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Isolated Angiopathy of the Central Nervous System

Matthew C. Jackson, MRCP; Graham Lennox, MRCP
Arch Intern Med. 1992;152(10):2144-2145. doi:10.1001/archinte.1992.00400220144032.
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To the Editor.—  We read with interest the article by Crane and colleagues1 presenting 11 patients with isolated angiitis of the central nervous system (IACNS), and we agree with their principle conclusion that a relatively benign syndrome characterized by headache and diffuse segmental cerebral arterial narrowing (with or without focal neurologic deficit) is not rare. Such cases have been previously described by various authors2-4 as isolated benign vasculitis or arteritis of the CNS. We feel that these terms are inappropriate, because angiitis is a histopathologic diagnosis, and a similar clinical and angiographic syndrome may result from a variety of other causes such as subarachnoid hemorrhage, pheochromocytoma with intermittent severe hypertension, benign sexual and exertional headache, presumed migraine, exposure to sympathomimetic drugs such as cocaine, and the postpartum period.5 The more neutral term isolated angiopathy of the CNS, as suggested by Hankey,5 better describes cases with


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