My experience with cerebral (CNS) vasculitis

[vivdunstan]

I have cerebral vasculitis, in a somewhat progressive form, similar in day-to-day symptoms to multiple sclerosis. I have been ill since 1994, when I was just 22. I was initially misdiagnosed with ME. I was finally diagnosed with cerebral vasculitis in 1997. My disease developed somewhat slowly initially, but has become more aggressive and life-threatening since then.

My main symptoms are mobility, bladder incontinence, memory/cognitive problems, fatigue, and losing control of my arms and legs. However I also have problems with vision, hearing, speech, falling over, headaches (though not until a few years ago), sleeping for up to 17 hours day after day, etc.

I have tried lots of treatments over the years. Cyclophosphamide was initially ruled out for fertility concerns - I was very young, and married, and hoped to have children sometime. More recently it has been ruled out again because I have significant (off the urologist's scale) bladder incontinence (including have to wear incontinence pads permanently), and the consultant fears that that symptom could be worsened. So we try other things. Also cerebral vasculitis has few patients, and few examples to draw on, so we can't be sure that treatments that work for other more commonly found vasculitis forms would work in my case, such as biologics and others.

Initially I was on Azathioprine and steroids. That stabilised things, between 1998 and 2004, but I was unable to come off the Azathioprine, though I managed to get my steroids down to 2.5mg every other day. Then I relapsed hugely in May 2004. Since then we've tried adding Ciclosporin, Mycophenolate Mofetil, Methotrexate, and now Thalidomide. I am currently on 4 different immunosuppressive drugs as well as high dose steroids. My consultant says it's as though we're treating me for cancer. But now I've recently started on Thalidomide we hope to get me off the Azathioprine, and hope to get my steroid dose down a little bit in future, all going well.

When I say my disease is progressive I'm not happy about this, but that's how things have gone for me. Every time I try to taper off the steroids, if it fails I get more brain damage and new or worsening disabling symptoms. And those are permanent. So it's hard to know how else to describe it. For example I didn't get the typical vasculitis headache until a few years ago, until after another unsuccessful attempt at tapering. Similarly my excessive sleeping only kicked in over the last 5 years again. Ditto. And that one is worsening in terms of hours slept a day as the years go on.

I can't work with this disease. I worked briefly, for a year, half-time, between Spring 2003 and Spring 2004, just before my big relapse. But I will never work again. When I fell ill I was a full-time computer science PhD student at St Andrews, and had to leave that, unable to put in enough hours as the disease developed. I could never return to that, and can't even write computer code any more. But I studied a history degree with the OU 1998-2000, partly to take my mind off the day-long nausea I got from Azathioprine in the early years of treatment, and then a part-time Masters at Dundee, and in 2010 I completed a part-time history PhD also at Dundee. So that's a big achievement, and I am currently - very slowly - turning my PhD thesis into more academic journal papers, and having lots of fun in the process.

On the downside, and this is a big regret, I have been unable to have children due to this disease and the drugs I've been on since I was 25. Many vasculitis patients are older, or already have had children. I was caught in a trap. But I've had to accept this, even if it's still difficult at times to cope with. I will never be able to come off the immunosuppressive drugs, and am on some stonkers now (Thalidomide obviously, but also Methotrexate which is used for drug abortions), so pregnancy is never going to happen. I'm not sure it even could. My periods stopped completely for 5 years, due to the cytotoxic cocktail I'm on, and anyway I'll never get off those drugs so it's an academic question.

I also have huge problems reading now, which is particularly ironic because my history PhD investigated historic reading habits. These problems have developed since the late 1990s. It's not so much physical problems managing a book, although that's a problem too - I lose control of my hands a lot due to the brain damage. But the bigger problem is concentrating on a page of printed text. It swims, it's too much for me to read for any length. But I have a Kindle now, and whereas a couple of years ago I wasn't reading at all I've so far managed nearly 70 books in 2011, and am a very happy reader again. The Kindle screen is that bit smaller than a printed book, so I find it easier to concentrate on. And I can adjust the font size and spacing to be optimal.

But I'm happy. Maybe it helps that I have huge memory problems, and each day can be like a fresh start. Or rather each evening, by the time I finally wake up after up to 17 hours of sleeping! I take pleasure in the small things. I have a husband (who I married just before the disease struck) who is wonderful, and has stuck by me through thick and thin. So I have a lot to be thankful for, even if there are downsides too.

