Childhood Primary Angiitis of the Central Nervous System Presenting as Isolated Middle Cerebral Artery Dissection

Abstract

Isolated middle cerebral artery dissection and primary angiitis of the central nervous system are two rare but serious causes of ischemic stroke in children. We report a case of a patient who presented with headache, left-sided hemiparesis, and left hemi-hypoesthesia with acute cerebral infarction in the right middle cerebral artery territory. Brain magnetic resonance imaging showed isolated right middle cerebral artery dissection with arterial wall contrast enhancement extending beyond the length of the dissection and diffuse narrowing with stenosis in the right posterior cerebral artery suggesting angiitis. After exclusion of other diagnoses the patient was treated with corticosteroids and dual antiplatelet therapy. Follow up magnetic resonance imaging performed 4 months later showed partially improved contrast enhancement in the right middle cerebral artery and persistent narrowing and focal stenosis of the right posterior cerebral artery. Although rare, primary angiitis of the central nervous system should be considered in the differential diagnosis of acute stroke in children. High-resolution vessel wall imaging magnetic resonance studies can provide important information in determining the etiology of stroke.