Moyamoya Disease in an Infant With Autonomic Dysfunction: Angiographic and MRI Findings

An infant with persistent Harlequinism went on to develop a hemiparesis secondary to Moyamoya disease at 21h months of age. The sympathetic nervous system is proposed to be an etiologic factor in the pathophysiology of Moyamoya disease as well as Harlequinism. This is one of the youngest patients reported in the English literature with Moyamoya disease and the only report of the coexistence of Moyamoya disease and atypical Harlequinism. Magnetic resonance imaging led to the diagnosis, which was confirmed by cerebral angiography. (J Child Neurol 1988;3:110-113).