Primary angiitis of the central nervous system: Unusual clinical presentation

Abstract

Primary angiitis of the central nervous system (PACNS) is a form of vasculitis restricted to the CNS. Most patients with PACNS present with focal neurologic deficits suggesting stroke or with a syndrome of headache and confusion. Less commonly, seizures, dementia, cranial neuropathies, myelopathies and, rarely, radiculopathies are seen. We report a 14-year-old girl who presented with partial motor seizures complicating focal cerebral vasculitis of superior parietal gyrus on the left. Despite extensive evaluation, no etiology was apparent for the vasculitis. Steroid therapy was administered, and 6 months after the therapy, she remains healthy except for a mild weakness of her right leg. Follow-up brain magnetic resonance images were normal. We conclude that partial motor seizures is uncommon as a presenting complaint in PACNS and steroid therapy is successful for this patient.