Superficial Siderosis a Progressive Neurological Disease

[abprops]

Superficial Siderosis a Progressive Neurolgical Disease - Pseudomeningceles resulting from dural defects following surgery to remove intradural tumours being the most common cause.

Has anyone else been diagnosed as having Superficial Sidereosis (CNS-SS) following surgery to remove an intradural tumour, with or without the added complication of resultant cerebrospinal cyst or Pseudomeningocele? This rare CNS disease is the result of iron deposits on the surface of the spinal cord and brain, which progressively attacks the patients nerves. The main problem being deposits of iron on the Cerebelum which lead to problems with nerves to eyes and hearing, with speech, balance and gait ataxia problems resulting in falls and need for a wheel chair.

Superficial Siderosis results from leakages of blood into the cerebrospinal fluid circulating around the spinal cord and brain. Bleeding can be due to a variety of reasons, strokes, accidental damage or following brain and or spinal surgery. The medical literature on Superficial Sderosis suggests that Dural Tears or Defects following surgery which result in a Cerebrospinal Cysts or Pseudomeningcele, is the most common source of bleeding which results in Superficial Siderosis.

There is at this time no cure for Superficial Siderosis, though one drug is currently being used to hopefully help remove the iron which causes problems for some people or where the source or sources of repeated bleeding into the cerebrospinal fluid can't be determined or stopped. In some cases bleeding can be intermittent making it difficult to find the source of blood contamination so stopping the flow of blood or repairing the source can be difficult. However locating and repairing dural tears or defects associated with cerebrospinal cysts or pseudomeningceles is much easier.

The drug Deferiprone or Feriprox is the only one known to be capable of passing through the blood barrier which protects the CNS, but that drug is highly toxic and an expensive means of removing existing iron deposits or controlling continuing iron deposits.

Dr Michael Levy based in Johns Hopkins Hospital Baltimore completed a Pilot Safety Trial of deferiprone in 10 subjects (patients) with superficial siderosis. The results were published in Stroke. 2012 Jan; 43(1): 120-4. don: 10.1161/STROKEEAHA.111.628032.27. Levy M. Llinas R

PMID:22034002 DOI: 10.1161/STROKEEAHA.!11.628032

stroke.ahajournals.org/content/43/1/120

An abstract or free full text is available for MEDLINE

Blood which contaminated the cerebrospinal fluid circulating around my spinal cord and brain following surgical removal of an intradural extramedulary schwannoma tumour at T9/T10 in my Thoracic Spine on 3 February 2009 plus, blood leakages from the dural tear or defect at the location of surgery to my Dura following that surgery between June 2009, until Investigative and Corrective surgery located and repaired that dural tear/defect, dealt with the remains of the pseudomeningocele and closed and filled the 'space' or cavity in which the pseudomeningocele had formed were the source of the bleeding into my cerebrospinal fluid leading to the deposition of Iron onto my brain, as revealed by MRI imaging done in December 2016 resulting in the confirmation of Superficial Siderosis in 2016/17. Superficial Siderosis having been the cause of the additional CNS symptoms I had reported since late June 2009, which had been denied and indeed claimed to indicate that I was mentally ill. Though fortunately I first requested and then demanded psychiatric and psychological assessments and if necessary treatment when those claims were made by the neurosurgeon and a GP in 2009. Fortunately for me two assessments by a Consultant Psychiatrist and two assessments by a Clinical Psychologist confirmed that I had no mental health problems and I was provided with copies of the psychiatric and psychological reports and discharged from the mental health service as requiring no form of treatment.

Spinal MRI imaging was done at 6 monthly intervals between 4 June 2009 and 9 May 2001, and continued at yearly and since 2 yearly intervals since 2012. A brain MRI was done three weeks after the first of two emergency admissions as a suspect stroke in 2010 and repeated in 2015. However the MRI scanners used between 2009 and 2015 were not capable of showing the deposits of Iron onto my brain and CNS. The diagnosis of Superficial Siderosis was made in 2016/17 flowing an MRI scan using a much more powerful MRI scanner, specifically to check the my claims about increasing neurological problems/deficits since June 2009.

