Diffuse T1 reduction in gray matter of sickle cell disease patients: evidence of selective vulnerability to damage?

Neuroimaging abnormalities in adults with sickle cell anemia

“…[17][18][19][20][21] However, structural brain abnormalities of cortical thickness or basal ganglia volumes in neurologically asymptomatic adults with SCA have not been reported. Therefore, our observation that reduced cortical thickness and reduced volumes of subcortical gray matter are evident in adults with SCA is important.…”

“…5,6,8,9,14,15 However, because a significant proportion of both children and adults with SCA do not have cerebrovascular lesions, 5,13,16 additional CNS factors likely contribute to cognitive dysfunction. Frontal lobe cortical atrophy [17][18][19][20][21] and gray matter growth delays in the basal ganglia and thalamus reported in children and adolescents with SCA [22][23][24] may represent factors contributing to poorer intellectual function in adults with SCA. This study was conducted to evaluate MRI characteristics of neurologically asymptomatic adults with SCA to determine the relationship of these characteristics with IQ.…”

White Matter Damage in Asymptomatic Patients with Sickle Cell Anemia: Screening with Diffusion Tensor Imaging

“…2 While several studies have demonstrated neurocognitive deficits in the presence of overt [3][4][5] and silent stroke, [6][7][8] recent studies of large numbers of neurologically asymptomatic children and adults with SCA have demonstrated that cognitive impairment occurs even in the absence of brain abnormalities on conventional MR imaging. [9][10][11][12] Even though the method of neurocognitive assessment varied in these studies, the shared evidence supports both global IQ deficits, as well as domain specific deficits, in executive function and visual-spatial memory. 13 These observations support an emerging consensus that brain injury in SCA is diffuse and insidious, 14 and that conventional neuroimaging often underestimates the extent of injury.…”

Nocturnal Enuresis in Children and Adolescent with Sickle cell Anemia

“…Various brain injuries were described in children with SCA. A significant reduction of the grey matter involving the cortex, thalamus and caudate nucleus was reported [107]. Using a quantitative MRI increased T1 was observed in the thalamus, frontal white matter, genu, and occipital white matter [108] Regional cortical thinning particularly involving the precuneus and the posterior cingulate was described [109].…”