Pediatric Central Nervous System Vasculitis

Abstract

A 6-year-old right-handed female presented for recurrent episodes of CNS dysfunction. She initially presented with several weeks of frontal headaches and intermittent fevers (38.4–39.5 °C). Her symptoms progressed to include fatigue, mental status changes, and an inability to walk unassisted due to her mental status and overall lack of coordination. She was admitted 20 days into her illness, where her exam demonstrated encephalopathy with hyperreflexia and an extensor plantar response. She was treated with steroids, but had recurrent optic neuritis twice in the following year, both times when weaning off steroids. Her headache with associated steroid-responsive neurologic symptoms, in the setting of fever and fatigue, made multiple sclerosis less likely. Given suspicions of CNS vasculitis, a brain biopsy of an enhancing parietal lobe lesion was performed and diagnostic for primary angiitis of the central nervous system (cPACNS). cPACNS is a rare inflammatory brain disease which targets the vasculature of the central nervous system to include the brain and/or the spinal cord. CNS angiitis remains important to distinguish from pediatric-onset multiple sclerosis because long-term treatment differs. She was treated with cyclophosphamide and prednisone which was eventually transitioned to mycophenolate mofetil (MMF).