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The answers on this page cover a wide range of issues that couldn't be placed in one particular category. If you're looking for something specific please use your internet browser's page search facility (usually in the top right hand corner of your screen) to search particular keywords on this page or alternatively, browse down the page to learn more about how Superficial Siderosis can affect everyday life and what can be done to help. If you'd like to broaden your search, you may find what you're looking for on another part of the Silent Bleed site - please use the search box in the header above.
Question:
Are superficial siderosis and hemosiderosis the same thing? They were both mentioned on my MRI
Answer:
No. Hemosiderosis is simply “deposition of iron complex” seen by MRI. You can have hemosiderosis in the heart, and other organs. In contrast, superficial siderosis is a specific hemosiderosis of the outside of the brain and spinal cord.
Question:
Are you aware of a specialists in Australia or particularly in Brisbane who might specialise in SS?
Answer:
Unfortunately, no. I do not know any neurologists in Australia who specialize in superficial siderosis. I have been in communication with a Dr. Compton in Epping about a superficial siderosis patient but I don't know if this is his specialty.
Question:
Can a person who is diagnosed Superficial Siderosis and also has Marfan's Syndrome take Ferriprox safely?
Answer:
As far as I know, yes. I have had two other Superficial Siderosis patients with Marfan’s Syndrome.
Question:
Can an intermittent bleed stop on its own?
Answer:
Yes. But in superficial siderosis, the bleeding occurs over years to decades so there is no reason to expect that the bleeding will stop after all that time.
Question:
Can you explain the relationship between iron and ferritin? My iron levels are staying between 10 and 30 and my ferritin is 15. Is that good?
Answer:
Ferritin is a protein that binds to and carries iron around the body. A ferritin of 15 is fairly low and indicates that your circulating iron reserves are low. Maybe due to Ferriprox, if you're taking Ferriprox. That would be expected as Ferriprox depletes total body iron stores as well as depleting the iron salts from the surface of the brain and spinal cord. We follow ferritin levels to make sure that over time it continues to decline indicating effective chelation.
Question:
Can you see the difference between new iron deposits and old iron deposits on an MRI scan?
Answer:
Unfortunately, no.
Question:
Care home. Hi, a good friend of mine has superficial siderosis and I’m desperate to find a care home for him in Norfolk or possibly Sussex. Can you recommend any? He is fortunate enough to have funds available to pay for his care but is currently in an NHS care home for mentally ill patients in South London. He would probably need to be assessed prior to a move. Covid hasn’t helped with trying to get him moved. Any advice would very welcome, we’re desperate to get him moved from a facility that we don’t think caters specifically enough for his needs.
Answer:
I have to admit that I have only been to London twice in my life. I truly enjoyed my visits and would love to visit more of the UK someday! I don’t know how to advise you about your friend with superficial siderosis.
Inosine
Question:
Cerebral Spinal superficial siderosis. Hi, my husband and I have been trying to find help for three years. We have been to countless doctors including the Mayo Clinic in Minnesota, looking for help. He had surgery in December and his most recent MRI shows the surgery was not a success. Looking for any amount of help we can find.
Answer:
Dr. Kumar at the Mayo Clinic in Rochester, MN, and I are the only two neurologists who see patients with superficial siderosis in the US. Dr. Kumar’s expertise is in the surgical approach to finding and stopping bleeds. I can’t imagine a more experienced team. But I also have neurosurgery colleagues at Johns Hopkins and UCLA who I’ve worked with before. My area of expertise is in the non-surgical management of superficial siderosis although I do refer patients to surgery when they really need it.
Question:
Checking our children for Marfan's syndrome. Hello Dr Levy. You may recall I sent you a message asking 'When I was 12 I had a brain aneurysm, my sister had a stroke a year ago, could weak blood vessels be hereditary?’ to which you replied ' Marfan’s syndrome is one such disorder of blood vessel walls that can be leaky and cause aneurysms and superficial siderosis.' As we both have children, should we get them checked for Marfan’s syndrome?
Answer:
Marfan’s syndrome is not a difficult diagnosis to make. Although it’s a genetic illness, there are many physical features of Marfan’s that should be evident on physical exam. There are other genetic causes of ‘weakened’ blood vessel walls but I’m not really an expert in this area. If you are keen to investigate further, I recommend a visit with a medical geneticist.
