INTRODUCTION

Central nervous system vasculitis is an increasingly recognized inflammatory brain disease characterized by an angiocentric inflammation of the vascular wall of cerebral blood and/or the perivascular space. Based on the primary vessel size affected it is divided into large-medium vessel angiits (angiography positive) and small-vessel CNS vasculitis (angiography negative). Isolated blood vessel inflammation of the brain is the hallmark of primary CNS vasculitis or angiitis (PACNS).

AIM

To report five children who met clinical and histopathological criteria of childhood small vessel vasculitis.

METHODS

We describe 5 patients, 3 women and 2 men. Symptoms at onset were seizures and headaches with a chronic and progressive course. Neuroimaging showed multifocal, unilateral hyperintense grey and white matter lesions on FLAIR and T2 sequences without a defined vascular territory. All of them had contrast enhacement. Only one patient had a VZV PCR positive in CSF. Because Conventional Angyography was negative in all cases, we decided to perform lesional brain biopsy which showed an angiocentric, transmural, perivascular infiltrate making the definite diagnosis of small vessel vascultis. We found EBV and HSV PCR positive in brain tissue in 3 patients, with nor evidence of acute/chronic infection neither a causal correlation between this finding and vessel wall inflammation.

CONCLUSIONS

In children with an acquired neurological deficit and CNS lesions we need to suspect Central Nervous system Vasculitis as a possible diagnosis, even with a normal angiography, considering the small vessel involvement. In this cases, cerebral biopsy is the gold standard to achive a definite diagnosis.