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Clinical Services at Rush Types of Vasculitis

Primary angiitis of the central nervous system (PACNS)

What is the cause of primary angiitis of the central nervous system?

What are the symptoms of primary angiitis of the central nervous system?

How is primary angiitis of the central nervous system diagnosed?

What is the treatment for primary angiitis of the central nervous system?

What is the prognosis for primary angiitis of the central nervous system?


Primary angiitis of the central nervous system (PACNS) refers to vasculitis that affects only the brain and, occasionally, the spinal cord. By definition, these are cases where no other part of the body is affected and where there is no known infection within the central nervous system.

“Primary angiitis of the central nervous system” is a more recent term for a range of central nervous system (CNS) diseases included in what used to be known as “isolated angiitis of the CNS (IACNS)” or “granulomatous angiitis of the nervous system (GANS).” Since the 1990s, a category called benign angiopathy of the central nervous system (BACNS) has been used to apply to a range of CNS vasculitis cases that do not fit the parameters of PACNS. BACNS affects mostly younger women, and while its symptoms tend to be acute, the disease can be less deadly than PACNS. Because both groups of CNS disorders do share many symptoms, it can be difficult to distinguish between the two.

What is the cause of primary angiitis of the central nervous system?

The cause of PACNS is not known.

What are the symptoms of primary angiitis of the central nervous system?

Some classic symptoms of PACNS include:

  • Headache
  • Focal weakness (as occurs with a stroke)
  • Seizures
  • Fever
  • Cerebral hemorrhage
  • Confusion
  • Disorders of memory
  • Altered consciousness
  • Personality changes

How is primary angiitis of the central nervous system diagnosed?

Diagnosing PACNS is very difficult because its symptoms tend to be subtle, subacute and often nonspecific in nature, which requires an invasive procedure such as an angiogram or brain biopsy. In fact, until advanced imaging techniques such as magnetic resonance imaging (MRI) and angiography were developed, the disorder was usually diagnosed postmortem.

Many diseases mimic vasculitis of the central nervous system, most notably a group of diseases known as reversible cerebral vasoconstriction syndromes (RCVS), so an angiogram and/or brain biopsy is usually required for diagnosis. Angiograms typically will show “beading,” a pattern of alternating dilations and constrictions within the affected blood vessels, although this is not unique to PACNS. Ideally, a brain biopsy would include the meninges, the brain’s covering, for the most complete picture of cerebral conditions.

Magnetic resonance imaging (MRI), magnetic resonance angiography (MRA) studies and lumbar punctures (spinal taps) are also helpful in the work-up of a patient who is suspected of having PACNS.

What is the treatment for primary angiitis of the central nervous system?

Powerful immunosuppressive therapy is standard in treating this otherwise severe disease. It is desirable to try to control the disease initially with high doses of steroids (prednisone), and for some patients that suffices. Other cases, however, require an additional immunosuppressive medication such as cyclophosphamide.

What is the prognosis for primary angiitis of the central nervous system?

Treatment of PACNS often must be continued for a year or more. The prospects seem to be much improved for people with a disease whose prognosis formerly was poor.





Contact Name
Rush Vasculitis Clinic
Contact Phone
(312) 563-2800
Contact E-mail
contact_rush@rush.edu



Location
Orthopedic Building
1611 W. Harrison St., Suite 510
Chicago, IL 60612



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