The neurosurgeon who had performed the laminectomy and removed the intradural tumour in February 2009, claimed in 2011, that he had been unable to fill the 'space' or cavity he had formed by removal of bone and muscle during the laminectomy necessary to access my spinal canal, to remove the intradural schwanoma, before he closed the muscles over the back of my spinal canal and closed the external wound. He claimed that as there was no tissue nearby to allow him to close the 'space' r cavity what he had previously claimed to be a perfectly normal clinical finding following a laminectomy and opening of the dura was a Meningocele. He also claimed that the only possible alternative, in his opinio, of extensive harvesting of muscle from there parts of my body with plastic transportation into the space or cavity was not justified due to the risk of such surgery to me the patient. The Neurosurgeon who performed the Investigative and Corrective surgery in January 2012, used what he described as ver routine and very necessary methods evolved to fill such cavities created by removal of bone and muscle during the many laminectomies carried out for a wide variety of reasons other than neurosurgery. Following the very successful, Investigative and Corrective surgery in 2012 the neurosurgeon provided copies of photographs showing the larger than expected defect in my dura. That defected had expanded considerably due to the flow of cerebrospinal fluid pumped from within my dura and into the large pseudomeningcele between June 2009 and January 2012. The Radiolgist who reported the presence of the Pseudomeningocele following the first post surgery monitoring MRI scan on 4 June 2009, recorded that the pseudomeningcel was within the Epidural Space and impinging upon the posterior column of spinal fluid. The Radiolgist who reported on the second post surgery monitoring MRI scan done on 8 December 2009, recorded that the pseudomeningocele was till expanding to fill the 'space' available as the trauma to surrounding tissue by the surgery on 3 February 2009 subsided. Rather obviously the pseudomeningocele had slowly expanded to fill the 'space' or cavity over the back of my spinal canal between 3 February 2009 and 8 December 2012.

Dr Levy has been conducting a follow up study which will hopefully prove the deferiprone is safe to use and a potentially a cure for means of limiting the progressive effects of Superficial Siderosis. The research results are due to be published later this month, June 2017.

Dr M.Levy's CV is available on the Johns Hopkins website.

[abprops]

Transcript of Dr Levy's Webinar on Superficial Siderosis follows:
Slide 1
Hi, my name is Dr. Michael Levy. I'm an assistant professor at Johns Hopkins University in Baltimore and a neurologist. I see patients with Superficial Siderosis of the nervous system. This webinar was really a joint effort with my patients on Facebook, who recruited me to present a twenty to thirty-minute overview about Superficial Siderosis. Today I'll go through just kind of the broad strokes of the pathophysiology of the disease, at least the parts we understand. We'll work out the treatment options that are available.
First, what is Superficial Siderosis? In broad brush strokes, Superficial Siderosis is an iron overload condition of the brain and spinal cord. Patients with Superficial Siderosis do not have an overall iron overload condition. The total body iron levels are normal, but the iron is distributed to the wrong place. There should not be iron on the surface of the brain and spinal cord. It is toxic in those locations, and that is the basis of the disease. I'll get into more about how the disease occurs biologically in a future slide
The condition is defined as a slowly progressive nerve-degenerative condition. Slow, I mean that it takes years between the time that patients have their first bleed, and the time that they have their first symptoms of Superficial Siderosis. It could be ten, twenty, thirty years, in one case forty years before the bleeding catches up with the symptoms and once the symptoms begin, the course is also very slow, so year by year the patient will notice that the hearing loss and the balance is getting worse. But it's not suddenly worse, and it doesn't go up and down. It's not a relapse and remitting condition and progressive really speaks to the course of the diseases. It's continuous, so that year after year you're worse than the previous year.
The triad of symptoms are hearing loss; ataxia, which is balance; and myelopathy, which is spinal cord dysfunction. The hearing loss can occur usually in one ear and then the other. It tends to involve higher pitched sounds first, and then lower pitched sounds later and it eventually becomes complete so patients at the end of this process will become deaf. The ataxia, or the balance trouble, is often second. Sometimes it's the presenting symptom, and it mostly is obvious in walking. The patients tell me that they bump into things, or that they fall more often. Rather than the fine motor tasks of the hands, it more involves the balance of the trunk, the balance of staying upright. Especially walking and running. The myelopathy is more of a heterogeneous process. Myelopathy just means spinal cord dysfunction. The spinal cord is involved in strength, sensation, bladder function, autonomic function. There are many different symptoms that can result from myelopathy, including

weakness, numbness, pain, spasticity, urinary retention, urinary incontinence, constipation. All those symptoms can be due Superficial Siderosis.
Slide 2
Superficial Siderosis is incredibly rare. It's one of the rarest conditions in the world. We estimate that there are approximately one hundred patients in the U.S. The incidence may be climbing a little bit because MRI is widely available in the U.S., and it's the basis for making the decision. Worldwide it may be harder to make that diagnosis because MRI is not as available, and so we estimate that the incidence is approximately one in three million people. Again, worldwide we don't know if those numbers are higher or lower. The incidence between men and women is approximately the same, and the age of onset tends to be in the later years, mostly because it takes ten to twenty to thirty years before symptoms begin. So if you have your traumatic even in your teenage years or in your twenties, it may be until you're fifty when you finally present with hearing loss.

