GasconAlex

@bombsh3ll the pots symptoms are just ridiculous heart rate changes. I feel pretty awful being vertical and it gets worse the longer I'm up. After the operation (5 days absolutely flat including eating, toilet) felt much better. Then had a month mostly flat no effort. When I started being up more it hurt more, but I don't want to be horizontal all day so take pain pills for the headaches and when too much lie on floor / bench or recline wheelchair. ( I have balance issues and mobility issues). The leak was right sided, now left sided so is new. The surgeon has little expectation that the patch will be lifelong as 'I have very little bone there', if / when it leaks again on that side I can look at other options but in absence of definitive imagery he operated to look and fix rather than having all the repair options available. Neurologists in particular seem to be very compartmentalized, the headache neuro looked at only the headaches, the movement ones ignored the headaches, the dysautonomic one showed the mild dysautonomic dysfunction but that's all. The local general neurologists are approaching retirement and not interested in 'new' stuff. I found a goodish one but since he cannot see the link between leak fixed - rapid improvements, leaking again problems restarting (he see's only the improvement so carry on.....) and why should a CSF leak cause odd neuro dysfunction? At that point gave up as a bad day and will speak to my nice gp. The great thing about it being leak related as it isn't neuro degenerative - which when I cannot think clearly is a great help! Hope you find a solution but it's taken 3 years for the leak diagnosis even when I'd already had a CSF leak surgically proven. The difficulty in my view is that with better imagery many doctors look at the pictures and not the patient.

@bombsh3ll the treatment helped, but never touched the POTS. I think I always had this but was never symptomatic, again a history of a connective tissue disorder but I have it mildly (my boys are showing much more in the way of symptoms) After some success now I am more vertical I am leaking again, but from a different point. I do have a little nasal discharge but mostly only after I've done way too much. I have a metallic taste issue, orthostatic headaches and muscular rigidity and tremors. The skull base surgeon is leaving it 3 months, then he'll do an MRI, then try to fix this new different leak. The MRI is expected to show nothing as the leak is intermittent and much better when lying down. So lying down in the MRI means the leak is very unlikely to be active. Looking around there is quite a bit of stuff that shows cranial leaks can go unremarked with no exterior signs or images (like my first CSF leak) and suffer from the same headaches etc. as spinal leaks.

I disliked my first blue badge, even though I use a wheelchair. I have disability but hate applying for it. It's hard having medical assessments that say you're not really functional, but I try to do as much as possible. It's good news to have stuff fixed, and maybe you will get better - it is always possible.

@bombsh3ll I'm not sure about fainting or pre syncope as the only time I am sure I fainted was in hospital waiting for an xray on my shattered and visible kneecap. This happened hours after the injury and was chalked up to significant blood loss. Sometimes (even with taking lots of painkillers) the headaches were so intense that I had to lie down before falling down. I knew I could not remain vertical and conscious so perhaps that counts as presyncope.

I haven't had lumber puncture opening pressure done (even though I pushed for it when being tested for chronic meningitis), but after my first lp which I cannot comment on as I had meningitis at the time I've found that whenever they took a sample I have the exact same headache but even more intense than normal. When they tried blood patches it helped the headaches, the best one was 36 hours headache free. But the neurologist have always gone with the radiologist and he was wrong. I have the full scans, CD and cover letters. I've got to contact several neurologists with these and the surgical report as dismissing a diagnosis which (according to ent doc) the MRI is incorrect 20-30% of the time is not a acceptable dismissal %'tage. One quite enthusiastic but now retired neurologist believed cranial leaks should not give headaches as back when his old boss took cranial CSF out with a huge caliber cannula so he was certain a leak would need to be lower down the spine. I have lost a lot of confidence in neurologists, the neurosurgeon had it spot on 30 months ago but as he said MRI doesn't show everything (but a surgeon cannot fix it if he cannot see it), and to regard it as a tool not an Oracle. If you are thinking of opening pressure measurement can this be combined with a blood patch? That way you don't risk adding a leak source for no benefit as you also try a blood patch. They hurt quite a lot - but for me the 36 hours was worth the pain (as I have difficult veins the blood for the patch was extracted, after multiple failures, from the front base of my thumb).

