Challenging presentation of primary vasculitis of the central nervous system

Abstract

Objective: To show a patient with a diagnosis of primary vasculitis of the nervous system and review this entity's literature.

Clinical case: Male 32 years, with two events; first, with a transient monoparesis of the right upper extremity, improve with steroids in days. Now, with a motor Jacksonian progression from the upper to the lower right extremities and generalized seizures. After; aphasia, right hemiparesis, and delirium. In the antiresonance, the finding of multiple arterial cerebral "beading." With an in-depth study, the diagnosis of primary cerebral vasculitis was made. The management with levetiracetam, steroids, and Azathioprine offers a satisfactory evolution.

Discussion: The primary CNS vasculitis is an exclusion diagnosis, with angio-MRI is possible to suspect it, but it is always obligate to discard a secondary etiology by infection, systemic diseases, neoplasia, and drugs.

Conclusion: Although this problem is infrequent, we must consider this possibility. Opportune treatment can restore the quality of life.

Fig. 1

Axial FLAIR T2 (a) and DWI (b) show subacute-chronic, cortical and subcortical cerebral stroke in both frontal lobes that restrict diffusion.

Fig. 2

3D TOF MRA (a) and CT angiography (b) shows irregularities and occlusion of the middle cerebral arteries bilaterally, predominantly in their proximal M1 segments. The right internal carotid artery is smaller in diameter compared to its contralateral counterpart. Findings consistent with vasculitis.