Diagnostic challenge in a middle-aged woman with recurrent ischemic strokes: a case of primary central nervous system vasculitis

Abstract

Introduction: Primary vasculitides of the central nervous system (CNS) are rare vascular disorders whose pathophysiology involves inflammatory infiltration exclusively in the vessels of the CNS and clinically without constitutional symptoms. The aim is to report a case of primary vasculitis of the CNS. Case report and brief bibliographic review with Health Sciences Descriptors. Case report: This case portrays a 46-year-old woman with a history of multiple ischemic strokes. She previously had systemic arterial hypertension, prediabetes, obesity, and smoking. During follow-up after the second ischemic event, a small patent foramen ovale was detected with a positive microbubble test and the use of edoxaban was chosen. Transthoracic echocardiogram and magnetic resonance angiography of the cerebral arteries were without relevant changes. However, she recurred with new neurological deficits three more times. Edoxaban was replaced by dabigatran. Cerebrospinal fluid analysis was done twice and both were normal. Cerebral angiography was performed at the time of the last stroke and revealed suggestive signs of vasculitis. The characteristics of this case that corroborate the hypothesis of angiitis, to the detriment of reversible cerebral vasoconstriction syndrome, are the patient’s age, chronic headache, and focal symptoms not temporally associated with the headache. Secondary causes of vasculitis have been extensively investigated with negative results. Intravenous methylprednisolone pulse therapy was performed for five days. She evolved with progressive improvement of the motor deficit. On hospital discharge was prescribed prednisone and azathioprine. Conclusion: This report reinforces the importance of investigating CNS vasculitis in patients with recurrent ischemic strokes.