Summary: Sickle cell disease can be complicated by cerebral white matter hyperintensities (WMHs), which are associated with diminished neurocognitive functioning. The influence of the total volume of WMHs on the degree of neurocognitive dysfunction has not yet been characterized. In our study of 38 patients (mean age 12·5 years) we demonstrated that a higher volume of WMHs was associated with lower full-scale intelligence quotient (IQ), verbal IQ, Processing Speed Index and more fatigue. Our results suggest that volume of WMHs is an additional parameter to take into account when planning individual diagnostic and treatment options.

Neurocognitive functioning, Sickle cell disease, Silent cerebral infarcts
dx.doi.org/10.1111/bjh.13179, hdl.handle.net/1765/89098
British Journal of Haematology
Department of Pediatrics

van der Land, V, Hijmans, A, de Ruiter, M, Mutsaerts, H.-J, Cnossen, M.H, Engelen, M, … Fijnvandraat, K. (2015). Volume of white matter hyperintensities is an independent predictor of intelligence quotient and processing speed in children with sickle cell disease. British Journal of Haematology, 168(4), 553–556. doi:10.1111/bjh.13179