Ito hypomelanosis and moyamoya disease

Abstract

A transient ischemic attack with nearly complete progressive recovery occurred at age 20 months in a girl with Ito's hypomelanosis. Outcome included mental deficiency and behavioral difficulties requiring special education, but without recurrence of ischemic attack. The angiographic investigation performed at 9 years of age disclosed bilateral stenosis of the internal carotid artery characteristic of moyamoya disease. This association has not been reported previously.