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Published on: July 19, 2019
White matter lesions in myotonic dystrophy type 1 co-locate with dilated perivascular spaces
Dimitri Renard1, Guillaume Taieb1
1Department of Neurology, CHU Nîmes, Hôpital Caremeau, Place du Pr Debré, 30029 Nîmes Cedex 4, France.
Background:
The pathophysiological mechanism of white matter lesions (WML) and dilated Virchow-Robin spaces (DVRS), both frequently encountered in myotonic dystrophy 1 (DM1), is unclear.
Methods:
We studied the spatial co-existence of WML and DVRS in 6 DM1 patients with a 1.5T or 3T brain MRI.
Results:
Co-localisation with DVRS was found in 86% of the WML. Some WML had a droplet, ovoid, of finger-like aspect following the exactly the orientation of the DVRS. Some WML were linear (indistinguishable from DVRS on T2-weighted imaging) whereas some other WML had a streak- or a dotted-like aspect.
Conclusion:
Our data suggest a pathophysiological relationship between the presence of WML and DVRS in DM1. Some WML might represent, pathologically modified, DVRS.
Insights
The study suggests a link between white matter lesions (WML) and dilated Virchow-Robin spaces (DVRS) in myotonic dystrophy 1 (DM1). Some WML may be pathologically altered DVRS, offering new insights into DM1 brain changes.
Area of Science:
- Neurology
- Neuroimaging
- Myotonic Dystrophy Research
Background:
- White matter lesions (WML) and dilated Virchow-Robin spaces (DVRS) are common in myotonic dystrophy 1 (DM1).
- The underlying pathophysiological mechanisms linking WML and DVRS in DM1 remain poorly understood.
Purpose of the Study:
- To investigate the spatial relationship between WML and DVRS in DM1 patients.
- To explore potential shared pathophysiological pathways.
Main Methods:
- Brain MRI scans (1.5T or 3T) were analyzed in 6 DM1 patients.
- The co-existence and spatial arrangement of WML and DVRS were examined.
Main Results:
- A significant co-localization of WML with DVRS was observed in 86% of cases.
- WML exhibited various shapes (droplet, ovoid, finger-like, linear, streak, dotted) often oriented along DVRS.
- Some WML appeared indistinguishable from DVRS on T2-weighted imaging.
Conclusions:
- The findings suggest a pathophysiological connection between WML and DVRS in DM1.
- Pathologically modified DVRS may contribute to the formation of WML in DM1.
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