CNSV and brain damage

I have posted in the past few months about my "relapse" symptoms of CNSV diagnosed and treated in 2005. I just received the report written after my first visit to new rheumatologist. He writes that the clinical picture is less likely a relapse of my CNS vasculitis and is "most likely due to previous damage in her brain white matter." I do not meet with him again for another month or two after another MRI is done. Does anyone have any ideas or knowledge about how the damaged matter can be causing headache, numbness, fatigue, brain fog, seizure, livedo, petechia, etc. These symptoms have been intense for the last three months. I probably have experienced some of these for years but why all of a sudden would I have a seizure and be quite ill if my brain damage is likely responsible. Thanks!!!

29 Replies

  • headache, numbness, fatigue, brain fog, seizure, livedo, petechia.. you just asked one of the hardest questions..I doubt drs understand these things, even neurologists. Do we have a list of drs we could discuss these? No. Not yet. I hope VUK would be able to compile a vetted dr list here in UK..this is one of my wishes. CNSV is probably one of the hardest type but poorly understood. Patients are isolated not knowing who to see for many years... a problem area.....if VUK could, that may help hundreds..possibly thousands of patients.

    Who is your Rheumy? Is he generally good? Is he the one who's arranging MRI? it sounds he's doing everything he could do for you? what maintenance treatments are you on if it would be okay to ask? I'm assuming that you have lupus or other autoimmune disease. Take care

  • Hi lancrose,

    The problem with a list of vetted Dr's is who would do the vetting, what criteria would we use and how would patients use that information?

    VUK have a helpline that people can phone for help, advice or just a listening ear. We try our very best to give patients names of Dr's that we know have a special interest in or experience of treating Vasculitis. Unfortunately no Dr is 100% perfect and we can never guarantee what the outcome of a consultation will be.

  • Oh yes, I know. I mean, I do know what you mean. Most of us are already aware of rheumy drs who say they have interest in V but you never know if they are really right ones for YOU (but soon, you will find this out yourself, anyway).

    Having said that, I am a member of charities of related illnesses. they do have a vetted dr list / drs who are active in these charities. Without those lists, I could not have made any sort of progress (over the past few years). The main issue about V, it affects multiple organs. You really need to know who to see when things strike and these are specialists areas a single rheumy would be unable to handle for himself. Especially, CNSV, it's a minefield for patients. Do we know many neuros who have interest in this? No, we don't. Do Neuros know enough about VCNS? I have seen multiple Neuros over the years, VCNS must be a specialist sub-speciality. It's something very few drs could deal with. Who are they? I don't know. Is this an issue? Yes, to patients, I believe it is.

  • Part of the problem is that there are over 14 different types of Vasculitis, it's not like phoning the Lupus charity and getting names of Lupus specialists.

    VUK does have medical advisors and we do our best to match patients with a local Dr. I am not sure that " publishing " Dr's names would be helpful. Unfortunately I don't think there are any UK CNSV " experts ", but am happy to be corrected if there are!

  • Well, everyone's situation is uniquely different, Keyes. Some people are on amend and some people are still struggling. Some are mildly affected and others are more severely affected.

    Perhaps, you do not need a list but there are others who may consider this to be useful (especially with life-threatening multi organ involvement - finding the options available would be immensely helpful) especially other charities do this quite often. They understand patients needs in trying to locate the specific drs. I think you are more focusing on "what will it happen if patients did not like the dr"? I know VUK's medical board have mainly rheumies (?) I don't know but it may help to know other drs in different areas other than rheumy. For patients going through "this maze" in medical care, anything will help in my honest opinion. But I think you are risk assessing if you did? Fear of uncertainty? I only speak as a patient who had to suffer from multiple issues without getting much help. Certainly, sadly, if you had a severe condition, you would end up needing an army of specialists. some patients do kindly mention who they received help from and THAT is hugely helpful. In fact, these patients may be saving other people's life by doing so.

  • With reference to discussion about the idea of creating a list of specialists who have experience and an interest in treating specific diseases such as CNSV, I know for me personally, it would have been extremely valuable. I am in Toronto so a UK list would not have helped me though I would travel to London to go to Addenbrookes if it turned out I had Hughes. Their list of specialists I am sure is a godsend to those diagnosed with Hughes.

  • Kat13

    We certainly seem to have similar symptoms. I hope that you will get to the bottom of this very soon. We can all say, "well, we don't know what it is as your condition is so rare and complex" and shrug this off almost as if you had such a bad luck.. Networking with other patients did help me too.. if you have a rare disorder, info gained from other patients going through the similar situation is totally vital. Not everyone is lucky to be placed on a multi-disciplinary team. Good luck with your next neurology appointment. Thank you for sharing. Best wishes,

  • Thank you Lancrose. Being able to connect with people with CNSV and other vasculitis diseases has been so important to me. I have received so much support and understanding from people with similar experiences. Keep in touch!

