Hogan, Alexandra Marie; (2003) Development of executive function in infants and children with sickle cell disease: A neuropsycholological and electrophysiological study. Doctoral thesis (Ph.D), UCL (University College London).

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Abstract

Sickle Cell Disease (SCD) is an inherited disorder of haemoglobin, mainly affecting people of Afro-Caribbean origin. SCD often becomes symptomatic from the first year of life, when foetal haemoglobin is replaced by adult haemoglobin. Up to 25% of patients have discrete white matter brain lesions by mid-childhood, which may be detected on MRI but are not usually associated with neurological signs or symptoms. There is evidence that the frontal lobes are the most common site of neuropathology, but few studies have systematically attempted to examine executive function and/or relate the locus of lesion to cognitive dysfunction. Moreover, although neuropathology is evident very early in life, its possible relationship to neuro-developmental delay in infants has been largely ignored. Two longitudinal studies are reported in this thesis. The first was a prospective study of a group of infants with SCD (n=15) and controls (n=14) at three critical ages of early development (3-, 9-, and 12-months). Infants with SCD were impaired relative to controls from age 3-months on a neurodevelopmental screen, but not on experimental measures purporting to assess the early development of executive function. There were differences, however, in the event related potentials (ERPs) of SCD infants compared to controls, suggesting early abnormality in attention and recognition memory. The second study examined long-term cognitive outcome in children and young adults with SCD (n=50) and a group of sibling controls (n=26). Patients with and without brain lesions (confirmed by MRI) showed deficits in attention, whereas only patients with brain lesions were impaired on tests of working memory and response inhibition. Abnormalities were also evident in two ERP experiments assessing neural response to novelty and action-monitoring. It is concluded that patients with SCD may be at risk of neuro-developmental delay from infancy, and that this may manifest as executive dysfunction as the child matures into adolescence.

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