Childhood Cerebral Vasculitis

Abstract

Central nervous system vasculitides are defined as the invasion of the vascular wall by blood-borne inflammatory cells. In childhood, they may be classified according to their trigger event (infectious vs. non-infectious), their temporal course (time-limited vs. chronic), and the size of the affected vessel. Diseases apparently confined to the central nervous system are also distinguished from secondary forms, associated with infection or rheumatic or systemic inflammatory disorders. Large-vessel vasculitis, the most frequent form, causes stroke and presents with acute focal deficits. MR, or more seldom contrast angiography is required for the positive diagnosis, while the child's medical history conveys the etiological diagnosis. The clinical manifestations of small-vessel vasculitis include headaches, seizures, focal deficits, cognitive decline, and behavior changes that can occur insidiously over a few weeks or a few months. The diagnosis is based on the associated clinical and biological symptoms in secondary forms and on cerebromeningeal biopsy in primary forms. Secondary forms of vasculitides are treated according to the etiology. The injury of large basal arteries is often observed after infection, especially varicella, and is also called transient focal cerebral arteriopathy (TCA) or post-varicella arteriopathy (PVA). This focal, monophasic, and time-limited entity is highly specific of childhood. There are no arguments in the current literature supporting the hypothesis that an aggressive immunomodulatory treatment would be more effective, in terms of recurrence rate or functional outcome, than aspirin alone. In contrast, the diffuse, prolonged, and aggressive course of the rare primary vasculitis of the central nervous system requires a prolonged immunosuppressive treatment. The management of associated symptoms, treatment-related adverse effects, and sequelae is based on a multidisciplinary approach.