CNS Lupus or hemiplegic migraines?? Any experience/advice please!

Hi, I wonder if anyone has any experience of neurological issues from lupus and can help me decide which consultant to believe please as their treatment plans are different!

I have had lupus for about 6 years with various manifestations, approx. 2 or 3 flares a year, some more serious like neutropenic sepsis and pericarditis and some with sore joints/fevers/fatigue and 3 with neuro issues like this current attack just less severe.

In May I became very unwell with confusion, difficultly walking and talking, uncoordinated, dizzy and severe lethargy. The GP gave me a steroid injection and then I've had 2 doses of tapered steroids since which have an enormous impact on my energy and relief of symptoms. The really severe symptoms have lessened but still very fatigued and pins and needles in various limbs a lot of the time especially at night. My left side is weaker and my left hand still not right. My left side of the face was numb and I started having problems swallowing and choking so went to A&E a few weeks ago where I saw many Drs and consultants who said the following:

A&E Dr - (very young but very thorough) - you have vasculitis (admitted me)

Hospital general Dr - Your blood tests are normal, you've been here with a few different things in different parts of your body and your ESR is always normal (think he was implying I was a hyperchondriac!)

Rheumy registrar - it seems a very neurological attack, I'm handing you to neuro.

Neurologist registrar - your body is just weak, your ANA isn't even positive today so I don't think you even have lupus (I explained I'd just stopped high dose steroids and that can happen) - you have fibromyalgia, you just need to motivate yourself to get stronger and should stop taking steroids like smarties. Nice guy...obviously I have ignored him in my decision making because he was that terrible combination of arrogant and ignorant!

Neurologist consultant - you have hemiplegic migraine and you have become 'stuck' in a constant chronic migraine for 3 months. There is no treatment (I have had hemiplegic migraines before but for hours rather than months and I had none of the normal flashing lights or awful headache this time)

So I then had an appointment with my rheumatology consultant (different hospital) who said - you have CNS lupus and must start immunosuppressents immediately as it is life threatening and why did you stop the steroids and why didn't the hospital MRI and lumbar puncture you?? You need to take 40mg a day for at least 6 months.

So I don't know what to think!! I hate medication but I also hate having no energy to do anything, cognitive problems, keeping on falling over, my kids saying I've been 'weird' since May etc. The rheumy was adamant that I had no choice so I've just started all his drugs but what if the neurologist is right and it's a paralysing form of constant migraine and all these drugs are unnecessary and harmful?!!

Has anyone had anything similar please? Any suggestions? Thank you!

14 Replies

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  • Hi

    Do you have any Antiphospholipid antibodies? I have all three and apparently they are causing my nuerologic issues rather than my lupus. They can't tell if it's blood flow are the antibodies attacking brain waiting on more tests. (MRI was normal, PET scan abnormal)

    L x

  • Did your rheum go ahead and arrange an MRI and lumbar puncture? Hope so but if not perhaps contact his secretary and ask for it if you are unsure about the meds. I have had a few hemiplegic migraines that lasted from1-3 days. They are very scary and I feel for you. So so confusing with so many opinions. For what it is worth I was diagnosed with cons involvement and given more steroids

  • Thanks Bacardibabe (love the name!) , no I don't have the antiphospholipid antibodies although I do know they can cause neuro problems - sorry you are affected this way :( . The rheumy thinks mine is more the blood vessels and nerves in my CNS and PNS being inflamed but hard to definitively diagnosis.

    Thanks Rolybear - yes the hemiplegic migraines are scary - especially the first one! Although I'm not so bothered now I know what they are and they always follow the same pattern (and stop within a day or two). This has felt quite different though although still left sided weakness and pins and needles but much longer term cognitive difficulties and falling over. The rheumy has written to the other hospital and said to do the tests when I'm in an acute flare rather than wait 3 months on a waiting list when you can then see very little but that will crossover with their letter to him saying I don't need these tests because they think it's chronic hemiplegic migraine! Although I'm thinking that the two are probably caused by the same thing. I'm seeing another specialist next week so hopefully she'll be on one side or another (or maybe have a completely different idea!) but I've started the mycophenolate and high steroids because I can't seem to get rid of this on my own.

    x

  • So who do you feel you have the most faith in? (Obviously discount the moron registrar!!!) Sometimes we have a gut feeling?

