Paracentral acute middle maculopathy represents deep capillary ischemia that can cause acute symptoms of scotoma. This case series describes two cases of symptomatic paracentral acute middle maculopathy that reflect the more severe spectrum of associated etiologies. Case 1: A 65-year-old male with recent history of hypertensive encephalopathy presented with sudden onset central visual disturbance described as amorphic in shape. Dilated fundus exam revealed severe hypertensive retinopathy with paracentral acute middle maculopathy. The patient died one year after the initial discovery of the retinal lesions. Case 2: A 87-year-old male presented with paracentral vision loss in one eye for three days. Clinical examination revealed multiple Hollenhorst plaques and paracentral acute middle maculopathy at presentation, and a subsequent branch retinal artery occlusion two days later. The patient was found to have 76% left carotid artery stenosis with numerous brain infarcts. The detection of paracentral acute middle maculopathy indicates an investigation through ocular and systemic risk factors to determine an attributable etiology. The search for possible etiologies should be tailored to the particular patient, based on age, associated ocular findings, systemic history, and symptomatic versus incidental presentation. The presence of symptomatic paracentral acute middle maculopathy, particularly in at-risk vasculopathic patients, should be addressed urgently as it may be an indicator of an underlying severe systemic disease process. CE Notification: This article is available as a COPE accredited CE course. You may take this course for 1-hour credit. Read the article and take the qualifying test to earn your credit. Click here to Enroll (https://www.crojournal.com/case-series-paracentral-acute-middle-maculopathy-as-a-sign-of-severe-systemic-vasculopathy)
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