Neuroimaging of Vascular Lesions in Schimke Immuno-Osseous Dysplasia

Abstract

Rivista di Neuroradiologia 16: 914-916, 2003 www.centauro.it 914 paired consciousness associated with focal motor seizures on the right arm and hypertensive crisis. He had SIOD diagnosed since the age of five at another institution 2 and suffered from chronic renal insufficiency.Brain CT performed at day two, cytochemical and microbiological CSF analysis disclosed no abnormalities. The diagnosis of encephalopathy secondary to arterial hypertension and hydroelectrolytic equilibrium disorder was then considered. The clinical manifestations resolved in 24 to 48 hours after electrolyte correction and arterial pressure treatment.Brain MRI performed at day 20, revealed high signal areas in T2-weighted images, without restricted diffusion, corresponding to non-acute ischemic lesions, located in left lentiform nucleus, corpus callosum genu and frontal white matter (figure 1). Additionally, atrophic-hipoplastic cerebellar vermis was also observed.Magnetic resonance angiography obtained at the same time disclosed occlusion or near occlusion of left internal carotid artery (figure 2). Acetylsalicylic acid and dipyridamole were added to maintenance therapy.Follow-up MRA study nine