Table 1.

Common CNS and PNS involvements of primary systemic vasculitis.

PSV	CNS involvement	PNS involvement
Takayasu arteritis	Dizziness (78.1%), headache (25.5%); visual disturbances (4.6–59.3%); syncope (4–21.9%); stroke (10–20%);	Rare
Giant cell arteritis	Neuro-ophthalmological damage (20–28.8%), stroke (1–3%), vertebrobasilar artery vascular accident (35%);	1–14% of cases; cranial neuropathies, multiple mononeuropathy, polyneuropathies;
Polyarteritis nodosa	20–40% of cases; Diffuse encephalopathy, cerebral infarction (13–17%);	60–70% of cases; Mononeuropathy, polyneuropathy, mononeuritis multiplex;
Granulomatosis with polyangiitis	8–28% of cases; Cranial nerve palsy (4.7–6%, mainly II, VI, and VII), pituitary damage (1.1–1.3%), meningeal pachymeningitis, ischemic, and hemorrhagic complications of brain and spinal cord, PRES	11–44% of cases; Recurrent mononeuropathies, mononeuritis multiplex, symmetric polyneuropathy
Eosinophilic granulomatosis with polyangiitis	6–10% of all cases; Cerebral infarctions and intracerebral hemorrhage	~60% of cases; Multiple mononeuropathy (68% of PNS cases), distal symmetric polyneuropathy (28%) and asymmetric polyneuropathy (4%)
Microscopic polyangiitis	Rare	55–79% of cases; polyneuropathy, mononeuropathy
Behçet's syndrome	CNS parenchymal inflammation (67–76% of all NBD cases): subacute cranial neuropathy, ophthalmoparesis, meningoencephalitis, alteration of cerebellar, pyramidal, and extrapyramidal function; Vascular complications in the nervous system (12–20% of all NBD cases): cerebral venous thrombosis, aneurysms	Extremely rare: 0.8% of BD cases
Cogan syndrome	Ischemic stroke (2.5–3%), encephalitis (5–6%), meningitis (5–22%), encephalopathy, myelopathy, optic nerve disorders, aneurysm, and cerebral venous thrombosis	Peripheral neuropathy (1–12.5%), cranial neuropathy (1–10%, mainly II, V, VI, and VII) and myopathy