Rudebeck, Sarah

This study investigated the cognitive trajectory and brain changes of those with Rasmussen syndrome (RS), a rare childhood disease characterised by atrophy of one hemisphere of the brain. Neuropsychology performance was estimated from historical neuropsychology assessments and 10 new neuropsychological assessments carried out as part as clinical practice by the first author. This resulted in 39 RS participants (right hemisphere affected=21, left hemisphere affected=18). Analyses were conducted to elucidate the changing cognitive trajectory at two time points: (1) pre-surgery and (2) pre- to post-op. Differences between left and right hemisphere groups were also investigated. Our study also explored cortical changes pre-surgery in a subset of RS participants using Voxel Based Morphometry. The possible neurobiological substrates of cognitive change were explored through correlational analyses.  Our results revealed that pre-surgery there were significant declines in both left and right RS groups in a number of cognitive domains. In addition, between groups analyses showed the right RS group exhibited more difficulties with tasks of perceptual reasoning, whereas the left RS group had weaker abilities on tasks requiring verbal faculties. From pre- to post-op the left RS group declined in all IQ abilities, whereas the right group’s abilities remained better preserved. The left RS group was also significantly weaker on tasks that required language than the right RS. VBM analyses showed that in a subset of our RS participants brain regions within the unaffected hemisphere of the brain significantly atrophied from 3 to 6 years post-onset of seizures. The decline in grey and white matter of the bilateral parietal and occipital lobes was significantly associated with decline in non-verbal IQ. These findings may have important implications for the medical and psychological care of those with RS, in particular, in regard to optimisation of clinical outcome. <br/

Rasmussen Syndrome: Cognitive Trajectories and Brain Changes

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