Myotonic dystrophy type 1 (DM1) is caused by a CTG expansion in the DM protein kinase
(DMPK) gene; furthermore, there is a correlation between CTG repeat size and age of
onset.
1
There have been few reports on the psychiatric features of DM1 in older patients
because patients with adult-onset DM1 have a shorter life expectancy,
1
and late-onset oligosymptomatic DM1 often remains undiagnosed. Here, we describe
a case of late-onset oligosymptomatic DM1 mimicking prodromal dementia with Lewy bodies
(DLB) accompanied by severe psychosis.To read this article in full you will need to make a payment
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References
- The myotonic dystrophies: molecular, clinical, and therapeutic challenges.Lancet Neurol. 2012; 11: 891-905
- Diagnosis and management of dementia with Lewy bodies: fourth consensus report of the DLB consortium.Neurology. 2017; 89: 88-100
- Cognitive/personality pattern and triplet expansion size in adult myotonic dystrophy type 1 (DM1): CTG repeats, cognition and personality in DM1.Psychol Med. 2010; 40: 487-495
- Abnormal functional brain connectivity and personality traits in myotonic dystrophy type 1.JAMA Neurol. 2014; 71: 603-611
- Research criteria for the diagnosis of prodromal dementia with Lewy bodies.Neurology. 2020; 94: 743-755
Article info
Publication history
Published online: January 18, 2021
Identification
Copyright
© 2021 American Association for Geriatric Psychiatry. Published by Elsevier Inc. All rights reserved.