    What medication have you been on for lupus? Hydroxy?

    Presumably you also have a diagnosis of these migraines alongside your lupus diagnosis?

    Did the steroids help? If so doesn't it point to something inflammatory and not a migraine?

    Sorry for all the questions but sometimes I find it helps to bounce ideas! 🤗

  • Well I suppose I have most faith in the rheumy because I gave them all a diary of the symptoms (because my memory's so bad!) etc since May and he read it thoroughly, asked questions, examined me and said he'd seen it all before with CNS lupus. He was nice but very firm and authoritative and said I really had no choice and must do as he says. Normally I like to be active in my healthcare and feel I have a say but in a way it was quite nice to be told what to do when it all seems so unclear, I've felt rubbish for ages and my brain doesn't feel competent at the moment to make decisions myself. Even though he was quite brutal with the statistics and risks he made me quite 'safe' if that's not a strange thing to say? My previous rheumy (who was perfect but moved hospitals) had told me last visit that she wanted to try the next stage drugs soon so it wasn't entirely unexpected.

    However, the A&E Dr was also really good and convinced it was vasculitis but he was very new and maybe he spent so long examining and questioning me because I was the first person he'd seen with lupus - he was clearly interested in everything, spent ages looking at the little red spots on my face and shins that were probably just bites, rashes or spots! The neuro consultant was obviously very experienced in his field and I do have a diagnosis of hemiplegic migraines too but he didn't really read any of the symptoms and didn't listen to me saying that the steroids had helped - just insisted they wouldn't help. It felt a little like he was fobbing me off because he didn't offer any treatment, advice or follow up. I didn't feel many of the normal migraine symptoms either and I have a very specific pattern but I suppose a chronic version may be different. He also admitted he didn't know much about lupus and I should get advice from the rheumy's.

    I looked up migraines, CNS lupus, lupus vasculitis and other people's experiences and I can't decide myself really as I have a lot of the symptoms and many of the symptoms are shared anyway. I also found an old discharge letter from another hospital from when I had neutropenic sepsis and it said 'autoimmune mediated attack on bone marrow related to her ANCA positive SLE'. I was too sick to research it at the time and my Drs never received a copy but from then looking up vasculitis it talks about ANCA. The neurologist said it must just be a typo and laughed and said 'you can't get ANCA +ve SLE, they must mean ANA' and I suppose he is right (but I forgot to ask the rheumy to clarify!)

    Yes, I've been on hydroxy for years now and it's certainly helped with the fatigue, nose ulcers, chest pains etc although the fatigue has been dreadful in this flare - every set of stairs feels like a mountain!

    I already feel so much better with 3 days of high dose steroids, the pins and needles are much less, my brain feels clearer, balance better and my body has some (limited!) energy. The mycophenolate will obviously take longer but I read another ladies report and she said after 3 months it gave her back the brain she had pre-lupus - I can't imagine what that would feel like as I've just accepted my lupus fog brain which is bad even when not in a flare.

    I expect all the conditions are quite linked (certainly migraine and CNS lupus) and maybe they're all correct, well except nasty neuro but he's getting an email about the danger of arrogance and ignorance which I'm looking forward to writing ;)

    Thanks for your help, it does help to think it though with other people who understand!