@bombsh3ll Thanks. I can stay seated all day at the moment no problems, which is really great. I also lost a whole host of secondary neuro symptoms in the two or three days after the operation - I have fine motor control again and can do alternating movement. My point with the POTS is I think I had it before but it never affected me. I'm not too concerned about POTS if it is asymptomatic for me. I have other fairly serious issues that need sorting. My gp is concerned with either Marfan's or EDS for my boys who both have more symptoms than me but I and one side of my family is marfanoid so I'd guess there is a genetic connective tissue issue (relatives have been DX' d) This is my second confirmed cerebral CSF leak with loads of top grade MRI's all saying absolutely no signs of a leak or communication between ear / nose and brain. However the specific CT scans showed significant bone loss in both areas, suggesting communication. Luckily I found a surgeon who didn't believe in the all powerful MRI and was willing to operate to investigate the overwhelming clinical suspicion of a leak. Hope you get fixed too, spending a life horizontal is no fun for you or your kids.

Thanks @Pistol, I really hope the headaches have been mastered now been 24hrs + with no pain meds which is really really good. I don't 'feel' my heart rate it's the doctor who worries! In one of the post op problems I suffered heart rate was over 160 whilst horizontal - I don't feel this - I was obviously a little stressed at not being able to breathe. Ruined the doctors Saturday night spending an hour or so clearing my throat properly.

Back home after the operation and 8 days (mostly absolutely horizontal only on back - no turning to side allowed) in hospital (not fun in an older non air con hospital at 38°C/100F) but there you go. Firstly a recap I've had a CSF leak and serious ear/brain work on the left in 2016 including meningitis. For that, and since that, I've had 11 Cranial MRI's (and a full 3 Tesla complete brain/ spine MRI - about 1 hour in machine time). None of those, particularly those tasked with looking for a leak, have given any indication of any CSF leak. One radiologist (who helps define the national protocols for cranial MRI) who has personally done 5 of these scans said on the last CT scan report 'Is it really necessary to do another MRI?'. They were not sure they couldn't see the leak but no associated signs were present either. But I also had the traditional orthostatic headache, which over the years moved to being an all the time headache which got slightly better lying down when the huge number of pills could not drown out the headache. The neurologists all (relying on all those MRI) said no leak possible, therefore medication misuse /psychological or other factor. End last year one of my ent guys actually looked when I complained of still having a slight right sided nasal drip. The ent guys I see are all specialist ear guys and don't do noses. He immediately ordered a none classic cone beam (CT scan) and sent me to a nose guy (doesn't do ears....) With my symptoms he was certain there was a leak and believed the CT which showed bone defects would be proven correct. In the operation he confirmed a 'large' break and 'significant CSF leak' with very little bone present. He then tried to patch this. Since then I've had a couple of issues, but most of the patch is holding. So far: My normal headache has gone! I've got a small sinal headache, but it comparison it is nothing. The hospital kept trying to offer extra pain meds but have basically stopped. I was taking a *significant* amount of tramadol every day and a couple of times when I had colds I was upped onto morphine / fentanyl. I can spend all day sitting - no problems. A lot of my fairly difficult neurological muscle problems just disappeared instantly. I have lost the salty metallic taste. But : The POTS is actually worse. Instead of a relatively slow increase my pulse really jumps on standing the cardio didnt like my 24hr holter for pots as the change was not abrupt enough, but the TTT got a 50bpm increase to 145bpm (ish) so well into POTS.

@bombsh3ll I know you have the headaches too, I was really surprised at how little faith in the MRI he had compared to the CT scans - normally it's the reverse. That said MRI scans have little risk (with the macrocyclic gadolinium contrast) which is why I keep having them, the CT scans (especially the ones I've been having) have the xray risks so I try to minimize the number I have as I have already had a few... Hopefully I will be released in about 5-7 days, but that's what they said in 2016 when it took 142 days before they were comfortable releasing me from hospital and rehab. At least my kids are a little bit older now so the absences are easier to manage.

