Description
A middle-aged African-American male with homozygous sickle cell disease presented with vaso-occlusive crisis and suffered a generalised tonic-clonic seizure while in the emergency department. He had been seizure-free for more than a decade, thus was not taking antiepileptic medications. CT head revealed diffuse cerebral oedema, effacement of the fourth ventricle and obstructive hydrocephalus (figure 1). An external ventricular drain was placed with improvement of hydrocephalus, and a repeat CT head revealed right parietal hypodensity (figure 1). MRI brain and conventional cerebral angiogram showed right parietal cerebral oedema, a large arteriovenous malformation, right internal carotid artery occlusion, moyamoya disease and basilar artery aneurysm (figure 2). The patient's cerebral oedema and neurological examination initially improved after exchange transfusion; however, he developed acute subarachnoid haemorrhage from basilar artery aneurysm rupture and brainstem strokes causing coma. He underwent palliative extubation after discussion with family and is now deceased.
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