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Reduced hypercapnic vasoreactivity in moyamoya disease

Departments of Neurology, Psychiatry, and Neurosurgery, College of Physicians and Surgeons, Columbia University, New York, NY.

We measured cerebral perfusion at rest and in response to CO₂ in eight patients with moyamoya disease (MMD), using the ¹³³xenon inhalation method to determine the effect of large-vessel occlusive disease on vasoreactivity. We studied three other groups for comparison, including four with bilateral internal carotid artery occlusions (BICAO), 11 with unilateral carotid occlusion (UICAO), and six with unilateral middle cerebral artery stem occlusion (UMCAO). Resting flows appeared to correlate with the severity of occlusive disease overall. Normocapnic perfusion was lowest in the group with BICAO and decreased in proportion to the degree of contralateral stenosis in the group with UICAO. Hypercapnic perfusion correlated with the apparent adequacy of angiographic collaterals. Reactivity was lowest in the MMD group (0.79%/mm Hg) whose collateral supply was limited to leptomeningeal anastomosis from the posterior cerebral artery, but highest in the patients with BICAO (2.72%/mm Hg), each of whom showed excellent posterior communicating artery flow. The clinical course of the MMD group was compatible with the syndrome of perfusion insufficiency with repeated ischemic attacks or a saltatory progression of an ischemic deficit; CT showed infarction in the borderzone territory. These results suggest that a severely reduced hypercapnic response may help to identify patients with ischemic syndromes due to perfusion failure in the borderzones, as in MMD.

Address correspondence and reprint requests to Dr. Tatemichi, Stroke Service, Neurological Institute, 710 West 168th Street, New York, NY 10032.

Supported in part by NIH grant AG 05433, a grant from the Jean and Louis Dreyfus Foundation, and from the Horace W. Goldsmith Foundation.

Presented in part at the thirty-ninth annual meeting of the American Academy of Neurology, New York, NY, April 1987.

Received January 18, 1988. Accepted for publication in final form April 11, 1988.

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