Evidence map›Paper›PMID 42787039›Full record

SynthesisFrontiers in neurology2026

Cognitive decline over time in myotonic dystrophy type 1: a systematic review of longitudinal studies.

Ainara Clemente-Fernández, Irati Larranaga, Joana Garmendia, Andone Sistiaga, Garazi Labayru

Abstract readSystematic Review
In one paragraph

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

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

0 citing papers in PubMed.

No citing paper in PubMed yet.

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

5 authors.

Ainara Clemente-Fernández *School of Postgraduates, Faculty of Psychology, Universitat Autònoma de Barcelona (UAB), Barcelona, Spain.
Irati Larranaga *Department of Clinical and Health Psychology and Research Methodology, Faculty of Psychology, University of the Basque Country (EHU), San Sebastian, Spain.
Joana GarmendiaCentro de Investigación Biomédica en Red sobre Enfermedades Neurodegenerativas (CIBERNED), Carlos III Health Institute, Madrid, Spain.
Andone SistiagaDepartment of Clinical and Health Psychology and Research Methodology, Faculty of Psychology, University of the Basque Country (EHU), San Sebastian, Spain.
Garazi LabayruDepartment of Clinical and Health Psychology and Research Methodology, Faculty of Psychology, University of the Basque Country (EHU), San Sebastian, Spain.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Background: Myotonic Dystrophy Type 1 (DM1) is a multisystem genetic disorder and the most common muscular dystrophy in adults. Central nervous system involvement is well-documented in DM1, including cognitive impairment. Cross-sectional studies have described a heterogeneous neuropsychological profile; however, the trajectory of cognitive functioning over time remains unclear, highlighting the need to focus on longitudinal studies. This systematic review aimed to examine longitudinal evidence on cognitive progression in DM1 and to analyze patterns of decline across clinical phenotypes. Methods: Following PRISMA 2020 guidelines, a systematic search was conducted in three databases: PubMed, Web of Science, and Scopus. Eligible studies were original longitudinal investigations assessing cognitive performance in DM1 using standardized measures. Risk of bias was assessed using an adapted Newcastle-Ottawa Scale. Results: Sixteen studies met the inclusion criteria, with follow-up periods ranging from 1 to 20 years. In pediatric phenotypes, intellectual disability and global cognitive impairment were evident at baseline, with largely stable trajectories over time. In contrast, adult- and late-onset phenotypes reported a pattern of slow, progressive, domain-specific cognitive decline, particularly in visuoconstruction and processing speed. Age and disease duration emerged as the most consistent predictors of decline over time. Conclusion: The available evidence supports a dual-process model of cognitive dysfunction in DM1, combining early neurodevelopmental impairment with later domain-specific neurodegenerative decline. These findings highlight the importance of longitudinal cognitive monitoring and suggest that specific cognitive domains may serve as sensitive markers for disease progression and potential endpoints in clinical trials. Systematic review registration: https://www.crd.york.ac.uk/PROSPERO/view/CRD420251148417, identifier (CRD420251148417).

Indexed as

Cognitive DysfunctionMyotonic DystrophyDisease ProgressionHumansLongitudinal Studiescentral nervous systemcognitive declinelongitudinal studiesmyotonic dystrophy type 1Steinert’s diseasesystematic review

Identifiers

PMID42787039
PMCPMC13600866

What Socratic holds

Textmetadata
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 Socratic graph.