COGNITIVE FUNCTION IN EARLY ADULT AND ADULT-ONSET MYOTONIC-DYSTROPHY

Introduction - Most studies revealing intellectual deficits in myotoni c dystrophy (MyD) involved heterogeneous groups of patients with respe ct to intelligence and onset of disease. The present study was underta ken to investigate whether patients with early adult and adult MyD sho w subtle cognitive deficits despite a normal intelligence. Materials a nd methods - We compared 26 MyD patients of normal intelligence with m ild symptoms and early adult and adult onset to 25 matched control sub jects (CS) on a range of neuropsychological tests and a number of moto r tasks of increasing complexity, which required increasing cognitive control. Results - The groups did not differ as far as the neuropsycho logical tests were concerned, with the exception of the Stroop Color W ord Test. With respect to motor performance, the MyD patients were poo rer scorers on simple and automatic motor tasks than CS, but the diffe rence disappeared as the complexity of the tasks increased and require d correspondingly more cognitive control. Conclusion - We found hardly any evidence of cognitive dysfunction in our group of MyD patients wi th early adult and adult onset.