Question:
Dear Dr Levy, I am glad I found this site and the possibility to contact you. My husband, 51 years old, became the diagnosis Superficial Siderosis 2 years ago. This is his story. My husband has a history of headache for decades. He's had many scans, MRI's and examinations during this period and 2 years ago, after a scan of his head because of hearing loss, by coincidence, a radiologist found iron on his small brains and brainstem. They took a scan of his back and the conclusion was a paraganglioma in his back, between the third and fourth lumbar vertebrae. (it am not sure about this, it could also be between the fourth or fifth). In September 2014, the tumor was removed at UMC Utrecht and it was a benign paraganglioma. During the operation, the surgeon made by accident a hole in the dural sac. Because of this my husband had water in his head but according the surgeon this was not severe and would be gone within a week. A week after the surgery, my husband was already at home, he developed such serious headache and we returned to the UMC. They found nothing and sent my husband home. This was the beginning of the nightmare. Every 2 weeks my husband is ill because of headache attacks and lies in bed for 3 or 4 days. Besides heavy headache he's sick, with vomiting and has a strange pressure in his head. Between the attacks he has headaches, but is able to go to his work. He now works only 4 hours a day and my husband was always busy with his hobby, cycling and now he just goes to his work but at home he is lying on the couch because he is exhausted. Our lives have changed dramatically and the world is getting smaller for us. The neurologist said to us that the paraganglioma caused the bleeding in the subarachnoid space, but the neurosurgeon denies this. What should we believe? Other symptoms of my husband: bad balance, bladder problems with blood in his urine, intestinal problems, dizziness, extreme tiredness, hearing loss, extreme sweating and high heartbeats. The neurologist of UMC Utrecht did not know what to do anymore, and referred us to the neurologist of the Radboud UMC Nijmegen for a second opinion. The conclusion there was clear: superficial siderosis. But it added nothing for us. There was no solution.
My husband tried so much medication, nothing works. The last medicine, Depakine, does also not work and last Friday my husband visited the neurologist at the Radboud and she sent him home and told him that she could not help him anymore. We are very desperate and frustrated about this. Since yesterday my husband is ill again. Besides the SS, my husband is under treatment by a endocrinologist at Radboud UMC, because he had a paraganglioma they are looking for other tumors (feochromocytom). His upper body is clean of tumors, the adrenaline and noradrenaline are ok and now we are waiting for the result of the MRI from his shoulders till his ears. There is planned a genetic research (exome sequencing) , this endocrinologist investigates the case of my husband very thoroughly. Concerning the SS, I have read your site and have some questions. 1 What is the prognosis of SS? 2. Is surgery possible to remove iron from brains? According the doctors in Holland this is not possible 3. I have heard about the medicine Ferriprox, but the doctors in Holland are negative about this medicine. What is the result of this medicine? 4. Is cannabis or weed treatment positive for the severe headaches? 5. Do you know what happened with in the body that my husband constantly has this headache attacks? Thank you that I can tell my story and hope that you are able to give a reaction to us.
Answer:
Prognosis of superficial siderosis depends on many factors including the extent and degree of iron deposition, age, and vulnerability of the underlying brain tissue. Generally, progression is linear – which means you will likely continue to worsen at the same rate over time.
It is not possible to surgically remove iron deposits off the surface of the brain.
We are still working to demonstrate a benefit using Ferriprox but what I can tell you is that some people respond well to it and show reduced iron deposits on the brain by MRI. The key unanswered question is, does that translate to improve neurological function? That’s what we're trying to answer.
Cannabis is useful for treatment of many forms of pain. It's probably not too harmful to try it for headaches.
I suspect the paraganglioma is responsible for the superficial siderosis but the headaches are not due to siderosis. They are due to either bleeding or CSF leak. Bleeding headaches are usually not-positional (it hurts in any position), associated with fever and stiff neck. CSF leak headaches are classically positional which get worse the longer you stand and get better the longer you lie down.
Question:
Degree of progression. Is there any other procedure beside MRI or observable symptoms to determine the extent of the progression of the disease? For example, monitoring the level of ferritin. Also, can hemosiderin be monitored? Can free-radical iron molecules be numbered and monitored?
Answer:
Great question. We’re working on that. Currently, there are no biomarkers in superficial siderosis. Ferritin in the spinal fluid (not the blood) has been proposed but we haven’t studied that yet. Ferritin in the blood is not a useful biomarker. It’s only useful to monitor chelation by deferiprone.
