Cognition and the Default Mode Network in Children with Sickle Cell Disease: A Resting State Functional MRI Study.
Cerebrovascular complications are frequent events in children with sickle cell disease, yet routinely used techniques such as Transcranial Doppler (TCD), Magnetic Resonance (MRI) and Angiography (MRA), insufficiently explain the cause of poor cognitive performances. Forty children with SS-Sβ° (mean age 8 years) underwent neurocognitive evaluation and comprehensive brain imaging assessment with TCD, MRI, MRA, Resting State (RS) Functional MRI with evaluation of the Default Mode Network (DMN). Sixteen healthy age-matched controls underwent MRI, MRA and RS functional MRI.Children with SCD display increased brain connectivity in the DMN even in the absence of alterations in standard imaging techniques. Patients with low neurocognitive scores presented higher brain connectivity compared to children without cognitive impairment or controls, suggesting an initial compensatory mechanism to maintain performances. In our cohort steady state haemoglobin level was not related to increased brain connectivity, but SatO2<97% was. Our findings provide novel evidence that SCD is characterized by a selective disruption of connectivity among relevant regions of the brain, potentially leading to reduced cognition and altered functional brain dynamics. RS functional MRI could be used as a useful tool to evaluate cognition and cerebral damage in SCD in longitudinal trials.
Clinic of Pediatric Hematology-Oncology, Department of Child and Maternal Health, Azienda Ospedaliera-University of Padova, Padova, Italy.
Raffaella
R
Colombatti
Department of Neurosciences, Azienda Ospedaliera-University of Padova, Padova, Italy.
Marta
M
Lucchetta
Clinic of Pediatric Hematology-Oncology, Department of Child and Maternal Health, Azienda Ospedaliera-University of Padova, Padova, Italy.
Maria
M
Montanaro
Department of Neurosciences, Azienda Ospedaliera-University of Padova, Padova, Italy.
Patrizia
P
Rampazzo
Department of Neurosciences, Azienda Ospedaliera-University of Padova, Padova, Italy.
Mario
M
Ermani
Department of Neurosciences, Azienda Ospedaliera-University of Padova, Padova, Italy.
Giacomo
G
Talenti
Department of Neurosciences, Azienda Ospedaliera-University of Padova, Padova, Italy.
Claudio
C
Baracchini
Department of Neurosciences, Azienda Ospedaliera-University of Padova, Padova, Italy.
Angela
A
Favero
Clinic of Pediatric Hematology-Oncology, Department of Child and Maternal Health, Azienda Ospedaliera-University of Padova, Padova, Italy.
Giuseppe
G
Basso
Department of Neurosciences, Neuroradiology Unit, University of Salerno, Salerno, Italy.
Renzo
R
Manara
Clinic of Pediatric Hematology-Oncology, Department of Child and Maternal Health, Azienda Ospedaliera-University of Padova, Padova, Italy.
Laura
L
Sainati
D000293:Adolescent; D000755:Anemia, Sickle Cell / Q000150:complications / Q000473:pathology* / Q000523:psychology; D001931:Brain Mapping / Q000379:methods*; D002648:Child; D002675:Child, Preschool; D003072:Cognition Disorders / Q000209:etiology / Q000473:pathology* / Q000523:psychology; D003430:Cross-Sectional Studies; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods*; D008297:Male; D009415:Nerve Net / Q000473:pathology*; D009483:Neuropsychological Tests