Primary angiitis of the central nervous system with myelopathy as initial clinical presentation

Primary angiitis of the central nervous system (PACNS) is a rare vasculitis restricted to the central nervous system without systemic involvement. Delay in diagnosis and treatment is common due to its non-specific symptoms and lack of non-invasive diagnostic tests. Myelopathy can occur in PACNS, during the clinical course of the illness, with or without cerebral symptoms. We describe here a 51 year-old ethnic Chinese woman who presented initially with paraparesis without cerebral symptoms. The diagnosis of PACNS was eventually made from brain biopsy when she subsequently developed cerebral involvement. Despite aggressive treatment, the patient developed progressive neurological deterioration and died. This patient demonstrates the rare occurrence of myelopathy as the sole initial presentation of PACNS.