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Context: Primary central nervous system angeitis (APSNC) is a vasculitis confined to the central nervous system (CNS). Its incidence rate is 2.4 cases per 1,000,000 person-years. It affects predominantly small and medium sized arteries of the cerebral parenchyma, spinal cord and leptomeninges. The most common manifestations are headache, cognitive impairment, stroke and transient ischemic attack. The fact that it results in multifocal inflammation of the arteries and veins can lead to ischemic or hemorrhagic infarctions in multiple vascular territories, more common in the subcortical white matter. Case report: A woman with a past of epilepsy and headache present to us with acute intraparenchymal hemorrhagic stroke, after extensive investigation, arrived at the presumed diagnosis of APSNC. Conclusion:Tests such as MRI and arteriography have low sensitivity and specificity for APSNC and brain biopsy is still the gold standard method for diagnosis, but in practice it is performed in less than half of the cases in clinical practice, this is due to several factors. Next, it is important to note that the biopsy efficiency is around 53-74% in unselected areas, reaching 80% if the site is selected affected by non-invasive exams. The literature specifies brain biopsy as a procedure with relatively low morbidity and mortality, however, it was not performed in more than a half of cases. |