  • I have to say it sounds like lupus and not a migraine to me. The other thing to remember is that you've had multiple rheumis say and treat you for lupus and they have specialized training in this field that is unpredictable and complicated. The A and E dr doesn't have that training and won't understand the conditions as well. And the fact that you are better after taking the steroids tells me it's lupus and not a migraine. I have sle and it took me a long time and several rheumis to find a great one that actually helped me. All of them agreed that my symptoms showed sle but my ANA showed negative. The first rheumi said it seems like lupus but I won't treat you with a negative ANA, go back to your dr have her keep doing steroids tapers when you have problems and NSAIDs and what not. Then I got worse because I wasn't being treated. Went to the next rheumi who he a lot more experience and he said that it was definitely sle and that while it is rare yes it is possible to have sle and a negative ANA. In fact if you look up the lupus website and all the other medical websites about it the list percentages of people that have sle with a negative ANA. It is a very old school teaching that you can't have sle if your ANA is negative and that there are many healthy people with a positive ANA. It can mean something and is kind of s guide toward a autoimmune condition but can also mean nothing. All the medical sources clearly state that there isn't one test that can diagnosis sle. That is a combination of tests and symptoms that a rheumatologist then diagnosis you off of. That's another thing that is frustrating to me is when drs that aren't specialist try and tell you about your sle when technically they aren't even qualified to diagnosis or treat autoimmune condtions. So the 2nd rheumi started treating me but was 3 hrs away and as he got close to retired would tell me to go see my local dr when I was having problems. Then my loca dr and her supervising physician started treating me under the rheumatology department of the big state hospital we have. I'm in the US so I know our system work differently. They put me plaquenil which I should have been started on way sooner and wanted me to make the jump to immune supressents like methotrexate. I was hesitant about this but because of lack of serious treatment my sle kept progressing. Plus just to fully disclose I'm told my sle is severe and kind of weird because I also have a blood condition and other overlapping disorders. They tell me that is part of why my labs are strange and why it's so hard to control my auto immune issues with them attacking my body in more ways than us even usual for sle. Anyways thru this time my primary Dr and her supervisor where researching for the right rheumatologist that they felt would know enough that to help me. I finally got into him and he immedietly put me in Benlysta infusions. He is the first one that has really helped me after being with him for several years now has slowed the disease progression. I honestly don't think I would still be alive if it wasn't for my current medical team. I'm on Benlysta infusions, weekly mtx injections, and still haven't been able to get off steroids without flaring. I'm just sharing some background so you can see how sometimes even the specialist don't know what to do and you have to keep looking for the right one for you so that your disease isn't allowed to just keep getting worse. All the things you talk about with the cognitive disorders, pine and needles, falling down, nose ulcers, skin rashes, etc really sound like CNS lupus to me as I get all of this whenever I flare. Since being on my treatments my symptoms have become better but some are still there but less persistent but at least when I'm not flaring I have my mind back and am me again. I hope you find some answers and help. I just wanted to encourage you that it's normal to get opinions like this from other drs but since the steroids are helping it makes me believe its auto immune and you should trust your rheumatologist. He has more information on both you and the disease then the other drs you have seen plus you have your old rheumi who also thought you should start immune suppressive treatment. I am so thankful that I finally did as it has helped me so much. I even look forward to my infusions and injections now as I know I will feel better once they start working again. Yes they are horrible drugs that can be dangerous but drs only prescribe them if they beilive that your disease is more dangerous then the drugs. They are trained to weight the benefits and the risk reward factors. They wouldn't put you on immune suppression if they didn't feel your sle was life threatening. Just some info to hopefully help you make and decision. Hang in there. :)

  • Thanks very much, that is very helpful to see other people's experience and so pleased your drugs are working for you. I have heard about the Benlysta but think US is ahead of the UK on that one maybe? Yes my ANA was initially negative for 2 years even when the rest of my symptoms were starting to point to lupus. It was then usually positive the last few years but tends to go negative when I'm on high steroids. Who knows? It's a strange disease!

    Yes I now feel quite confident (if not happy with) the diagnosis. I really trust my old rheumy and she also knows my new one and says she highly recommends him and they both said the same so think I have to go with their suggested drug regime and see if it works. Glad you get your brain back between flares - I can't imagine what it must be like to remember things and not lose your car in the car park! I will have so much extra time that currently I spend looking for things I've forgotten or lost...

    Best wishes and thanks for the advice!

  • Hello Melba1.

    Your post interests me and I'm sorry that I have taken so long to reply.

    I am in the process of getting my CNS symptoms investigated. But I do not think I have CNS lupus. But I think I have Lupus with CNS involvement. I am not sure that my experience will help or not. But just in case, here's my story so far.

    I am 43 years old. I have SLE, osteoarthritis hips knees and back, various allergies, back ground hypermobiity, varicose veins since aged 17/18, headaches since early teens.

