Evidence map›Paper›PMID 42258595›Full record

ArticleNeuroepidemiology2026

Occurrence and Mortality of Stiff Person Syndrome in Denmark.

Henrik Toft Sørensen, Trine Toft Sørensen, Holly Elser, Erzsébet Horváth-Puhó

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Article in Neuroepidemiology, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

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0citing papers in PubMed
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1 · What the graph read from it

What it found

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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

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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

0 citing papers in PubMed.

No citing paper in PubMed yet.

4 · The record

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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

4 authors.

Henrik Toft SørensenDepartment of Clinical Epidemiology and Center for Population Medicine, Aarhus University and Aarhus University Hospital, Aarhus, Denmark, hts@clin.au.dk.
Trine Toft SørensenNational Institute of Public Health, University of Southern Denmark, Copenhagen, Denmark.
Holly ElserDepartment of Clinical Epidemiology and Center for Population Medicine, Aarhus University and Aarhus University Hospital, Aarhus, Denmark.
Erzsébet Horváth-PuhóDepartment of Clinical Epidemiology and Center for Population Medicine, Aarhus University and Aarhus University Hospital, Aarhus, Denmark.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

introductionStiff person syndrome is a rare and progressive neurological disorder, yet evidence on long-term outcomes is limited. We examined the occurrence and mortality associated with stiff person syndrome in Denmark.

methodsWe conducted a population-based cohort study including all Danish citizens from 2001 to 2022. Individuals with a first-time diagnosis of stiff person syndrome (N = 30) were identified in the Danish National Patient Registry covering all Danish hospitals and matched 1:100 on sex and birth year with persons from the general population (N = 3,000). Incidence, prevalence, cumulative mortality, and mortality rates were estimated, and Cox proportional hazards regression was used to compute hazard ratios (HRs) as an estimate of mortality rate ratios adjusted for sex, year of birth, and comorbidity with 95% confidence intervals (CIs).

resultsThirty patients were diagnosed during the study period; 53.3% were male, and median age at diagnosis was 50 years. Incidence increased from 0.11 per 1,000,000 person-years (2001-2008) to 0.43 (2017-2022). Prevalence rose from 1.32 per 1,000,000 in 2016 to 3.94 in 2022. The 10-year cumulative mortality in the stiff person syndrome cohort was 27.5% (95% CI: 13.8-50.1) (HR = 4.1 [95% CI: 1.9-8.9).

conclusionStiff person syndrome is a rare disorder with increasing incidence and prevalence and high mortality.

Indexed as

Cohort studyIncidenceOverall mortalityPrevalenceStiff person syndrome

Identifiers

PMID42258595
PMCPMC13412219

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