Sickle cell disease (cerebral manifestations)
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.
Approximately 25% of patients with sickle cell disease have a neurological event in their lifetime, many of these in childhood 1.
Ischaemic strokes and cognitive impairment are common, but in many instances, infarcts may be asymptomatic 1-2.
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
- predilection for aneurysms and subsequent intraparenchymal or subarachnoid haemorrhage 1
- 1. Lonergan GJ, Cline DB, Abbondanzo SL. Sickle cell anemia. Radiographics. 21 (4): 971-94. Radiographics (full text) - Pubmed citation
- 2. Kotb MM, Tantawi WH, Elsayed AA et-al. Brain MRI and CT findings in sickle cell disease patients from Western Saudi Arabia. Neurosciences (Riyadh). 2012;11 (1): 28-36. Pubmed citation
- 3. Saito N, Nadgir RN, Flower EN et-al. Clinical and radiologic manifestations of sickle cell disease in the head and neck. Radiographics. 2010;30 (4): 1021-34. doi:10.1148/rg.304095171 - Pubmed citation
- 4. Dhakal LP, Bourgeois K, Barrett KM, Freeman WD. The "starfield" pattern of cerebral fat embolism from bone marrow necrosis in sickle cell crisis. (2015) The Neurohospitalist. 5 (2): 74-6. doi:10.1177/1941874414554300 - Pubmed