Neuropsychological implications of Crouzon's syndrome:: A case study

Introduction. Crouzon syndrome (CS) has traditionally been associated with moderated mental retardation. However, very few studies have quantified in these patients the degree of this mental retardation, and even less research has been carried out to evidenciate the typical neuropsychological profile, including affective, cognitive and executive functions. Clinical case. We present a single case-study of a 16 year-old, right -handed female affected of CS. Neuropsychological functions assessed included attention, concentration, memory, visual perception, language, arithmetic processes, praxis, executive functions, reasoning and general intelligence. Deficient results have been obtained in learning and memory, attention, language, graphic praxis, comprehension of social situations and executive functions (frontal lobes). Nevertheless, the patient kept within normal limits her spatial and temporal orientation, visual perception, visual-spatial and manipulative abilities and arithmetic processes. Conclusions. Results suggest that the intellectual defect in CS is not general and uniform and that some intellectual abilities are preserved, while others are affected in different degrees. The importance of carrying out a thorough neuropsychological assessment in these cases is emphasized [REV NEUROL 1999; 29: 1040-4].