title: 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.affiliation: Department of Neurology.
authors.forename: Hazuki
authors.initials: H
authors.lastname: Watanabe
authors.forename: Naoki
authors.initials: N
authors.lastname: Atsuta
authors.forename: Ryoichi
authors.initials: R
authors.lastname: Nakamura
authors.forename: Akihiro
authors.initials: A
authors.lastname: Hirakawa
authors.forename: Hirohisa
authors.initials: H
authors.lastname: Watanabe
authors.forename: Mizuki
authors.initials: M
authors.lastname: Ito
authors.forename: Jo
authors.initials: J
authors.lastname: Senda
authors.forename: Masahisa
authors.initials: M
authors.lastname: Katsuno
authors.forename: Yuishin
authors.initials: Y
authors.lastname: Izumi
authors.forename: Mitsuya
authors.initials: M
authors.lastname: Morita
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Tomiyama
authors.forename: Akira
authors.initials: A
authors.lastname: Taniguchi
authors.forename: Ikuko
authors.initials: I
authors.lastname: Aiba
authors.forename: Koji
authors.initials: K
authors.lastname: Abe
authors.forename: Kouichi
authors.initials: K
authors.lastname: Mizoguchi
authors.forename: Masaya
authors.initials: M
authors.lastname: Oda
authors.forename: Osamu
authors.initials: O
authors.lastname: Kano
authors.forename: Koichi
authors.initials: K
authors.lastname: Okamoto
authors.forename: Satoshi
authors.initials: S
authors.lastname: Kuwabara
authors.forename: Kazuko
authors.initials: K
authors.lastname: Hasegawa
authors.forename: Takashi
authors.initials: T
authors.lastname: Imai
authors.forename: Masashi
authors.initials: M
authors.lastname: Aoki
authors.forename: Shoji
authors.initials: S
authors.lastname: Tsuji
authors.forename: Imaharu
authors.initials: I
authors.lastname: Nakano
authors.forename: Ryuji
authors.initials: R
authors.lastname: Kaji
authors.forename: Gen
authors.initials: G
authors.lastname: Sobue
mesh_terms: D017668:Age of Onset; D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000453:epidemiology* / Q000401:mortality / Q000503:physiopathology* / Q000601:surgery; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007564:Japan / Q000453:epidemiology; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D018908:Muscle Weakness / Q000503:physiopathology*; D011175:Positive-Pressure Respiration; D011379:Prognosis; D012042:Registries; D014139:Tracheostomy
keywords: ALS; ALSFRS-R; functional decline; survival