[vivdunstan Volunteer]

I didn't say much about how I had to fight for my new diagnosis. Often cerebral vasculitis is mistaken for something else - it can mirror many illnesses. I was originally misdiagnosed with ME by my GP in Cupar. But the symptoms changed over the next few years, and became more like MS, including severe bladder incontinence, and other neurological indicators like lots of visual disturbances. But I had to fight for a change of diagnosis. Even when I was finally referred to a hospital consultant he assumed that the ME diagnosis was correct. I had to argue, very clearly, why I thought it was wrong, and why I thought more tests were needed. I also said I feared that I had something that could turn out to be much more dangerous, and needed to be treated properly, and quickly, to prevent more progression/damage. The consultant agreed to run a MRI brain scan, but didn't think it would show anything. Wrong! Multiple lesions in my brain, which should never be there for someone in their early 20s. More tests were then needed to distinguish between MS and other inflammatory diseases of the brain. I didn't have a brain biopsy or an angiogram because they can often give false negatives. But I had many other tests, like a lumbar puncture (mega mega ouch - never again please!), visual evoked potential test (if positive would have pointed towards MS), scans of lots of other organs like heart/lung/kidneys, and a huge number of blood tests. My main tests were conducted on Bonfire Day 1997 and I came home, shortly to be in agony due to a botched lumbar puncture, to the sound of fireworks and smell of bonfires. And I got the official new diagnosis weeks later.

In the early years after my cerebral vasculitis diagnosis my blood tests were all perfectly normal, not showing any signs of inflammation. So we would go purely on my symptoms. This is quite normal for cerebral vasculitis. Then the inflammation markers (PV - sort of like ESR - and CRP) started to follow the disease activity. Now we can usually use them as a guide, but my symptoms are the ultimate guide. I know very quickly when my disease is going badly out of control. I remember well when I went to see my new GP in May 2004 and said "I think the inflammation in my brain is going out of control." He said "You'll be the one to know", which was nice, but up until that point I hadn't had too much experience of what happens when my vasculitis goes very haywire. Years later I've had too much experience of it!

For many years I got monthly blood tests (PV, CRP, LFT, U+E, FBC), but for the last year I've had weekly blood tests, ever since I've been on a quite incredible cocktail of multiple immunosuppression drugs. But that works ok. Hubby and I have a routine now. He works at home that day, takes me for my blood test first thing, we get a nice breakfast in a local coffee shop (lines my stomach for all my morning pills), then a bit of shopping, then home, me back to bed, and him to the computer to work.

Oh and when I ended up in hospital with chickenpox (I never had it as a child) a few weeks after starting my Azathioprine and steroids in 1998 I showed signs of vasculitis in my hands. I was on an anti-viral drip for a week (I was told I only just got to hospital in time - the chickenpox could easily have killed me), and although it started painless initially the blood vessels in my hand where the drip was going in would become inflamed and painful over time = vasculitis. The nurses initially moved the drip around, but then refused to move it any more. So I just had to put up with an awful lot of pain. That's the only time I've ever showed any sign of vasculitis outside my brain.

I probably caught that chickenpox from someone I passed in the street or elsewhere who had shingles - it's easy for people with shingles (usually older people) to infect adults who've never had chickenpox before, especially those on immunosuppression drugs. Thanks to that chickenpox I developed shingles in March 2010, just weeks before my PhD viva. Wasn't sure I'd even be non-infectious in time for the big day. But I managed it, bandaged up my leg well, got comfortable in my manual wheelchair, and got through. But it didn't help viva preparation. Then again it took my mind off getting worried!

[longtimegca]

My first post on this site. V. I am 77 now and my med history is parallel to yours since I was 30. Medics didn't have the tools or knowledge back then and I was diagnosed with atypical MS. Only been diag'd since I showed the syptoms of CGA 6mths ago and since then the jig saw has made a picture. Currently not responding to high steroids except for side effects so starting on cyclo. Hope that works.

[hof-man]

hi viv - i have wegeners granulomatosis since 2007. have had migraines since teenager, now 48. last couple of years migraines more problemmatic, lasting weeks rather than days and more symtpoms than before - now get visual disturbance, numbness everywhere and anywhere and more pronounced global headaches. your story is interesting in that i am fairly sure my disease is progressing despite methotrexate,arava and prednisolone. like u, i know my disease more than the doctors, though it helps that i am a doctor!! i had to retire last year as a GP (family doctor) as the job was too intense to cope with - my symptoms are chronic fatigue to varying degrees, migraines , muscle and joint pains. i still remain active during my better times, even continuing to ski once or twice a year and sail through the summer months but energy is limited and i will pay, usually heavily, for any activities i do. i would value your comments and hope u are doing ok. hof-man

[vivdunstan Volunteer in reply to hof-man]

Thanks for the reply. It does sound as though you are having increasing neurological involvement, so you should be querying this with your doctors. Even if your blood tests are good the disease could be going out of control and need increased treatment. Blood inflammation markers etc. are an imperfect guide and can be normal even when the disease activity is not. Good luck!

