Abstract
BackgroundCerebrovascular diseases are well-known complications of systemic lupus erythematosus (SLE). Among them, cerebral arterial dissection is a rare vascular complication, in which an intimal tear of the blood vessel leads to an intramural hematoma. Cerebral arterial dissection leads to arterial stenosis, thrombosis, and aneurysm, resulting in cerebral infarction or subarachnoid hemorrhage (SAH). Herein, we report a case of posterior cerebral artery (PCA) dissection in SLE that presented as unilateral thalamic infarction followed by SAH and intraventricular hemorrhage (IVH). Case PresentationA 16-year-old boy hospitalized with prolonged fever, hair loss, and skin eruption was newly diagnosed with SLE based on the 2019 EULAR/ACR SLE classification criteria. He suddenly complained of headache, diplopia, and impairment of lateral gaze during hospitalization. Brain magnetic resonance imaging revealed left thalamic infarction, although cerebral vessel inflammation or thrombosis was not observed. Antiphospholipid antibodies such as lupus anticoagulant, anti-cardiolipin antibody, and anti-β2-glycoprotein antibody were not detected. His symptoms improved with high-dose steroid, low-dose aspirin, and mannitol therapy. Five days later, he experienced severe headache and generalized tonic-clonic seizures. Brain computed tomography revealed SAH and IVH with hydrocephalus. Even though emergent external ventricular drainage was performed, the ventricle size did not decrease. Transfemoral cerebral angiography revealed a ruptured dissecting PCA pseudoaneurysm, and immediate coil embolization was successfully performed. The patient fully recovered without any neurologic sequelae, although he underwent ventriculoperitoneal shunting for hydrocephalus following SAH. ConclusionsTo the best of our knowledge, this is the first reported case of PCA dissection in a patient with childhood-onset SLE. Moreover, the subsequent progression from cerebral infarction to SAH caused by PCA dissection makes this case unique. In SLE patients presenting with cerebral infarction and hemorrhage, cerebral arterial dissection and pseudoaneurysm should be considered to achieve favorable outcomes.
Distal Middle Cerebral Artery Fusiform Aneurysm Associated with Systemic Lupus Erythematosus
