: clinical evidence and potential molecular processes. Curr Opin Neurol 28(5):455–461

    Article  Google Scholar 

  7. Tortelli R et al (2016) Time to generalization and prediction of survival in patients with amyotrophic lateral sclerosis: a retrospective observational study. Eur J Neurol 23(6):1117–1125

    Article  CAS  Google Scholar 

  8. Tortelli R et al (2016) Time to generalisation as a predictor of prognosis in amyotrophic lateral sclerosis: table 1. J Neurol Neurosurg Psychiatry 87(6):678–679

    Article  Google Scholar 

  9. Liao SJ et al (2020) The rostral to caudal gradient of clinical and electrophysiological features in sporadic amyotrophic lateral sclerosis with bulbar-onset. J Int Med Res 48(9):300060520956502

    Article  Google Scholar 

  10. Turner MR et al (2010) The diagnostic pathway and prognosis in bulbar-onset amyotrophic lateral sclerosis. J Neurol Sci 294(1–2):81–85

    Article  Google Scholar 

  11. Vasta R et al (2021) Neck flexor weakness at diagnosis predicts respiratory impairment in amyotrophic lateral sclerosis. Eur J Neurol 28(4):1181–1187

    Article  Google Scholar 

  12. Miller RG et al (1988) Prognostic value of electrodiagnosis in Guillain-Barré syndrome. Muscle Nerve 11(7):769–774

    Article  CAS  Google Scholar 

  13. Rubin DI (2019) Normal and abnormal spontaneous activity. Handb Clin Neurol 160:257–279

    Article  Google Scholar 

  14. Shayya L et al (2018) Distal predominance of electrodiagnostic abnormalities in early-stage amyotrophic lateral sclerosis. Muscle Nerve 58(3):389–395

    Article  CAS  Google Scholar 

  15. de Carvalho M et al (2008) Electrodiagnostic criteria for diagnosis of ALS. Clin Neurophysiol 119(3):497–503

    Article  Google Scholar 

  16. Chio A et al (2011) Phenotypic heterogeneity of amyotrophic lateral sclerosis: a population based study. J Neurol Neurosurg Psychiatry 82(7):740–746

    Article  Google Scholar 

  17. Raymond J et al (2019) Clinical characteristics of a large cohort of US participants enrolled in the National Amyotrophic Lateral Sclerosis (ALS) Registry, 2010–2015. Amyotroph Lateral Scler Frontotemporal Degener 20(5–6):413–420

    Article  Google Scholar 

  18. Demetriou CA et al (2019) Retrospective longitudinal study of ALS in Cyprus: clinical characteristics, management and survival. PLoS One 14(9):e0220246

    Article  CAS  Google Scholar 

  19. Wei QQ et al (2018) Clinical and prognostic features of ALS/MND in different phenotypes-data from a hospital-based registry. Brain Res Bull 142:403–408

    Article  Google Scholar 

  20. Chen L et al (2015) Natural history and clinical features of sporadic amyotrophic lateral sclerosis in China. J Neurol Neurosurg Psychiatry 86(10):1075–1081

    Article  Google Scholar 

  21. Fujimura-Kiyono C et al (2011) Onset and spreading patterns of lower motor neuron involvements predict survival in sporadic amyotrophic lateral sclerosis. J Neurol Neurosurg Psychiatry 82(11):1244–1249

    Article  Google Scholar 

  22. Sekiguchi T et al (2014) Spreading of amyotrophic lateral sclerosis lesions—multifocal hits and local propagation? J Neurol Neurosurg Psychiatry 85(1):85–91

    Article  Google Scholar 

  23. Nakamura R et al (2013) Neck weakness is a potent prognostic factor in sporadic amyotrophic lateral sclerosis patients. J Neurol Neurosurg Psychiatry 84(12):1365–1371

    Article  Google Scholar 

  24. Turner MR et al (2010) Pattern of spread and prognosis in lower limb-onset ALS. Amyotroph Lateral Scler 11(4):369–373

    Article  Google Scholar 

  25. Manera U et al (2020) Regional spreading of symptoms at diagnosis as a prognostic marker in amyotrophic lateral sclerosis: a population-based study. J Neurol Neurosurg Psychiatry 91(3):291–297

    Article  Google Scholar 

  26. Roche JC et al (2012) A proposed staging system for amyotrophic lateral sclerosis. Brain 135(3):847–852

    Article  Google Scholar 

  27. Sato Y et al (2015) Prediction of prognosis of ALS: importance of active denervation findings of the cervical-upper limb area and trunk area. Intractable Rare Dis Res 4(4):181–189

    Article  Google Scholar 

  28. Fileccia E et al (2020) Denervation findings on EMG in amyotrophic lateral sclerosis and correlation with prognostic milestones: data from a retrospective study. Clin Neurophysiol 131(8):2017–2022

    Article  CAS  Google Scholar 

  29. Zhang HG et al (2017) Clinical features of isolated bulbar palsy of amyotrophic lateral sclerosis in Chinese population. Chin Med J (Engl) 130(15):1768–1772

    Article  Google Scholar 

  30. Burrell JR, Vucic S, Kiernan MC (2011) Isolated bulbar phenotype of amyotrophic lateral sclerosis. Amyotroph Lateral Scler 12(4):283–289

    Article  Google Scholar 

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Authors and Affiliations

  1. Medical School of Chinese PLA, Beijing, China

    Zhengqing He & Xusheng Huang

  2. Neurological Department of the First Medical Center, Chinese PLA General Hospital, No. 28 Fuxing Road, Haidian District, 100853, Beijing, China

    Zhengqing He, Bo Sun, Jiongming Bai, Haoran Wang, Hongfen Wang, Fei Yang, Fang Cui & Xusheng Huang

  3. Geriatric Neurological Department of the Second Medical Center & National Clinical Research Center for Geriatric Diseases, Chinese PLA General Hospital, Beijing, China

    Bo Sun

  4. Department of Neurology, PLA Rocket Force Characteristic Medical Center, Beijing, China

    Feng Feng

  5. College of Medicine, Nankai University, Tianjin, China

    Jiongming Bai & Haoran Wang

  6. Department of Neurology, Hainan Hospital of PLA General Hospital, Hainan, China

    Fang Cui

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  1. Zhengqing He
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  2. Bo Sun
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  8. Fang Cui
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  9. Xusheng Huang
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Correspondence to Xusheng Huang.

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This study was approved by the Medical Ethics Committee of Chinese PLA General Hospital.

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He, Z., Sun, B., Feng, F. et al. Time of symptoms beyond the bulbar region predicts survival in bulbar onset amyotrophic lateral sclerosis. Neurol Sci 43, 1817–1822 (2022). https://doi.org/10.1007/s10072-021-05556-w

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  • DOI: https://doi.org/10.1007/s10072-021-05556-w

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