Question:
Delirium/Dreams
My 80 year old father was diagnosed with SS about 10 years ago and now is confined to a wheelchair. He has started having vivid dreams which when woken can take a little time to realise reality from dream. But on other occasions he is delusional, hearing and seeing things and unable to reality from fiction. Is this potentially the next step in SS?
Answer:
I have had two patients with audio hallucinations but delirium is unusual in superficial siderosis. I would look into other potential causes.
A: Superficial siderosis is very, very rare and most doctors have not heard of it. If your doctor is interested, they are more than welcome to contact me by email and I can advise them: mlevy11@mgh.harvard.edu.
Question:
Do you know of a neurologist in Britain that specializes in SS?
Answer:
I don’t know any that have a particular interest in SS.
NOTE FROM THE SILENT BLEED TEAM: We are in contact with Dr Werring at University College Hospital , London who is involved with research into Superficial Siderosis. Dr Wren based at St George's in Tooting and Frimley Park hospital also has an interest.
Question:
Do you think there is a genetic component? My friend's mother has SS. Her neurologist was unable to answer the question about whether this can be genetic. Please can you advise?
Answer:
We are actively engaged in a study to determine if there is a genetic component to superficial siderosis. This project is being spearheaded by a group in London, UK, and we are participating.
Generally I'd say there is no known genetic component. There are some conditions that can increase the risk of superficial siderosis like Marfan's disease but the chance that any two related people would both sustain an injury that leads to chronic bleeded into the spinal fluid is exceedingly unlikely.
Question:
Doctor search. Hello, my mother has recently been diagnosed with superficial siderosis. We are trying to find a doctor that has knowledge of this condition. Do you have any recommendations in the Midwest?
Answer:
Dr. Raj Kumar at the Mayo Clinic in Rochester, MN, is a world expert in superficial siderosis.
Question:
Dr Levy, my neurologist in Auckland NZ rang today and asked me how do you find the source of the brain bleed? They did an MRI and told me 2 years ago that it was coming from my brain stem. That sounds right to me as when I am going to have a bleed it burns and is really sore in that area. Can you help me? Thanks a lot for all your help.
Answer:
If the bleed is most likely in the brainstem, a CT myelogram will not help because the dye only goes up to the neck and not the brainstem. There is no test that can identify a bleed in the brainstem. It’s probably not worth an exploratory surgery of the brainstem unless it’s very obvious that is the source of the bleed for example because of a previous surgery in that area.
Question:
Hello, and thank you for offering to take questions. I can only imagine how busy you must be. I wrote once before, but that question may have been a bit too long and rambling, so I'd like to try something more specific. Please feel free to edit as needed for your use. I've just recently been diagnosed, via brain MRI, as having Superficial Siderosis along the left cerebellopontine angle. My current neurosurgeon read that report, looked at the images and shrugged it off, saying it was very minor and "nothing to worry about". While I've had at least 5 Brain MRIs in the last ten years, this is the first to receive this diagnosis. But, my question is this: when I received this diagnosis, my first thought was that it must be due to a brain bleed I had right after having a Posterior Fossa Meningioma removed. I had a heart attack while in recovery, and treatment for that contributed to the cause of the bleed. BUT and this is where I don't understand, during my first follow up with one of the two neurosurgeons who did my surgery, I asked why it was called a bleed and not a stroke. He replied that a true stroke takes place within the brain, beyond the Meninges and Arachnoid spaces/layers. My bleed had been outside the protective layers around the brain, and placed great pressure on the brain - hence my EVD, hydrocephalus, Tonsillar herniation, etc. So, what I don't understand then is, how would I get SS within these protective layers, if the only known bleed I've had had been outside these layers? My only thought is that this would suggest that there are/have been other bleeds of some sort, inside the protective layers around the brain. Is this logical thinking, or is there some medical/anatomical answer that I may not have the education to consider on my own? I hope this is a valid question for you and look forward to your reply.
Answer:
You can think of three layers of the central nervous system. There is the brain tissue itself. Then there is a lining around the brain, which is made up of three individual linings: the pia, the arachnoid and the dura. And then there is the skull. A bleed in the space between the skull and the dura is called an epidural bleed. A bleed in the space between the dura and the arachnoid is called a subdural bleed. A bleed in the space between the arachnoid and the pia is called a subarachnoid haemorrhage. This is where the bleed is in patients with superficial siderosis. There is no space between the pia and the brain – they are stuck together. A bleed inside the brain is called a haemorrhagic stroke.
