Advertisement

Neurological Sciences

, Volume 40, Issue 12, pp 2509–2514 | Cite as

Under-recognized primary spontaneous pneumothorax in ALS: a multicenter retrospective study

  • Jin-Sung Park
  • Young Woo Do
  • Jin-Mo Park
  • Hung Youl Seok
  • Donghwi ParkEmail author
Original Article

Abstract

Primary spontaneous pneumothorax (PSP) is not an uncommon disease, especially in patients with risk factors such as male gender, history of smoking, and low body mass index (BMI). Amyotrophic lateral sclerosis (ALS) is a rare disease caused by neurodegeneration of the motor neurons that share risk factors with PSP. The aim of this study was to determine the prevalence of PSP in ALS and find the significant risk factors related to PSP. We retrospectively reviewed the data from 86 patients with clinically probable or definite ALS from three different centers. Clinical characteristics, including age, sex, subtype, disease duration, body mass index, history of smoking, tracheostomy state, and ventilator use, were obtained. The ALS Functional Rating Scale-Revised Form (ALSFRS-R) total score and subscores were also retrieved from the medical records. In the results, six of the 86 patients (7%) had PSP. There were no statistically significant differences among the clinical characteristics and the ALSFRS-R scores between the patients with and without PSP, except for BMI and smoking (p < 0.022 and p < 0.019, respectively). A multivariate logistic regression analysis of smoking and BMI showed an odds ratio of 19.25. In conclusion, the existence of PSP in ALS may be under-recognized. Further well-designed, large studies are needed to elucidate the prevalence and pathophysiology of pneumothorax in ALS.

Keywords

Amyotrophic lateral sclerosis Dyspnea Pneumothorax Smoking Body mass index 

Notes

Funding information

This research was supported by Basic Science Research Program through the National Research Foundation of Korea (NRF) funded by the Ministry of Science, ICT and Future Planning (NRF-2017R1D1A1B03033127). This work was supported by the National Research Foundation of Korea (NRF) grant funded by the Korean government (Ministry of Science and ICT) (No. 2017R1C1B5076264).

Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.

