好色先生

好色先生

Explore the latest content from across our publications
Join The AAN

Log In

Forgot Password?
Create New Account

Loading... please wait

Abstract Details

Multimodal Investigation of Brain Structural Changes, Neurocognitive Function, and Sleep Disruptions in Myotonic Dystrophy Type 1
General Neurology
P5 - Poster Session 5 (8:00 AM-9:00 AM)
2-008
The primary objective of this study is to investigate the alterations in brain white matter integrity, neurocognitive functioning, and sleep architecture in individuals living with Myotonic Dystrophy Type 1 (DM1). This study aims to identify neuroimaging biomarkers and patterns in polysomnography (PSG) that are associated with cognitive impairment, structural brain changes, and disrupted sleep. 
DM1 is the most common form of adult-onset muscular dystrophy. While DM1 primarily impacts skeletal muscles, CNS involvement has become increasingly recognized. Cognitive deficits, including impairments in executive function and memory, as well as brain structural abnormalities have been reported. Additionally, many individuals with DM1 experience disrupted sleep patterns. However, the relationship between brain structural changes, neurocognitive performance, and sleep dysfunction remains unexplored.
A cross-sectional study was conducted on a cohort of DM1 patients. The following data were collected: MRI scans, including Diffusion Tensor Imaging (DTI) and high-resolution functional MRI (fMRI), Polysomnography (PSG), and cognitive assessments. The following methods were applied: DTI-derived Fractional Anisotropy and Mean Diffusivity were used to assess white matter integrity in major tracts. PSG metrics were analyzed to explore correlations between sleep disturbances and neurocognitive performance. Supervised learning algorithms were employed to classify structural brain changes and sleep disruptions. These models were trained to predict cognitive impairments based on neuroimaging and PSG features.
The study identified significant reductions in white matter integrity. PSG analysis revealed decreased sleep efficiency and prolonged REM latency, with sleep disturbances correlating with cognitive impairments.

This study provides evidence of widespread white matter alterations and disrupted sleep patterns in  DM1 patients. These changes are strongly associated with cognitive impairments, suggesting that both structural and functional brain changes, along with sleep architecture disruptions, play a role in the cognitive dysfunction observed in DM1. The findings emphasize the value of combining multimodal data for comprehensive CNS monitoring in DM1.  
Authors/Disclosures
Tahereh Kamali, PhD (Stanford University)
PRESENTER 		Dr. Kamali has nothing to disclose.
Katharine A. Hagerman, PhD Dr. Hagerman has nothing to disclose.
Jacinda B. Sampson, MD, PhD Dr. Sampson has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Dyne Therapeutics. Dr. Sampson has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Viking Therapeutics. Dr. Sampson has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Biogen. Dr. Sampson has received research support from Marigold Foundation. Dr. Sampson has a non-compensated relationship as a Scientific Advisory Committee with Myotonic Dystrophy Foundation that is relevant to AAN interests or activities.
John W. Day, MD, PhD (Stanford University School of Medicine) Dr. Day has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Novartis Gene Therapy. Dr. Day has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Biogen. Dr. Day has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Roche/Genentech. Dr. Day has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Sarepta. Dr. Day has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Avidity. Dr. Day has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for PepGen. Dr. Day has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Epirium Bio. Dr. Day has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Solid Biosciences. Dr. Day has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Muscular Dystrophy Association. The institution of Dr. Day has received research support from Astellas Pharma. The institution of Dr. Day has received research support from Novartis Gene Therapy. The institution of Dr. Day has received research support from Biogen. The institution of Dr. Day has received research support from Roche/Genentech. The institution of Dr. Day has received research support from Sanofi/Genzyme. The institution of Dr. Day has received research support from Sarepta. The institution of Dr. Day has received research support from Scholar Rock. The institution of Dr. Day has received research support from AMO Pharma. The institution of Dr. Day has received research support from AnnJi. Dr. Day has received research support from CureSMA. The institution of Dr. Day has received research support from Muscular Dystrophy Association. The institution of Dr. Day has received research support from Ionis Pharmaceuticals. The institution of Dr. Day has received research support from NMD Pharma. The institution of Dr. Day has received research support from SMA Foundation. Dr. Day has received intellectual property interests from a discovery or technology relating to health care.