Abstract

SILVA SOLER, Miguel Arturo; QUINTERO, Ana María  and  GONZALEZ, Aura Virginia. Primary vasculitis of the central nervous system, a diagnostic challenge. Case report. Acta Neurol Colomb. [online]. 2018, vol.34, n.3, pp.189-194. ISSN 0120-8748.  https://doi.org/10.22379/24224022211.

Primary angiitis of the central nervous system is a rare condition in which there is destruction and inflammatory involvement of the vessels of the central nervous system. It is often underdiagnosed given its multiple forms of clinical presentation and neuroimaging findings that can mimic other pathologies delaying diagnosis and management. This type of vasculitis is very poorly understood and there are not enough studies at present regarding the pathophysiology and management. Inflammatory markers are usually normal with abnormal CSF in 80-90 %% of cases and cerebral arteriography may show a typical pattern described as in "Rosario" however with very low sensitivity. Given the persistence of high clinical and absence of non-invasive diagnostic tests suspect the final diagnosis is made by biopsy of brain lesions and meninges. Finally, treatment is based on a combination of corticosteroids and cyclophosphamide however has not controlled studies support it.

Keywords : primary angiitis; vasculitis; central nervous system; cyclophosphamide; dementia; brain neoplasms (MeSH).

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