Cognitive function and its relationship with brain structure in myotonic dystrophy type 1

Kathleen E Langbehn; Ellen Plas; David J Moser; Jeffrey D Long; Laurie Gutmann; Peggy C Nopoulos

doi:10.1002/jnr.24595

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Cognitive function and its relationship with brain structure in myotonic dystrophy type 1

Journal article

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Peer reviewed

Cognitive function and its relationship with brain structure in myotonic dystrophy type 1

Kathleen E Langbehn, Ellen Plas, David J Moser, Jeffrey D Long, Laurie Gutmann and Peggy C Nopoulos

Journal of neuroscience research, Vol.99(1), pp.190-199

01/2021

DOI: 10.1002/jnr.24595

PMCID: PMC7872331

PMID: 32056295

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https://www.ncbi.nlm.nih.gov/pmc/articles/7872331View

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Abstract

Studies have shown relationships between white matter abnormalities and cognitive dysfunction in myotonic dystrophy type 1 (DM1), but comprehensive analysis of potential structure–function relationships are lacking. Fifty adult‐onset DM1 individuals (33 female) and 68 unaffected adults (45 female) completed the Wechsler Adult Intelligence Scale‐IV (WAIS‐IV) to determine the levels and patterns of intellectual functioning. Neuroimages were acquired with a 3T scanner and were processed with BrainsTools. Regional brain volumes (regions of interest, ROIs) were adjusted for inter‐scanner variation and intracranial volume. Linear regression models were conducted to assess if group by ROI interaction terms significantly predicted WAIS‐IV composite scores. Models were adjusted for age and sex. The DM1 group had lower Perceptual Reasoning Index (PRI), Working Memory Index (WMI), and Processing Speed Index (PSI) scores than the unaffected group (PRI t(113) = −3.28, p = 0.0014; WMI t(114) = −3.49, p = 0.0007; PSI t(114) = −2.98, p = 0.0035). The group by hippocampus interaction term was significant for both PRI and PSI (PRI (t(111) = −2.82, p = 0.0057; PSI (t(112) = −2.87, p = 0.0049)). There was an inverse association between hippocampal volume and both PRI and PSI in the DM1 group (the higher the volume, the lower the intelligence quotient scores), but no such association was observed in the unaffected group. Enlarged hippocampal volume may underlie some aspects of cognitive dysfunction in adult‐onset DM1, suggesting that increased volume of the hippocampus may be pathological. Myotonic dystrophy type 1 (DM1)‐affected adults (blue) exhibit lower scores on measures of cognitive function than unaffected adults (pink). These differences are associated with enlarged hippocampal volume in DM1‐affected individuals for Wechsler Adult Intelligence Scale‐IV index measures of perceptual reasoning (Panel a) and processing speed (Panel b).

DM1

magnetic resonance imaging

WAIS‐IV

neuroanatomy

hippocampus

cognitive impairments

Details

Title: Subtitle: Cognitive function and its relationship with brain structure in myotonic dystrophy type 1
Creators: Kathleen E Langbehn - University of Iowa Hospitals & Clinics
Ellen Plas - University of Iowa Hospitals & Clinics
David J Moser - University of Iowa Hospitals & Clinics
Jeffrey D Long - University of Iowa Hospitals & Clinics
Laurie Gutmann - University of Iowa Hospitals & Clinics
Peggy C Nopoulos - University of Iowa Hospitals & Clinics
Resource Type: Journal article
Publication Details: Journal of neuroscience research, Vol.99(1), pp.190-199
DOI: 10.1002/jnr.24595
PMID: 32056295
PMCID: PMC7872331
NLM abbreviation: J Neurosci Res
ISSN: 0360-4012
eISSN: 1097-4547
Number of pages: 10
Grant note: National Institute of Neurological Disorders and Stroke (5R01NS094387‐04) Wyck Foundation
Language: English
Date published: 01/2021
Academic Unit: Neurology; Psychiatry; Stead Family Department of Pediatrics; Iowa Neuroscience Institute; Biostatistics; Medicine Administration
Record Identifier: 9984070545802771

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