Js. Rubinsztein et al., MILD MYOTONIC-DYSTROPHY IS ASSOCIATED WITH MEMORY IMPAIRMENT IN THE CONTEXT OF NORMAL GENERAL INTELLIGENCE, Journal of Medical Genetics, 34(3), 1997, pp. 229-233
Previous investigations of cognitive function in myotonic dystrophy (D
M) suggested that DM subjects have impairment of general intelligence
and severe defects in executive function. These studies had to neglect
patients with mild clinical disease, since they were generally perfor
med before the availability of DNA testing for DM. We have investigate
d a group of milder DM cases (defined both by their degree of disabili
ty and CTG expansion size) and have not found any defect in general in
telligence using the National Adult Reading Test, Standard Progressive
Matrices, and Mini-Mental State examination. Three executive function
tests were used. The cognitive estimates test and the Wisconsin Card
Sorting Test showed tendencies towards impairment, but the verbal flue
ncy task was performed normally. However, in the face of normal genera
l intelligence, mild DM subjects as a group show impairment of memory
function. This suggests that memory function may be more sensitive to
small CTG repeat expansions than general intelligence.