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SynopsisCodesLook ForDiagnostic PearlsDifferential Diagnosis & PitfallsBest TestsManagement PearlsTherapyReferences
Cerebral vasculitis
Other Resources UpToDate PubMed

Cerebral vasculitis

Contributors: Andrea Wasilewski MD, Richard L. Barbano MD, PhD
Other Resources UpToDate PubMed

Synopsis

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.

Codes

ICD10CM:
I67.7 – Cerebral arteritis, not elsewhere classified

SNOMEDCT:
427020007 – Cerebral vasculitis

Look For

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

  • Multiple sclerosis
  • Meningitis (Bacterial meningitis, Aseptic meningitis, Fungal meningitis)
  • Paraneoplastic encephalomyelitis or Antibody-mediated encephalitis
  • Cerebral stroke
  • Reversible cerebral vasoconstriction syndrome
  • Systemic Vasculitis involving the brain
  • Systemic lupus erythematosus
  • Rheumatoid arthritis
  • Behçet syndrome
  • Polyarteritis nodosa
  • Tertiary syphilis
  • Central nervous system Tuberculosis
  • Human immunodeficiency virus primary infection
  • Angioendotheliomatosis
  • Sarcoidosis
  • Moyamoya disease
  • Sinus thrombosis

Best Tests

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Management Pearls

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Therapy

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References

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Last Reviewed:10/29/2018
Last Updated:03/14/2023
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Cerebral vasculitis
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A medical illustration showing key findings of Cerebral vasculitis : Seizures
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