Cerebral manifestations of sickle cell disease contribute significantly to the overall morbidity of the disease. Sickle cell disease is among the most common causes of stroke in the paediatric population.
For a general discussion of sickle cell disease, please refer to sickle cell disease.
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Epidemiology
Approximately 25% of patients with sickle cell disease have a neurological event in their lifetime, many of these in childhood 1.
Clinical presentation
Ischaemic strokes and cognitive impairment are common, but in many instances, infarcts may be asymptomatic 1-2.
Radiographic features
Cerebral radiological findings in sickle cell disease include 1-3:
- tortuosity of intra and extracranial arteries
- intracranial ICA stenosis and occlusion results in moyamoya
- moyamoya syndrome
- cerebral atrophy, most common 2
- cerebral ischaemia
- chronic small vessel ischaemia
- cortical infarcts
- cerebral fat emboli from bone marrow necrosis (starfield pattern) 4
- predilection for aneurysms and subsequent intraparenchymal or subarachnoid haemorrhage 1
- PRES 5
- spontaneous epidural haematoma (rare) 6