对Mbius综合征成年患者认知功能的评估

Cognitive evaluation in adult patients with Mbius syndrome

Objective: To establish the occurrence of mental retardation in a group of patients with Mbius syndrome and subsequently, if mental retardation is absent, to screen major aspects of memory and attention, in order to assess possible pervasive dysfunction in these cognitive domains which might be responsible for the current view that mental retardation occurs frequently in Mbius syndrome. Meth ods: In a group of 12 Dutch Mbius patients, intellectual performance, memory f unction and attention were assessed using a number of standardized neuropsycholo gical tests. Results: The mean general intellectual performance did not differ s ignificantly from that of the Dutch population. Screening of selective attention and memory did not provide indications of pervasive dysfunctions in these domai ns. Conclusion: The rate of occurrence of mental retardation in our group of M bius patients did not differ from that in the normal Dutch population. Furthermo re, there was no evidence of attention and memory dysfunction in our group of M ¨obius patients.