Primary central nervous system vasculitis: A diagnostic and therapeutic challenge. A series of 7 patients

Abstract

IntroductionPrimary central nervous system vasculitis (PCNSV) is a rare disease affecting medium- and small-calibre blood vessels of the central nervous system. ObjectiveThe aim of this study was to analyse clinical findings and diagnostic aspects, with special attention to histopathological findings, as well as the treatments used and treatment response in patients diagnosed with PCNSV at our hospital. Patients and methodsWe conducted a retrospective descriptive analysis of patients with a diagnosis of PCNSV at discharge from our centre and meeting the 1988 Calabrese criteria. To this end, we analysed the hospital discharge records of Hospital General Universitario de Castellón between January 2000 and May 2020. ResultsWe analysed a series of 7 patients who were admitted with transient focal alterations and other less specific symptoms such as headache or dizziness; diagnosis was based on histological findings in 5 cases and on suggestive arteriographic findings in the remaining 2. Neuroimaging results were pathological in all cases, and CSF analysis detected alterations in 3 of the 5 patients who underwent lumbar puncture. All patients received initial treatment with megadoses of corticosteroids followed by immunosuppressive treatment. Progression was unfavourable in 6 cases, with fatal outcomes in 4. ConclusionsDespite the diagnostic challenge of PCNSV, it is essential to attempt to reach a definitive diagnosis using such tools as histopathology and/or arteriography studies, in order to promptly establish appropriate treatment and thus reduce the morbidity and mortality of this condition.