Slide 3
What causes Superficial Siderosis? What's really the biology underlying this disease? It starts with the concept of spinal fluid. Spinal fluid is produced deep in the brain, and it's forced out of the brain through these small channels. The spinal fluid then circulates around the entire brain and around the entire spinal cord. The cause of the disease is bleeding into the spinal fluid. The bleeding can take place anywhere. It could be this part of the brain, it could be down to the spinal column. Wherever the bleed occurs, the spinal fluid will then circulate around and deposit those blood products all around the brain and spinal cord.
There are many different potential etiologies. Trauma is the most common, trauma to the spine. That can occur in car accidents, diving into pools, falling off ladders, and getting kicked by bulls. There are a lot of different reasons people have trauma, and the basis of the traumatic event is not that there's a one-time bleed, but that there is a tear in the lining around the spinal cord that does not heal completely, and tears and re-tears over and over and over again such that the bleeding persists or is intermittent over the course of years and years. That doesn't typically happen after trauma, and that is why there are only a hundred patients in the U.S. with this condition cuz most of the time you heal well after a trauma, but for one in three million of the time the healing is not complete, or the bleeding remains intermittent, and you develop superficial siderosis.
The next most common cause are tumers. There are some tumers that bleed. The tumers generally lie within, with access to the spinal fluid, so when they bleed they bleed into the spinal fluid. Same problem. There are surgical procedures, very common. It's probably the second most common reason people get Superficial Siderosis. Again, when the neurosurgeon goes in there to remove a tumor, or fix the spine, or do whatever, if there's incomplete healing and persistent bleeding into the spinal fluid, then after years and decades you can get superficial siderosis.
Finally, there are some people with rare causes, such aneurysms, that leak into the spinal fluid, or tears in other parts of the lining that surround the spinal cord and brain that can lead to superficial siderosis.

Slide 4
Now, chronic bleeding into the spinal fluid, what that causes is a breakdown of the blood cells that leak into the spinal fluid. Within those blood cells are compounds that contain iron. The iron is normally used to transport oxygen around your body, and when these compounds break down they release the iron. Iron is very toxic, especially to the brain, and so we've evolved over billions of years to produce a protein called ferritin, and excrete it to bind the free iron and make sure that it's not toxic. That ferritin and iron compound is called hemosiderin, and that's what's seen on the MRI is hemosiderin.
The hemosiderin that floats around the spinal fluid that's shown here in this representative graph, representative picture, it floats all around the brain and the spinal cord, and then by gravity it tends to stick wherever it hangs around. So, if you're laying on your back to go to sleep for eight hours a night, then it tends to deposit here around the cerebellum, where the balance problems come from. When you're upright for the rest of the two-thirds of the time you're alive, the iron tends to deposit by gravity around the spinal column. The nerve that goes through the brain to the ear is particularly susceptible to the floating hemosiderin deposits because that nerve spends most of its life in the spinal fluid, as opposed to any other nerve coming off of the brain. So that's why the hearing is lost, because that nerve is particularly vulnerable.
99.9% of the time the ferritin is actively bound to that free iron. It keeps it from being toxic, but that .1% of the time where it's not actively bound, the free iron escapes and causes damage, and over years and years and years and years of this kind of release and damage, that's how superficial siderosis develops. That's why it takes such a long time. The big question is, if we remove the iron, do the symptoms improve? Or, if we remove the iron, have we, ah, already gone down a pathway that's not fixable. That's really the big question that we're trying to answer, and we'll show you how we're trying to answer that question in the treatment options later.

Slide 5
The primary way we make the diagnosis is by MRI. Here are MRI shots of the brain and um, the brain stem here, identified by that white arrow, the cerebellum identified by that white arrow, and the cortex, identified by that white arrow and what they're all pointing to are these dark, black deposits. That's the hemosiderin that we're talking about. It's unmistakable on the MRI. You don't see these kind of deposits in any other condition, and so if the patient gets an MRI for any reason, for hearing loss, balance problems, whatever, the radiologist should be able to identify this particular pattern and make the diagnosis of Superficial Siderosis.
The lumbar puncture is an important test to see if you're actively bleeding. So, if you've undergone a procedure, for example, to fix the problem, and one patient who had a bleeding tumor had the tumor resected, then add the question, "Is there ongoing bleeding?" The only way to find out is to do a lumbar puncture, and that involves inserting a needle into the lower spine, well below the end of the spinal cord but well within the spinal sack, withdraw spinal fluid and analyze it. Fresh blood can be detected within two to three days of a bleed. Blood products, those breakdown products, will remain floating around in the spinal fluid for up to two months. So if you do a lumbar puncture, you can detect any bleeding that occurred within the past two months. A clear spinal tap suggests that you haven't had a bleed within the last two months. It doesn't mean you're not bleeding ever. It just means you haven't bled for the past two months.

