Magnetic Resonance Imaging and Clinical Correlates of Intellectual Impairment in Myotonic Dystrophy

Cognitive Function in Myotonic Dystrophy: A Follow-Up Study

“…In more recent studies, however, the intellectual level has been at least slightly below the normal level [2][3][4][5][6][7][8]. The relative proportion of DM patients with mental retardation has usually varied from 10 to 24% [3,5,[6][7][8], but Cencori et al [9] found significant global intellectual impairment in 50% of the patients (using the Raven's Colored Progressive Matrices as a test). In two studies no patient scored in the mentally retarded range [2,4], The present material includes about 10% of the Finnish DM patients, and 50% of the esti mated number of DM patients living in this area.…”

“…Impairment of intellectual and cognitive function in DM has been found in psychometric studies [2][3][4][5][6][7][8][9], some patients showing substantial mental retardation. Impair ment seems to be more pronounced among patients who have inherited the disease from the mother [3,5,7], Two studies [3,9] claim more pronounced intellectual impair ment among female DM patients. Studies on the relation ship between physical disability and impairment of cogni tive function are controversial [2,3,7,9].…”

“…Impair ment seems to be more pronounced among patients who have inherited the disease from the mother [3,5,7], Two studies [3,9] claim more pronounced intellectual impair ment among female DM patients. Studies on the relation ship between physical disability and impairment of cogni tive function are controversial [2,3,7,9]. Although mus cular involvement is progressive, it is unknown whether this is also true regarding mental function over a period of time as anticipated by some authors [10,11].…”

Dilated Virchow-Robin Spaces in Myotonic Dystrophy: Frequency, Extent and Significance

“…Neuropathological [4, 5], magnetic resonance imaging (MRI) [6], single-photon emission computed tomography [7], positron emission tomography [8], proton MR spectroscopy [9], MR relaxometry [10]and neurophysiological [11]studies have demonstrated several brain abnormalities in patients with DM. However, many issues, such as the parts of the brain primarily involved in the disease and the fundamental differences between the likely developmental abnormalities in congenital DM and the apparently degenerative changes in adult-type DM, remain to be investigated [12].…”

“…Glantz et al [14]found periventricular WMLs in 43% of patients, while Bachmann et al [15]found mainly subcortical WMLs. Some authors [6, 16]described characteristic anterior temporal WMLs, which were considered a volume averaging effect with the surrounding subarachnoid spaces by others [7]. Bachmann et al [15]reported a high frequency of dilated Virchow-Robin spaces (VRSs) in patients with DM but did not evaluate their location, extent and strength of association with the disease.…”