Question:
Help in the UK. Hi I was diagnosed recently with superficial siderosis, caused by a bleed following a brain tumour. I am really struggling to see any doctor or consultant who even has the basic knowledge of SS. My current consultant just brushed off any effects I have like loss of balance lower back pain. One year on, I have seen no one and it's a fight to even try to get help.
Answer:
My collaborator in the UK, Dr. David Werring at Queens Square, London, is very knowledgeable about SS. He is also a friend and a good person.
Note from the Silent Bleed Team - there are now a few consultants around the UK who are engaging with treatment and management of superficial siderosis. Members of our Facebook Group (search The Silent Bleed - Superficial Siderosis) are always happy to share their experiences and local knowledge. We've also met David Werring in London and endorse what Dr Levy has to say about him.
Question:
Hi Dr Levy, I have been to see the neurologist in NZ. They have told me that the front of my brain is also bleeding as well as the cerebellum. The tear I have is in the brain stem and bottom of the spinal cord. They don't seem in a hurry to do anything about it, even tho I sent you my lumbar puncture results, which they have. I would like to be on Ferripox like you suggested, you also suggested that they stop the source of bleeding. Over here they don't see the need to do this. Is there anyway I can ask them for the help I need. I bleed twice a week. Also does every one with SS have aphasia and speech problems? Thanks for your input it is great
Answer:
Aphasia and speech problems is common SS because of siderosis around the brainstem. I’m really not sure how to advise you regarding your country’s healthcare system but it’s worth pushing for your treatment as you are your best advocate.
Question:
How are SS and ALS similar yet different?
Answer:
Superficial Siderosis is a condition of brain and spinal cord iron overload that causes toxicity to tissue exposed to the iron. The most common areas affected are the nerves going to the ears causing hearing loss, the cerebellum causing balance problems, and the spinal cord causing weakness, numbness and bowel/bladder problems. ALS is a neurodegenerative condition that selectively targets the motor nerves in the brain and spinal cord. About 10% of cases are familial (genetic). It causes weakness and death within a few years. They are very different diseases in my view
Note from the Silent Bleed team: ALS or Amyotrophic lateral sclerosis is also known as motor neurone disease (MND).
Question:
I had a big bleed four weeks ago. I bled for two and a half weeks, through the bleeding I had a lumbar puncture which showed large amount of blood, these are the neurologist's words. I told the doctor doing it that I would have lots of blood owing to knowing I was bleeding. My neurologist wants another Lumbar puncture done again, the third with blood in it as she feels that I was pricked. I asked her if I could have a myelogram and if the source of bleeding could be found so as they could repair it. She has declined my request and just wants to know if another lumbar puncture will have blood in it. I am sure it will as I have a bleed every week to ten days. I know because my neck is so stiff I can't turn my head, my headache feels like it is going to explode and my balance and speech go out the door. I have to just sleep. Please can I have an input to as to where I should go and what I should say to the neurologist. I would like the bleeds slowed down as they really drain me.
Answer:
You can tell your neurologist that a CT myelogram starts with insertion of a needle into the lumbar space. She can obtain spinal fluid first. And then proceed with the myelogram. Ultimately, you are probably right about your condition. I think you are bleeding every few days as you say, and that’s causing your symptoms. You might have to find a new neurologist who will proceed with the CT myelogram.
Question:
I had an MRI and Lumbar puncture on Monday 18th August. The results haven't been printed yet, but the Drs who were watching the MRI said they hadn't seen blood in the cerebellum ever like I have got it. They did a head to tail bone MRI and the bottom of my spine has shown something is not good down there. Can SS affect the spine as well? As I have a lot of pain down my back and also my bowel is leaking and my bladder is either going all the time or only empting 6%. This seems to happen when I have had a bleed and get the vasospasms. Is this all connected to the bleeds and SS? I had a brain bleed while having the lumbar puncture, they thought it was at first from the lumbar puncture, but it may have caused something. They kept me in hospital and my speech went completely like a stroke victim, that is what happens every time I get a bleed. Unable to understand my speech at all. Would love your input into this as I am waiting to go to Auckland City Hospital Neurologist for help.
