Evidence map›Paper›PMID 41316718›Full record

ArticleAnnals of neurology2026

The Epidemiology of Primary Lateral Sclerosis: Results from a Population-Based Cohort.

Rosario Vasta, Enrico Matteoni, Giorgio Pellegrino, Antonio Canosa, Umberto Manera, Francesca Palumbo, Maurizio Grassano, Sara Cabras, Alessandra Maccabeo, Fabrizio D'Ovidio and 8 more

Abstract read
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 1 paper.

0numbers the graph read from it
0cells of the map it votes in
1citing 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

1 citing paper in PubMed.

  1. 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

18 authors.

Rosario Vasta *ALS Center, Department of Neuroscience "Rita Levi Montalcini", University of Turin, Turin, Italy.ORCID 0000-0002-0393-4736
Enrico Matteoni *ALS Center, Department of Neuroscience "Rita Levi Montalcini", University of Turin, Turin, Italy.
Giorgio PellegrinoALS Center, Department of Neuroscience "Rita Levi Montalcini", University of Turin, Turin, Italy.
Antonio CanosaALS Center, Department of Neuroscience "Rita Levi Montalcini", University of Turin, Turin, Italy.ORCID 0000-0001-5876-4079
Umberto ManeraALS Center, Department of Neuroscience "Rita Levi Montalcini", University of Turin, Turin, Italy.
Francesca PalumboALS Center, Department of Neuroscience "Rita Levi Montalcini", University of Turin, Turin, Italy.
Maurizio GrassanoALS Center, Department of Neuroscience "Rita Levi Montalcini", University of Turin, Turin, Italy.
Sara CabrasALS Center, Department of Neuroscience "Rita Levi Montalcini", University of Turin, Turin, Italy.ORCID 0000-0002-4988-2855
Alessandra MaccabeoALS Center, Department of Neuroscience "Rita Levi Montalcini", University of Turin, Turin, Italy.
Fabrizio D'OvidioALS Center, Department of Neuroscience "Rita Levi Montalcini", University of Turin, Turin, Italy.
Gabriele MoraALS Center, Department of Neuroscience "Rita Levi Montalcini", University of Turin, Turin, Italy.
Salvatore GalloneALS Center, Department of Neuroscience "Rita Levi Montalcini", University of Turin, Turin, Italy.
Elisa D'AngeloALS Center, Department of Neuroscience "Rita Levi Montalcini", University of Turin, Turin, Italy.
Letizia MazziniALS Center, Department of Neurology, Azienda Ospedaliero Universitaria Maggiore della Carità, and University of Piemonte Orientale, Novara, Italy.
Fabiola De MarchiALS Center, Department of Neurology, Azienda Ospedaliero Universitaria Maggiore della Carità, and University of Piemonte Orientale, Novara, Italy.
Cristina MogliaALS Center, Department of Neuroscience "Rita Levi Montalcini", University of Turin, Turin, Italy.
Adriano Chiò *ALS Center, Department of Neuroscience "Rita Levi Montalcini", University of Turin, Turin, Italy.ORCID 0000-0001-9579-5341
Andrea Calvo *ALS Center, Department of Neuroscience "Rita Levi Montalcini", University of Turin, Turin, Italy.ORCID 0000-0002-5122-7243

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

objectiveIn this population-based study, we described the epidemiology of primary lateral sclerosis (PLS) in northern Italy and compared the clinical characteristics of patients with PLS to those with predominant upper motor neuron (PUMN) involvement and classic amyotrophic lateral sclerosis (ALS).

methodsPatients from the PARALS registry diagnosed with probable or definite PLS between 2007 and 2021 were included. Crude annual incidence rates were calculated, along with age- and sex-specific rates. A survival analysis was performed to identify prognostic factors at diagnosis. Covariates included sex, age at onset, site of onset, diagnostic delay, forced vital capacity (FVC), change in ALS Functional Rating Scale (ΔFRS), and change in body mass index (ΔBMI).

resultsA total of 57 PLS patients (2.7%) were included, with a crude incidence rate of 0.084 per 100,000 person-years. Compared to PUMN and classic ALS, PLS patients were younger (median onset age 63.5 years, interquartile range [IQR] 54.9-70.4) and predominantly female (male-to-female ratio 0.58). Bulbar onset occurred in 11 cases (19.3%), all of whom later developed spinal symptoms. At censoring, 38 patients (66.7%) were still alive (median survival 8.3 years, IQR 5.7-12.3), corresponding to a point prevalence of 0.89 per 100,000. Survival was significantly associated with age at onset (hazard ratio [HR] 1.17, 95% confidence interval [CI]: 1.05-1.33, p = 0.001), male sex (HR 4.41, 95% CI: 1.24-15.6, p = 0.02), and FVC at diagnosis (HR 0.95, 95% CI: 0.93-0.98, p = 0.006).

interpretationPLS was confirmed to be rarer than other ALS phenotypes. Patients had a higher age at onset than previously reported and a female predominance. Sex, age at onset, and respiratory function were key prognostic factors. ANN NEUROL 2026;99:606-613.

Indexed as

Motor Neuron DiseaseAdultAgedAge of OnsetAmyotrophic Lateral SclerosisCohort StudiesFemaleHumansIncidenceItalyMaleMiddle AgedRegistries

Identifiers

PMID41316718
PMCPMC12954144

What OpenQuestion holds

Textmetadata
LicenceCC BY
Read underepoch 390

Registered trials

None linked

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.