Angiography-negative primary angiitis of the central nervous system in childhood

Abstract

Childhood primary angiitis of the CNS is a recently characterized, potentially reversible disease process. A favorable outcome requires early diagnosis and appropriate treatment. The histological findings of childhood primary angiitis of the CNS are characterized by a lymphocytic, nongranulomatous vasculitis. This disorder can lead to neurological deficits, seizures, and strokes. Laboratory and radiographic investigation are part of the evaluation, but are often nonspecific. Conventional angiography can fail to show any abnormality, and biopsy may ultimately be required for diagnosis. Although there can be significant rates of morbidity and mortality if untreated, patients who receive appropriate therapy can experience excellent outcomes, and in many cases will demonstrate near-complete or total clinical and radiographic resolution. The case of a previously healthy 13-year-old girl with new-onset generalized tonic-clonic seizures is presented, with a review of the literature.