Slide 6
Ok, I mentioned the majority of people have Superficial Siderosis through trauma, and the trauma is mostly in the spine, in the back, and in the neck. In fact, in the recent Mayo study 70% of patients had a spinal malformation that leads to bleeding, and by far the most common is what's called a dural tear. The dura is the orange membrane around the spinal cord. The orange membrane surrounds another membrane called the subarachnoid tissue, which surrounds the spinal cord itself. The dura is really a membrane, it's as tough as leather, that does not heal well, so if you tear your dura, which is very difficult to do, but if you tear it what happens is your body tries to heal it by bringing in new blood vessels, new cells. The immune system goes in there and really tries to do its best to heal that tear and keep any spinal fluid from leaking out. The problem is that the recruitment of those small blood vessels and the inflammation really doesn't do the job well, and instead that weak healing process just leads to more bleeding over time as you tear and re-tear over the course of years.
How do we know if the bleeding is coming from the spine? The best clue is to look at the brain MRI. If the hemosiderin deposits are equal on both side of the brain, it almost certainly came from below, and, as I mentioned, by gravity the iron deposits layer on the bottom of the brain when you lay down. That's how we know the bleeding is coming from the spine. The other way is to do an MRI on the spine and look for the dura that's been torn and displaced. It's not always detectable, and about a third of our patients we cannot find the source of the bleed. The best way to identify the exact precise site of the tear of the dura is to do what's called a CT myelogram. And that’s where, it's like a lumbar puncture, but you don't go in as far, and you inject a dye that travels on the outside of this membrane, and wherever the tear is it quickly travels on the other side. When you see that dye moving, then you know that you found the tear. As I mentioned, sometimes the dura is sealed by the healing process. Even if it's not that good of a seal, the dye can't get across, and so by the myelogram it will normal. You have to actually be bleeding or have a torn dura for the CT myelogram to detect the tear.
I pose here a theoretical question. Does it matter where the bleed is coming from? The reason I pose that question is because if you're on an iron chelator like [ferriprox 00:15:26], which is much better at chelating active iron than it is at chelating hemosiderin, then if you're on ferriprox when you bleed you've pretty much chelated all that blood, and so it doesn't really matter that you can't find the source of the bleed, it doesn't matter that you should be really trying hard to find the source of the bleed if the ferriprox is just going to chelate it. That's something to consider, and we'll talk more about that later.

Slide 7
The typical course of this disease is slow. I put that in caps because there are no emergencies with Superficial Siderosis. Things do get slowly worse over time, and that's concerning to most people, but they get worse very slowly. I mentioned it takes a long time for the disease to progress, but it is progressive, so once you start to get worse, you're going to continue to get worse. Usually, hearing starts first, but sometimes bladder problems start first. Most of my patients have trouble walking, and many of them have bowel or bladder dysfunctions. These tend to occur later. As I mentioned, there are many functions within the spinal cord, including autonomic function. That's blood-pressure control, sexual function that can be affected as well.

Slide 8
I get this question a lot, so I thought I would address it in this presentation the question a lot of patients ask is, does superficial siderosis lead to an early death? We don't know the answer to that. We don't know if superficial siderosis causes an earlier mortality. The reason we don't know that is because we haven't studied enough patients over time to be able to see that. I can give you my personal experience. I've seen at least seventy patients, and so far two have died. Those were men in their seventies. One died of a stroke, and one died of cancer. They were in their seventies. The average lifespan is somewhere around seventy-six to seventy-eight for men, so they were slightly younger than that. Whether or not that's hastened by superficial siderosis, I don't know.
Now we have had three strokes in this patient population so far. They were all men, at least sixty years of age with other stroke risk factors. It could be that superficial siderosis is a risk factor for stroke. We haven't seen too many other comorbidities so I can't say that there are any other particular diseases we should be looking for in patients with superficial siderosis that may cause an early death.
The one phenomenon I want to mention is called neurological reserve. Neurological reserve is really the capacity of the brain to handle stress. If you think about a baby who's under stress, it really can't handle it and they quickly lose their neurological function very quickly. Same with older folks. An older person may have an infection and then slow thinking is a result. You treat the infection and the thinking improves. That's really because the neurological reserve in young and the very old is reduced over time. The same is true with superficial siderosis. The neurological reserve is reduced, so when you undergo a stressful or metabolic stressor, you may find that your symptoms are exacerbated. A classic presentation is a patient going under

anesthesia for surgery requiring more time to recover from the anesthesia. That's a classic presentation.
Slide 9
Ok, let's talk about treatment options. The way we used to manage this condition is to just kind of stop the bleeding. We know from patients who bleed all the time, one-time bleeds from trauma or hemorrhages or strokes, that the brain is capable of removing the iron, so we were thinking that if we stop the bleed that the brain would be able to just remove the iron over time and we found that didn't work, and probably the reason it didn't work is because when patients finally presented with symptoms the capacity of the brain to remove more iron was finally overwhelmed, and so stopping the bleeding at that point did not help. So our current approach is really to try to not just stop the bleeding if it helps, but also to remove the hemosiderin and iron that's bound to the ferritin; to try to remove that as well.

