Cerebral Vasculitis (mis)diagnosis/RCVS/vasospasms

Early Tuesday morning on April 28th, 2015 I woke up at 3:30am with an extremely painful migraine that I have come to associate as “thunderclap headache.” I had blurred vision, sweats, and a pounding in my head that was unlike anything I had ever experienced in my life. It was so bad my first thought was that I was having an embolism or heart attack. I woke up my roommate and pleaded with her to take me to the emergency room.

We arrived at Imperial Point Hospital in a panic and were not received with the same urgency as we had hoped. I was in so much pain that I was hysterical, my blood pressure was extremely high, and I kept demanding that someone please help me. I was disoriented and felt as if I was having a seizure. My friends were pretty shaken up and it was clear the nurses and doctors thought I was either on drugs or detoxing from drugs. Understandable, as my symptoms of vomiting (by this time I was very nauseous and had been sick into a bucket a few times) screaming, crying, etc. probably mimicked that of a drug user. The nurse was less than sympathetic and seemed more annoyed than anything about my condition.

After about 4.5 hours I was finally admitted to the hospital and given pain medication. (They gave me dilaudid, morphine, and toradol.) Surprisingly, toradol was the only thing that made the pain remotely subside. (I would later find out toradol is used as an anti-inflammatory.) I was seen by a neurologist and a Dr. but don’t remember much of the experience since I was sedated. I was given a lumbar puncture and a CT scan. I was discharged the next day and given the diagnosis of a severe migraine. I left the hospital with an aching head and extreme confusion. I had just experienced one of the most bizarre episodes of my life. I was given a prescription to two different types of muscle relaxers and told to “take it easy.”

I returned to work the next day May 30th (for half a day) and felt extremely confused/disoriented/weak and uneasy. I went directly home after work and slept until the next day, head pounding. My family, being extremely concerned, told me to come to their house so they could monitor the progress. So, I packed a bag and went to my parent’s house on Friday evening, May 1st. I went to bed early, complaining of head and neck pain. I would wake up later at approximately 1:30am with the same symptoms of thunderclap headache, crying hysterically and pleading for help.

My stepmom rushed me to the emergency room at Holy Cross Hospital in a panic. I had the same symptoms of vomiting, hysteria, high blood pressure, disorientation, and confusion. Once again, the staff was less than urgent and treated me once again as if I was a drug addict/user. I was put into a room and my stepmom was almost arrested for pleading with the staff to treat the situation with more urgency.

Once again, I can’t recall much except for the extreme pain and the seizure-like distortion of my body. I was constantly rolling around on the table looking for a comfortable position for my neck and head to rest.

I was finally admitted to the hospital and given a room, where the symptoms of vomiting and hysteria continued. A neurologist came to visit me, where they once again treated me with pain medication, while I screamed for the torodol that I knew would be the only relief. I was given a shot in the stomach (which the neurologist would later admit made the condition worse) and released the next day.

I went home, still disoriented, confused, nauseous, and hysterical. I wandered around my house crying, seeking relief and screaming in pain for help. My parents were dumbfounded as to how this could be just a migraine. On Sunday they were so overwhelmed with my condition they took me back to the ER for A THIRD TIME in the same week.

However, this time it was different. My father made a phone call to alert the doctors I would be returning. (We have a strong connection at the hospital and he was hoping they could expedite the ER process this time.) Thankfully, when I arrived at the ER they were more receptive and didn’t treat me as they had previously. I was seen immediately by three doctors and given pain medication to relax me. Once again, I hardly remember any of this- but I do remember my blood pressure (which is usually normal) hit 190/91. At that point it was clear this was not your average migraine.

One of the doctors recommended I have an angiogram. So, I was put onto a stainless steel table and told that they would take a camera through my groin and into my artery to look at the activity in my neck. When I heard the Dr. say, “We have a diagnosis…” it seemed like music to my ears. Finally, someone was agreeing that this was not just a migraine.

When we were briefed on the results of the angiogram it was revealed that my arteries were extremely narrowed and restricted. I was told this was common with cerebral vasculitis. I was admitted to the critical care unit and put on a slew of different medications, such as celebrex and nimodipine. They also started me on high doses of steroids to treat cerebral vasculitis. They were trying to fight both RCVS and cerebral vasculitis, at the same time.

