Primary central nervous system vasculitis: a review of this challenging diagnosis

The primary vasculitis of the central nervous system is still a controversial subject and in constant study for a better understanding of all its aspects. It is a pathophysiology disease that is not yet fully understood, and with probable immunological and biological mechanisms of very high complexity, which go beyond the current technical and scientific limits. Its clinical manifestations are challenging even for the most experienced neurologists, which requires, in addition to academic knowledge, a unique ability to interpret clinically and to weigh the results presented in complementary tests. This is because we do not have any non-invasive diagnostic tool that confirms, or discards, with full security, the possibility of VPSNC. In practice, the confirmatory examination is considered the gold standard and is still the brain biopsy, which is usually performed only in exceptional cases. Correspondence to: Oliveira, FTM. Department of Neurology, Santa Casa at São PauloSP, Av. Dr Cesario Mota Jr, 112 – V. Buarque. São Paulo-SP, Brazil, CEP 01221-010. E-mail: towmaz@gmail.com Received: September 27, 2017; Accepted: October 23, 2017; Published: October 24, 2017 Introduction Primary Central Nervous System Vasculitis (VPSNC) is a rare and poorly defined clinical condition, which began to be better understood in the late 1950’s, when pathological studies performed by Cravioto and Feigin [1] described some associated neurological events to an inflammatory process restricted to the brain and spinal cord of granulomatous histological characteristics. It was initially referred to as idiopathic or non-inflammatory granulomatous arteritis of the CNS, or giant cell arteritis of the CNS. At that time, such condition was related to usually unfavorable developments, usually with outcomes with moderate to severe disability or death, despite the available treatments, which due to the lack of studies based on clinical trials, used to be nonstandardized and had no scientific basis to support the rationalization of its uses. Over time, the understanding of VPSNC-related conditions has expanded significantly, as well as the clarification of other conditions with clinical presentation and similar pathophysiological features, but which can now be understood as distinct diseases, such as, for example, reversible cerebral vasoconstriction syndrome and reversible posterior encephalopathy syndrome. In addition, the development of diagnostic techniques, both for laboratory analysis and increasingly accurate and accurate imaging methods, have helped to build the pathophysiological details of the NSDS. The treatment protocols for this condition are not yet standardized and are generally elaborated based on diseases of similar pathophysiological understanding, such as systemic vasculitis and immune-mediated diseases. However, we already have well-developed cohort studies, designed by specialized centers, with an emphasis on diagnosis and treatment, which have been modifying the evolutionary course of the disease, transforming the vital and functional prognosis of the patients affected by this disease. This work aims to carry out a thematic review of the current literature, based on recent work and carried out by specialized study centers. Method The research conducted in this study was carried out essentially with the help of the PubMed portal (https://www.ncbi.nlm.nih.gov/ pubmed/), managed by the US National Institute of Health (NIH). The terms “Primary angiitis of Central Nervous System”, “Vasculitis in Central Nervous System”, were inserted for research. Articles with a publication dating from 2005 to 2017, including also articles of historical relevance, and published in indexed journals and considered as publications of high academic impact, were selected for consultation. It was decided to give greater emphasis to articles related to primary vasculitis, although discussions related to secondary vasculitis cannot be completely ignored. The study of the involvement in adults was prioritized, since the NSVC has particular characteristics in the pediatric population, besides being usually excluded from the sampling of the largest and most important studies. Primary vasculitis of the central nervous system