My own case is doing a little better at the moment, amazingly. There's an update on my story at

vasculitis.org.uk/living-wi...

It explains the treatments I've tried since the post above, including Thalidomide (caused peripheral neuropathy, so had to stop suddenly), and Cyclophosphamide (didn't seem to help). I may need to go onto a biologic therapy (maybe Tocilizumab), but the rheumatologist wouldn't approve that while I was still on 3 immunosuppression drugs simultaneously (Myco Mof, Azathioprine, and Methotrexate) in addition to steroids. So I had to try to get off one of them first. And I've got off - yay! - the Methotrexate. Currently not having joy lowering my daily steroids below 20mg, but I'm off Metho. Woo! So feeling a bit more positive at the moment.

But, yes, do get your neurologically-sounding things investigated properly. The doctors may not be able to detect anything in scans or bloods, so may have to go on your symptoms as the guide to disease activity. But they should really be doing that anyway.

[hof-man in reply to vivdunstan]

great viv, your story really helps. i have tried rituximab in past, no good. recently my ferritin was up as was my haemoglobin which is not a good indicator apparently!!!! i will let u know how i get on, neuro expert likely needs involved soon, seeing my gp next week. cheers again for your advise and support. hof

[Wellsie]

I'd agree with Viv on ESR and CRP inflammation markers, although I have PAN, not CNS. I relapsed very quickly and it took a long time for it so show in my ESR and CRP bloods, which have forever bamboozled my consulting team. For me, the first sign of trouble is normally the rapid weakening and loss of sensation in a peripheral nerve... but we are all different aren't we

[hof-man in reply to Wellsie]

cheers wellsie. you are spot on. presume pan = polyarteritis nodosa. also presume u are saying u do not get neuro symptoms??? what are your main symptoms? as a doctor i am also interested as i never came across a case of pan in my 24 years as a doctor before retiring last year.

[Wellsie]

Hof-man; lucky for you not seeing a case of PAN is all I can say, it's so hard to diagnose Here's a summary, just message me if you want to know anything more specific, as this isn't the compete list of symptoms and outcomes:

Neurological manifestations rapidly formed (within 24 hours) conduction blocks in peripheral nerves, affecting motor, sensory or both. Confirmed by NVC and EMG tests. Monoeneuropthy multiplex, never polyneuropathy, following a distal to proximal nerve patten.

Unusually, my neuropathic symptoms started in my L ulnar in a block at the elbow (which lead to unnecessary ulnar transposition and decompression surgery, d'oh, it certainly wasn't cubital tunnel). Then I developed a compete block in the L radial about mid humerus. When admitted to hospital with severe arm pain and livido reticularis in both arms my R ulnar nerve collapsed. CRP and ESR spiked through the roof, and there strong evidence of inflammation markers from a lumbar puncture I had. ANCA tests were all -ve. I had lost 3 stone in 4 weeks, and developed acute night fevers, joint pain and intermittent temperatures. I was whacked with a lot of methyl pred and IV-IG with a possible systemic vasculitis diagnosis and discharged 3 weeks later.

4 months later, I relapsed developing leg pain, heavily swollen legs, and several punched-out ulcers on my legs and hands. At the same time I developed rapidly acute ochitis and lower bowel pain and found evidence of internal bleeding in my stools. At the same time, my L sural and peroneal nerves started to experience sensory deficiencies, and all it went downhill very quickly. When I was admitted to hospital there was lots blood and protein in my urine and my kidney function poor. End result = more time in hospital, sural nerve biopsy and 12 cycles of cyclophosphamide and methyl prednisilone, which were successful.

Discharged and placed on Azathioprine as maintenance. Felt better for 6 months, then started to go down hill again last summer, 6 months later. Massive joint pain, night fevers, maliase. Rapid weakening of lower joints, foot drop, loose L hip. NVC and EMG showed SNAP issues and lowered latencies across L and R Peroneal nerves and R sural now too (L sural already cut out). Placed back on chemo and current treatment plan is to continue on chemo once a month until June and then once every 6-weeks until September and then review again. Neurologist, Urologist and Rheumatologist want to be sure that remission sticks before reverting back to maintenance, and then make a decision whether maintenance is Methotrexate, Azathioprine or Mycophenolate.

It's had a massive effect on my life, but I still work, full-time, and will never ever let it beat me. Take care, and look after your WG.

