Primary central nervous system vasculitis in a child mimicking parasitosis

Abstract

Sirs: Inflammatory diseases of the central nervous system (CNS) in childhood remain a diagnostic and therapeutic challenge. Of these, CNS vasculitis may occur isolated (primary CNS vasculitis) or secondary to systemic conditions such as infectious processes, systemic vasculitis or collagen vascular diseases [3, 5]. Angiography-negative childhood primary angiitis of the central nervous system (cPACNS) has only recently been recognized as a novel rare disease entity [4], with only four cases reported thus far. Whereas large or medium-sized vessel primary CNS angiitis usually presents as transient ischemic attack or stroke, symptoms of small-vessel CNS vasculitis are more variable, including progressive neurological deficits [7, 9]. Typically, MRI reveals multifocal lesions of both gray and white matter with enhancement in T2 and FLAIR (fluid attenuated inversion recovery) images, whereas diffusion-weighted images and conventional angiography are normal [2, 4]. Brain biopsy shows intramural mononuclear lymphocytic inflammation of small and medium-sized vessels in both gray and white matter. In a small series of four patients, immunosuppressive treatment led to rapid clinical improvement and vanishing of MRI FLAIR lesions [4].