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Central Nervous System Vasculitis
Kristi Sloan
other names for Central Nervous System Vasculitis (CNSV):
- recently the preferred name for this form of vasculitis has been primary angiitis of the central nervous system ("PACNS")
- isolated angiitis of the CNS ("IACNS"), emphasizing the confinement of the disease process to the brain and the spinal cord
- granulomatous angiitis of the nervous system ("GANS")
- a subgroup of patients appear to have milder disease courses has led to the designation of benign angiopathy (as opposed to "angiitis") of the CNS, or "BACNS"
Granular material and abnormally large cells (giant cells) accumulate in the elastic lining of the arteries. Chronic inflammation is sometimes confined to the different branches of the heart's main artery (aorta) and any large arteries can become inflamed. However, the temporal arteries (large medium-sized arteries) of the head are most frequently affected (temporal arteritis).
symptoms:
CNSV symptoms and signs are often subtle, subacute, and non-specific in nature. Presentating symptoms are highly variable, but the triad of headache, organic brain syndrome, and multifocal neurologic deficits suggests CNSV. Most affected patients do not have systemic symptoms, signs, or abnormal routine blood tests, including ESR.
diagnosis:
Diagnosis requires that an invasive procedure (either an angiogram or a brain biopsy) be performed on a delicate organ system – the brain. This makes CNSV one of the most challenging diagnoses to make in medicine.
treatment:
Many patients are treated with prednisone and cyclophosphamide
Source references:
VASCULITIS OF THE CENTRAL NERVOUS SYSTEM, Meg Reitmeyer, M.D.,
Clinical Medicine Conference, January 30, 1997
Johns Hopkins CNS vasculitis page
Central Nervous System Vasculitis,
A discussion of Central Nervous System Vasculitis written in medical terms by David Hellmann, M.D. (F.A.C.P.)
Central Nervous System Vasculitis resources:
VASCULITIS OF THE CENTRAL NERVOUS SYSTEM -article by Meg Reitmeyer, M.D., from Clinical Medicine Conference, January 30, 1997
Johns Hopkins CNS vasc page
Central Nervous System Vasculitis A discussion of Central Nervous System Vasculitis written in medical terms by David Hellmann, M.D. (F.A.C.P.)
Diagnosis: Vasculitis of the central nervous system -a case summary of a CNSV patient and A Review of Central Nervous System Vasculitis (Primary Angiitis of the CNS-PACNS) by Sara Westgate, M.D., Ph.D., Resident, Department of Neurology, Baylor College of Medicine
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