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Original research
Prognosis of amyotrophic lateral sclerosis patients undergoing tracheostomy invasive ventilation therapy in Japan
  1. Naoki Hayashi1,
  2. Naoki Atsuta1,
  3. Daichi Yokoi1,
  4. Ryoichi Nakamura1,
  5. Masahiro Nakatochi2,
  6. Masahisa Katsuno1,
  7. Yuishin Izumi3,
  8. Kazuaki Kanai4,5,
  9. Nobutaka Hattori4,
  10. Akira Taniguchi6,
  11. Mitsuya Morita7,
  12. Osamu Kano8,
  13. Kazumoto Shibuya9,
  14. Satoshi Kuwabara9,
  15. Naoki Suzuki10,
  16. Masashi Aoki10,
  17. Ikuko Aiba11,
  18. Kouichi Mizoguchi12,
  19. Masaya Oda13,
  20. Ryuji Kaji3,
  21. Gen Sobue14,15
  1. 1 Department of Neurology, Nagoya University Graduate School of Medicine, Nagoya, Japan
  2. 2 Data Science Division, Data Coordinating Center, Department of Advanced Medicine, Nagoya University Hospital, Nagoya, Japan
  3. 3 Department of Clinical Neuroscience, Institute of Biomedical Sciences, Tokushima University Graduate School, Tokushima, Japan
  4. 4 Department of Neurology, Juntendo University School of Medicine, Tokyo, Japan
  5. 5 Department of Neurology, Fukushima Medical University, Fukushima, Japan
  6. 6 Department of Neurology, Mie University Graduate School of Medicine, Tsu, Japan
  7. 7 Division of Neurology, Department of Internal Medicine, Jichi Medical University, Shomotsuke, Japan
  8. 8 Division of Neurology, Department of Internal Medicine, Toho University School of Medicine, Tokyo, Japan
  9. 9 Department of Neurology, Graduate School of Medicine, Chiba University, Chiba, Japan
  10. 10 Department of Neurology, Tohoku University Graduate School of Medicine, Sendai, Japan
  11. 11 Department of Neurology, National Hospital Organization Higashinagoya National Hospital, Nagoya, Japan
  12. 12 Department of Neurology, National Hospital Organization, Shizuoka Medical Center, Shimizu-cho, Japan
  13. 13 Department of Neurology, Mifukai Vihara Hananosato Hospital, Miyoshi, Japan
  14. 14 Brain and Mind Research Center, Nagoya University Graduate School of Medicine, Nagoya, Japan
  15. 15 Aichi Medical University, Nagakute, Japan
  1. Correspondence to Professor Gen Sobue, Brain and Mind Research Center, Nagoya University Graduate School of Medicine, Nagoya, Aichi 464-8601, Japan; sobueg{at}


Objective The aim of this study is to describe and clarify the factors affecting the prognosis of Japanese patients with amyotrophic lateral sclerosis (ALS) undergoing tracheostomy invasive ventilation (TIV) therapy.

Methods We conducted a prospective longitudinal observational case-control study using a multicentre registry. ALS patients who started TIV therapy after registration (TIV group) and those who did not receive TIV (non-TIV group) were included. We compared the survival time between the TIV group and the non-TIV group using a propensity score matching analysis and evaluated the prognostic factors in the TIV group.

Results From February 2006 to January 2018, 190 patients in the TIV group and 1093 patients in the non-TIV group were included in this study. The mean age of disease onset and usage rate of gastrostomy and non-invasive ventilation therapy differed between the groups. In the propensity score matching analysis using known prognostic factors, the median overall survival time of the TIV group was significantly greater than that of the non-TIV group (11.33 years vs 4.61 years; p<0.001). Analysis using the Cox proportional hazard model suggested that older age of onset and respiratory onset was an independent factor for poor prognosis after starting TIV therapy.

Conclusion We showed that there was a significant difference of approximately 7 years in life expectancy between Japanese ALS patients who did and did not receive TIV therapy.

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  • Collaborators Tomohiko Ishihara, Osamu Onodera (Niigata University), Yasuyuki Ohta, Toru Yamashita, Koji Abe (Okayama University), Yukio Fujita, Yoshio Ikeda (Gumma University), Kazuko Hasegawa (Sagamihara National Hospital), Tomoko Nakazato (Juntendo University), Tomokazu Obi (Shizuoka Institute of Epilepsy and Neurological Disorders), Hiroaki Ito (Miyagi National Hospital), Seiya Noda, Satoshi Kuru (National Hospital Organization Suzuka National Hospital), Ikuko Iwata (Hokkaido University), Akihiro Kawata (Tokyo Metropolitan Neurological Hospital), Tadashi Kanouchi, Takanori Yokota (Tokyo Medical and Dental University), Yasuhiro Watanabe (Tottori University), Hiroyuki Ishiura, Shoji Tsuji (Tokyo University), Yukiko Tsuji, Toshiki Mizuno (Kyoto Prefectural University), Takashi Ayaki, Ryosuke Takahashi (Kyoto University), Takuji Fujita (Takumi-kai Neurology Clinic), Hiroshi Doi, Hideyuki Takeuchi, Akikage Tanaka (Yokohama City University), Takamura Nagasaka, Yoshihisa Takiyama (Yamanashi University), Wataru Shiraishi, Jun-ichi Kira (Kyushu University), Hitoshi Aizawa (Tokyo Medical University), Mie Nakamura (National Hospital Organization Tokyo National Hospital), Yuji Takahashi (National Center Hospital, National Center of Neurology and Psychiatry) and Japanese Consortium for Amyotrophic Lateral Sclerosis research.

  • Contributors NaH, NA and GS designed the study, contributed to analysis and interpretation of data and wrote the manuscript. MN analysed the data and reviewed the manuscript. DY, RN, MK, YI, KK, NoH, AT, MM, OK, KS, SK, NS, MA, IA, KM, MO and RK contributed to data collection and interpretation and critically reviewed the manuscript. NaH, NA, DY, RN, MN, MK, YI, KK, NoH, AT, MM, OK, KS, SK, NS, MA, IA, KM, MO, RK and GS approved the final version of the manuscript and agreed to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.

  • Funding This work was supported by grants (15Aek0109071h0002, 17ek0109284h0001, 16lk0201057h0001, 181k1601002h0001) from the Japan Agency for Medical Research and Development, Health, and Labor Sciences Research grant (H29-033), and Grants-in Aid (17K09778) for Scientific Research from the Ministry of Education, Culture, Sports, Science, and Technology (MEXT) of Japan.

  • Competing interests None declared.

  • Patient consent for publication Not required.

  • Ethics approval The ethics committees of all participating facilities approved the study.

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • Data availability statement Data are available upon reasonable request. Data of participants are deidentified.The reuse of the data needs permission of the steering committee of Japanese Consortium for Amyotrophic Lateral Sclerosis.