  • Thank you, Kat13 :) ideally, CNSV does need its own support group as our needs are quie different from those without CNS involvement. We understand each other and our symptoms. Best wishes,

  • I wish there was greater support for CNS patients specifically. I agree with lancrose that our form of vasculitis - if a primary CNS in particular - is vastly different from more common forms like Wegener's etc. Not just in day to day symptoms, but also in treatment. So much research focuses on ANCA-associated vasculitis, but that does little to help people with CNS vasculitis.

    I know this is probably not a popular view to share here, but I'm going to share it anyway ;) CNSV is still largely little understood. When I first got in touch with Vasculitis UK not too many years ago the charity had never even heard of CNS or cerebral vasculitis as I have had since 1994. Even now there is a lack of knowledge, though I and several other CNS/cerebral vasculitis patients are happy to act as volunteers for the charity - and do already - and are often contacted to advise on CNS/cerebral queries.

    There is at least one CNS vasculitis expert in the UK: Neil Scolding at Bristol, who has been actively researching and studying it for a couple of decades at least. But it's little understood other than that. Personally I think neurologists are often the worst people to see about it - they often know so little about this very rare neurological disease, and don't always recognise the signs, ignoring blood tests showing active inflammation, and MRI / lumbar puncture etc. isn't always informative. It's why I always recommend people see someone like a good rheumatologist who has dealt with many vasculitis patients, and, ideally, some CNS/cerebral ones too.

  • I know Vivian that none of the many doctors who have seen me over the years has actually treated a patient with CNSV with the exception perhaps of my current rheumatologist (not sure) and I hope the neurologist I will be seeing for the first time next week (not sure). My experience is that when asked (even if I ask in a gentle way ie. Have any of your previous patients with CNSV...) doctors will lie about this. I assume it does not I still a lot of confidence to tell your patient that you have never encountered this disease in person before. Personally, I would prefer that a doctor tell me straight and then tell me they are willing to contact the experts, do the research etc.

  • vivdunstan

    Thank you for your insights in your most helpful post. I much appreciated your excellent input :-) Wishing you well. Best wishes,

  • I wasn't thinking of my need for a " list " or otherwise Lanrose, neither was I speaking on behalf of VUK. I was musing out loud on the complexity of Vasculitis and how that meant it couldn't be pigeonholed along with other charities.

    You are wrong to say that there are mainly Rheumies on the medical advisory board, there is at least 1 nephrologist. There is also a lot of networking and medical advice going on behind the scenes, John Mills is also on the main European and British medical panels for Vasculitis.

    VUK is a small charity, staffed entirely by volunteers the majority of which have Vasculitis themselves. You may not have intended it but your post comes across as critical of the work that we try to do, much of which goes


    So my challenge to you is please come and join us if you think you can do better, get involved and actually speak to the many people that VUK have assisted over the years, whether that be help with finding an appropriate specialist or just a listening ear.

  • Keyes, I didn't read her post that way. I think the problem is that the vasculitides are all lumped together when they're all so different both in severity and in presentation/symptoms.

  • Thank you orsen-trapp. I'm a member of VUK but I have been very ill. The sheer delays of diagnosis despite the onset of the condition. I will be admitted to hospital this month. I only speak based on my own experience and how hard it has been. If you get my point, that's great! But if you don't, I am not planning to dispute as I have no energy for doing so-I have enough stress from life and from this illness. Everyone is different. Take care and thank you again, orsen-trapp! you made my day. It's nice to have a "friend" who understands. Best wishes,

  • Just out of interest Keyes, how does one get more involved? Does VUK actively recruit volunteers or does someone interested in volunteering approach VUK?

    I think the work done by VUK is remarkable, considering it's a small charity, it certainly proved useful to me.

  • Hi rasp,

    There was a push for more volunteers recently as the work the charity is doing has grown. I know they advertised on this site for people who had IT and clerical experience to help with admin and updating the website etc.

    Otherwise it's just becoming a contact for a geographical area and helping with local support groups, fundraising or ultimately going on the board and committees. I have got involved with the helpline and the Facebook and this group as I have some medical knowledge. I would really like to go on the board but I stay in Scotland and had to give up my driving licence recently due to eye problems ( which may or may not be connected to whatever auto immune condition I have! ).

    If you are interested then e mail

    My tasks for this month include writing a report of the Vasculitis Patient Symposium and putting together a healthy eating/ diet tips/ recipe page for the Autumn newsletter!

    VUK has given so much to me personally that I really wanted to give something back. I find it a welcome distraction from my symptoms as well.

  • Hi Lancrose

    I do not have lupus or any other autoimmune diseases as far as I know. The rashes, numbness, etc. all are new this time. My original onset was brain bleed, 10/10 headaches, seizure, chronic neck pain and the memory issues, fatigue etc. that followed with prednisone and cyclophosphamide treatment.

  • Hi Kat13,

    I probably don't need to tell you that you have one of the hardest to diagnose and treat forms of Vasculitis. Unfortunately there are very few Dr's up and down the country with experience of CNSV due to its rarity.

    I agree it does seem strange that old brain damage should suddenly cause such severe symptoms. Does your new Rheumy have experience in CNSV and have you ever seen a Neurologist?

