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Neurology. 2018 Jun 5;90(23):e2042-e2050. doi: 10.1212/WNL.0000000000005644. Epub 2018 May 11.

White matter integrity and processing speed in sickle cell anemia.

Author information

1
From Developmental Neurosciences (H.S., F.J.K., M.K., P.B., J.D.C., S. Sahota, S. Sakaria, C.A.C., J.M.K.), UCL Great Ormond Street Institute of Child Health, London; University Hospital Southampton NHS Foundation Trust (F.J.K.); Clinical and Experimental Sciences (F.J.K.), University of Southampton; Department of Radiology (D.E.S.), Great Ormond Street Hospital NHS Foundation Trust, London; Department of Haematology and Evelina Children's Hospital (J.H., R.K.-A., B.I., M.P.), Guy's and St Thomas' NHS Foundation Trust, London; King's College Hospital NHS Foundation Trust (S.C., D.C.R., M.A.), London; North Middlesex University Hospital NHS Foundation Trust (O.W.), London; and Department of Haematology (M.L.), Imperial College Healthcare NHS Foundation Trust, London, UK.
2
From Developmental Neurosciences (H.S., F.J.K., M.K., P.B., J.D.C., S. Sahota, S. Sakaria, C.A.C., J.M.K.), UCL Great Ormond Street Institute of Child Health, London; University Hospital Southampton NHS Foundation Trust (F.J.K.); Clinical and Experimental Sciences (F.J.K.), University of Southampton; Department of Radiology (D.E.S.), Great Ormond Street Hospital NHS Foundation Trust, London; Department of Haematology and Evelina Children's Hospital (J.H., R.K.-A., B.I., M.P.), Guy's and St Thomas' NHS Foundation Trust, London; King's College Hospital NHS Foundation Trust (S.C., D.C.R., M.A.), London; North Middlesex University Hospital NHS Foundation Trust (O.W.), London; and Department of Haematology (M.L.), Imperial College Healthcare NHS Foundation Trust, London, UK. Fenella.Kirkham@ucl.ac.uk.

Abstract

OBJECTIVE:

The purpose of this retrospective cross-sectional study was to investigate whether changes in white matter integrity are related to slower processing speed in sickle cell anemia.

METHODS:

Thirty-seven patients with silent cerebral infarction, 46 patients with normal MRI, and 32 sibling controls (age range 8-37 years) underwent cognitive assessment using the Wechsler scales and 3-tesla MRI. Tract-based spatial statistics analyses of diffusion tensor imaging (DTI) and neurite orientation dispersion and density imaging (NODDI) parameters were performed.

RESULTS:

Processing speed index (PSI) was lower in patients than controls by 9.34 points (95% confidence interval: 4.635-14.855, p = 0.0003). Full Scale IQ was lower by 4.14 scaled points (95% confidence interval: -1.066 to 9.551, p = 0.1), but this difference was abolished when PSI was included as a covariate (p = 0.18). There were no differences in cognition between patients with and without silent cerebral infarction, and both groups had lower PSI than controls (both p < 0.001). In patients, arterial oxygen content, socioeconomic status, age, and male sex were identified as predictors of PSI, and correlations were found between PSI and DTI scalars (fractional anisotropy r = 0.614, p < 0.00001; r = -0.457, p < 0.00001; mean diffusivity r = -0.341, p = 0.0016; radial diffusivity r = -0.457, p < 0.00001) and NODDI parameters (intracellular volume fraction r = 0.364, p = 0.0007) in widespread regions.

CONCLUSION:

Our results extend previous reports of impairment that is independent of presence of infarction and may worsen with age. We identify processing speed as a vulnerable domain, with deficits potentially mediating difficulties across other domains, and provide evidence that reduced processing speed is related to the integrity of normal-appearing white matter using microstructure parameters from DTI and NODDI.

PMID:
29752305
PMCID:
PMC5993179
DOI:
10.1212/WNL.0000000000005644
[Indexed for MEDLINE]
Free PMC Article

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