1. Cranial magnetic resonance imaging in genetically proven myotonic dystrophy type 1 and 2
- Author
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Thomas Klockgether, Jürgen Reul, Niki Amanatidis, Wolfram Kress, Christoph Grothe, Rolf Schröder, and Cornelia Kornblum
- Subjects
Adult ,Male ,musculoskeletal diseases ,congenital, hereditary, and neonatal diseases and abnormalities ,medicine.medical_specialty ,Pathology ,Neurology ,Neuropsychological Tests ,Myotonic dystrophy ,Proximal myotonic myopathy ,Atrophy ,medicine ,Humans ,Myotonic Dystrophy ,Aged ,Neuroradiology ,Brain Mapping ,medicine.diagnostic_test ,business.industry ,Brain ,Magnetic resonance imaging ,Middle Aged ,medicine.disease ,Myotonia ,Magnetic Resonance Imaging ,Hyperintensity ,Female ,Neurology (clinical) ,Cognition Disorders ,business - Abstract
Cranial magnetic resonance imaging (MRI) in 19 German patients with genetically proven myotonic dystrophy Type 1 (DM1, n = 10) or Type 2 (DM2, n = 9) showed pathological findings consisting of white matter lesions (WML) and/or brain atrophy in 9/10 DM1 and 8/9 DM2 patients. Anterior temporal WML (ATWML) were exclusively seen in DM1 patients. Our findings indicate a high frequency of central nervous system (CNS) involvement in both disorders. However, temporopolar pathology, previously associated with intellectual dysfunction, seems to be restricted to DM1.
- Published
- 2004
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