[John_Mills Volunteer]

Hi Hof-man.

Send me a pm or email me at [e-mail].

I'm not sure if we can help you - but at worst, as an ex GP, you might be able to help us!! :0)

(One has to grab these rare opportunities when they come one's way)

Seriously, it's worth pursuing every alternative. As you know, in medicine and healthcare, as in every sphere, there are the average, the good and the superior. My mission is to get the average to become good etc. Yours is to get the very best for Hof-man. There are new drugs in clinical trials - as you will know the numerous mabs are breaking boundaries and there are others, like anti-CD20s, waiting to be explored. There are places where you can get access to the latest.

You do realise that that now you are in the HU community there are 1300 "experts" to advise you!!

Very best wishes

John - and Susan

[Atkins2014]

Hi there, your story is very inspiring. My sister & I are extremely close and she was diagnosed with CNS vasculitis 1 year ago. So many similarities with my sister's experience. She is just about to be medically discharged from her Employer after working all her life as she is too sick to continue. We are all filled with worry this week as her Neurologist thinks she may be having a flare up of her CNS vasculitis. Have read your story out to family members and will read it my sister also. Many Thanks. Hannah.

[vivdunstan Volunteer in reply to Atkins2014]

Thanks for the kind comments. There's a slightly updated version of my story at vasculitis.org.uk/living-wi...

I had Cyclophosphamide infusions in 2012, which didn't seem to work, but may have helped after all. I've been able to come off my Methotrexate, and am currently trying to lower my steroids a bit.

It's now nearly 20 years since I first fell ill, and I'm still here, and still hopeful. So I hope that may be encouraging for you and your family to hear.

[anthony01]

Hi Viv

Very interesting reading about your symptoms as I am P - Anca and now there is a query???? of Cerebral Vasculitis ??? but that is it. I suffer with minor forms of strokes and recently suffered with one and all symptoms of a stroke but they do the Scans and do not see any blood clots so literally laugh it off! and got move from Atkinson Morley to Kingston Hospital. Atkinson Morley doctor said it was not Cerebral vasculitis as I did not react?? like a Cerebral Vasculitis patient? ( so your information is interesting as there seems to be a mood?? that one gets into?? is this true from your experience?) since this last stroke I have become a great deal weaker on my right side, although starting to walk again and have physio coming in. As they can not find a blood clot it seems to write out it is a stroke? I have chronic tiredness and body clock completely twisted. I find remembering things terribly hard and can watch something on TV like a soap and have no idea or remember names. My partner is fantastic and copes with me as I am. The hospital is going to send me to someone who is to work out why my brain flips into a stroke form? So it has being interesting reading your experience and sharing it. I am on Rituximab which was working a treat and then flipped??

[vivdunstan Volunteer in reply to anthony01]

It's difficult for me to comment on the "mood" thing, though I know when I'm not right. I have a huge range of neurological symptoms, and some, like extremely severe bladder incontinence, are very difficult for medics to brush aside that. Having said that, I recently saw a neurologist for a second/third opinion, and he could find no trace of active illness, which could be a result of the high dose Cyclophosphamide infusions of 2012 being effective after all, IMHO, albeit belatedly. Though I also asked him if my ongoing cocktail of immunosuppression might be affecting the results: he didn't respond to that at all. But my main consultant who made the original diagnosis in 1997 is happy with my diagnosis, and continuing to look after me. We have been able to reduce some of my immunosuppression drugs (down from 4 simultaneously to 3 now), and I'm trying to lower my steroids further now, very very slowly and cautiously.

I think the biggest problem with cerebral vasculitis is that it is so rare, and few specialists will have seen it. Where are you being seen? If you're in the UK then Cambridge and Bristol would probably be the specialist centres to go to for cerebral vasculitis care - Neil Scolding at Bristol is particularly experienced with this form of vasculitis. The risk with seeing a general neurologist etc. is that they are unlikely to have seen enough cases like yours, if any at all, and may not know what to do well enough, and could dismiss you, without you having adequate treatment.

[anthony01]

Wow thanks Viv, interesting. I do feel there is lack of knowledge and shocked when I said it to the Neurologist could it be Cerebral Vasculitis "No, you have got the moods and temperament ?? that's why i wondered how he knew. On my records show Possible Cerebral Vasculitis, so that is why I asked? As they do not see it as a stroke, they are sending me to a form of Neurologist Phycatrists who will put me through mental tests and discover what it is? (well that is what it feels like and sounded like) My concern is how do I manage the form of stroke? and that is what I am asking them for?? as it is a waste of time going to A&E. I am with St.Georges in London and have a good Vasculitis Consultant but the Neurologist I have never being certain with!? thanks again and hope we both resolve our mysteries!!?

