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Aug 14, 2006
CNS Vasculitis
Vasculitis is a condition of inflammation affecting blood vessels, including veins, arteries, and capillaries. Most conditions of vasculitis have an autoimmune origin and are characterized by inflammation, bleeding under the skin, ruptured surface (cutaneous) blood vessels, and bruising. Vasculitis of the central nervous system (CNS) is a rare autoimmune disorder characterized by inflammation of the blood vessels that supply the tissues that make up the brain and spinal cord. Vasculitis in these areas leads to blood flow blockage, causing a condition of ischemia or oxygen deprivation. Consequently, the affected blood vessel wall and the tissue supplied by the blood vessel die. In CNS vasculitis the immune system produces antibodies that react with protein antigens in cerebrovascular (blood vessels of the nervous system and brain) tissue.
These antibodies form a lattice-like antigen-antibody complex that lodges within the blood vessel walls. In addition, the presence of these complexes causes an immune response in which the immune system's white blood cells infiltrate the blood vessel wall. Immune system chemicals and enzymes are released during this infiltration, which causes swelling and cellular destruction.
Symptoms of CNS vasculitis are variable. Most patients will have a cluster of several symptoms rather than all associated symptoms. Headache is one of the earliest symptoms of CNS vasculitis and it may be accompanied by cognitive changes or symptoms of depression or seizures. Other symptoms include weakness, gait disturbances, seizures, ataxia (an inability to coordinate muscle reflexes), stroke-like symptoms and coma.
Several procedures are used to diagnose CNS vasculitis. Because CNS vasculitis is an inflammatory disorder, patients typically have an elevated erythrocyte sedimentation rate and C-reactive protein. These are the usual screening tests for CNS vasculitis. Negative results suggest other causes.
Diagnosis of CNS vasculitis usually requires a lumbar puncture and a cerebrospinal fluid (CSF) examination. CSF surrounds the spinal cord. In CNS vasculitis, levels of protein are typically elevated although there is no evidence of bacteria, which are typically seen in conditions of meningitis, and the oligoclonal band pattern seen in multiple sclerosis is absent. Magnetic resonance imaging (MRI) studies typically show evidence of multiple small blood vessel infarcts or signs of inflammation and breakdown of the normal blood-brain barrier. Cerebral angiography, an imaging procedure used to study the blood vessels of the brain after injection of a contrast dye, may show irregularities or "beading" of blood vessels. Although these procedures may all be used to help diagnose CNS vasculitis, a definitive diagnosis requires a brain biopsy. A brain biopsy involves removing a small piece of the brain for microscopic examination.
Immunosuppressant medications such as dexamethasone and corticosteroids are the primary therapies used for CNS vasculitis. Patients with severe cerebral edema and inflammation may also require monitoring with an intracranial pressure monitor. Some patients may also require therapies such as plasmapheresis or intravenous immunoglobulins to quickly reduce the number of immune complexes in an effort to reduce inflammation quickly.
Comments
Sep 25, 2008 9:09 AM
Guest :
We recently lost someone at the cleveland clinic for cns vasculitis
autoimmune disorder. We had never heard of this disorder untill she was
told she had it 2 months ago. This is the first web site I found that
explains the diorder where I can actually understand it. I still don't
understand how someone can go all there life and not know they have it. She
was 39 years old.
Oct 4, 2008 7:33 PM
Guest :
My name is Pattie Iwas at Cleveland Clinic on JUne 25 of this year with
symptoms of CNS vasculitis i couldnt walk i lost motor skills and cognitive
skills Ihad had a seizure and felt like i was litterly going to die it is
now October and i am still having symptoms Ihave been unable to work at my
job that ilove and had been there 13 years .and they told me it was some
sort of virus i had and i would get better but I know i was not diagnosed
propperly and im scared. because ive had a terrible headache and neck
pressure for 25 days with stringy mucas coming from my eyes and hacking up
thes weird mucas little balls perfectly round this is not normal and
because CNS is rare they dont take my symptoms seriously.
Oct 20, 2008 8:46 PM
Guest :
My husband was diagnosed with CNS vasculitis October of 2006. He had a
brain biopsy to determine this after about a month of every other test
possible including lumbar punctures, MRIs, CT scans, etc. By the time he
was diagnosed and before they began to treat him at UVA in Charlottesville,
VA, he lost the use of his entire right side (arm and leg), and also his
speech and vision, in addition to a loss of cognitive ability...I could go
on and on. He is still with us, but has only regained some of his vision
and speech, no use of arm or leg and his mind is still altered (mood,
reasoning, attention span). He requires assistance for everything, except
that he can feed himself. Its been a hard road and I read everything I can
about this disorder, but still feel a little lost.
Sandra Lee, King
George, Virginia
Oct 21, 2008 9:46 AM
Elaine Moore :
Dear Sandra
I'm sorry to hear of your husband's problems. If the
standard therapies of corticosteroids and dexamethasone aren't helping you
might ask your doctor about plasmapheresis or IVIG therapy. I hope your
husband begins to improve. Best, Elaine
Nov 17, 2008 2:17 PM
Guest :
My 12-year-old daughter was diagnosed with CNS Vasculitis and it has been a
long an difficult road. She was admitted to CHOP for 50 days and endured
months physical therapy to learn how to walk, talk, eat, etc.
It's
been 15 months now and she is doing well. She is back to school without
any assistance physically or is able to complete all of her studies. Her
chemo is almost done, along with all of the other meds.
Below is a
link to a site that explains everything in great detail, from diagnosis
through treatment and recovery. In most cases there is a light at the end
and it isn't all bad, just keep the faith and good luck!
http://members.cox.net/cnsv
Jan 22, 2009 6:55 AM
Guest :
I am a 60+ year old female diagnosed with CNS vasculitis 1 month ago
following a mild TIA. It is very difficult to find literature on long term
issues of this disease. Dr. says it is rare and mostly found in males, not
helpful to me. It feels very scary and lonely-every little twinge makes me
wonder if I have some new problem. Thanks for listening
Apr 7, 2009 7:40 PM
Elaine Moore :
Hi,
Since I'm not a physician I'm not really qualified to say what
other disorders might be causing your wife's symptoms. But I am qualified
to interpret laboratory test results. A WBC count of 20 with a slightly
increased protein isn't an unusual finding in a lumbar puncture and it
could signify many things including contamination from the lumbar puncture
itself. I don't think you have enough information for a definitive
diagnosis at this point. If your insurance covers it you might want to see
about getting a second opinion and you might want to ask if a PET scan
would help with the diagnosis.
If the meds are effective in
reducing intracranial pressure they should reduce symptoms, but for some
patients plasmapheresis or IVIG therapy are needed.
Best to both
of you, Elaine
Sep 4, 2009 6:33 PM
Elaine Moore :
Hi Chad,
Did you have a sed rate and a C-Reactive Protein (CPR)
test? These blood test results will be markedly elevated in CNS vasculitis.
You can get a copy of your tests results from your local hospital from the
medical records department?
Are you on corticosteroids to lower
antibody production? This often helps. IVIG therapy is also a good choice.
See this article:
www.suite101.com/blog/daisyelaine/ivig_therapy
and ask your doctor about it. It's expensive but often very effective as
is plasmapheresis. I wish you well, Elaine
8 Comments
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