Factors affecting longitudinal functional decline and survival in amyotrophic lateral sclerosis patients

Hazuki Watanabe, Naoki Atsuta, Ryoichi Nakamura, Akihiro Hirakawa, Hirohisa Watanabe, Mizuki Ito, Jo Senda, Masahisa Katsuno, Yuishin Izumi, Mitsuya Morita, Hiroyuki Tomiyama, Akira Taniguchi, Ikuko Aiba, Koji Abe, Kouichi Mizoguchi, Masaya Oda, Osamu Kano, Koichi Okamoto, Satoshi Kuwabara, Kazuko Hasegawa & 6 others Takashi Imai, Masashi Aoki, Shoji Tsuji, Imaharu Nakano, Ryuji Kaji, Gen Sobue

Research output: Contribution to journalArticle

32 Citations (Scopus)

Abstract

Our objective was to elucidate the clinical factors affecting functional decline and survival in Japanese amyotrophic lateral sclerosis (ALS) patients. We constructed a multicenter prospective ALS cohort that included 451 sporadic ALS patients in the analysis. We longitudinally utilized the revised Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS-R) as the functional scale, and determined the timing of introduction of a tracheostomy for positive-pressure ventilation and death. A joint modelling approach was employed to identify prognostic factors for functional decline and survival. Age at onset was a common prognostic factor for both functional decline and survival (p <0.001, p <0.001, respectively). Female gender (p = 0.019) and initial symptoms, including upper limb weakness (p = 0.010), lower limb weakness (p = 0.008) or bulbar symptoms (p = 0.005), were related to early functional decline, whereas neck weakness as an initial symptom (p = 0.018), non-use of riluzole (p = 0.030) and proximal dominant muscle weakness in the upper extremities (p = 0.01) were related to a shorter survival time. A decline in the ALSFRS-R score was correlated with a shortened survival time (p <0.001). In conclusion, the factors affecting functional decline and survival in ALS were common in part but different to some extent. This difference has not been previously well recognized but is informative in clinical practice and for conducting trials.

Original languageEnglish
Pages (from-to)230-236
Number of pages7
JournalAmyotrophic Lateral Sclerosis and Frontotemporal Degeneration
Volume16
Issue number3-4
DOIs
Publication statusPublished - Jun 1 2015

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Amyotrophic Lateral Sclerosis
Survival
Upper Extremity
Riluzole
Positive-Pressure Respiration
Tracheostomy
Muscle Weakness
Age of Onset
Lower Extremity
Neck
Joints

Keywords

  • ALS
  • ALSFRS-R
  • Functional decline
  • Survival

ASJC Scopus subject areas

  • Clinical Neurology
  • Neurology

Cite this

Factors affecting longitudinal functional decline and survival in amyotrophic lateral sclerosis patients. / Watanabe, Hazuki; Atsuta, Naoki; Nakamura, Ryoichi; Hirakawa, Akihiro; Watanabe, Hirohisa; Ito, Mizuki; Senda, Jo; Katsuno, Masahisa; Izumi, Yuishin; Morita, Mitsuya; Tomiyama, Hiroyuki; Taniguchi, Akira; Aiba, Ikuko; Abe, Koji; Mizoguchi, Kouichi; Oda, Masaya; Kano, Osamu; Okamoto, Koichi; Kuwabara, Satoshi; Hasegawa, Kazuko; Imai, Takashi; Aoki, Masashi; Tsuji, Shoji; Nakano, Imaharu; Kaji, Ryuji; Sobue, Gen.

In: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, Vol. 16, No. 3-4, 01.06.2015, p. 230-236.

Research output: Contribution to journalArticle

Watanabe, H, Atsuta, N, Nakamura, R, Hirakawa, A, Watanabe, H, Ito, M, Senda, J, Katsuno, M, Izumi, Y, Morita, M, Tomiyama, H, Taniguchi, A, Aiba, I, Abe, K, Mizoguchi, K, Oda, M, Kano, O, Okamoto, K, Kuwabara, S, Hasegawa, K, Imai, T, Aoki, M, Tsuji, S, Nakano, I, Kaji, R & Sobue, G 2015, 'Factors affecting longitudinal functional decline and survival in amyotrophic lateral sclerosis patients', Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, vol. 16, no. 3-4, pp. 230-236. https://doi.org/10.3109/21678421.2014.990036
Watanabe, Hazuki ; Atsuta, Naoki ; Nakamura, Ryoichi ; Hirakawa, Akihiro ; Watanabe, Hirohisa ; Ito, Mizuki ; Senda, Jo ; Katsuno, Masahisa ; Izumi, Yuishin ; Morita, Mitsuya ; Tomiyama, Hiroyuki ; Taniguchi, Akira ; Aiba, Ikuko ; Abe, Koji ; Mizoguchi, Kouichi ; Oda, Masaya ; Kano, Osamu ; Okamoto, Koichi ; Kuwabara, Satoshi ; Hasegawa, Kazuko ; Imai, Takashi ; Aoki, Masashi ; Tsuji, Shoji ; Nakano, Imaharu ; Kaji, Ryuji ; Sobue, Gen. / Factors affecting longitudinal functional decline and survival in amyotrophic lateral sclerosis patients. In: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration. 2015 ; Vol. 16, No. 3-4. pp. 230-236.
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AU - Nakamura, Ryoichi

AU - Hirakawa, Akihiro

AU - Watanabe, Hirohisa

AU - Ito, Mizuki

AU - Senda, Jo

AU - Katsuno, Masahisa

AU - Izumi, Yuishin

AU - Morita, Mitsuya

AU - Tomiyama, Hiroyuki

AU - Taniguchi, Akira

AU - Aiba, Ikuko

AU - Abe, Koji

AU - Mizoguchi, Kouichi

AU - Oda, Masaya

AU - Kano, Osamu

AU - Okamoto, Koichi

AU - Kuwabara, Satoshi

AU - Hasegawa, Kazuko

AU - Imai, Takashi

AU - Aoki, Masashi

AU - Tsuji, Shoji

AU - Nakano, Imaharu

AU - Kaji, Ryuji

AU - Sobue, Gen

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