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PACNS - Part 2: Benchmarks, Eyesight
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PACNS - Part 2: Benchmarks, Eyesight

THIS UPDATES MY PREVIOUS POSTING (4/17/2001) IN RESPONSE TO YOUR QUESTIONS.

About four months ago, my wife was diagnosed with Vasculitis of the Central Nervous System.  ANOTHER TERM IS PACNS, AND THE DIAGNOSIS OF PACNS -- PRIMARY ANGIITIS OF THE CENTRAL NERVOUS SYSTEM -- WAS CONFIRMED BY DR. CALABRESE OF THE CLEVELAND CLINIC AFTER A VISIT THERE.  She is taking Prednisone and Imuran.  A recent MRI shows that the vasculitis has diminished significantly, and she feels better overall -- recognizing that there are bad days as well as good.  However, my wife's eyesight has been declining steadily over these months.  Recently, the eyesight became much worse -- with pressure, cloudiness and a further reduced visual field, and she often cannot see well enough to read.  (It's like looking through crumbled cellophane.)  MY WIFE HAS BEEN SEEING A NEURO-OPTHAMOLOGIST GROUP FOR MORE THAN FOUR WEEKS.  THEIR FIELD VISION TESTS CONFIRM THE REDUCED VISION.  THEY HAVEN'T IDENTIFIED A CAUSE OF THE VISION PROBLEMS.  THE DAY AFTER YOUR MESSAGE, THEY RULED OUT YOUR POSSIBILITIES -- CATARACTS OR PSEUDOTUMOR CEREBRI.  My wife does her best to keep active and positive, and answers to two questions would help us greatly.

What are the benchmarks for PACNS?  We need some perspective on what we might expect over time, even in general terms.  Milestones will help us better recognize progress and accept setbacks.  How does it go with PACNS -- slow and steady progress or decline, or what?  In general, after how many months might we expect what kinds of changes?

Is it expected that eyesight would be affected by PACNS?  My wife's vision is much, much worse now than at any other time.  Has the PACNS affected the part of the brain that handles vision?  Can the medications play a part this decline?  Could the vision problem be unrelated?  What should we, and her doctors, be doing right now to reduce or eliminate the likelihood of permanent vision damage?

Thank you for any information you might provide.  WE WILL APPRECIATE YOUR SUGGESTIONS AND OF COURSE UNDERSTAND THAT THEY WOULD BE GENERAL.

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Avatar_n_tn
Dear Dave:

It sounds, at least looks on type, that you have highlighted things for me to see.  Thanks.  One finds that patients will make abbreviations of many things and it is best to be sure when giving advice, sorry but I don't like giving information when things are not clear to me.  

So, what has your neurologist told you?  You must have volumes of literature concerning primary or isolated CNS angitis.  The paper by Lie in Human Pathology 1992, 23:164-171 reviewed the literature and described 15 new cases.  So, you know that IACNS (this is his abbreviation for the disorder, so... one can see the confusion that can arise) embraces a full spectrum of clinical and pathologic manifestations and degrees of severity.  The angitis can affect small vessel, medium vessels, or large vessels or a combination of the above.  Some varieties are only found in the spinal cord, some in brain and some in both. What and where were the vessels were affected in your wife?  How was the diagnosis made-biopsy, MRA/MRI, angiography?  Not knowing makes some prognostication difficult because outcomes and severity are associated with the size of vessel affected.

I will assume that if your wife had only medium and large arteries affected that benign angiopahty has been ruled out.  What did the latest MRI/MRA of the brain show?  How has the changes of the lesion on MRI progressed and how has the treatment limited or changed the findings on MRI.  The outcome in small vessel types is not encouraging.  Symptoms are progressive and depending of where the lesions are, manifest corresponding brain areas.  I would search for lesions that affect vision in your wife.  Lesions in this case are likely better picked up by high resolution MRI than angiography.  Multifocal neurologic defects, cognitive decline and often seizures are seen in this type of angitis.  Medium to large vessels are usually seen having strokes or subarachnoid hemorrhage.  Again the MRI is the best tool to follow.  Aneurysm can also be found in this type of angitis and MRA is a good tool to follow and even clipping by surgery can be done if warranted.  Again, the brain region that is compromised by the lesion would show neurological signs and therefore MRI would show a lesion of the vision cortex or optic tracts in your wife's case.  If these are found then, unfortunately this would be the progressive neurolgical course your wife is facing.  

If one plays the odds, then in the adult world, most adult have the small vessel type of disease.  Most will have a gradual but variable course, with headaches, focal seizures, multifocal neurologic deficts and neurobehavior impairment-reflecting diffuse brain damage.  Here, as stated above, the MRI is the tool of choice to follow.  Treatment has shown, at least in the literature (I have not seen complete remission, but it is reported) a spectrum of responses to remission to gradual but progressive decline.  Medium to large vessel disease usually presents with stokes or TIA.  This is usually a more aggressive form of the disease but treatment again, is as with the small vessel variety.  Two good resources to read about treatment can be found in an article by Moore in Neurology 1989, volume 39: 167 and in Ilhan et al. in Int J Angiol, 2000, volume 9, 23-26.

I do hope your wife responds to treatment.  

Sincerely,

CCF Neuro MD

5 Comments
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Avatar_f_tn
Dave,
Just read your entry. Wonder if you'd like a web site for support? I am in the process of being further diagnosed MS or CNSV. Wonderful group of people! I am not very computer wise but try these sites:
CNSV.net
***@****
if that doesn't work there is a little symbol that says e-mail, an address will flash with the founder of the site address, she can help you too.
Good luck and hope to see you there, it is a post forum through your e-mail, like i said lots of info and good folks.
Allie
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Avatar_n_tn
Allie --

I'll check that site out.  Thanks and good luck to you.

Dave
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Avatar_n_tn
Hi Dave,
Did your neuro-opthamologist say anything about epiretinal disease or macular degeneration?  My mom has crumpled celephane type vision and she has these two problems.  Just a thought.
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Avatar_n_tn
kd --

Thanks for your suggestions.  The neuro-opthamologists have not mentioned either of these.  We'll read up on those and ask them if they might account for her vision problems.

Dave
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