Evidence map›Paper›PMID 41020440›Full record

ArticleAnnals of neurology2026

Primary Lateral Sclerosis Natural History Study: Primary Lateral Sclerosis Functional Rating Scale and Other Outcomes Assessment.

Ikjae Lee, Grace Jang, Ying Kuen Ken Cheung, Alexander V Sherman, Wendy S Johnston, Frank Diaz, J Americo M Fernandes, Ali A Habib, Nicholas J Maragakis, Sabrina Paganoni and 30 more

Abstract readMulticenter Study
PubMed Full text at PMC Publisher
In one paragraph

Article in Annals of neurology, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 2 papers.

0numbers the graph read from it
0cells of the map it votes in
2citing papers in PubMed
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

2 citing papers in PubMed.

  1. Article
  2. Article
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

40 authors.

Ikjae LeeDepartment of Neurology, Columbia University Irving Medical Center, New York, New York, USA.ORCID 0000-0003-0537-9622
Grace JangDepartment of Neurology, Columbia University Irving Medical Center, New York, New York, USA.
Ying Kuen Ken CheungDepartment of Biostatistics, Mailman School of Public Health, Columbia University, New York, New York, USA.
Alexander V ShermanDepartment of Neurology, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts, USA.
Wendy S JohnstonDivision of Neurology, University of Alberta, Edmonton, Alberta, Canada.
Frank DiazDepartment of Neurology, Cedars-Sinai Medical Center, Los Angeles, California, USA.ORCID 0000-0003-3526-1852
J Americo M FernandesDepartment of Neurological Sciences, University of Nebraska Medical Center, Omaha, Nebraska, USA.
Ali A HabibDepartment of Neurology, University of California Irvine, Orange, California, USA.
Nicholas J MaragakisDepartment of Neurology, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.ORCID 0000-0002-7311-9614
Sabrina PaganoniDepartment of Neurology, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts, USA.
Katherine BurkeDepartment of Neurology, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts, USA.
Bjorn OskarssonDepartment of Neurology, Mayo Clinic, Jacksonville, Florida, USA.ORCID 0000-0002-1725-9866
Jaimin ShahDepartment of Neurology, Mayo Clinic, Jacksonville, Florida, USA.
Lorne ZinmanDepartment of Neurology, Sunnybrook Health Sciences Centre, Toronto, Ontario, Canada.
Mary Kay FloeterClinical Neurosciences Program, NINDS, Bethesda, Maryland, USA.
Senda Ajroud-DrissDepartment of Neurology, Northwestern University, Chicago, Illinois, USA.
Kelly GwathmeyDepartment of Neurology, Virginia Commonwealth University, Richmond, Virginia, USA.
Terry Heiman-PattersonDepartment of Neurology, Temple University Lewis Katz School of Medicine, Philadelphia, Pennsylvania, USA.
Omar JawdatDepartment of Neurology, University of Kansas Medical Center, Kansas City, Kansas, USA.
Edward J KasarskisDepartment of Neurology, University of Kentucky, Lexington, Kentucky, USA.
Yaz Y KisanukiDepartment of Neurology, The Ohio State University Wexner Medical Center, Columbus, Ohio, USA.
James WymerDepartment of Neurology, University of Florida College of Medicine, Gainesville, Florida, USA.
Christina FournierDepartment of Neurology, Emory School of Medicine, Atlanta, Georgia, USA.
Ghazala HayatDepartment of Neurology, Saint Louis University, Saint Louis, Missouri, USA.
Daragh HeitzmanTexas Neurology, Dallas, Texas, USA.
Catherine Lomen-HoerthDepartment of Neurology, University of California San Francisco, San Francisco, California, USA.
Michael T PulleyDepartment of Neurology, University of Florida College of Medicine, Jacksonville, Florida, USA.
Stephen N ScelsaDepartment of Neurology, Icahn School of Medicine at Mount Sinai, New York, New York, USA.
David WalkDepartment of Neurology, University of Minnesota, Minneapolis, Minnesota, USA.
Stephen A GoutmanDepartment of Neurology, University of Michigan, Ann Arbor, Michigan, USA.ORCID 0000-0001-8780-6637
Christen ShoesmithDepartment of Neurology, Western University, London, Ontario, Canada.
Zachary SimmonsDepartment of Neurology, Penn State University College of Medicine, Hershey, Pennsylvania, USA.
Eric SorensonDepartment of Neurology, Mayo Clinic, Rochester, Minnesota, USA.
Lauren ElmanDepartment of Neurology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, USA.
Matthew B HarmsDepartment of Neurology, Columbia University Irving Medical Center, New York, New York, USA.
Benjamin N HooverDepartment of Neurology, Columbia University Irving Medical Center, New York, New York, USA.
Rebecca Y YunDepartment of Neurology, Columbia University Irving Medical Center, New York, New York, USA.
Regina M SantellaDepartment of Environmental Health Sciences, Mailman School of Public Health, Columbia University, New York, New York, USA.
Hiroshi MitsumotoDepartment of Neurology, Columbia University Irving Medical Center, New York, New York, USA.
PLS NHS Study Group

