What to expect

"The first "modern" description of CNSV appeared in 1959. However, review of the medical literature reveals sporadic reports of probable CNSV as early as 1922. Before the availability of modern diagnostic techniques, CNSV was rarely diagnosed during life; rather, the diagnosis generally was rendered only at autopsy. The availability of advanced imaging techniques for the CNS (e.g., magnetic resonance imaging, angiography) now frequently permit the diagnosis to be made during life, and allow treatment to begin.

"CNSV symptoms and signs are frequently subtle, subacute, and often non–specific in nature. These characteristics, combined with the fact that confirmation of the diagnosis requires that an invasive procedure (either an angiogram or a brain biopsy) be performed on a delicate organ system — the brain — qualifies CNSV as one of the most challenging diagnoses to make in medicine."

No matter how bad I might be feeling, mentally or physically, I am lucky to be alive. If I had this condition 20 years ago I’d most likely be dead. Like I said, keeps things in perspective.

CNSV is a scary thing to have but it does not have to be the end of your life. We’ve taken the combined experiences of a few hundred folks with CNSV and come up with a basic outline of what to expect over the course of this condition.

Diagnosis

This is the easy version; if you want a more detailed medical explanation see Diagnosing Central Nervous System Vasculitis.

If CNSV is suspected it is of the utmost importance that you find a doctor who is familiar with the condition or is willing to confer with one who is. This is not a condition you can mess around with. Waiting is not an option. The vessels and tissue in your brain are being damaged. This is not a good thing!! You could have a TIA or a stroke if the swelling is not stopped.

Currently, there are several reliable methods for diagnosing CNSV which range from non-invasive tests to more complicated invasive procedures that may be used to verify the disease. The "Gold Standard" has been the brain biopsy for confirming CNSV. However, it is difficult to get a correct reading unless they biopsy tissue from a piece of brain that is active with CNSV. Also if taking Prednisone the results will be unreliable.

Here is a list of some tests that should be done when diagnosing CNSV.

• Measurement of the erythrocyte sedimentation rate, which is nonspecific but indicative of an ongoing inflammatory process.


• Detection of inflammation in the cerebrospinal fluid (CSF). This is performed by a lumbar puncture (or spinal tap). A needle is inserted into the small of the back to recover CSF, which circulates around the spinal cord. Levels of protein are elevated in 85% of patients with CNS vasculitis.


• Magnetic Resonance Imaging (MRI) almost always detects evidence of widespread small vessel infarcts, or unusual patterns of enhancements resulting from inflammation and breakdown of the blood-brain barrier.
  
• Cerebral angiography, which is an x-ray examination of blood vessels supplying the brain after the patient has been injected with a dye. This test may demonstrate irregularities of the cerebral arteries, known as "beading."

In addition, we recommend neuropsychological testing as another diagnostic tool to evaluate any decline in cognitive functioning, comprehension, information processing abilities, etc. Also, these tests can determine the extent and location of damaged areas of the brain. This information is very helpful when applying for disability benefits.

     Once you have a diagnosis of CNSV your treatment will begin.

Treatment

To get the CNSV under control we take Immunosuppressive and cytotoxic drugs. Here is a list of the most common medicines used to treat CNSV:

• Prednisone
• Imuran
• Cytoxan
• Methotrexate
• Cyclophosphamide
• Cyclophosphamide
• Cytoxan
• CellCept

The medications used to treat CNSV are toxic to your body but at this point these are the medications that work.

These are the most common experiences reported by those being treated for CNSV. There are also some tips on how to handle things:

If you have not done so already, we suggest taking an extended leave of absence from your job.

Until treatment is completed life is very difficult. You basically feel exhausted all the time, from the medication and the CNSV. You want to sleep all day and feel bad because your not accomplishing anything. Listen to your body! You need a lot of sleep to recover. Don't feel bad, it's just part of the condition.

If taking Cytoxen drink at least 64 oz of water a day, especially if it's Intravenously. It's imperative to flush the toxins from your body. It also helps avoided bleeding in the bladder and lessen the chances of kidney problems later on.

With a lowered immune system it's easy for infection to set in. When you take Prednisone it will ”mask” signs of infection. It is important to tell your doctor if you have a cough or begin to feel sick. It's hard to tell when your on all the medication so you need to pay close attention to what your body is doing.

You might feel depressed, angry, violent or suicidal. Some of these feelings are side effects from the medication, some are a result of feelings of loss from such an abrupt and unwelcome change in your lifestyle. You need a lot of help right now and you resent having to ask. You might want to ask your doctor about taking an antidepressant at this time.

Because your cognitive skills are affected by the CNSV and the medication, simple things like adding and subtracting become impossible. You have problems counting money and become upset if in a store because people are standing behind you tapping their feet and sighing while you try to count out the correct change.

You become forgetful and leave things cooking on the stove or in the oven. As you might suppose this can be a dangerous problem.

Words fail you. You can see them in your head but it just won't come out or words come out in an unrecognizable format. While family members might find this amusing, my kids loved it when mommy talked funny, it can be frustrating and embarrassing to you.

It becomes difficult to concentrate when sitting with a lot of people. With many conversations going on around you, you tend to ”zone out” and stare off into space trying to hang onto your composure. All you really want to do is find a nice quite room and take a nap!

Driving becomes difficult or downright dangerous! The brain cannot handle all the visual and mental stimulation driving creates.

Speaking of vision, You might notice you have problems with seeing correctly, reading might be difficult. Your depth perception might not work right.

Your balance gets out of wack and you might have vertigo. Going up and down stairs becomes difficult.

Your appetite is out of control. You eat as if it's you last meal at every sitting. Prednisone can cause cause drug-induced diabetes, which usually goes away after stopping the drug but it's better to avoid it if possible. You can do this by eating like a diabetic. A great place to read about this is Dave Mendosa's Diabetes website. The other side of the coin is loss of appetite. Not too many people have reported this problem.

Your joints hurt, watch out for Osteoporosis, take a calcium pill but not vitamins. You don't want to boost your immune system at this time. The treatment for CNSV is to wipe out your immune system so it can "re-set" itself. Taking vitamins goes against the treatment!

Make sure you taper off the Prednisone slowly. People have become very sick if they come off it to fast. They’ve reported back pain, joint pain, exhaustion, nausea and body aches. The first time I started tapering I ended up in the Emergency Room because I could not sit up without great pain and I could barely walk. After 5 hours in the ER we realized it was because I was coming of the Prednisone too fast.

Stay out of the sun! The medication makes your skin ”thin” which leads to major sunburn and bruising easily.

Another fun side effect of Prednisone is you start to look like an oompa-loompa or a demented Cabbage Patch doll. You might get a hump between your shoulders. Your skin gets tight and hugs are painful!

You might not get all these things but these are the most reported problems among people with CNSV.