References

  1. 1.
    Park JS, Park D (2017) The terminal latency of the phrenic nerve correlates with respiratory symptoms in amyotrophic lateral sclerosis. Clin Neurophysiol 128(9):1625–1628CrossRefGoogle Scholar
  2. 2.
    Bourke SC, Tomlinson M, Williams TL, Bullock RE, Shaw PJ, Gibson GJ (2006) Effects of non-invasive ventilation on survival and quality of life in patients with amyotrophic lateral sclerosis: a randomised controlled trial. Lancet Neurol 5(2):140–147CrossRefGoogle Scholar
  3. 3.
    Park D (2017) Application of different ventilator modes in patients with amyotrophic lateral sclerosis according to certain clinical situations: a case report. Medicine 96(34):e7899CrossRefGoogle Scholar
  4. 4.
    Sassoon CS (1995) The etiology and treatment of spontaneous pneumothorax. Curr Opin Pulm Med 1(4):331–338PubMedGoogle Scholar
  5. 5.
    Jee SH, Sull JW, Park J, Lee SY, Ohrr H, Guallar E, Samet JM (2006) Body-mass index and mortality in Korean men and women. N Engl J Med 355(8):779–787CrossRefGoogle Scholar
  6. 6.
    Park D, Lee JH, Han S (2017) Underweight: another risk factor for cardiovascular disease?: a cross-sectional 2013 Behavioral Risk Factor Surveillance System (BRFSS) study of 491,773 individuals in the USA. Medicine 96(48):e8769CrossRefGoogle Scholar
  7. 7.
    Park D, Park JS (2017) Terminal latency abnormality in amyotrophic lateral sclerosis without split hand syndrome. Neurol Sci 38(5):775–781CrossRefGoogle Scholar
  8. 8.
    Park D (2018) Recurrent complex regional pain syndrome type I in a patient with amyotrophic lateral sclerosis: a case report. Neurol Sci 39(8):1487–1488CrossRefGoogle Scholar
  9. 9.
    Park JS, Park D (2017) Reply to “prolongation of terminal latency of the phrenic nerve in amyotrophic lateral sclerosis - is it clinically useful, and what are the mechanisms?”. Clin Neurophysiol 128(10):2095CrossRefGoogle Scholar
  10. 10.
    Park D, Lee GJ, Kim HY, Ryu JS (2017) Different characteristics of ventilator application between tracheostomy- and noninvasive positive pressure ventilation patients with amyotrophic lateral sclerosis. Medicine 96(10):e6251CrossRefGoogle Scholar
  11. 11.
    Crescimanno G (2019) Early non-invasive ventilation in patients affected by amyotrophic lateral sclerosis: revisitingliterature in view of new scientific knowledge. Eur J Neurol 2019;26(7):e72Google Scholar
  12. 12.
    Benditt JO (2002) Respiratory complications of amyotrophic lateral sclerosis. Semin Respir Crit Care Med 23(3):239–247CrossRefGoogle Scholar
  13. 13.
    Onuki T, Ueda S, Yamaoka M, Sekiya Y, Yamada H, Kawakami N et al (2017) Primary and secondary spontaneous pneumothorax: prevalence, clinical features, and in-hospital mortality. Can Respir J 2017:6014967CrossRefGoogle Scholar
  14. 14.
    Okutani D, Kotani K, Nagai T, Makihara S (2009) Bilateral pneumothorax ofan amyotrophic lateral sclerosis patient under mechanical ventilation. Kyobu Geka 62(3):231-4Google Scholar
  15. 15.
    Kaumi L, Diaz-Lobato S (2013) Acute respiratory failure in a female patient with amyotrophic lateral sclerosis as a result of a pneumothorax that was not visible in the imaging study. Rev Neurol 56(2):127–128PubMedGoogle Scholar
  16. 16.
    Armon C (2003) An evidence-based medicine approach to the evaluation of the role of exogenous risk factors in sporadic amyotrophic lateral sclerosis. Neuroepidemiology 22(4):217–228CrossRefGoogle Scholar
  17. 17.
    Weisskopf MG, Gallo V, O’Reilly EJ, Vineis P, Ascherio A (2010) Smoking may be considered an established risk factor for sporadic ALS. Neurology 74(23):1927–1928 author reply 8-9CrossRefGoogle Scholar
  18. 18.
    Shimizu T, Nagaoka U, Nakayama Y, Kawata A, Kugimoto C, Kuroiwa Y et al (2012) Reduction rate of body mass index predicts prognosis for survival in amyotrophic lateral sclerosis: a multicenter study in Japan. Amyotroph Lateral Scler 13(4):363–366CrossRefGoogle Scholar
  19. 19.
    Jawaid A, Murthy SB, Wilson AM, Qureshi SU, Amro MJ, Wheaton M et al (2010) A decrease in body mass index is associated with faster progression of motor symptoms and shorter survival in ALS. Amyotroph Lateral Scler 11(6):542–548CrossRefGoogle Scholar
  20. 20.
    Bense L, Eklund G, Wiman LG (1987) Smoking and the increased risk of contracting spontaneous pneumothorax. Chest 92(6):1009–1012CrossRefGoogle Scholar
  21. 21.
    MacDuff A, Arnold A, Harvey J, Group BTSPDG (2010) Management of spontaneous pneumothorax: British Thoracic Society pleural disease guideline 2010. Thorax 65(Suppl 2):ii18–ii31CrossRefGoogle Scholar
  22. 22.
    Tan J, Yang Y, Zhong J, Zuo C, Tang H, Zhao H, Zeng G, Zhang J, Guo J, Yang N (2017) Association between BMI and recurrence of primary spontaneous pneumothorax. World J Surg 41(5):1274–1280CrossRefGoogle Scholar
  23. 23.
    Noppen M, Baumann MH (2003) Pathogenesis and treatment of primary spontaneous pneumothorax: an overview. Respiration 70(4):431–438CrossRefGoogle Scholar
  24. 24.
    Wormser U, Mandrioli J, Vinceti M, Fini N, Sintov A, Brodsky B, Proskura E, Finkelstein Y (2016) Reduced levels of alpha-1-antitrypsin in cerebrospinal fluid of amyotrophic lateral sclerosis patients: a novel approach for a potential treatment. J Neuroinflammation 13(1):131CrossRefGoogle Scholar
  25. 25.
    Takubo Y, Guerassimov A, Ghezzo H, Triantafillopoulos A, Bates JH, Hoidal JR et al (2002) Alpha1-antitrypsin determines the pattern of emphysema and function in tobacco smoke-exposed mice: parallels with human disease. Am J Respir Crit Care Med 166(12 Pt 1):1596–1603CrossRefGoogle Scholar
  26. 26.
    Dupuis L, Corcia P, Fergani A, Gonzalez De Aguilar JL, Bonnefont-Rousselot D, Bittar R et al (2008) Dyslipidemia is a protective factor in amyotrophic lateral sclerosis. Neurology 70(13):1004–1009CrossRefGoogle Scholar

Copyright information

© Fondazione Società Italiana di Neurologia 2019

Authors and Affiliations

  1. 1.Department of Neurology, School of Medicine, Kyungpook National UniversityKyungpook National University Chilgok hospitalDaeguSouth Korea
  2. 2.Department of Thoracic and Cardiovascular SurgeryKyungpook National University HospitalDaeguSouth Korea
  3. 3.Department of NeurologyDongguk University College of MedicineGyeongjuSouth Korea
  4. 4.Department of NeurologyKeimyung University School of MedicineDaeguSouth Korea
  5. 5.Department of Rehabilitation Medicine, Daegu Fatima HospitalDaeguRepublic of Korea

Personalised recommendations