Finally had the consultation with the ENT nose guy. After yet another MRI he is going to operate next week. The MRI does not show any CSF leak (none of mine ever have - even the one a few days before a surgically confirmed leak), however one of my problems is with the skull base. The skull base is a bit of a surgical battleground between various groups of neurosurgeons and different factions of ENT surgeons. Even though the problems are in basically the same bone and are a few centimeters apart the surgery is done a different way (endoscopic rather than microscopic) by different surgical teams and, in my case, in a different site of the hospital. For those with classic CSF leak symptoms (including POTS) the surgeon (specialist rhino skull base) said some interesting stuff. Firstly that I have a definite problem visible on the cone beam (type of CT scan) that is not visible at all on the MRI. In these cases the scanner is almost always correct and the MRI is a false negative, he is more than willing to discard the MRI as it misses smaller or intermittent leaks. Secondly, with my symptoms he would be absolutely astounded if there was not a leak, so seeing as it is not fixing itself he will try to repair the damage. Notwithstanding the other neuro problems (which may be caused by other stuff), hopefully this sorts the continual headaches (started as really orthostatic, over the years (!) have become constant with orthostatic worsening).

At last a neurologist who listened, and did a thorough exam too. Unfortunately he has a strong suspicion of Corticobasal degeneration, which frankly isn't great news. He is not convinced that the tumor, CSF leak, skull base problems and meningitis fit some of the neuro symptoms (although the headaches may well be - hoping the operation in July works) so he is arranging a brain scintigraphy to see if this turns up indications. So a mixed day overall.

Prism glasses can help, even if they aren't perfect. The other option is an eye patch. An orthoptist (OK I didn't know it was a thing before I was sent) is usually someone dealing with squints and the like. They are a bit like physio for the eyes and this can help. However having your vision split by various prisms to 'exercise' the eye muscles is surprisingly tiring and headache inducing. It does help some people. My orthoptist did several tests with different prisms over several sessions before deciding what compromise would work best for me. I never wore glasses before and had plain glass (with slight astigmatism adjustment) made with prisms. They help a bit.

You may want to try an orthoptist for your diplopia. They have lots of headache inducing tests and some people respond to the reconditioning they can do. Don't plan on driving straight afterwards as having your vision split really does your head in. Prismatic glasses can also help in my experience and the orthoptist should be able to add this to a current prescription, and if you don't have a prescription you can always have just the prism.

Neurology Reviews. 2017 March;25(3):23-24 Imaging of patients with CSF leaks often initially is read as normal, and MRI is not an adequate evaluation in the high clinical suspicion of a leak, Dr. Carroll said. “It is a good place to start, because if you see a leak on your MRI, maybe you do not have to get a CT myelogram,” he said. “But if you have a clinical suspicion of a leak … you should pursue that in the face of your radiologist telling you that there is nothing.”

CFS leaks are tricky, and the MRI is no way definitive on this, except for huge leaks. Smaller and intermittent leaks are missed. The headache neurologist I saw was 100% confident in the MRI, even though I had a confirmed CSF leak (by surgery) and the MRI 3 days before suggested there were no problems. She dismissed any possibility of a leak because of the new MRI. The only headache relief that worked was a blood patch, this worked for 36 hours before stopping abruptly. During that time I was still orthostatically intolerent with some bp issues - but no headache. The ENT who is now treating me believes that I have another CSF leak from a different skull base defect and will (hopefully) be operating in July. They however said that 20-30% of nasal CSF leaks do not show up on the MRI. They are also not considering any neuro or pots symptoms for their diagnosis only ent ones. I (and some of my doctors) are considering that there may well be an underlying problem, probably eds that is causing repeated problems. So even if the next leak gets fixed the pots might well still be there. But if fixing the leak fixes the headaches that would be great. There is a huge disagreement on this within the professions, the neuro surgeon believed the symptoms justify a blood patch (hurts but not hugely risky) and if that makes things better then his view was that is confirmation. Some neurologists say only a spinal leak can cause the headaches, some believe cranial leaks cause problems but not by low pressure. There are many opinions so look at your case carefully and don't necessarily accept the first assurance you come across - it depends on your particular symptoms. If you can get an opening pressure reading from a lumbar puncture / blood patch that can help. I've never had this despite numerous lumbar punctures and blood patches, but some doctors say it is useful in CSF leak diagnosis.

