• Chun-Hsin Teng, I-Hsiao Yang, Meng-Ni Wu, and Ping-Song Chou.
• Department of Neurology, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung, Taiwan.
• Medicine (Baltimore). 2021 Aug 6; 100 (31): e26837e26837.

IntroductionMoyamoya disease (MMD) and posterior reversible encephalopathy syndrome (PRES) share similar pathophysiological characteristics of endothelial dysfunction and impaired cerebral autoregulation. However, there have never been any published studies to demonstrate the relationship between these 2 rare diseases.Patient ConcernsA 26-year-old Asian man presented with a throbbing headache, blurred vision, and extremely high blood pressure. We initially suspected acute cerebral infarction based on the cerebral computed tomography, underlying MMD, and prior ischemic stroke. However, the neurological symptoms deteriorated progressively.DiagnosisCerebral magnetic resonance imaging indicated the presence of vasogenic edema rather than cerebral infarction.Interventions And OutcomesAn appropriate blood pressure management prevents the patient from disastrous outcomes successfully. Cerebral magnetic resonance imaging at 2 months post treatment disclosed the complete resolution of cerebral edema. The patient's recovery from clinical symptoms and the neuroimaging changes supported the PRES diagnosis.ConclusionThis report suggests that patients with MMD may be susceptible to PRES. It highlights the importance of considering PRES as a differential diagnosis while providing care to MMD patients with concurrent acute neurological symptoms and a prompt intervention contributes to a favorable clinical prognosis.Copyright © 2021 the Author(s). Published by Wolters Kluwer Health, Inc.

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