Case Report Case of Moyamoya Disease in a Patient With Advanced Acquired Immunodeficiency Syndrome Sophia R. Sharfstein, MD,* Shadab Ahmed, MD,† Mohammed Q. Islam, MD,§ Mamoun I. Najjar, MD,† and Vladimir Ratushny, BS,¶ Background: Moyamoya disease is an occlusion of the terminal portion of internal carotid arteries and proximal portion of middle and anterior cerebral arteries of unknown origin. Moyamoya syndrome is associated with meningitis, tuberculosis, syphilis, head trauma, head irradiation, brain tumor, von Recklinghausen’s disease, tuberous sclerosis, Marfan syndrome, sickle cell anemia, arteriosclerosis, hyperten- sion, and oral contraceptive use. To our knowledge, acquired immunodeficiency syndrome (AIDS) as a cause of moyamoya syndrome has not been reported in an adult population. Objective: We report a case of moyamoya syndrome in a patient with AIDS and without other conditions associated with occlusion of the circle of Willis and formation of collateral network at the base of the brain and basal ganglia. Methods: We present a case report. Results: A 29-year-old woman with an 8-year history of AIDS on multiple antiretroviral medications presented with recurrent tingling of the left extremities which 1 month later progressed to mild hemiparesis and dysarthria. During the next few months the patient developed progressive cognitive decline and on-and-off fluctuations in the degree of hemiparesis. Brain magnetic resonance imag- ing showed multiple small subcortical infarct’s in both parietal lobes. Magnetic reso- nance angiography showed occlusion of middle cerebral arteries distal internal carotid arteries, with prominent collateral network. Cerebral angiography confirmed moya- moya pattern. Lumbar puncture showed: white blood cell count 1, red blood cell count 418, protein 56, glucose 53, negative bacterial and acid-fast bacilli smear and culture, negative VDRL test, India ink, cryptococcal antigen, cytology and negative polymerase chain reaction for cytomegalovirus, Epstein-Barr virus, varicella-zoster virus, and herpes simplex virus type 1 and 2. Electroencephalography showed diffuse back- ground slowing. Conclusions: We hypothesize that human immunodeficiency virus (HIV) caused central nervous system vasculitis, which eventually led to formation of moyamoya pattern. No other definite causes of central nervous system vasculitis were found in our patient. Cerebrovascular disorders should be considered in patients with HIV/AIDS with focal neurologic deficit. Moyamoya syndrome as a cause of stroke should be considered in patients with HIV/AIDS, especially as survival improves. Key Words: Stroke—moyamoya disease—acquired immunodeficiency syndrome. © 2007 by National Stroke Association Moyamoya disease is an occlusion of the circle of Willis of unknown origin associated with the formation of col- lateral network at the base of the brain and basal gan- glia. 9-11 Occlusion of the terminal portion of internal carotid arteries (ICAs) and proximal portion of middle cerebral arteries (MCAs) and anterior cerebral arteries are From the Department of *Neurology, SUNY Downstate Medical Center, New York; †Department of Medicine; §Department of Neu- rology, Nassau University Medical Center, East Meadow; and ¶Third Year MD/PhD candidate, Drexel University College of Med- icine, Philadelphia, PA. Received April 16, 2007; revision received June 25, 2007; accepted July 3, 2007. Address correspondence to Sophia R. Sharfstein, MD, Department of Neurology, SUNY Downstate Medical Center, Box 1213, 450 Clarkson Ave, Brooklyn, NY 11203. E-mail: Sophia.Sharfstein@ Downstate.edu. 1052-3057/$—see front matter © 2007 by National Stroke Association doi:10.1016/j.jstrokecerebrovasdis.2007.07.001 Journal of Stroke and Cerebrovascular Diseases, Vol. 16, No. 6 (November-December), 2007: pp 268-272 268