Abstract

We report the clinical case of AB, a right-handed 19-year-old woman who presents severe developmental topographical disorientation, a relatively rare syndrome, leading to difficulties in navigating in familiar (and novel) environments. This symptomatology appears without acquired cerebral damage (MRI described as normal) nor more global cognitive disability (high degree of education achieved).An extensive assessment of spatial cognition with different aspects of underlying cognitive processes is first presented. Second, the patient's preserved cognitive abilities and her major difficulties in calculation, as well as her attention deficit, as seen in a detailed neuropsychological assessment, are reported.For the first time to our knowledge, we show that developmental topographical disorientation can be associated with other developmental cognitive disorders affecting number processing (dyscalculia) and attention (Attention Deficit-Hyperactivity Disorder (ADHD)). We discuss the links between these different cognitive processes in relation to visuo-spatial working memory and magnitude representation, which could represent common denominators for all these syndromes.This case report highlights the importance of thoroughly assessing potentially associated neurocognitive disorders in developmental topographical disorientation. In addition, it highlights the necessity to keep in mind the prevalence of spatial difficulties in the assessment of children and adolescents with other neurodevelopmental syndromes. Finally, this case study raises a new question about the nosology of developmental disorders affecting the visuo-spatial and spatial domains.

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