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ArticleNeurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology2026

Early respiratory decline around diagnosis and short-term post-landmark outcomes in amyotrophic lateral sclerosis: a 6-month landmark cohort study.

Kazuhiro Horiuchi, Shuntaro Nakamura, Kaede Ishikawa, Sumire Nunomura, Kazuki Yamada, Takashi Inoue, Kei Oiwa, Shintaro Fujii, Yuki Oshima, Akihiko Kudo and 1 more

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Article in Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper.

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1citing papers in PubMed
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1 · What the graph read from it

What it found

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2 · The registry

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3 · Its place in the literature

Who cites it

1 citing paper in PubMed.

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4 · The record

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5 · Who and what money

Authors and funding

11 authors.

Kazuhiro HoriuchiDepartment of Neurology, Hakodate Municipal Hospital, 1-10-1 Minato-cho, Hakodate, Hokkaido, Japan. horiuchi-kazuhiro@hotmail.co.jp.ORCID http://orcid.org/0000-0002-7040-3273
Shuntaro NakamuraDepartment of Neurology, Faculty of Medicine, Graduate School of Medicine, Hokkaido University, Sapporo, Japan.
Kaede IshikawaDepartment of Neurology, Hakodate Municipal Hospital, 1-10-1 Minato-cho, Hakodate, Hokkaido, Japan.
Sumire NunomuraDepartment of Neurology, Hakodate Municipal Hospital, 1-10-1 Minato-cho, Hakodate, Hokkaido, Japan.
Kazuki YamadaDepartment of Neurology, Faculty of Medicine, Graduate School of Medicine, Hokkaido University, Sapporo, Japan.
Takashi InoueDepartment of Neurology, Faculty of Medicine, Graduate School of Medicine, Hokkaido University, Sapporo, Japan.
Kei OiwaDepartment of Neurology, Faculty of Medicine, Graduate School of Medicine, Hokkaido University, Sapporo, Japan.
Shintaro FujiiDepartment of Neurology, Faculty of Medicine, Graduate School of Medicine, Hokkaido University, Sapporo, Japan.
Yuki OshimaDepartment of Neurology, Faculty of Medicine, Graduate School of Medicine, Hokkaido University, Sapporo, Japan.
Akihiko KudoDepartment of Neurology, Faculty of Medicine, Graduate School of Medicine, Hokkaido University, Sapporo, Japan.
Ichiro YabeDepartment of Neurology, Faculty of Medicine, Graduate School of Medicine, Hokkaido University, Sapporo, Japan.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

backgroundIn amyotrophic lateral sclerosis (ALS), respiratory decisions rely on serial trends rather than a single value. We evaluated whether early respiratory decline around diagnosis provides prognostic information in a real-world landmark framework.

methodsThis single-center retrospective cohort screened 94 consecutive patients diagnosed between April 2019 and December 2025. A 6-month landmark was used. Early decline was estimated from %FVC values between - 30 and + 180 days around diagnosis. The primary model included age and early %FVC decline; robustness analyses included time-varying Cox, piecewise Cox, RMST, included-vs-excluded comparison, death-only analysis, and slope-quality filtering.

resultsOf 94 screened patients, 62 met baseline eligibility, 56 had calculable early slope, and 45 entered the landmark cohort; 28 post-landmark composite events occurred. In the Cox model, faster early %FVC decline was associated with higher hazard of death or invasive mechanical ventilation via tracheostomy (HR 1.33 per 1%/month faster decline, 95% CI 1.14-1.55, p < 0.001). PH diagnostics suggested non-proportionality (%FVC p = 0.031; NIV p = 0.034 in the expanded model), so this HR was interpreted as an average follow-up association and complemented by PH-robust analyses. The signal was stronger early than late, remained consistent in a death-only analysis, and favored the slower-decline group by RMST at 24 and 36 months.

conclusionsIn this selected measurement-capable landmark cohort, early respiratory decline provided a clinically meaningful short-to-medium term prognostic signal for post-landmark adverse outcomes. External validation is required before broader generalization beyond measurement-capable landmark populations.

Indexed as

Amyotrophic Lateral SclerosisAgedCohort StudiesDisease ProgressionFemaleHumansMaleMiddle AgedPrognosisRetrospective StudiesVital CapacityAmyotrophic lateral sclerosisForced vital capacityLandmark analysisProportional hazardsRestricted mean survival time

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.