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CNS vasculitis

  I would like to get some information on CNS Vasculitis as my Mother-In-Law was just diagnosed with it yesterday. I would like to know what it and any current research information on treatment possibilities.  The Doctors told us that it is very rare.  I Thank You for any information that you can give me.
Vasculitis is inflammation of blood vessels. There are different types of vasculitis, but most appear to involve an attack by the immune system on self (auto-immune disease). Vasculitis can appear in different parts of the body, including the central nervous system. In diseases like lupus, for example, vasculitis of the CNS appears in the setting of vasculitis in other parts of the body.
Isolated CNS vasculitis is its own disease - that is, no other part of the body is involved inthe vasculitis, just the brain. Other names for this include "primary angiitis of the CNS (PACNS)", "isolated CNS vasculitis", "granulomatous angiitis of the CNS".
PACNS is difficult to diagnose and treat. Symptoms depend on what part of the nervous system is being more affected, which is sort of random. It's basically like having a lot of small (or even large) strokes. After all, if the blood vessels are affected, you can see how they could get plugged up, depriving parts of the brain of blood supply, which is basically what happens in stroke. The difference is that instead of "hardening of the arteries" the process is inflammation.
Untreated, the bad news is that PACNS is devastating, most often fatal. The good news is that if the diagnosis is made in time, before much permanent damage has taken place, the vasculitis can be arrested and some degree of recovery made.
It's pretty rare. I've personally had experience with just three patients over about 5 years. One in particular experienced a dramatic mental decline and another neurologist thought she had Alzheimer's (but the progression was over months, not years). We did the appropriate studies, gave her aggressive treatment, and when I saw her in follow-up, she was able to talk again and was pretty with it and cheerful.
It's probably not realistic to expect a complete recovery - there may be some residual neurologic deficit after successful treatment. But there really is hope that the disease process can be arrested and the damage minimized.
Treatment usually includes Cytoxan (cyclophosphamide), which if you look it up you'll find is a chemotherapy agent. It's pretty rough stuff, but it's what works. In the past, doctors tried high dose steroids. Those are part of the treatment too, but usually cannot be the sole agent used.
The above discussion is predicated on the correct diagnosis. This can be a very difficult diagnosis to make, requiring spinal tap (LP), angiogram, and often brain biopsy. If this doesn't sound like your experience, be sure your doctor clarifies what he means by "CNS vasculitis" and how the diagnosis was made.
The stroke specialists here at CCF have more expertise than I about this disorder - they treat quite a few patients with it (even as rare as it is).
This information is posted for your medical education. Specific comments regarding diagnosis, prognosis, and treatment options must come from your doctor after appropriate evaluation. I hope this helps. CCF MD mdf.

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