Cerebral vasculitis is inflammation of blood vessel walls in the central nervous system (CNS; brain, spinal cord). It may be caused by autoimmune disease, infection, malignancy, or a systemic vascular disorder, or it may be idiopathic. Primary angiitis of the CNS refers to vasculitis that is limited to the CNS and leptomeninges. Secondary vasculitis involving the CNS can occur from systemic vasculitides. The small- and medium-sized arteries of the CNS are typically affected.
Clinical manifestations are variable but include severe headaches, transient ischemic attacks or strokes, cranial neuropathies, seizures, and encephalopathy. In primary angiitis of the CNS, systemic symptoms such as fever, myalgias, rash, or weight loss are usually absent.
More recently, cerebral vasculitis also has been reported in children, but its incidence is unknown due to both its rarity as well as the possibility of it having gone undiagnosed. The signs and symptoms are the same.
Cerebral vasculitis
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Codes
ICD10CM:
I67.7 – Cerebral arteritis, not elsewhere classified
SNOMEDCT:
427020007 – Cerebral vasculitis
I67.7 – Cerebral arteritis, not elsewhere classified
SNOMEDCT:
427020007 – Cerebral vasculitis
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Differential Diagnosis & Pitfalls
- Multiple sclerosis
- Meningitis (bacterial, viral, fungal)
- Paraneoplastic or autoimmune encephalitis
- Embolic stroke
- Reversible cerebral vasoconstriction syndrome
- Systemic vasculitis involving the brain
- Systemic lupus erythematosus
- Rheumatoid arthritis
- Behçet syndrome
- Polyarteritis nodosa
- Neurosyphilis
- Central nervous system tuberculosis
- HIV infection
- Intravascular lymphoma
- Sarcoidosis
- Moyamoya disease
- Sinus thrombosis
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Last Reviewed:10/29/2018
Last Updated:03/14/2023
Last Updated:03/14/2023