Abstract

GRANA, Diego et al. Central Nervous System Primary Vasculitis: a diagnostic challenge. Arch. Med Int [online]. 2015, vol.37, n. 2, pp.74-79. ISSN 0250-3816.

The primary central nervous system vasculitis is an uncommon variety of vasculitis that exclusively compromises the encephalon and the bone marrow. It arises as heterogeneous and unsystematized and it could compromise more than one encephalic structure at the same time. The diagnosis is made based on compatible symptoms supported by an angiography showing evidence of a vasculitis pattern or/and an encephalic parenchyma or meninges biopsy. The alterations documented in the Nuclear Magnetic Resonance are frequent but unspecific and it is possible to find no evidence of the injury if only a small vessel is affected. We present three clinical cases of probably PCNSV in which the diagnosis was made based on: the clinical presentation, imaginologicfindingsand therapeutic response. In the cases studied the alterations of the NMR were confirmed, the results suggested that several encephalic structures were compromised with CSF (cerebrospinal fluid) inflammatory and alterations on the EEG. The induction therapy included Methilprednisolone boluses; and the maintenance therapy, locally applied steroids. The response to the treatment was excellent clinically as well as imaginologically.

Keywords : primary central nervous system vasculitis.