HgbSS (on hypertransfusion protocol at La Rabida) and poorly controlled epilepsy (refractory to multiple AEDs) secondary to left MCA stroke in 2007 (with residual right sided weakness), who presents with worsening generalized weakness and gait changes over the past 4 weeks. The study is limited by patient motion.MRI: There is unchanged extensive encephalomalacia in the left frontal, left parietal and to lesser extent left temporal lobes, with Wallerian degeneration of the left brainstem. However, there is no evidence of acute infarct. The ventricles and basal cisterns are unchanged in size and configuration, with ex vacuo dilatation of the left lateral ventricle. The orbits are grossly unremarkable. There is diffuse low T1 and T2 signal in the bone marrow and findings related to Dyke Davidoff Masson on the left side. EEG leads are in place on the scalp. MRA: There is unchanged severe steno-occlusive disease of the left supraclinoid internal carotid artery. The proximal left anterior and middle cerebral arteries appear to be diffusely narrowed as well. There is a paucity of cortical vessels overlying the areas of left cerebral hemisphere encephalomalacia. There also appears to be narrowing of the supraclinoid right internal carotid artery, which is more pronounced than previously, and minimal collateral vessel formation in the region. The vertebral, basilar, and posterior cerebral arteries are intact. There is no evidence of cerebral aneurysms.
1. Chronic extensive left middle cerebral artery territory infarction related to sickle cell disease, without evidence of acute infarction.2. Severe steno-occlusive disease of the left supraclinoid internal carotid artery, proximal left anterior and middle cerebral arteries stenoses are also unchanged, with associated moyamoya. Stenosis involving the right supraclinoid internal carotid artery is more pronounced, although this might be artifactual. 3. The appearance of the skull is likely related to sickle cell disease.