Aim: To determine the type of neuropsychological dysfunction in myotonic dystrophy type 1 (DM1).
Methods: This study included 38 patients (17 males; mean age 41.6 ± 9.7 years) with the adult onset DM1 and the same number of matched healthy volunteers. Following neuropsychological tests were used: Mini Mental Score Examination (MMSE), Wechsler Adult Intelligence Scale (WAIS), Rey Auditory Verbal Learning Test (RAVLT), Rey−Osterrieth Complex Figure Test (RCF), Trail Making Test A and B (TMT-A and -B), Wisconsin Card Sorting Test (WCST), Boston Naming Test (BNT) and verbal fluency test (FAS).
Results: Average score on the MMSE of DM1 patients was 26.5 ± 2.0 and total IQ was 92.6 ± 13.2. RAVLT showed no impairment of immediate memory while recall and recognition were lower in DM1 patients in comparison to controls (p< 0.01). Copy, immediate and delayed recall of RCF were worse in patients than in controls (p< 0.01). The results of the TMT-B were decreased in patients (p< 0.01). Significant difference on WCST between patients and controls was observed only in the number of attempts to complete categories (p < 0.05). Score on the BNT test was worse in patients with DM1 in comparison to controls (p< 0.05). Score on the FAS did not differ in patients and controls (p>0.05).
Conclusion: Patients with DM1 had impaired cognitive status with executive dysfunction, visuoconstructional deficit, impaired memory and moderate impairment of speech function. These results are suggestive of frontal and to lesser degree of temporal lobe dysfunction in DM1.
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