Symptoms of Primary Angiitis of the Central Nervous System
What are the typical presenting manifestations of PACNS/PCNSV?
The onset is most often insidious (over 1–3 months). Patients almost always have a headache. Common presentations are:
• Headaches (60%), cognitive impairment and strokes (30%–50%).
• Recurrent focal neurologic symptoms.
• Unexplained diffuse neurologic dysfunction (subacute encephalopathy, behavioral changes, seizures, cerebral hemorrhage): most common in older patients with ABRA.
• Unexplained spinal cord dysfunction (myelopathy, radiculopathy).
• Rare symptoms: cranial neuropathies, ataxia, seizure, and coma.
• Note that symptoms of systemic vasculitis, such as fever, rash, and mononeuritis multiplex, are usually absent.