Atypical progressive stroke syndrome associated with oral contraceptives and cigarette use

We report a case of progressive multifocal intracranial distal arterial branch occlusive disease in a young adult who smoked heavily and used oral contraceptives. The distribution of the angiographic lesions and the associated telangiectasias and rete mirabile resemble Moya-Moya disease except for clearly absent proximal carotid arterial disease. This type of distal multifocal cerebral arterial occlusive pattern appears to be a very rare manifestation of cigarette and oral contraceptive use. The patient has remained without symptoms for >3 years off oral contraceptives and cigarettes and on antiplatelet agents.