I stayed in CCU for about 6 days. I had another lumbar puncture (which showed elevated protein) and an MRI. (At first observation of the MRI no one noticed the restriction of arteries, until they saw the angiogram and went back to look at it a 2nd time.) At such a small community hospital, the doctors were baffled and clearly out of their element. They didn’t have a “team” to deal with this and we soon found out the politics of medicine. No one wanted to step on anyone’s toes and no one really knew much about the condition. A rheumatologist explained that my blood test results didn’t show infectious diseases or any abnormalities (except an elevated white blood cell count) but it would be smart to see a specialist. (She recommended Cleveland Clinic in Ohio.) She was confident my symptoms were more similar to that of RCVS. I had one other episode of “thunderclap headache” while in CCU, immediately following a nauseous spell where I began to vomit, my knees buckled, and I went into a fit of seizure-like symptoms. I begged for the toradol, as I knew it would be the only thing to give me real relief.

I was transferred out of CCU on May 7th and moved to another room and considered stabilized. I was told I would be discharged the next day, May 8th if no other episodes occurred.

When May 8th came around I was very excited to finally leave the hospital and continue on with my life. (And more importantly, find a specialist.) But here I sit, May 9th, unable to leave because they are unable to find my medication anywhere in the country. (Apparently, when you have a rare condition, you also have rare medication.) Nimodipine can only be immediately found in Mexico and Canada, leading me to develop conspiracy theories about the monopolization of the pharmaceutical industry. (But I digress…)

After 3 trips to the ER in one week, being misdiagnosed and treated like a drug addict I always kept a positive attitude. I smiled at my nurses, told them jokes, and was pleasant and polite to everyone in the hospital. But my patience is wearing thin. My doctors seem to be clueless as to whether this is in fact cerebral vasculitis, a life-threatening condition, or RCVS, something that can be resolved in 1-3 months and never return. I understand being at a community hospital has been a huge disadvantage. I should be at Jackson Memorial or a center where the doctors are more familiar with these conditions. But as of now I’m not even allowed to be discharged until they can fill this prescription.

I wanted to share this story with you because I am very scared and confused as to what to do from here. Everyone agrees it’s best to seek the opinion of a specialist at Cleveland Clinic or a larger center… Which I plan on doing first thing when I am able to leave here. But any other advice or insight into cerebral vasculitis/RCVS would be greatly appreciated.

Some things I didn’t mention above:

I am 27 years old and have never had ANY health problems in my life. Except, March 27th, 2015 I developed a staph infection in my armpit (likely from shaving) and had the abscess removed. I am curious as to whether this could have played a role in developing vasculitis.

I am also an avid scuba diver, softball player, and previous gymnast. I have put a lot of stress on my body over the years.

Since 2008 I have traveled to approximately 30 countries as an English teacher. (Many of them 3rd world…)

I had another MRI 2 days ago that showed my vasospasms are still there but doctors have not been able to elaborate much about anything—adding to my frustration.

I would be happy to provide any other information regarding details, medications, examinations, etc.

Thank you.

19 Replies

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  • I am so sorry to hear about your situation. I thought we had problems here in the UK with poor professional care. Here they say that white caucasian (Nordic) genes predominate in GCA. What steroid dose are you on? What is your ESR, CRP? I would not be without my asprin, also an anti-inflammatory that doctors here often don't bother prescribing despite its low cost. Hope you get the transfer soon to a vasculitis specialist. Rheumatologists, in my experience, are trained in keeping joints moving but are not trained to treat very serious medical emergency conditions, such as vessels which supply the brain with its blood supply.

  • Very useful thread and posts. "Here they say that white caucasian (Nordic) genes predominate in GCA"

    I hear this all the time. This could well be another medical myth similar to: " you won't get it because you aren't 80 years old yet". "you won't get it because you are still in your Fifties" Distorted belief system which drive drs to continue dismissing patients from getting diagnosed.

    Friends live in East Asia (Japan, Hong Kong, Singapore..) I have been told they have "GCA" over there as well. I googled and there's loads on GCA in Japanese language. I haven't looked in Korean language yet.

