Developmental topographical disorientation: a newly discovered cognitive disorder

A variety of lesions in different cerebral regions may affect the human ability to orient in the environment, resulting in ‘topographical disorientation’. In a recent study, we documented the first case of Developmental Topographical Disorientation (DTD), in a person with a life-long inability to orient despite otherwise well-preserved cognitive functions, and in the absence of a cerebral injury/malformation or other neurological condition. This selective topographical disorientation was due to her inability to form a ‘cognitive map’, a mental representation of the environment, which in turn impaired her ability to orient in both familiar and unfamiliar surroundings. Here, we describe 120 new cases of DTD recruited via the internet and assessed with an online battery testing their cognitive and orientation skills. We found that people with DTD differ from matched (age, gender and education) healthy controls only in those skills confined to the orientation/navigation domain, among which the ability to form a cognitive map was the most significant factor that distinguished a person affected by DTD from control subjects.