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Primary vasculitides of the central nervous system are rare vascular disorders whose pathophysiology involves inflammatory infiltration exclusively in the vessels of the central nervous system. Its spectrum extends from primary angiitis of the central nervous system to cerebral amyloid angiopathy. This case portrays a 46-year-old woman with a history of multiple strokes. History of multiple comorbidities such as systemic arterial hypertension, prediabetes, and smoking. During follow-up after the second ischemic event, a patent foramen ovale was detected with positive microbubbles stand. Therefore, she was using oral anticoagulants. Transthoracic echocardiogram and magnetic resonance angiography without relevant changes. However, she resorted to the neurological deficit three more times. Cerebrospinal fluid (CSF) analysis was done twice; both were normal. Cerebral angiography was performed at the time of the last stroke and revealed suggestive signs of vasculitis. Discrimination of angiitis and reversible cerebral vasoconstriction syndrome was challenging and based on the demographic and clinical features of the patient. The characteristics of this case that corroborate the hypothesis of angiitis are the patient's age, which is in the median age range (40-60 years), chronic headache, and focal symptoms not temporally associated with the headache. Intravenous methylprednisolone pulse therapy (one gram/day) was performed for five days. Evolved with progressive improvement of the motor deficit. On hospital discharge, it was prescribed prednisone, and after ten days, Azathioprine 2.5mg/Kg/day was started. Diagnosis of primary central nervous system vasculitis requires high clinical suspicion due to the low specificity of the clinical features. This report reinforces the importance of investigating CNS vasculitis in young patients with a history of recurrent stroke.
Raposo et al. (Thu,) studied this question.