Patient With Severe Moyamoya Disease Who Presents With Acute Cortical Blindness
- cerebral hemorrhage
- encephaloduroarteri osynangiosis
- moyamoya disease
- pial synangiosis
A previously healthy 16-year-old girl presented to a local emergency room with intense headache, severe nausea, and vision loss. The week before, she had intermittent headaches and nonspecific visual changes that resolved with acetaminophen or NSAIDs and rest. On admission to the hospital, she was found to be extremely hypertensive and had bilateral occipital hypodensities consistent with infarcts on noncontrast computed tomography.
On transfer to the regional pediatric hospital, she had severe vision loss (light perception only), and magnetic resonance imaging showed bilateral infarctions, especially affecting the posterior circulation and occipital lobes (Figure 1). Magnetic resonance perfusion revealed severe bilateral hemispheric hypoperfusion, and magnetic resonance imaging fluid-attenuated inversion-recovery sequence demonstrated linear hyperdensities along sulci—the ivy sign often seen in moyamoya. Cerebral angiography revealed complete occlusion of the bilateral internal carotid arteries distal to the anterior choroidal arteries with dense moyamoya collaterals and extreme attenuation of the posterior cerebral artery circulation, also with moyamoya collaterals (Figure 2). Biochemical, hemostatic, and genetic evaluations were normal, with the exception of a significantly elevated d-dimer level (1850 ng/mL; normal range: 0–543 ng/µL). Apart from her mother experiencing a deep venous thrombosis during pregnancy, the patient’s family history was unremarkable for coagulopathies.