Determining the neurocognitive dysfunction in children with Sickle Cell Disease

Prof. Fenella Kirkham (ICH, UCL)

The commonest neurological complication in children with sickle cell disease is stroke, with United States estimates suggesting that 25% of SCA will have had a stroke by the age of 45 years. This is thought to be due to an occlusive vasculopathy and measurement of cerebral blood flow velocity (CBFv) using Transcranial Doppler (TCD) ultrasonography can be used to predict high risk children. There is now increasing evidence that there is a high prevalence of ‘silent' cerebral infarcts occurring in 15-40% children with SCA who do not have a clinical history of stroke, in association with significant impairment of higher cognitive and executive function with reduced intellectual and academic performance and predictive of overt stroke. Detection of silent infarcts requires magnetic resonance imaging (MRI) which is not practical or economically feasible particularly in sub-Saharan Africa (SSA). Therefore, a combination of TCD examination and careful neuropsychometric evaluation has been suggested to be helpful in screening and identifying those children with early CNS pathology or silent infarcts which can then be confirmed by MRI.

In this project we will study children with SCA with careful measurement of CBFv by TCD, neuropsychometric evaluation followed by MRI imaging. The aim will be; (1) To determine the prevalence of silent cerebral infarcts in children with SCA children. (2) To evaluate the usefulness of TCD and neuropsychometric testing in identifying SCA children with CNS pathology.