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. 2024 May 8;10(10):e30875.
doi: 10.1016/j.heliyon.2024.e30875. eCollection 2024 May 30.

Neurocognitive disorder in Myotonic dystrophy type 1

Stefan Winblad  1 , Olöf Eliasdottir  2 , Sara Nordström  2 , Christopher Lindberg  2   3
Affiliations

Neurocognitive disorder in Myotonic dystrophy type 1

Stefan Winblad et al. Heliyon. .

Abstract

Cognitive deficits and abnormal cognitive aging have been associated with Myotonic dystrophy type 1 (DM1), but the knowledge of the extent and progression of decline is limited. The aim of this study was to examine the prevalence of signs of neurocognitive disorder (mild cognitive impairment and dementia) in adult patients with DM1. A total of 128 patients with childhood, juvenile, adult, and late onset DM1 underwent a screening using the Montreal Cognitive Assessment (MoCA). Demographic and clinical information was collected. The results revealed that signs of neurocognitive disorder were relatively rare among the participants. However, 23.8 % of patients with late onset DM1 (aged over 60 years) scored below MoCA cut-off (=23), and this group also scored significantly worse compared to patients with adult onset. Age at examination were negatively correlated with MoCA scores, although it only explained a small portion of the variation in test results. Other demographic and clinical factors showed no association with MoCA scores. In conclusion, our findings indicate a low prevalence of signs of neurocognitive disorder in adult patients with DM1, suggesting that cognitive deficits rarely progress to severe disorders over time. However, the performance of patients with late onset DM1 suggests that this phenotype warrants further exploration in future studies, including longitudinal and larger sample analyses.

Keywords: Cognition; Dementia; Montreal cognitive assessment; Myotonic dystrophy type 1; Neurocognitive disorder.

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Conflict of interest statement

The authors declare the following financial interests/personal relationships which may be considered as potential competing interestsChristopher Lindberg reports financial support was provided by NEURO Sweden.

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Further reading

    1. Perini G.I., Colombo G., Armani M., Pellegrini A., Ermani M., Miotti M., Angelini C. Intellectual impairment and cognitive evoked potentials in myotonic dystrophy. J. Nerv. Ment. Dis. 1989 Dec;177(12):750–754. doi: 10.1097/00005053-198912000-00007. PMID: 2592965. - DOI - PubMed
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    1. Antonini G., Soscia F., Giubilei F., De Carolis A., Gragnani F., Morino S., Ruberto A., Tatarelli R. Health-related quality of life in myotonic dystrophy type 1 and its relationship with cognitive and emotional functioning. J. Rehabil. Med. 2006 May;38(3):181–185. doi: 10.1080/16501970500477967. PMID: 16702085. - DOI - PubMed

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