Slide 10
So let's discuss how we stop the bleeding. There are three ways that we approach this. The first way is through an exploratory surgery. You may be able to find a neurosurgeon or orthopedic surgeon who can go into your spine, look around, and try to stop the bleeding at the source. Sometimes exploratory surgery identifies the tear or the site of bleeding, but sometimes it does not. There are risks, obviously, going under the knife, but there are potential benefits. If you find a surgeon, who is really keen on finding the leak and can do it and feels confident doing it, give them at least a chance to explain themselves.
Another approach is called direct fibrin injection. This is when we know the site of the bleed. We'll insert a needle between the bones to the space right near the site of the tear or the bleed, and we inject the blood product called fibrin. Fibrin is a glue which seals the membrane that's torn and provides a good seal for at least a few weeks, gives the immune system and the healing process a chance to really try to fix that tear better than without the injection.
The third approach is what's called the blood patch. Blood patch is where we insert a needle into the lower spine. No matter where your tear is the needle goes into the lower spine, blood is injected from your arm, blood is taken out, and then re-injected back into your body through this needle, and the blood goes on the outside of the spinal cord and pushes that lining around your spinal column and triggers an inflammatory reaction. The blood there keeps the dura in place, triggers an inflammatory process that really gives the dura a chance to heal. Sometimes it works, sometimes it doesn't. The blood patch is by far the safest of all the approaches, followed by the fibrin injection, then followed by the surgery after that.

Slide 11
Ok, the approach we've taken recently is to use what's called an iron chelator. This is a molecule that binds the iron. Very simply, the goal here is to try to remove the iron that's on the surface of the brain and spinal cord, and to see if the brain and spinal cord can heal once the iron is removed. The reason that we use Deferiprone or Ferriprox is because it's the only iron chelator that gets in the brain. There are three or four or five other iron chelators on the market, but none of them get in the brain. So they circulate in the blood, but they won't get in the brain.
We performed this study of ten subjects with Ferriprox using the drug over three months and followed them by clinical measures and by MRI, and we found that in four of the ten subjects the MRI improved in as little as three months. So then we extended it out as an observational study to thirty-seven subjects, following them for two years to see if we can detect a clinical benefit, and what we found is that most subjects take at least two years to really see that improvement on MRI. Almost everyone had improved, at least by MRI, and what we really need now is to see what happens after that iron is gone.
So in many of these patients, as the iron disappears, can we then detect a clinical benefit? Is there a healing period that's required? We don't know. Does all of the iron have to be gone before the healing can begin? We don't know. We do know that in the first two years as the iron is being chelated there's no real clinical benefit. We don't see patients getting much better, at least initially, because the iron is still there, and it's still being toxic, and so the healing process is really limited early on. What we really need to see is, when that iron is finally gone, then can we see a clinical benefit?

Slide 12
Here are the alternatives, tested and untested. There are copper chelators that get in the brain. They do not work. Less common is Triamterene. It doesn't work for Superficial Siderosis, although it is a weak iron chelator. There are other iron chelators, as I mentioned, available on the market, but they don't get in the brain. One example is Exjade. There's no point in taking Exjade because it's just going to chelate the iron in the rest of your body. What you need is to chelate only the iron in your brain and spinal cord. People have thought, well if you're bleeding into the spinal fluid can you shunt that spinal fluid out into the body instead of letting it circulate around? It's a good thought. The truth is, though, is that you need spinal fluid to buffer your brain, and so you can't shunt all of it. Shunting part of it, it delayed the onset of disease, but it doesn't change the progression of the disease. Doing nothing is not an option. Doing nothing means that you're going to progress. We were hoping that some patients, that they maybe could stabilize and maybe do fine in the long term. That doesn't happen. Everyone progresses.
Here are some untested alternatives that I personally do not have experience with, but my patients brought to my attention. One is Inositol Hexaphosphate. It's a vitamin, IP6, that apparently gets in the brain and apparently is an iron chelator. That's the combination we're looking for. I don't have any experience with it, but if other people do I'd love to hear from you. Curcumin is a derivative from turmeric, and it's been purported to be an iron chelator and get in the brain. And we are aware of new iron chelators that are being developed by pharmaceutical companies, that have penetration into the brain. Those are maybe promising.