    I have wondered whether I have vasculitis at times but Dr Kaul at London Bridge Lupus Centre ruled this out at my first appointment with him in April this year.

    My GP has told me that I have SLE with CNS involvement. This scared me when she said it, as no one else has been that honest or frank with me. But when I really thought about it, it shouldn't have been a surprise for me, as it made sense. I get bad headaches/migraines/cluster mygraine/migraine with myalgia etc, brain fog, I am often short tempered and my moods can swing - with no explanation and I wonder myself why I am so angry, I have also suffered bursitis in my hip and forms of tendonitis in my wrists and ankles - which I think can be symptoms of nervous system involvement. I am definitely not as physically strong as I used to be, possible muscle weakness. Plus I have 2 areas of inflammation on my spinal cord, which I have been told I was probably born like, although I have to say that I am not 100% convinced and also white matter lesions - high signal - which could be migraine on my brain MRI. These are being investigated. I have still not been referred to a Neurologist.

    My brain fog has been quite bad during flares. On one occasion I couldn't remember my age, so think to myself, I'll work it out from my date of birth, but then couldn't remember the year I was born! I was so scared. I asked my kids what my date of birth was and how old I was, they thought I was nuts. It confused me so much. I just could not think straight at all.

    I replied to another member yesterday about their CNS lupus. ange726 and I attach the link for my reply to her. In that reply I explain about my spinal inflammation and the white matter lesions on my brain.

    healthunlocked.com/lupusuk/...

    This is my post re my brain scan on Friday.

    healthunlocked.com/lupusuk/...

    Headaches are probably my number 1 lupus issue along with fatigue.

    First of all I put my headaches down to hormones and when I had my periods in early teen years. I often remember taking paracetamol for headaches and rocking with period pain on the stairs, awake a lot of the night etc.

    Then when about 14-16 year old, whilst studying for my GCSEs I was prescribed glasses and told it was eye strain? The headaches got a lot worse around 18-22 years old. I had a part time job through my A-levels and worked long shifts on my feet. I found that if I did not drink enough during those long, hot shifts or have a meal break at the right time I would get a headache. I noticed that sleep was also important. I went home with a terrible migraine one day and threw up - the only time I have ever actually been sick. I slept in a darkened room until it was gone. I used paracetamol, ibuprofen or Migraleve - over the counter - but not sure they still make those. They were yellow and pink tablets. You took one at the onset and then if the first colour didn't work, you could take the next stage 2 hours later.

    So basically I have suffered headaches all my life.

    Then, with hindsight I can see that my lupus was triggered by the birth of my 3rd child in 2008. By 2009 I had my first real bad flare - it went un-diagnosed by the doctor. But I had a terrible 3 week long migraine. It was so debilitating. I actually woke one morning and found hard bits in my mouth, which I realised were bits of my tooth! I had ground it in my sleep. So I was flaring (not that I knew it then) and being bullied at work. Luckily for me the dentist saved my tooth. The GP prescribed me diazepam for night time use only, to enable me to sleep with my mouth open & facial muscles relaxed, to let the tension release from my head. It worked. But I didn't want to get addicted to those and stopped after 2-3 weeks. I could have asked the GP to write me off work sick but my employers realised how bad I was and let me off working my notice and paid me for it anyway. So I handed my notice in at work, as I knew I wasn't right and needed a break. I thought it was just having 3 children within 4 years and being super busy.

    Anyway, I flared again most of 2013 and this did lead to my diagnosis. I had lots of other symptoms by then too. I get nausea and fatigue with my headaches. Confusion. Lack of concentration. Loss of appetite. No patience. Etc.

    As time has gone on, my headaches have got longer. I had one this Feb-into April. 5/6 weeks. That's the longest so far. Tenderness on my head and pains shooting down my neck. A feeling of a tight band around my head. Pain in my jaw.

    When I am in a flare, paracetamol & ibuprofen do not work. I have found that those combined with codeine do work, mostly. (I had codeine in the house for bursitis in my hip last year). Also, when on steroids I do not get headaches. In fact I do not get any lupus symptoms and have good energy levels too. But am not on daily steroids.