Funding

True Metal fac coreP30ES009089 · NIEHS · COLUMBIA UNIVERSITY HEALTH SCIENCES · PI MATTHEW S PERZANOWSKI · 1998 to 2026
$44.7M
Metabolic Alteration in Presymptomatic and Symptomatic ALS Study (MAPS ALS Study)K23NS131586 · NINDS · COLUMBIA UNIVERSITY HEALTH SCIENCES · PI Ikjae Lee · 2023 to 2026
$919k
NIEHS NIH HHS P30 ES009089NINDS NIH HHS K23 NS131586
6 · The paper itself

Abstract

objectiveThe primary lateral sclerosis (PLS) consensus diagnostic criteria and functional rating scale (PLSFRS) were recently established to facilitate and optimize future PLS clinical trials. We examined the trajectory of the PLSFRS and other functional outcome measures and biomarkers in the PLS Natural History Study (PLS NHS) to understand their performance in this prospective cohort.

methodsThe PLS NHS is a prospective, longitudinal, multicenter study of people living with PLS in different diagnostic categories: early (disease duration <2 years); probable (2-4 years); and definite PLS (4-15 years). PLSFRS scores and other functional outcome measures were collected at baseline, 3-, 6-, 9-, and 12-month follow-up visits. Baseline characteristics were compared between the groups. The slopes of the PLSFRS and other functional outcome measures over 12 months were examined in the overall cohort and subgroups using linear mixed-effect models. The associations between baseline characteristics and the rate of PLSFRS decline were analyzed with linear regression models.

resultsA total of 76 participants were included: early (n = 6); probable (n = 26); and definite (n = 44) PLS. Baseline PLSFRS total scores were highest in the early PLS group, followed by the probable and definite PLS groups. In the overall cohort, the PLSFRS total score declined by 0.33 points/month (95% confidence interval [0.27-0.39], adjusted p < 0.05). The rate of decline was steepest in the early PLS group, followed by the probable and definite PLS groups. Baseline neurofilament light chain level was associated with the rate of PLSFRS decline over 1 year (p = 0.001).

interpretationIn PLS, the rate of functional decline, as measured by the PLSFRS total score, is faster during the early phase of the disease. Neurofilament light might serve as a prognostic biomarker in PLS. ANN NEUROL 2026;99:418-428.

Indexed as

Motor Neuron DiseaseOutcome Assessment, Health CareAdultAgedBiomarkersDisease ProgressionFemaleHumansLongitudinal StudiesMaleMiddle AgedNeurofilament ProteinsProspective StudiesSeverity of Illness IndexBiomarkersneurofilament protein LNeurofilament Proteins

Identifiers

PMID41020440
PMCPMC12871045

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Registered trials

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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.