The spectrum of cerebrovascular abnormalities (CVA), including moyamoya, was evaluated in a retrospective chart review of 353 patients with neurofibromatosis type 1 (NF1) seen at the Children’s National Medical Center, Washington, DC, from 1995 to 2003. Of 316 with brain MRI, eight (2.5%) had a CVA. MR angiography at age 1 to 13 years (mean 7.3 years) identified the following: 1) narrowed left posterior cerebral artery, 2) ectasia of carotid and cerebral arteries, 3) moyamoya with infarcts, 4) aneurysm, 5) carotid arterial stenosis with moyamoya and thalamic infarction, 6) internal carotid stenosis, 7) occlusion of middle cerebral artery, and 8) hypoplastic internal carotid artery. Vasculopathy was diagnosed in 7 patients, one presenting with acute hemiparesis and seizures at age 2 years. Three children required surgery. At 5.8 years follow-up (range 10 months to 9 years), only one (with moyamoya and strokes) had residual neurologic deficits. [1]

COMMENT. Early recognition of a cerebral vascular abnormality by magnetic resonance angiography may help to prevent complications. The authors cite 43 cases of CVA in the NF1 literature, 25 (58%) in children. The supraclinoid ICA is involved most frequently. Ischemia with hemiparesis, often complicated by convulsions, is the most common presenting symptom. In 7 of the authors’ 8 cases, the CVA was an incidental finding.