Symptoms of Primary Angiitis of the Central Nervous System

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.

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