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July 1981

Moya-Moya Disease Associated With Renal Artery Stenosis

Author Affiliations

From the Department of Neurology (Dr Ellison), The Medical College of Wisconsin, Milwaukee; and the Department of Pediatrics (Dr Largent), and the Division of Neurosurgery (Dr Popp), Albany (NY) Medical College.

Arch Neurol. 1981;38(7):467. doi:10.1001/archneur.1981.00510070101025

This is a description of a child with arterial hypertension, who was found to have bilateral renal artery stenosis and moya-moya disease.

REPORT OF A CASE  At age 14 years, the patient was referred to us following two years of severe hypertension that was resistant to medication. Four years previously, transient hypertension had been recorded during hospitalization for presumed viral meningitis associated with transient right-sided weakness. Family history was negative for hypertension.Except for elevation of blood pressure (160/120 mm Hg), the results of physical examination were normal and showed the patient to be thin, with no abdominal bruit or retinopathy. Extensive studies demonstrated no kidney damage. The results of rapid-sequence intravenous pyelography and renal flow studies were normal. Transfemoral angiography (Fig 1), however, showed marked bilateral renal artery stenosis, with an elevated right renal vein renin level of 41 mg/mL/hr compared with 24 mg/mL/hr in the inferior vena

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