    Last year my GP gave me sumatriptan 50mg tablets. I found that they didn't really work. But I took them anyway as I had no other choices. This year I couldn't get them despite asking for them repeatedly on my prescription. So I quizzed the pharmacist. they couldn't get 50mg tabblets for some reason but had 100mg in stock. I asked if I could get those instead as the 50mg didn't touch the sides anyway. She spoke to the GP that day and got my repeat prescription amended to 100mg tablets. Now these work a treat. But I think due to my daily medication being right too by now.

    The right medication and sleeping it off is always good for me.

    I can now see that headaches have been part of my flares all along. I have been told I have headaches, migraine, cluster migraine, migraine with myalgia (pain around my head, ears and face), ice pick headaches - the short, sharp burst of intense pain in my head that can be a one off, or have a series of them, but they are totally unpredictable and you cannot take pain relief effectively for them.

    Lupus seems to be the cause. And my body certainly does not like stress. I can see the direct link. But even though I no longer work and try to control my lupus with life style and meds (2x200mg hydroxy daily, 3g MMf daily and 50mg mepacrine x 3 times per week) stress still happens. That's life right.

    I still think hormones and periods are linked to my lupus too.

    Like I said, my GP told me sometime ago that I have lupus with CNS involvement (Central Nervous System) and I guess most of us do to a certain extent. It's finding out the extent we as individuals have it. I had an MRI to try to find the cause of some inflammation on my spinal cord last year and this showed small bilateral lesions - high signal - on the front of my brain.

    I do not have Hughes (tested 2014 & 2017) and my blood are mainly good. I have only ever had 2 positive ANA tests. (But only seem to get tested at my rheumy appts and they are 11 months apart - this one 13 months apart). My ESR is around 6/7/8. Nothing alarming.

    I also still waiting for a referral to Neurology.

    I do not suffer from headaches when I've been on 2 courses of steroids - 5 weeks each - last summer. 20mg for 2 weeks and tapering.

    Fibro has never been mentioned to me.

    I also keep a daily health diary, to I can see when I have flares and headaches, how long they last, new symptoms and what meds work for me.

    Local care hasn't been great for me so far and I am waiting to meet a new NHS Rheumy on 13th September. I am intending on seeing Dr Kaul again in November in London, as I trust him and want him to lead my care.

    The Neuro might be right. But likewise, the Rheumy seems pro-active, understands and wants to help - I haven't seen much of that with my local NHS care and he also has a good treatment plan. My steroids last year were an experiment - if the steroids made all of my symptoms better and I felt well/good energy levels, it was the final tick on the check list for me to go on an immune suppressant - as lupus is inflammatory and therefore responds well to steroids. Might be worth a try? To see how good the steroids are for you?

    I hope I have helped a little. Take a look at ange726's post and my other link.

    I would love to hear from you again.

    Good luck with your doctors and I hope one of them really steps up to the mark to help you.

    Best wishes.

    Wendy

  • Melba1 ange 726 is posting re similar issues.

    healthunlocked.com/lupusuk/...

  • Hi Wendy, Thanks very much for your reply. Very strange in that we have similar personal circumstances (I'm 43 too and had 3 children in 4 years!! - maybe led to our increased stress?!).

    I've just had a second opinion from my old rheumy who I loved but moved to another hospital. She was very firm and clear it is 'neurolupus' and said it must be treated aggressively and laid out exactly the same treatment regime as the other rheumy. That doesn't mean that yours is definitely that though as some of our symptoms are the same but some different. I think it's a worrying diagnosis but like you said probably many of us (some scientific papers say up to 80%) have CNS involvement and there are different degrees of it like with all lupus. You should definitely ask to be referred to a neurologist although my rheumy today said a CNS lupus specialist is better as the neuros often lack knowledge of even the basics of lupus. She told me the name of someone who she will refer me to if I don't get better from this treatment regime (but she thought I would get better in a few months) but I forgot the name! Dodgy brain!! I will let you know it if you like when I get the letter if you're in the South - sometimes you just need to tell your GP who to refer you to?

    I completely sympathise with the cognitive problems, forgetting your age is scary (I forgot my lovely dog's name when I was really bad and my youngest son burst into tears this weekend at my lack of memory/concentration) but please be reassured by what my rheumy said today that these problems are temporary effects of the lupus and once the lupus is well controlled things should calm down.

