Myotonic dystrophy type 1: Neuropsychological and brain magnetic resonance image study
نویسندگان
چکیده
منابع مشابه
Structural and functional cardiac changes in myotonic dystrophy type 1: a cardiovascular magnetic resonance study
BACKGROUND Myotonic dystrophy type 1 (MD1) is a neuromuscular disorder with potential involvement of the heart and increased risk of sudden death. Considering the importance of cardiomyopathy as a predictor of prognosis, we aimed to systematically evaluate and describe structural and functional cardiac alterations in patients with MD1. METHODS Eighty MD1 patients underwent physical examinatio...
متن کاملMyocardial fibrosis in patients with myotonic dystrophy type 1: a cardiovascular magnetic resonance study
BACKGROUND Myotonic dystrophy type 1 (DM1) is associated with increased cardiac morbidity and mortality. Therefore, assessment of cardiac involvement and risk stratification for sudden cardiac death is crucial. Nevertheless, optimal screening-procedures are not clearly defined. ECG, echocardiography and Holter-monitoring are useful but insufficient. Cardiovascular magnetic resonance (CMR) can p...
متن کاملMyotonic dystrophy type 1
Myotonic dystrophy type 1 (DM1) is a distal myopathy and a multisystem disease occurring with an incidence of 1/8000, as a result of a CTG trinucleotide repeat expansion in the serinetreonine-protein kinase (DMPK) coding gene on chromosome 19q13.3. In DM1 patients the length of the CTG expansion ranges from 50 to 4000. Disease severity correlates with repeat length and the phenomenon of genetic...
متن کاملBrain gray matter structural network in myotonic dystrophy type 1
This study aimed to investigate abnormalities in structural covariance network constructed from gray matter volume in myotonic dystrophy type 1 (DM1) patients by using graph theoretical analysis for further clarification of the underlying mechanisms of central nervous system involvement. Twenty-eight DM1 patients (4 childhood onset, 10 juvenile onset, 14 adult onset), excluding three cases from...
متن کاملBrain proton magnetic resonance spectroscopy and brain atrophy in myotonic dystrophy.
OBJECTIVES To evaluate by magnetic resonance spectroscopy the age-related cerebral alterations present in myotonic dystrophy (MD) and to compare these results with those obtained by magnetic resonance imaging. DESIGN Twenty-one patients (aged 16-63 years) with MD were compared with 16 age-matched healthy control subjects. RESULTS In magnetic resonance spectroscopy, the mean (+/- SD) ratio o...
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ژورنال
عنوان ژورنال: Journal of the Neurological Sciences
سال: 2015
ISSN: 0022-510X
DOI: 10.1016/j.jns.2015.08.247