    People living in Phillipines are more prone to autoimmune diseases (1 in six suffering from SLE plus other comorbid autoimmune conditions RA, AS etc which are extremely common in Far east.) Knowing other autoimmune diseases are common in Asia, it makes sense GCA isn't a special disease only people from a single race would develop. "GCA" may not be a specific disease..Like Lupus, it could be a syndrome where people have similar "GCA" presentations but not completely the same (different gene influences would be likely to be at work?). Knowing the human beings of any race /gender / age could develop/contract similar diseases similarly, I doubt GCA is anything different. Drs shouldn't keep using this "tiny box" to expect all their patients to fit into them (causing the condition to remain "SO RARE"... People simply don't get diagnosis and sometimes people die as a result. There must be similar underlying etiologies which give rise to this condition. Guidelines should be used as a "guide" not the "absolute".

    I'm sure another 10 years, there are more articles "wait, no it wasn't just Nordic disease"..

  • Hi Ferntree, more elderly people get GCA than young people but middle age is a worrying time to get it and it is so inconvenient to someone with an active life. I think not enough emphasis is placed on the younger patient. Here they say 'Nordic' types get GCA presumably because the Vikings did a massive amount of travelling in their boats, over oceans and through rivers so a very large catchment for the 'genetic' hypothesis which is very convenient. I do wish they would do a little more research. Unfortunately, research is mostly funded by drug companies who are interested in finding a new drug to make some money for more research. Two things have been puzzling me. A few years ago I had a very strange mucous problem in my chest which I insisted was analysed. They only came back with an infection 'seen in children'. However, I had just adopted an abandoned dog who also had a cough and wondered whether he had picked up something in his previous home or in the rehoming centre. I wondered if it had affected my immune system which has never been especially good so I avoid chemicals where I can. I have also wondered whether stress affects it. It seems to. I'm putting my faith in spiritual healing now because nothing has been helpful so far.

    Definitely try to see a vasulitis specialist. If you compare online articles from rheumatology centres and that of the vasculitis sites, definitely its the vasculitis people who are far more interested than any of the others. Ask for a printout of your test results. My doctors overlooked a stack of stuff on my test results.

  • WTMuk

    "Hi Ferntree, more elderly people get GCA than young people but middle age is a worrying time to get it and it is so inconvenient to someone with an active life. I think not enough emphasis is placed on the younger patient."

    Thank you for sharing your intriguing experience and helpful thought process..Much appreciated. I bet you're right. It seems to be a major and recurrent issue which are often discussed over the forums (e.g. difficulties to get the type of attention from Rheuy drs for GCA if you are slightly "younger" 40s/50s) . I believe this is something both Vasculitis UK and GCA charity could try to "address" perhaps..? Not an easy task, I'm sure. The problem is, this condition would /might cause some nasty organ compromise a few years down the line. It's very helpful of you to share your thoughts on this.

    Take care and thanks again.

  • I don't know much about cerebral vasculitis specifically, but I do know that infections, such as the staph you had, are suspected to be a trigger of vasculitis. It sounds like you've been to hell and back. I have leukocytoclastic vasculitis, so far cutaneous, but possibly damaged my ear also. If I were you, I would not wait to contact someone at Cleveland Clinic and tell them what you've been through. Beg, if you have to. You need to talk to a specialist in vasculitis, even if it's over the phone. Don't be afraid to be a squeaky wheel. This is your life we're talking about. I will pray for you to get some answers. Hugs.

  • Sorry to hear of your troubles. I have also been diagnosed with CNSV. Fortunately mine is low grade (mild). Like you, my diagnosis comes mainly from symptoms rather than test results. The reason for this is that a confirmed diagnosis of CNVS involves a brain biopsy (and even then they can miss it if they don't sample the correct place). In my case it is the symptoms combined with an abnormal MRI together with a LACK of positive tests and the fact that the correct meds deal with my 'flares' that have led my neurologist to his diagnosis. I agree with him - why biopsy when we have a solution!

    It is odd (and sometimes frustrating) not to be 100% sure but this site helps through both its support and the ability to see other alternative diagnosis to discuss with him if they might apply.

