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

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.

• Authors: 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, Takashi Imai, Masashi Aoki, Shoji Tsuji, Imaharu Nakano, Ryuji Kaji, Gen Sobue
• Journal: Amyotrophic lateral sclerosis & frontotemporal degeneration (Amyotroph Lateral Scler Frontotemporal Degener) Vol. 16 Issue 3-4 Pg. 230-6 (Jun 2015) ISSN: 2167-9223 [Electronic] England
• PMID: 25548957 (Publication Type: Journal Article, Multicenter Study, Research Support, Non-U.S. Gov't)

Topics

• Age of Onset
• Aged
• Amyotrophic Lateral Sclerosis (epidemiology, mortality, physiopathology, surgery)
• Disease Progression
• Female
• Follow-Up Studies
• Humans
• Japan (epidemiology)
• Kaplan-Meier Estimate
• Male
• Middle Aged
• Muscle Weakness (physiopathology)
• Positive-Pressure Respiration
• Prognosis
• Registries
• Tracheostomy