Slide 13
As I mentioned before, our ongoing research is really to look at these patients who are on an iron chelator for a long time. Because again, what we really want to know is, once that iron is gone does the clinical benefit fall out? We know we're good now with removing the iron. The question now is, does that provide a clinical benefit?
So I would like to acknowledge my patients who set up this group in the U.K. called The Silent Bleed. They've been helping raise money for research, which we've been using towards these clinical trials.
That's the end of this webinar. You can always reach me at [e-mail]. If you have any personal questions, I'd be happy to answer them. Thank you very much for paying attention

[peaceandlove]

Dear Abprops,

I Read aprtielly your post (since I cant understand all), but I want to highlight that symptoms which accure in vision and hearing and speech are related to nerves placed at Tectum. I have similar to what you experience but cause is not the iron, I have a tectal glioma. Recently I started feeling the eyes problem in vision and I believe it is relative. Doctor tel me it will regress with time, but I don't think soo. Hydrocephalus is also a symptoms of the tectum glioma since the cerebro spinal fluid will be pressurized by the glioma if volume increased which can create headache pain.

I have another suggestion to you. Some doctors informed me that since the muscle of the back post laminectomy will touch the spine since no bones, this can be reason of neuropathic pain since this muscle may create electricity by friction and may be translated to the brain as pain. The same neurosurgeon advice me to make laminoplasty as corrective action. I don't know how much this is correct. The laminoplsty is a surgery to restore the removed bone by artificial one to remove the tension of muscle from the spine.

Good Day. Please advise.

Thanks

[abprops]

Thanks for your interest Peace & Love. I am not sure how to respond to your post but hope the answer I provided to a question from Coxen00 on 4 June will suffice. I wish you well and if there is anything else I can help with please ask.

Jun 4, 2017 at 1:22pm QuoteMessage Options
Hi

My previous posts should provide all details including the very successful Investigative and Corrective surgery to deal with the Symptomatic Pseudomeningocel by Prof. Dr. Westphal of the German NHS in Hamburg in January 2012. I accepted Prof. Dr. Westphals offer as his was the most straight forward, detailed what he would do and provided detailed costings.

The reason the Investigative and Corrective surgery was done by the German NHS (which I paid for) rather than by the UK NHS was the difficulty I had persuading the UK NHS to take my rapidly deteriorating condition seriously. But that had been the case since July 2008 when my neurological symptoms first appeared and despite my protest I was advised nothing would or could be done for what GPs, Hospital doctors and the consultant neurosurgeon claimed was sciatica.

The surgery in Hamburg was very successful and my recovery when I returned home was excellent and improving rapidly. I was exercising daily by walking, then running and cycling.

In 2011, I had requested advice from the Scottish Patients Association when second opinion from a local Consultant Neurologist and the Walton Centre Specialist Neurological Trust in Liverpool were less than helpful. Though the NHS Tayside neurologist did persuade his colleague the neurosurgeon to at last change his definition of the symptomatic pseudomeningcele from a "perfectly normal clinical finding following a laminectomy and opening of the dura" as he had since November 2009, to a MENINGOCEL. Though the neurosurgeon did not mention the defect in my the dura necessary for either the Pseudomeningocele or a Meningocele to form, the neurosurgeon did admit that the reason for the presence of the cyst was the 'space' or cavity he had formed over the back of my spinal canal. A 'space' or cavity the neurosurgeon explained he had been unable to fill or close as there was no tissue nearby which he could use to do so on 3 February 2009 before he closed the powerful muscles over the back of my spinal canal and the external wound. I managed to obtain a copy of that letter from the UK Consultant Neurosurgeon and send a copy to Prof. Dr. Westphal in Hamburg, who advised that the UK consultant neurosurgeons claim that he had not only been unable to close or fill the cavity over the back of my spinal canal, was a nonsense as due to the large number of laminectomies carried out for a wide variety of reasons other than neurosurgery very ROUTINE and very NECESSARY methods had evolved. Therefore there was no need for harvesting of muscle from other parts of my body with plastic transportation into the 'space' or cavity as the UK neurosurgeon had claimed in his letter to his colleague the neurologist. That was the final detail I needed to persuade me to accept Prof. Dr. Westphal's detailed formal offer and travel to Hamburg in December 2011 for the surgery completed in January 2012. Following that surgery Prof. Dr. Westphal provided photographs of the larger than expected defect in my dura which had obviously expanded considerably from the initial tiny defect which resulted in the slow accumulation of cerebrospinal fluid that filled the 'space' or cavity over the back of my spinal canal between 3 February 2009 and 12 December 2009. Though both the UK NHS consultant neurosurgeon claimed in letters that the MRI imaging done on 4 June 2009 and 8 December 2009 showed a perfectly normal clinical fining following a laminectomy and opening of the dura. However when I obtained copies of the reports by NHS Tayside Consultant Radiologists on the same MRI images and indeed all radiologists reports on 6 monthly MRI images due between 4 June 2009 and 9 May 2011, I found that all those consultant radiologists had reported NOT a perfectly normal clinical finding BUT a Pseudomeningcele in the Epidural Space impinging upon the posterior column of spinal fluid. And the Consultant Radiologist who reported on the second post surgery monitoring MRI scan done on 12 December 2009, recorded that the Pseudomeningocele was still expanding to fill the 'space' or cavity as the swelling to surrounding tussle subsided.

