Last modified: 2018-09-09
Abstract
As part of a larger study to determine the burden and spectrum of neurocognitive impairments in Ugandan SCA children, we selected 81 children enriched for possible brain pathology from Mulago Hospital SCA clinic out of a random sample of 262 stable children for non-contrasted brain MRI/MRA. None was receiving hydroxyurea. All had detailed clinical history, physical and neuro-cognitive exam and transcranial Doppler cerebral blood flow velocity determination
Results
Of the 81 participants imaged, 61 had one or more of a history of stroke, focal neurological deficit, cognitive impairment or abnormal TCD. The brain MRI/MRA had abnormalities in 35/61(63.9%) participants with possible brain pathology and in 4/20(20.0%) without. Different structural abnormalities were seen and in all brain regions ranging from T2 weighted hyper-intensities, white matter lacuna infarctions to bilateral multifocal ischemic cerebral infarcts and associated compensatory hydrocephalus. Vessel abnormalities ranged from cerebral microangiopathy, multiple stenoses to occlusions of major arteries. Severe vessel obstructions were seen in three children <36months.
Conclusions
Brain injury in Uganda children with SCA begins early in childhood and progressively increases in frequency and severity with age. Neonatal screening programs should be enhanced and interventions to prevent sickle cell brain vasculopathy initiated early after diagnosis. The risk factors for such early brain injury should be investigated.