Sickle cell disease (cerebral manifestations)
Sickle cell
disease cerebral vasculopathy. Sickle cell vasculopathy with occlusion of internal carotid arteries and moyamoya pattern supported by posterior circulation from vertebrobasilar system. Notice patent bilateral superficial temporal artery synangiosis.
Sickle cell
disease (cerebral manifestations) • Moyamoya syndrome with background sickle cell disease - Ganzer Fall bei Radiopaedia
Sickle cell
disease (cerebral manifestations) • Old multi-territory infarcts in the setting of sickle cell disease - Ganzer Fall bei Radiopaedia
Sickle cell
disease cerebral vasculopathy. Sickle cell vasculopathy with occlusion of internal carotid arteries and moyamoya pattern supported by posterior circulation from vertebrobasilar system. Notice patent bilateral superficial temporal artery synangiosis.
Preschooler
with sickle cell disease. AP MRA of the neck with contrast (above) shows near total occlusion of the left internal carotid artery. AP MRA of the brain with contrast (below) shows the left internal carotid artery to be small in caliber and to have a beaded appearance. The left middle carotid artery is reconstituted throug the anterior communicating artery.The diagnosis was sickle cell disease causing near total occlusion of the left internal carotid artery.
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 pediatric population.
For a general discussion of sickle cell disease, please refer to sickle cell disease.
Epidemiology
Approximately 25% of patients with sickle cell disease have a neurological event in their lifetime, many of these in childhood .
Clinical presentation
Ischemic strokes and cognitive impairment are common, but in many instances, infarcts may be asymptomatic .
Radiographic features
Cerebral radiological findings in sickle cell disease include :
- tortuosity of intra and extracranial arteries
- intracranial ICA stenosis and occlusion results in moyamoya
- moyamoya syndrome
- cerebral atrophy, most common
- cerebral ischemia
- chronic small vessel ischemia
- cortical infarcts
- cerebral fat emboli from bone marrow necrosis (starfield pattern)
- predilection for aneurysms and subsequent intraparenchymal or subarachnoid hemorrhage
- PRES
See also
Siehe auch:
Assoziationen und Differentialdiagnosen zu Sichelzellenanämie (zerebrale Manifestationen):
muskuloskelettale
Manifestationen bei Sichelzellanämie