Answer:
All superficial siderosis patients are susceptible to spinal cord involvement. The spinal fluid is made deep in the brain and circulates around the brain and the entire spine. Bleeding into the spinal fluid allows the iron (from inside blood cells) to circulate around both the brain and spinal cord. When you bleed into the spinal fluid, the fresh blood irritates both the lining of the brain which causes neck stiffness and fever, and the blood vessels around the brain which causes neurological dysfunction similar to stroke. To achieve this level of irritation, there usually has to be quite a bit of blood – on the order of 20,000 red blood cells per millilitre of spinal fluid.
Question:
I had yet another lumbar puncture, two in a row. The first one showed lots of blood in the vials, the second one showed large amounts of blood, the doctor doing it had to change the needle as it was clogging. The results showed no evidence of bilirubin, despite the large amounts of blood. I am writing what my neurologist wrote. She has discussed this with a Neuradiologist. The plan is to do another CSF again under the guidance of this doctor. Given it is the third attempt to answer questions regarding presence of persisting intracranial hypotension and presence of blood. I asked if I could have a myleogram and they aren't interested in finding the cause. I have written to you before and told you how my neck gets so stiff I can't turn my head and my neck stem burns and is very sore. you suggested that the tear maybe in my brain stem. I am now also experiencing the same pain down my whole spine and bad down the lower spine. My neurologist says given the stability of the findings on neuroimaging, that she would need to have more reason to pursue this. She has decided to take one step at a time and not do the myleogram. She has asked for a CSF pressure measurement and sequential tube collection for red blood cells and the presence of xanthochromia performed at the radiology. I don't understand the last paragraph, what is that. What are your thought on this letter and how would you approach it? I sometimes feel that my neurologist is lost and doesn't know what to do. In the meantime, my health is deteriorating. I haven't go a lot of energy and sweat a lot even when it cold. I have developed angina as well. I am not doing very well at all.
Answer:
I suspect they did not actually insert the needle into the lumbar space where the CSF is. That’s probably why your neurologist wants to do this with neuroimaging to guide the needle. Bilirubin is the iron product from red blood cells that break down in the CSF. Xanthochromia is the “yellowness” from bilirubin.
Question:
I have been taking Ferriprox for twenty months and my last MRI showed no change. Would an intermittent bleed need to be found? Is it likely the bleed could become a bigger bleed?
Answer:
Iron from active bleeding is much easier for Ferriprox to chelate than iron bound to hemosiderin already stuck to your brain. If you are seeing no progress by MRI, my only suggestion is to keep trying it. I’ve seen only a rare few cases that did not improve by MRI eventually (4 years or more in some cases).
Your bleed hasn’t changed in 15-40 years. It’s unlikely to change now unless you do something traumatic or surgical.
Question:
I have mild superficial siderosis and have been on Ferriprox for three years. I have just received my latest scan results and it shows no change. I do a lot of exercise, mainly free weights and low impact cardio and I walk my dog every day. Could the frequent change in blood pressure close an intermittent bleed? Could my workouts increase a bleed and could the Ferriprox be just working on new deposits?
Answer:
To answer your first question, it depends on the source of the bleeding. Exercise and exertion may cause re-bleeding if there is shearing pressure on the bleeding site, but I don't think it's due to changes in blood pressure. In answer to the second, Ferriprox is very good at chelating fresh blood. I think there is plenty of Ferriprox to chelate both the fresh blood and the continue to work on old iron deposits.
Question:
I recently viewed your webinar and learned a lot more. Can you explain a bit more about neurological reserve and the effect that SS can have on that. My OF (old fella) is often running on what I call a flat battery.
Answer:
“Neurological reserve” is your brain’s capacity to deal with stressors, including metabolic and psychological stressors. Everyone has a breaking point where the neurological reserve is depleted and dysfunction ensues. In the young and elderly, the neurological reserve is low so even an infection can cause them to become delirious. In SS patients, the neurological reserve is low too. So when considering surgery and anaesthesia, which are significant metabolic stressors, expect to have a prolonged recovery phase. Other conditions that depend on neurological reserve in SS patients are infections, change in medications, poor circulation and diabetes.
Question:
I recently went for a follow up MRI for arachnoiditis post subarachnoid haemorrhage/intraventricular haemorrhage . My new diagnosis is siderosis of the lumbosacral spine. Have you ever seen arachnoiditis and siderosis occur together in the spine? Are the physical pain symptoms the same?