Not entirely sure exactly what they are proposing as this gets explained just before the operation at the same time as I have the anesthetic consultation (about a week or so before the op.) The only information I have is that they need the MRI for the surgery to act as guidance. This specialist is a 'nose' guy who based on symptoms, and a cone beam (type of CT scan), and the fact that a couple of years ago the MRI said no leak and intact dura a couple of days before the surgeon had to stop because the dura was leaking and had collapsed into my ear, is convinced the last MRI (January) is not definitive and there is another, different leak from the nose. Several of the specialists have said that the MRI can miss 20-30% of cranial leaks so they cannot be taken as definitive. I'm 'lucky' in that I have several areas of 'defects' in my skull base visible on the CT scans, so they think this could explain some of the symptoms, but with a family history of eds it may well be that the leak just pushed me over the edge from coping with symptoms to being unable to fight through all the issues. The only thing that really helped my headaches was a blood patch which worked perfectly for 36 hours - no pain meds at all and about 5 hours out of hospital feeling human (still unstable and unable to walk unsupported but able to stand without headaches). They only tried the blood patch because of several lumber punctures I had with the meningitis. Shortly afterwards I had a consultation with the regional headache specialist neurologist who, unfortunately, is of the opinion if a leak isn't visible on the MRI then there isn't a leak. After that consultation they were unwilling to retry another blood patch. Cranial CSF leaks are notorious for being difficult to detect in those with spontaneous leaks so it might well be worth pushing down that road as at least there is a chance things might get better for you if this is your case.

After a fair bit of telephone work I have at least had some progress. The latest update is I get to have yet another cranial MRI (this makes 11 on the current problems) followed by consultation and surgery a couple of weeks later. (Early summer) The MRI is for a roadmap, they are not expecting the expected CSF leak to be visible. It would appear that a large portion of my skull base is defective with confirmed leaks (confirmed by surgery) and my brain dropping slightly out of its cavity. They currently believe I have a leak into the sinus area, the previous leak was into the ear. Not sure if this will change the dysautonomic problems, really hoping it solves the headaches.

Unfortunately MRI only shows so much, and often in neurological problems shows very little. In my case I have had repeated MRI's ( 10 in the last 3 years) and they have only shown expected damage and no explanations for my problems. Unless there is a largeish physical problem it is unlikely to help, but it is worth checking even so.

A problem with lists is that you list everything the neuro says it's psychological as you have a laundry list - but when you don't have a list they say why didn't you tell me about xyz. I take a list and start on the major stuff then bring up other stuff if it looks like it might be relevant. Unfortunately I have 'issues' with my local hospital general neuros who keep sending me for hyper specialist neuro assessments who come back with there are neurological issues but these are not my specific corner. I end up no further on. Hopefully you hit a good neuro who can help you.

Treatment for CSF leaks is (in the first place) staying horizontal for a few weeks to allow the membranes to heal. In lots of cases this apparently works. Another option is the blood patch. This is where your blood is taken and then injected into your spinal column. For me this worked perfectly for the headaches, 100% gone with no pain pills necessary for 36 hours. No headaches when vertical at all. (You can have this done if you are a Jehovah's witness by the way - I'm not but it came up when I was researching the treatment) But this is where it gets complicated for me, my gp, neurosurgeon think it is a leak that cannot be seen on the MRI (if he can't see it, or where it might be he can't fix it) on the otherwise the neurologists who can't agree but the one who is responsible (and did the patch last time) disagrees and doesn't want to retry. Without definite proof no one else wants to contradict him.