    You haven't mentioned any other symptoms other than the migraines. Everyone is different - Besides the migraines, I get pain, pins and needles and numbness in my arms and legs, also general fatigue. These symptoms 'flare up' when I am stressed, hormonal or have an infection, otherwise they tend to build up over a period of about 6 months. I also find it gets worse if I can't do things at my own pace. Exercise actually seems to help. Prednisolone deals with the flares.

    The 'route map' will help you find more info. I am sure someone will post a link - I am in a rush atm (Makes mental note to slow down!)

    I suggest that you monitor and record the pattern of your symptoms and your general health at the time so that when you see a doctor you are 'armed' with the correct info.

    Taking control of your situation helps.

    Hope that helps,

    Mel

  • Jennabug

    "I am also an avid scuba diver, softball player, and previous gymnast. I have put a lot of stress on my body over the years.

    Since 2008 I have traveled to approximately 30 countries as an English teacher. (Many of them 3rd world…)"

    You make excellent observations and thank you for your helpful thread. I'm sorry to read your hollowing experience.

    Have you had the viral /fungus / bacteria screens yet? I have seen a few articles re. different types of vasculitis arising from the autoimmune reactions to "common bugs"..are you possibly suggesting that you might have picked up some tropical disease? You say, you had some invasive surgical procedure..Could this surgery trigger the possible dissemination of the bacteria via blood vessels? Hope you would be discharged very soon and your medications sorted out quickly. Take care

  • My experience of the fruition of Primary Systemic Vascilitis of the Central nervous System goes back to 1997, I say fruition because like a dripping tap I had in fact been subjected to changes in health over the years that I just accepted as part of life and went by almost unnoticed that is until it all became so dramatically different.

    Can I ask you to google the question, 'Systemic vascilitis or not? That is the question' the article by Dr R Powell and a Greek Doctor who I call Vlassis relates to a 49yr old man, myself. It highlights the problems I faced and the medication prescribed at that time.

    No doubt medical procedures have moved on a bit but it is an exceptionally rare condition perhaps by reading of my problem it will give you thoughts as to the Doctors you should be seeing.

    Wish you well. I am now in my 67th year.

    I use to be able to access this the web page quite easy at one time but as their are more articles on the subject it does not quite pop up so fast.

  • EOS7D

    "Can I ask you to google the question, 'Systemic vascilitis or not? That is the question' the article by Dr R Powell and a Greek Doctor who I call Vlassis relates to a 49yr old man, myself. It highlights the problems I faced and the medication prescribed at that time."

    Thank you for sharing. Could anyone provide the link at all, please? I checked with google scholar but am unable to find the article (e.g. fogginess in my brain). Thank you so much in advance.

  • ncbi.nlm.nih.gov

    Sorry about brief reply but I am on move with tablet.

    Trust the above will get you in but please reply either way.

    Best wishes.

  • Sorry, no luck...but thank you all the same.

    Kind Regards,

  • Which area of the cranial artery is most affected jennabug? They often suggest a biopsy of the temporal artery if there is pain in the temples. This way they can look for Giant Cells. I refused the investigatory surgery so I was diagnosed with 'arteritis' plain and simple inflammation of the arteries but without evidence of giant cells!

  • "I was diagnosed with 'arteritis' plain and simple inflammation of the arteries but without evidence of giant cells"

    I hear you do not need "giant cells" to "qualify" for "GCA". I bet there are more than one type...the same treatment anyway.

    Good question..you get any arteries inflamed with "GCA" not just above the neck yes? (I expect inflammation travels everywhere)

  • AllyGY2013

    You are so wonderful - thank you so much xox

  • I aim to please - glad I could help!

  • You're so kind...have an enjoyable evening :-)

  • Oh my gosh - I feel so awful for you. What about Takayasu's? Have any doctors mentioned this? Your age and gender fit the usual TAK patient. How about starting you on some prednisone? Toradol is working and is anti-inflammatory and prednisone is something used to lower inflammation, I wonder if it could help. Please update. Do NOT let the doctors neglect you. Make them give you EVERY test until they figure it out.

  • I am having the same symptoms. RCVS Reversible Cranial Vasoconstriction Syndrome. There is a drug they used on me. Verapamil. Hope this helps. I am not on these forums usually. email connie@irwinandco.com may 21 2016

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