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Brain MRI abnormalities in the adult form of myotonic dystrophy type 1: A longitudinal case series study
- Source :
- The Neuroradiology Journal. 29:36-45
- Publication Year :
- 2016
- Publisher :
- SAGE Publications, 2016.
-
Abstract
- This study aimed to verify whether brain abnormalities, previously described in patients with myotonic dystrophy type 1 (DM1) by magnetic resonance imaging (MRI), progressed over time and, if so, to characterize their progression. Thirteen DM1 patients, who had at least two MRI examinations, were retrospectively evaluated and included in the study. The mean duration (± standard deviation) of follow-up was 13.4 (±3.8) years, over a range of 7–20 years. White matter lesions (WMLs) were rated by semi-quantitative method, the signal intensity of white matter poster-superior to trigones (WMPST) by reference to standard images and brain atrophy by ventricular/brain ratio (VBR). At the end of MRI follow-up, the scores relative to lobar, temporal and periventricular WMLs, to WMPST signal intensity and to VBR were significantly increased compared to baseline, and MRI changes were more evident in some families than in others. No correlation was found between the MRI changes and age, onset, disease duration, muscular involvement, CTG repetition and follow-up duration. These results demonstrated that white matter involvement and brain atrophy were progressive in DM1 and suggested that progression rate varied from patient to patient, regardless of age, disease duration and genetic defect.
- Subjects :
- Adult
Male
0301 basic medicine
Brain
Magnetic resonance imaging
Myotonic dystrophy type 1
White matter disease
Neurology (clinical)
Radiology, Nuclear Medicine and Imaging
medicine.medical_specialty
Pathology
Adolescent
Sensitivity and Specificity
Myotonic dystrophy
White matter
Young Adult
03 medical and health sciences
0302 clinical medicine
Atrophy
Nuclear Medicine and Imaging
Internal medicine
medicine
Brain mri
Humans
Myotonic Dystrophy
Radiology, Nuclear Medicine and imaging
Longitudinal Studies
Child
Brain Imaging
medicine.diagnostic_test
business.industry
Brain, magnetic resonance imaging, myotonic dystrophy type 1, white matter disease
Reproducibility of Results
General Medicine
Middle Aged
medicine.disease
Magnetic Resonance Imaging
Hyperintensity
030104 developmental biology
medicine.anatomical_structure
Disease Progression
Cardiology
Female
Radiology
business
030217 neurology & neurosurgery
Case series
Adult form
Subjects
Details
- ISSN :
- 23851996 and 19714009
- Volume :
- 29
- Database :
- OpenAIRE
- Journal :
- The Neuroradiology Journal
- Accession number :
- edsair.doi.dedup.....079f74f9a30a1b669cc91a87f5851fa1