Takayasu arteritis: a treatable cause of stroke in infancy.
Takayasu disease is an arteritis of unknown etiology involving the aorta and its major branches. Untreated, 75% of patients die within two years. A 6-month-old black female presented with a right focal seizure, a flaccid right hemiparesis, decreased pulses in the right arm, a large left frontoparietal hypodense area on computed tomography, and an elevated erythrocyte sedimentation rate. Cerebral angiography demonstrated irregular dilatation of both carotid arteries and narrowing of the left middle cerebral artery. Aneurysm of the right sinus of Valsalva, dilatation of the aortic root, narrowing of the origins of both carotid arteries, and beading of the descending aorta were demonstrated by cardiac angiography. The patient was treated with prednisone (2 mg/kg/day) and azathioprine (1 mg/kg/day). There was gradual return of the erythrocyte sedimentation rate to normal over the ensuing 3-10 months, resolution of the hemiparesis, and acquisition of normal developmental milestones. Digital subtraction angiography revealed improvement in the appearance of the descending aorta and of the common carotid arteries with the disappearance of the arterial wall irregularities. Early diagnosis and vigorous immunosuppression may improve outcome in this rare and often fatal vascular disease in childhood.
Kohrman
M H
MH
Department of Pediatrics, University of Chicago, Pritzker School of Medicine, Illinois.
Huttenlocher
P R
PR
D001015:Aortic Arch Syndromes / Q000150:complications*; D001379:Azathioprine / Q000627:therapeutic use; D002533:Cerebral Angiography; D002544:Cerebral Infarction / Q000209:etiology*; D005260:Female; D006801:Humans; D007223:Infant; D011241:Prednisone / Q000627:therapeutic use; D013625:Takayasu Arteritis / Q000150:complications* / Q000188:drug therapy; D014057:Tomography, X-Ray Computed
D001379:Azathioprine; D011241:Prednisone