    Sorry to hear you've had a lifetime of bad headaches. I think that the two are definitely linked with me and she said today that migraine/lupus are very linked and obviously both are linked with female hormone cycle too.

    Yes I've had steroids several times and they work brilliantly. I don't like taking this amount (my husband keeps sending me photos of moonfaces!) but both rheumys were very adamant that this amount is essential or it will start all over again (but I might reduce them a little without permission as I keep really laughing and behaving strangely so think 30mg is my limit for sanity!)

    So I feel much clearer in my head - well in my diagnosis at least, my head is still not very clear!!

    How have you found the immunosuppressant? Any side effects? I just can't seem to sleep but not sure whether that's the pred. I thought it would be just a few months but she told me today it's forever?!

    x

    PS migralive is still sold - I use it! I used to have Dr Kaul too - felt in very safe hands but he moved hospitals.

  • Hi Melba.

    Wendy sent me your link

    I have just been diagnosed with Lupus CNS with lesion on my spinal chord. I'm having SSEPs and a lumbar puncture on 4th October.

    I'm taking hydroxychloriqine, nortryptaline, tarpentadol and omeprazol. I was on steroids which took my pain away for 5 glorious weeks but then the pain came back tenfold. They increased my dose to 30mg a day but nothing. The only thing that dulls the pain is the tarpentadol.

    I'm not surprised you are confused about what to do. My memory is awful and i repeat conversations.

    I'm seeing Prof D'Cruz at guys hospital for my lupus and Dr Holmes neurologist.

    I have been told I have a strongly positive lupus serology with a positive ANA, RNP and SM antibodies. Every person I see on here has some symptoms similar and some completely different. I was convinced I had an injury and it wasn't lupus. But 3 MRIs later I have finally accepted it's not an injury but still not accepted the lupus part!

    How are you feeling overall? I'll let you know about my tests etc

    What is the next step for you? Are you going to ask for a lumbar puncture?

    Take care

    Ange x

  • Hi Ange,

    Sorry to hear that BUT I do think it's positive to know then they can get the right treatment for you? Lupus is such a strange disease that, like you say, effects most of us in some similar ways (especially the brain fog!) but we all have our own other parts too. I was asking my rheumy today about some other inflammatory things that have been going wrong, like awful abdo pain which comes with a raised CA125 inflammatory blood test which is normally an ovarian cancer marker (but they've checked ovaries and fine) but she explained that everyone's manifestation of lupus is different and this is just another example of 'my personal lupus' inflaming me somewhere.

    I hope your lumber puncture is OK - let us know how you get on. I must say I have found the steroids a huge help but both rheumys said the only way to stop a relapse is long term immunosuppressents - maybe they will try that with you? The one I'm having - mycophenalate - I think lots of lupus people are on and it seems fine for me so far (a little bit sick and loss of appetite but that's good balancing the steroids maybe!).

    Both rheumys have written to my GP saying I need a lumbar puncture, EEG and MRI but better to happen at the time when my symptoms were really bad a few months ago or if they (hopefully not!) get really bad again - she said that I could get long term remission if I take the drugs and I really hope you can too.

    I feel a bit better on the steroids, managed to run up the stairs today (have been crawling on hands and knees for months!), still got a lot of pins and needles, get 'zombie like' a lot, very forgetful and severe fatigue but overall on the up I think and actually felt like 'me' for a little while this morning - like a sort of heavy physical and mental cloud had lifted (it came back down but at least some light at the end of the tunnel!).

    Good luck to you; I will be thinking about you with your tests etc although I do think you shouldn't be waiting until October? The sooner treatment starts the better I think and if you're in loads of pain too that must be awful.

    xx

  • Melba

    What a wonderful positive reply from you.

    Thank you so much. You have just given me a huge smile and fantastic hope that I will have that same morning you have just had soon.

    Xx

  • I hope you do too! If not today then soon. Someone once said to me that the advantage of lupus is that we can feel really rubbish in a flare but to remember that just as quickly as the disease flares up it can go into remission again so fingers crossed for us all flaring at the moment that it will stop!

    x

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