Central Nervous System Angitis
Last updated: November 6, 2014
Synonyms: Primary CNS angiitis, isolated CNS angiitis, granulomatous angiitis of CNS.
ICD-9 Code: 437.4 (vasculitis).
Definition: The term CNS angiitis encompasses a variety of inflammatory conditions that result in decreased blood flow in cerebral vessels (Table 9). Neurologic defects may be focal or diffuse, depending on the location and extent of the lesions. Primary CNS angiitis is an idiopathic inflammatory disorder with characteristic clinical, angiographic, or histologic findings of vasculitis limited to the CNS.
| Table 9 Conditions Associated With or Resembling CNS Angiitis |
|---|
| Primary CNS angiitis
Biopsy proven Angiographically proven Secondary CNS angiitis Systemic vasculitides: giant cell arteritis, Takayasu’s arteritis, Wegener’s granulomatosis, polyarteritis nodosa, Churg-Strauss angiitis, Behçet’s syndrome Inflammatory disorders: rheumatoid arthritis, SLE, Sjögren syndrome, sarcoidosis Infections: tuberculosis, fungi (coccidioidomycoses, actinomycoses, cryptococcus), spirochetal disease (syphilis, Lyme disease), viruses (HIV, CMV, herpes) Drug induced: cocaine, amphetamines, sympathomimetics (ephedrine, phenylpropanolamine), thiazides Vasculopathic conditions Antiphospholipid syndrome Atherosclerotic disease Hypertension Thrombotic thrombocytopenia purpura Persistent or recurrent vasospasm CNS lymphoma Moyamoya disease |
| *CNS, central nervous system; SLE, systemic lupus erythematosus; HIV, human immunodeficiency virus; CMV, cytomegalovirus. |
Pathology: Primary CNS angiitis may demonstrate granulomatous or non- granulomatous vasculitis affecting the leptomeningeal and cortical small- and medium-sized vessels.
Demographics: This rare disorder is commonly seen in adults aged 20 to 60 years.
Cardinal Findings: Symptoms of primary CNS angiitis may include headache, confusion, neurocognitive dysfunction, cranial neuropathy, seizures, and focal motor or sensory defects. Physical examination may reveal fever, hypertension, focal neurologic deficits, papilledema, and funduscopic abnormalities. Other systemic features, such as arthralgias or myalgias, are uncommon. Nearly 10% of patients exhibit a myelopathy.
Diagnostic Testing: In patients suspected of having CNS angiitis, routine laboratory test results are frequently normal. Nonspecific findings may include an elevated ESR or leukocytosis. Serologies (ANA, RF, ANCA) should be normal. CSF analysis may reveal several abnormalities, including increased CSF protein, lymphocytic pleocytosis, and increased CSF immunoglobulins (see CSF studies). The CSF results may also be normal and be of greatest value in excluding other causes. The role of leptomeningeal biopsy is controversial. Although pathognomonic, granulomatous or nongranulomatous vasculitis with mononuclear infiltrates is found in <50% of patients. Sensitivity is increased by using biopsy specimens from both brain tissue and leptomeninges or by taking tissue samples from areas abnormal on MRI. However, the procedure may yield false- negative results in >25% of cases. Biopsy is particularly helpful in establishing or excluding other diagnoses such as lymphoma and sarcoidosis.
Imaging: CNS imaging results are often abnormal. However, they may not allow precise determination of the cause. CT scans may show infarcts or be entirely normal. It may be most useful in helping to rule out other conditions, such as subarachnoid hemorrhage or tumor. As many as 15% of patients have mass lesions on CT scans or MRI. MRI frequently reveals abnormal signals in the areas of affected vessels. MRI is most effective in identifying myelopathy owing to vasculitis. Angiography characteristically shows diffuse vascular “beading” with areas of alternating stenosis and dilatation. Although these changes are consistent with vasculitis, they are not diagnostic; they may also be seen in noninflammatory vasculopathies.
Keys to Diagnosis: Primary CNS angiitis should be suspected in young to middle-aged individuals presenting with headache, focal neurologic findings, normal or slightly abnormal CSF results, and angiographic evidence of intracerebral vasculitis. Medications, infections, or associated inflammatory and noninflammatory vascular disorders must be considered and excluded.
Differential Diagnosis: See Table 9.
Therapy: Aggressive immunomodulatory therapy is indicated for proven primary CNS angiitis. Usual recommendations include high-dose prednisone in conjunction with cytotoxic drugs such as cyclophosphamide for a period of 6 to 12 months.
Prognosis: The prognosis of CNS angiitis can be variable. Primary CNS angiitis may be fatal if untreated. However, early diagnosis and aggressive treatment have led to more favorable outcomes, although some may be left with fixed neurologic deficits.
BIBLIOGRAPHY
Calabrese LH, Duna GF, Lie JT. Vasculitis in the central nervous system. Arthritis Rheum 1997;40:1189–1201.PMID:9214418
Cohen SB, Hurd ER. Neurological complications of connective tissue and other “collagen-vascular” diseases. Semin Arthritis Rheum 1981;11:190–212.PMID:6116280
West SG. Central nervous system vasculitis. Curr Rheumatol Rep 2003;5:116–127.PMID:12628042
