Neuropsychological deficits in myotonic muscular dystrophy.

Abstract

Twenty patients with myotonic muscular dystrophy (MMD) were compared with twenty controls on a battery of standardised neuropsychological tests measuring motor and cognitive functions. The MMD patients performed significantly poorer on both motor and cognitive tests, particularly those assessing spatial functions. Although both motor and cognitive scores were correlated with age, significant diagnostic group by age interactions were present only for the motor measures. Therefore, while motor deficits in MMD may progress with ageing, cognitive deficits are mainly developmental and relatively stable.

Cerebral involvement in myotonic dystrophies

“…Several authors tried to correlate neuropsychological and neuroimaging findings. 6,24,27,28,45,62,101 Meola et al 71,72 confirmed a selective impairment on tests of frontal lobe function in DM1 and suggested that this type of deficit does not correlate with cortical atrophy and white matter hyperintense lesions, but might be associated with fronto-temporal lobe hypoperfusion on PET studies.…”

The Kohs' blocks test as an important instrument to investigate the visuo-spatial impairments in myotonic dystrophy: part I. Quantitative and qualitative analysis

“…Malloy et al 22 and Censori et al 23 have also described impairment of the visuo-spatial functions and constructive abilities. Tests of language, verbal memory and the prefrontal executive functions were intact, while spatial and nonverbal memory abilities were considered significantly impaired 22 .…”

“…Tests of language, verbal memory and the prefrontal executive functions were intact, while spatial and nonverbal memory abilities were considered significantly impaired 22 .…”

Cognitive deficits and personality patterns in maternally versus paternally inherited myotonic dystrophy

“…Previous neuropsychological studies have documented that MD is associated with declines in cognitive functioning, albeit with wide interpatient variability (e.g., Bird, Follett, & Griep, 1983;Censori, Danni, Del Pesce, ' & Provinciali, 1990;Franzese et al, 1991;Huber et al, 1989;Malloy, Mishra, & Adler, 1990;Sinforiani et al, 1991;Walker, Rosser, Mastaglia, & Walton, 1984;Woodward, Heaton, Simon, & Ringel, 1982). Beyond the consensus that MD can be associated with cognitive decline, previous studies have yielded many conflicting findings.…”

“…For example, even among patients with normal range global intellectual level, Censori et al (1990) documented relatively impaired performance on tasks of visual-spatial and constructional abilities (as assessed by Koh's Blocks, and pencil and paper copying). Malloy et al (1990) also observed particular impairment in nonverbal/ spatial abilities among MD pq$ients (measured by a three-dimensional constructional task, Judgment of Line Orientation, Block Design, and Picture Ardingement). In addition, while finding no significant differences in WAIS-R verbal subtests among MD patients versus a neurological patient control group (without CNS disorders), Walker et al (1984) observed that the MD patients had significantly lower scores on visual-spatialkonstructional subtests (i.e., Block Design, Picture Arrangement, and Object Assembly).…”