In my previous posts I mentioned that during the second post surgery clinic in August 2009, I was advised that none of my additional CNS symptoms were neurological in nature and I should return to have my GP reinvestigate my sciatica. NOTE: I was not aware of the existence of the cyst until November 2009 when I acting on advice from Prof. Jallo MD in Baltimore finally persuaded NHS Tayside to release copies of the MRI imaging, though at that time they claimed there were no associated radiologists reports. NOTE In January 2010, Prof Jallo MD confirmed that the MRI software I had forwarded to Baltimore showed a very abnormal pseudmenigcele and not the perfectly normal clinical finding as claimed by the UK NHS neurosurgeon and the CEO of NHS Tayside.

During the August 2009 clinic I was advised that the UK NHS Neurosurgeon claimed my failure to accept his advice, that I had no CNS symptoms following removal of the schwannma tumour and no additional CNS symptoms as from late June 2009, within weeks of the neurosurgeon writing to inform me that the first post surgery monitoring MRI scan showed no signs of problems at the site of surgery indicated I was mentally ill. When I first opened the MRI software in November 2009 and wrote asking why the neurosurgeon had not mentioned the cyst covering the combined length of the T9 and T10 vertebrae and as wide and deep as my spinal canal, the neurosurgeon wrote advising that there had been no need for him to mention that 'fluid' as it was perfectly normal clinical finding and similar MRI images had occurred for all previous patients for whom he had removed spinal schwanomas over the years. In August 2009 my then GP agreed with the neurosurgeon that my failure to accept the advice that I had no CNS symptoms and no additional CNS indicated I was mentally ill. My response was to request psychiatric and psychological assessment and if necessary treatment, when my GP ignored my request I demanded those assessments. Two assessments were done by a Consultant Psychiatrist in December 2009 and two assessments were done by a Clinical Psychologist in 2010. Both the psychiatrist and the psychologist concluded that I had no mental health problems and I was discharged with copies of all three reports on the four assessments. Those reports allowed me to obtain help from the Data Commissioner when the specialist solicitor the Scottish Patients Association had introduced me to as the only way to persuade the UK NHS to release copies of ALL medical files, informed me he could not release full copies of those files to me due to agreements he had been forced to make with the UK NHS. Those three assessment reports allowed the Data Commissioner to persuade the solicitor that withholding copies of the full reports from me the patient concerned would be illegal. The Data Commissioner had initially advised he was powerless to help as the Data Legislation gave special privileges to the UK NHS, which allowed them to prevent the patient obtaining access to his or her medical files other than in edited abstract or summary form if any doctor claimed releasing those files could cause mental health problems.

The result was copies of all the medical files released to my solicitor in 2011, were released to my by my solicitor in 2012. Though my concern was making a complaint to NHS Tayside abut my Neurological problems, when looking through those medical files in 2012, I found reason to suspect that there were problems with my Prostate Gland, despite advice that the gland was supposedly small, smooth feeling and benign. The result of my questions to Urologists being surgical removal of my prostate gland, left nerve bundle and a margin of tissue in December 2013, to hopefully prove the cancer which an MRI scan found was spread throughout my prostate gland and indeed had compromised the left nerve bundle. Had I not obtained those medical files and not pushed the problem forcefully as my experience with my neurological problems had indicated the cancer would have spread to other parts of my body and became inoperable.

The prostate problems took some time to settle and indeed I still have problems but I got back to exercising as soon as possible and since 2014 have progressed to cycling some 150 miles per week and participating in cycling spot rives and Audax (long distance cycling) of up to 150 miles per day. But I still had CNS symptoms which were becoming more and more problematic, though as had been the case between July 2008 and January 2011, I could not persuade GPs hospital doctors or the Constant Neurosurgeon to take my problems seriously.

