Primary Central Nervous System Angiitis in a 10-year-old Girl

Abstract

Primary angiitis of central nervous system (PACNS) is well reported, however its occurrence in the pediatric population is infrequent. We describe the clinical, neuroimaging and histopathological features of PACNS in a young girl.A ten year-old, previously healthy girl presented with a threeweek history of progressive left hemiparesis and facial weakness. Other findings included left hemineglect, impaired concentration and memory. She had no evidence of systemic disease. Head computed tomography (CT), with and without contrast, revealed no abnormalities. Magnetic resonance imaging (MRI) demonstrated multifocal, bilateral signal abnormalities within basal ganglia, thalami, right frontal cortex and subcortical white matter on T2 and FLAIR images. However, these areas did not show restricted diffusion on diffusion weighted imaging (DWI). Cerebral angiography revealed normal intracranial vessels (Figure 1). Neuroimaging, three weeks later, showed progression of these abnormalities. The areas of abnormal signal showed heterogeneous and nodular enhancement on gadolinium enhanced T1 weighted images (Figure 2). Rheumatologic, metabolic, prothrombotic and cerebral spinal fluid investigations were normal.