    There are a few people on here with CNSV so hopefully they will be along to offer advice from their experience.

  • Hi Keyes. Yes I saw my old neurologist who did eventually get a vasculitis work up done in hospital. Angiogram, LP, MRI, bloods. They cannot see active vasculitis and eventually sent me home to deal with "headache issues" disregarding the consistent numbness and tingling of face and extremities, severe fatigue, nausea, neck pain, brain fog and then the other vasculitis- type rashes. Saw this same neurologist last week with rheumatologist report in hand. He could not provide any links between my brain damage on recent scans and the current symptoms. I am taking baby aspirin and THAT's it at this point. Have second opinion neurologist on August 13 and am so hopeful that he is more knowlegeabke and interested in cerebral vasculitis. Recommended by Best Doctors insurance. I am in Toronto. My rheumatologist is well respected but extremely busy. I am trying to stay physically active when I can but am feeling depressed more frequently just with long term ongoing uncertainty and fear that the damage may be getting worse in the meantime. I am worried about the condition of my brain but feel I am not getting the straight story. Hopefully this new neurologist will be good. I know I can not cure this myself regardless of how great my diet or exercise regimes are. I understand reluctance to put me back on Prednisone while they are still testing. It is a tough waiting gig!!!

  • I suggest you prepare a list of questions and a detailed history of your disease over the last six months in advance of your next meeting (It is a pet peeve of mine that Drs wait to see you before they order an MRI then you get a letter with the results that ALWAYS raises questions that you want to discuss and have to wait months for the next appointment. I have 6 monthly appointments that often turn into a year because of my consultant's case load. It's a long wait to find out. :-( but I digress ).

    An important question would be "What do they think caused the 'damage' that they are referring to?" They may mean long term damage caused by the vasculitis or damage due to trauma.The many reasons for the same symptoms is what makes this disease so hard to diagnose and predict. There must be something in the scan which has made him come to the conclusion he has and he should be prepared to discuss this at your next meeting. A detailed history of your disease progress, together with a 'map' of potential contributing factors in your life (particularly in the build up to the event) will help the two of you TOGETHER confirm or counter this.

  • Thank you Melon. I should see him in the next month or two. I hope the most recent MRI with contrast will help provide some answers where the blood testing typically does not for CNSV.

  • Thank you Melon for giving me some great ideas. I especially liked the idea of doing a detailed history with contributing factors in my life. Though I knew that a relapse was possible when I was first diagnosed, by the time I was no longer being followed by any specialists (2010), I thought I had put CNSV completely behind me. I think perhaps the history completed with all health records and scans I now have copies of, a different story may emerge. Maybe I should contact my rheumatologist's office and just ask his secretary to provide me with a brief explanation about how he feels the previous damage is the cause of current symptoms. This would be important information to know before I meet with the neurologist.

  • Glad it was useful! Good luck :-)

  • Melon

    I just reread your post. What did you mean by the damage done by trauma? did you mean a specific kind of trauma ie brain bleed or trauma In a more general way? Thanks

  • By trauma I meant damage due to a specific event. This could be due to 'external' influences - my first MRI revealed a calcified haematoma due to a accident - or, as potentially in your case, damage due to a bleed caused by your CNSV in the past. I guess position would tell them this. I think that it would good to have an appreciation as to whether there are benefits to some medications that you take other than just the obvious reduction of symptoms. Is it necessary to take some meds regularly (or at least longer than others) to reduce/avoid long term damage to the brain caused by the inflammation? I say this just because I strongly believe that a patient should be aware to the reasoning behind doctors decisions. Something that they often forget to explain.

    Hope that helps,


  • Tell me about it... negative blood tests always make me feel a little like a fraud! (I know it's silly and unreasonable but I always wish there was more tangible evidence!)

  • Hello kat13, I was diagnosed with cerebral vasculitis in June 2010, after being admitted to hospital with confusion, difficulty with speech ,walking+ other odd symptoms, I was put under the care of a Rhuematologist, within 3 days I had an MRI scan . A diagnosis of ? Cerebral vasculitis was given . I was started on cyclophosphamide and high dose prednisilone intravenously ,during my 11days in hospital, this was continued for 6months every 3weeks,in the cancer unit. I suffered a pulmonary embolism during this time , which was treated by 6months of Warfarin, my Rheumatologist then started me o Methotrexate and a reducing regime of prednisilone

  • Sorry. My comment suddenly got transferred by post!! I was explaining about my reducing prednisilone, over the last 5 years I have had a couple of flares, when the dose has gone back up, at present I am on 20mgms of methotrexate, 6 mgms pred.and 75mgms aspirin + other bits and pieces to protect various organs and bones from the effects of the drugs . I keep active as much as possible and generally feel well, I have the odd day when I feel exhausted and under the weather. 5years before my diagnosis I suffered Uvieitis which they now think was retinal vasculitis. I'm sure I have probably suffered some minimal brain damage, but on the whole it hasn't impacted too much on my life. I hope these comments are useful to you , I consider my self very fortunate in being diagnosed so quickly,by my Rheumatologist and treated so promptly.

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