Alteration of grey matter volume is associated with pain and quality of life in children with sickle cell disease

Document Type

Journal Article

Publication Date



Translational research : the journal of laboratory and clinical medicine






DARTEL = diffeomorphic anatomical registration through exponentiated lie algebra; GMV = grey matter volume; IPL = inferior parietal lobule; MCC = middle cingulate cortex,; MIC = mid insula cortex; MRI = magnetic resonance imaging; PCC = posterior cingulate cortex; PIC = posterior insula cortex; QOL = quality of life; ROI = region of interest; SCD = sickle cell disease; SCI = silent cerebral infarcts; SPM12 = Statistical Parametric Mapping; VBM = voxel-based morphometry; pgACC = perigenual anterior cingulate cortex; sgACC = subgenual anterior cingulate cortex


Pain is the most common symptom experienced by patients with sickle cell disease (SCD) and is associated with poor quality of life. We investigated the association between grey matter volume (GMV) and the frequency of pain crises in the preceding 12 months and SCD-specific quality of life (QOL) assessed by the PedsQL SCD module in 38 pediatric patients with SCD. Using voxel-based morphometry methodology, high-resolution T1 structural scans were preprocessed using SPM and further analyzed in SPSS. The whole brain multiple regression analysis identified that perigenual anterior cingulate cortex (ACC) GMV was negatively associated with the frequency of pain crises (r = -0.656, P = 0.003). A two-group t-test analysis showed that the subgroup having pain crisis/crises in the past year also showed significantly lower GMV at left supratemporal gyrus than the group without any pain crisis (p=0.024). The further 21 pain-related regions of interest (ROI) analyses identified a negative correlation between pregenual ACC (r = -0.551, P = 0.001), subgenual ACC (r = -0.540, P = 0.001) and the frequency of pain crises. Additionally, the subgroup with poorer QOL displayed significantly reduced GMV in the parahippocampus (left: P = 0.047; right: P = 0.024). The correlations between the cerebral structural alterations and the accentuated pain experience and QOL suggests a possible role of central mechanisms in SCD pain.