Because the headaches became utterly ridiculous I take slow release tramadol all the time. With this I can be vertical for up to 120 minutes before it gets too bad. If I have vibrations (like car trips) it makes the headaches much much worse. Then I take more tramadol and also paracetamol until I have real problems. By that time I might need 12 to 36 hours flat. It's not perfect but the chronic pain people think it's better to be able to be vertical (and yes it doesn't get rid of the pain) than to decondition myself.

Voted yes but after about 3 years they are much less orthostatically sensitive. Now I take more pills and spend more time vertical.

@bombsh3ll never had a myelogram, did have a full spinal MRI looking for leaks. This took over 50minutes in the MRI (from image timestamps) nothing relevant found. Family has HEDS, but no genetic testing done. Some suggestion of Marfan's, from a range of doctors for different family members but consensus moving towards eds. (Last year I had an angio ct looking for aortic dilation the neuro came back with 'sorry signs of dilation' cardio said ' yes but 'just' within normal so not worrisome') I have quite a lot of dystonia, so no longer showing flexibility! @StayAtHomeMom depends on the leak. Constant high volume leaks are more obvious, intermittent lower volume leaks are much more difficult. http://dizziness-and-balance.com/disorders/central/csf-leak.html Has some interesting stuff but also quotes that no imaging is particularly useful. They look for diffuse symptoms but these are not definite. To really look you have to examine each area closely. I remember the neuro intern saying ' ah we'll need an MRI' Head neuro saying 'of what?' Intern - 'the head' HN - 'you can't say that, you need to look for something' @bombsh3ll if some of your stuff gets fixed, you can probably live with the rest. Its what I'm hoping for. Sorry this is a bit disjointed, I broke a rib on Monday and this has made life even more difficult.

The confirmed leak was spontaneous, but linked to an ear tumor. Since then the family has confirmed eds with pots which means the rest of us have looked at the symptoms. I'm fairly certain eds is there but the doctor wants to sort out the neuro deficits and severe headaches before looking into this - which might be the underlying cause. The otologist who examined me last was not sure where the fluid he found by rhinoscope was coming from, so needed to check. That is when the bony defects in the sinus were found. I have the next MRI in December to check, but the imaging is often not conclusive in leaks. He strongly suspects another (different) spontaneous cranial leak. The blood patches were tried because of the lumber punctures I've had, these were fairly difficult due to low bp. The neuro who did them is now chief neuro at the local hospital, he didn't want to do the patches and only did when told to do so by his now retired boss. This makes having another try difficult to say the least. My point is that even with issues and specialists thinking there might well be a leak, because the MRI's are not clear it is difficult to progress. I'm doing better this year, still have lots of problems but no inpatient stays so far this year. That's a lot better than the 25 ish days last year and 142 in 2016. It's certainly easier for my kids.

Very difficult getting a leak confirmed. Looking towards my 10th MRI and still no general signs visible. The problem being that I was operated on 3 days after an MRI, which they were sure meninges were still intact- they weren't- and I had a leak confirmed surgically (along with some brain herniation) this meant that operation wasn't successful. After meningitis still have problems, including postural headaches etc. However now have disagreements about causes. Neurosurgeon convinced is still leak, some neurologists think maybe, some no. The Neurotosurgeon thinks not because other than the tegmen tympani being mostly missing nothing else shows. The ENT has just run a new cone beam but that shows the ears fine but large bony bits missing in the brain / sinus border. This is with a very slow nose drip that increases when the hr goes into overdrive by standing up. Have had a few blood patches that work for a day or two (during which time my nose runs more) then stop. My gp is convinced its organic, some of the local neuros think might be related to eds, some say psychological - psychiatrist says organic. So my case is a little complex, I'm leaning towards the problems of pots and eds always being there and I thought this was normal (physio says partial dislocation of jaw if do move too far is not normal) but the other problems and leak just put it over the edge. If you have visible signs on the MRI it's easy to show a leak, if not you run around the houses again and again. The pots (from ttt) was easy to show, although the cardiologist here think pots should be an instant acceleration on standing not gradual over 2 or 3 minutes. Good luck on trying, I'm hoping at least some of my problems are sortable.