The authors studied in two occasions a group of 12 patients with myotonic dystrophy in a mean interval of three years and a half between the examinations. The neuro - psychological battery included the following tests: Raven's progressive matrices (coloured and general scales) , Wechsler children intelligence scale (WISC), Kohs' blocks and Piaget-Head. 50% of the patients had better scores on the second examination on RCPM, 81.89% on WISC-digit span, 63.67% on WISC-numbers, 44.44% on Piaget-Head 2 and 60% on Kohs' blocks. However, on Piaget-Head 1-3, the majority had worse results (87.56%) with statistical significative difference (p <0.05). Though the scores from Kohs' blocks were better in 60% of the patients with p< 0.05, we have to consider that 60% had 0 point on first examination being their scores a little better on second one. This is enough to result on statistical significative difference, however, very low if compared to normal subjects.
myotonic dystrophy; myotonia atrophica; psychological exam; longitudinal evaluation