Answer:
Yes, superficial siderosis and arachnoiditis co-occur. When bleeding occurs in the spinal column, the fresh blood triggers an inflammatory reaction in the lining around the spinal cord. That process is known as arachnoiditis. Symptoms of arachnoiditis all come from dysfunction of nerves trying to pass through the inflamed lining to the lower abdomen, lower back, pelvis and legs. It’s also very painful. Arachnoiditis is treated using anti-inflammatory medications.
In contrast, superficial siderosis is caused by the long term consequences of chronic or intermittent bleeding over a long time. In response to bleeding, the brain and spinal cord produce a protein called ferritin to chelate and neutralize the free iron from the blood breakdown products. This product of ferritin and iron is called hemosiderin and it is insoluble. It is unable to be filtered back to the bloodstream so it collects in the spinal fluid. By gravity, it collects around the spinal cord when you’re awake, in the back of the brain when you sleep on your back and in the front of the brain when you sleep on your tummy. For the most part, hemosiderin is effective at suppressing the damage caused by free iron but over years and decades, the iron can escape from time to time and cause damage to the brain/spine tissue underneath. The areas most vulnerable to iron damage are the nerves going to the ears causing hearing loss, the nerves in the balance center of the brain (cerebellum) and the nerves in the spinal cord causing weakness, numbness and bowel/bladder/sexual dysfunction. We treat superficial siderosis by stopping the bleeding and chelating the iron out of the brain with iron chelating medications.
Question:
I watched the NBC Dateline program on Sunday (6/7/15) which was about the use of cannabis oil. The show focused primarily on children with epilepsy and neurological conditions. It is believed cannibis has helped or could help if it were more readily available. A neurologist stated that it was her opinion that cannabis could help in people with MS. Since SS mimicks MS in some areas. What is your opinion about it's use in SS? How difficult is it to get for medical use? Is Johns Hopkins planning any research on the use of cannibis oill?
Answer:
Cannabis would be expected to help with pain (neuropathy) and spasticity. For those SS patients with neuropathy and spasticity, cannabis would be expected to help. Unfortunately, no, we have no plans for testing cannabis at Hopkins.
Question:
I watched the NBC Dateline program on Sunday (6/7/15) which was about the use of cannabis oil. The show focused primarily on children with epilepsy and neurological conditions. It is believed cannibis has helped or could help if it were more readily available. A neurologist stated that it was her opinion that cannabis could help in people with MS. Since SS mimicks MS in some areas. What is your opinion about it's use in SS? How difficult is it to get for medical use? Is Johns Hopkins planning any research on the use of cannibis oill?
Answer:
Cannabis would be expected to help with pain (neuropathy) and spasticity. For those SS patients with neuropathy and spasticity, cannabis would be expected to help. Unfortunately, no, we have no plans for testing cannabis at Hopkins.
Question:
I'm helping a loved one to find a neurologist who has experience with treating superficial siderosis near VA and NC line. Area from Roanoke VA to Greensboro NC line, any advice?
Answer:
Superficial siderosis is incredibly rare. The only two doctors I know of who routinely treat superficial siderosis are me and Dr. Kumar at the Mayo Clinic. However, any neurologist can learn about superficial siderosis and consult us for advice.
Question:
If I have an intermittent bleed is there anything I should not be doing? I work out doing weights, shadow boxing, low impact cardio, Zumba and yoga but have done since before I started Ferriprox and my MRIs have shown no deterioration.
Answer:
Your MRI will not show deterioration because the timescale is too short. You would have to compare an MRI 4-5 years from now to see a deterioration. And you would not necessarily be able to correlate a change in that MRI to a few things you are doing now. So it’s not useful to use MRI as a measure of whether something is harmful.
The best way to know if these activities are leading to bleeding is to do the activity vigorous a few times and then get a lumbar puncture. If you see fresh blood in your spinal fluid, you have your answer. If your spinal fluid is clear, you’re probably OK doing those activities.
Question:
If ferritin levels were very high, it would tell if I had an active bleed, is that right?
Answer:
No, not at all. Ferritin is a measure of total body iron. It is measured in the blood. If you have a bleed in the brain, it will not be reflected on the level of ferritin in the blood. The other thing to know about ferritin is that when you have an infection or acute metabolic problem (pancreatitis, for example), your body makes a lot of ferritin. The ferritin is produced to bind up all of the free iron and deprive bacteria of any. So sometimes if you check your ferritin level, you may notice that it suddenly increased markedly. For the purpose of monitoring ferritin as measure of total body iron, just disregard those occasional increased values.