[lucierna]

Hi To; whom it my concern..I've been diagnosed 4 a Selebral Vascular Diseases ..CNS.Vasculities..started one morning 2004 i have 5 kids one girl 4 boys at the time they're were very little and they were at the living room playing Playstation and i was looking 4 my robe towell 2 take a bath but i decided 2 sit 4 little bit and then in seconds i said 2 my kids ok be good boys ima go and take a bath so wen i got up tne chair i felted like if i was poses by a weid thing got inside my body felted heavy and i said 2 my kids i can't see i heard one of my kid said what's you mean you can't see and another approach towards me but i cant only see one side totally black the other side was wrinkle like a monster and then another brother i heard telling him reach to the phone call police then i try 2 reach the door and call 4 help and said help me pls help me pls and then i step back but i was stiff and the super A x housband and wife came up and said what happens and told me 2 sit but i couldn't i felt like iam a statue one of my kids try 2 put my sandals and said i cant lift your leg is hard mom is like glue to the floor and that's it that's all i remember at the hospital a Dr were tapping my sholder calling my last name 3 time mrs muñiz i want it 2 talk but i couldn't my tongue was so hard but telepathy in my mind i was mumbling try 4 him 2 understand what i wanted 2 said like what's happened 2 me he seem like understand and said mrs muniz 3 time you have a stroke but it was my first time at the time i was 38 and i didn't understand 4 me wen inside my ear and left through the other ear i dindt have understanding or knowledge about the sickness and i slept again end up at intesivecare unit.. ICU for 4 months will i was there i have experience that i was flying but i was like a cross my head up it was a bright light powerful and i talk 2 that light and i speak more Spanish but in English i said and what about my kids it was so peaceful no pain i felt it was so nice i saw little flowers green weird green grass then like i felt a weird sorceress a power magnetic pushing me down in seconds i had to started all over again lost memories recovered but not %100% learn 2 talk walk write took long but yeah i was at reabilitation and member of fam told me my eye went back i was no wonder Dr said no mirrors 4 me no were it was a night mare its easier to said but living this weird experience humm i started 2 have panic attacks and anxiety depressed do to the stroke i have 5 stroke 2008 living in a shelter i have a nother stroke make it short 4 months ago i have 2 min stroke and lost little memories i sturter talking i have now to much pain all over ones a moth i get a weird pain is like a volcano in one side of my head like vapor come out there is inespecable my joint hurt more my hands like i i have al tritis if i sit 2 long is bad if i laying down 2 much is bad i walk cus i love walking but wen i get home uff.. you don't have an idea how painful its is my bones cracked hips arm legs every time i move you hear my bone crak but i dont know how iam dealing with this iam losses my hair i have a angiogram orthe test like catheter i have 4 catheter blood passing so the one at the head is a thin wire but to see my head i saw like a tree branch and blood clot big med and small i call it Jupiter Mars pluton ect ect ..that how they foud out i have cns v never told my story up 2 now ..

[Melaniejoye in reply to lucierna]

Don't what the hell you experienced but that was NOT vacsulitis!!!!

[Melaniejoye]

I was 51 and had the first stroke in 2011. I have had migraines since age 26. After the first stroke I suffered multiple TIA's over the last 4 years. I ended up in the ED about every 6-8 weeks. They diagnosed me with either complex migraine or TIA. In 2015 I had another mild stroke. I knew I something was really wrong. I was of normal weight and healthy. They tested me for everything all came back normal. I went in the search for a new doctor. I found one and he went to work. The best neurologist in my area. Almost exactly one year later I had another stroke and then 2 more back to back. He brought in a rheumatologist who specializes in vacsulitis. I had a brain angiogram in Dec and and then a brain biopsy in Jan. They put me a high dose of prednisone. I was on 60mg to start. They were trying to wean me down but it seems I just had another episode. My advice is don't stop loooking for answers. This disease can look like a lot of different things. Also if the doctor you are seeing seems clueless find another one. Fast! The first doctor told me I just wasn't "walking enough". Seriously? There was nothing else wrong with me. Good luck to you all, and God bless.

[Melaniejoye]

Guess no one is on this sight anymore. I just realized it's been 3 years since someone posted.

[sorefeetsoldier in reply to Melaniejoye]

Hi Melanie

Im here and i just read your post.

Cant believe Doc said your not walking enough. When i went to A&E many times before being diagnosed i was told i had man flu!!!!

Keep posting

S