Then in 2015 an NHS Tayside Public Health Consultant wrote, telephoned and demanded that I sign a document confirming that I had been advised that contaminated instruments had been sued during the surgery to remove the schwanma tumour n 3 February 2009. The explanation being that the decontamination process for surgical instruments used by the UK NHS could not remove certain contaminants. There was therefore a risk that my CNS had been contaminated and any further surgery could pass that contamination to others if the surgical instruments were used again. The written information provided with the covering letter provided details of a specialist clinic based in the University College London Hospitals. So I contacted that clinic and used the advice provided by the Public Health Consultant to obtain a referral to that specialist clinic. The result was referral to UCHL's National Neurological Centre in December 2016. That initial clinic resulted in two further visits to UCHL NCC in December 2016 and confirmation that far from having no neurological symptoms by the consultant neurosurgeon since June 2009, I had a very rare Neurological Condition, Superficial Siderosis. A progressive neurological condition which has quite devastating consequences for patients with that condition.

Superficial Siderosis results from blood contaminating the Cerebrospinal Fluid circulating around the Spinal Cord and the Brain. The result being deposits of Iron on the spinal crd and brain, deposits which attack the nerves leading to problems with the eyes, hearing, balance and control leading to serious complications, defines, blindness and mobility problems.

At present there is only one form of drug treatment which is toxic and perhaps more importantly VERY EXPENSIVE. The UK NHS does not allow prescipition of that drug, though patients treated by the UK NHSand UCHL NCC are prescribed that drug. But not in my case as my condition is described as MILD, whatever that means.

The medical literature on Superficial Siderosis is interesting. The main cause of bleeding which results in Superficial Siderosis is a DURAL TEAR or DEFECT leading to a PSEUDOMENINGCELE.

Yes I have had problems and I have no doubt that many doctors and medical professionals within the UK NHS have been and are part of the problem. But then there are rouges and charlatans in all professions who are only interested in their own interests and progression within their hierarchy. But there are many medical professionals who have helped me, albeit reluctantly at times, but then they have to look after themselves and their families. My battle to survive is not yet over so I will continue to do the best I can to lead a long and happy life doing what I enjoy.

I wish you well and can only advise you not to give in or accept that nothing can be done.

Peace &Love in my case a pseudomeningocele filled the 'space' or cavity formed by removal of bone and muscle over the back of my spinal canal. That pseudomeningocele was described by the NHS Tayside Consultant Radiologists in there reports on 6 monthly monitoring MRI scans, as being in "the Epidural Space and impinging upon the posterior column of spinal fluid". As you will be ware the 'epidural space' is between the exterior of the Dura and the internal walls of the spinal canal (vertebrae). The powerful muscles over the back of my spine did NOT therefore come directly into contact with my spinal cord. Removal of the pseudomeningocele and repair of the defect which the NHS Tayside Neurosurgeon had left in my dura, has hopefully allowed my dura/meninges to return to normal, such that cerebrospinal fluid now surrounds, damps and protects my spinal cord from contact with the internal walls of the vertibrae/spinal canal. The tissue which Prof. Dr. Westpal used to fill the 'space' or cavity which the pseudomeningocele filled between 3 February 2009 and 9 January 2012 was formed to allow the equivalent of the epidural space. Prof. Dr. Westphal advised that there was a possibility that my meninges/dura might remain tethered to my spinal cord due to the three years during which the pseudomeningcele had displaced the cerebrospinal fluid from around my spinal cord. Tethering of the meninges/dura to the spinal cord being one of the neurological deficits mentioned in the medical literature which concluded that where a pseudomeningcele was symptomatic surgical investigation was necessary. Prof. Dr. Westphal also advised that given time he was hopeful that my dura/meninges would re-inflate but if tethering was evident on the next monitoring MRI scan, further surgical intervention might be required to prevent long term complications. I have not seen copies of the monitoring MRI images done on 2 December 2012 or the associated Radiolgists reports, and annually since, but the NHS Tayside Consultant Neurosurgeon wrote advising "the repairs to the pseudomeningocele have been successful" as he has following every monitoring MRI scan since December 2012.

July 2008 Sciatica, re-diagnosed 28 Nov 2008 SCT
Intradural Schwannoma removed by Mr Eric Ballantyne Consultant Neurosurgeon, Ninewells Hospital Dundee, Scotland UK 3 Feb 2009.
Second Tumour notified 19 Mar 2009 confirmed 17 Sep 2009.
CSF Fistual/Pseudomeningcele, dural defect & Cavity due to removal of bone and muscle during laminectomy repaired by Prof. Dr Westphal University Hospital Hamburg 09 Jan 2012.
Superficial Siderosis diagnosed UCLH NNC London Uk 2016/17.

[M. Matkov]

A million thanks for this excellent, comprehensive info. I sincerely appreciate your time, effort & energy. Blessings to you & yours always.