Compromised affect and learning associated with Crouzon Syndrome – a clinical case study

Abstract

Several reported case studies suggest that Crouzon Syndrome is characterized by a specific pattern of lesioned brain areas, which led to the exploration of how these structural brain lesions relate to the learner’s affective, social, and cognitive behaviour. However, these case studies were reported from highly specialized neurological and genetic perspectives, with no attempt at merging the triad, i.e., specific brain circuitry, learning and affect. This research project thus aims to describe compromised affect and learning associated with Crouzon Syndrome on the basis of a clinical case study, and of three critical inferences based upon a review of relevant literature. The first inference is that a craniofacial condition such as Crouzon Syndrome might be classified as a pervasive developmental disorder, since the brain is not fully developed at the time of diagnosis, while the concept pervasive suggests that these impairments significantly affect individuals throughout their lives. The second inference implicates a neural substrate to compromised learning associated with Crouzon Syndrome, therefore the expectation is that individuals diagnosed with Crouzon Syndrome might show a particular profile of compromised learning. As a result of specific lesioned brain areas, the third inference is that compromised affect associated with Crouzon Syndrome is delicately entwined with compromised learning. Human cognitive behaviour and emotions involve specific and delicately intertwined brain-operating systems, and it can be expected that the same brain-operating systems also underlie compromised affect associated with Crouzon Syndrome. Therefore, this project aims to scrutinize the neural makeup of Crouzon Syndrome based on a clinical case study, in order to compile a detailed explanatory profile of compromised affect and learning associated with Crouzon Syndrome. This is done using a mixed-method approach which involves both quantitative and qualitative research.