Question:
Inosine is available OTC. What would you advise people like me who are tempted to order and try it out on their own?
Answer:
Inosine was shown to be neuroprotective in Parkinsons disease and has nearly no side effects. The hexaphosphate form of inosine (IP-6) is also a weak iron chelator. I think its worthwhile to consider this supplement after a discussion with your doctor.
Question:
Intracranial Hypotension.
I'm currently suffering from both superficial siderosis and intracranial hypotension. Do you know whether there is any link between the two conditions and whether the interaction between them is of any significance?
My main curiosity is that could it be possible having low exacerbates the deposition of Hemosiderin within the CNS?
Answer:
Yes, superficial siderosis and intracranial hypotension are tightly associated. Both conditions are commonly due to a tearing in the lining around the spinal cord. A tear in the lining leads to a leak of spinal fluid. One of the functions of spinal fluid is to provide buoyant support to the brain. If spinal fluid is leaking out, the support is reduced and the brain literally drags along the base of the skull. When you lay down, you should feel better as the brain is no longer dragging. A tear in the lining around the spinal cord is the most common site of bleeding in superficial siderosis. With repeated tearing, the scar tissue becomes friable and bleeds easily. So from a single tear, you can develop intracranial hypotension from the spinal fluid leak and, in the long term, superficial siderosis from the bleeding.
Question:
Is the ferritin levels in CSF different from blood levels, if so, how?
Answer:
Ferritin is not measured in the CSF. Normally, there should be no ferritin in the CSF because there should be no iron in the CSF. In siderosis patients, ferritin is made and secreted into the CSF to sop up the free iron released by blood cells. We don’t usually measure the level of ferritin so I would not have a sense of normal or abnormal values if we did.
Question:
Is there likely to be CSF leak at the location of the bleed as I am currently suffering from low ICP which was confirmed by intracranial pressure monitoring?
Answer:
Yes, absolutely. The site of bleeding in the spinal column is usually a dural tear where spinal fluid leaks out too.
Question:
Knee surgery and superficial siderosis. My mother has recently been diagnosed with ss. She also is due to have a knee replacement in the next month. Can the knee surgery have any complications with the SS and vice versa? We have not been given any information apart from told to give the MRI to the consultant who is operating which we have. Just a bit wary of going ahead with surgery if it's going to make the SS worse.
Answer:
If my SS patients need surgery, I generally approve it. The only issue is that anaesthesia can take a lot longer to wear off and balance can be worse for several times after surgery. However, the anaesthesia will eventually wear off.
Question:
My 40 year old son has just been diagnosed with SS. He is totally deaf, manages a little bit of walking with a frame and has no smell or taste. He also has bladder and bowel incontinence problems. Short term memory loss and he has a dural ecasia which had made him very uncomfortable. He has Marfan's Syndrome and we think the bleed on the brain was caused by taking Warfarin. He had a rod fitted to his back for his curved spine when a teenager. This rod recently has caused his thinning skin to bleed and has become infected. He was rushed to theatre last week and the rod was removed. i am wondering about the dural sac at the base of his spine. How would it affect this? He says he can taste a few things now, could some of his symptoms have been from damaged nerves to the spine?
Answer:
This sounds more complicated than the usual case of superficial siderosis because we’re also dealing with Marfan’s and previous neurosurgical procedures.
One thing I can tell you is that nerves in the nose that connect directly to the brain can regenerate. So often my patients will regain their ability to smell when the iron deposition decreases.
Question:
My boyfriends sister is 31 and has been diagnosed with iron on the brain and early onset Parkinson's. She has a beautiful one year old little boy and her symptoms are memory loss, falling or tripping and tiredness - she is deteriorating quickly. Is there anyone out there that has this very rare condition and can give us advice on how to treat her illness or give us advice on what we can do? She has seen consultants at Salford Royal and Liverpool hospitals as well as being seen privately but it is so rare there is very little known about it and all different kinds of medication.
Answer:
It's unusual for someone as young as 31 to have such severe symptoms of superficial siderosis unless the iron deposits have been present since birth. For example, there are some babies who require brain surgery and develop chronic bleeds a result. Unless your boyfriend's sister falls into this category of patient, I am a little suspicious of the diagnosis. I wonder if there are other issues that may be contributing to her neurological symptoms.
Question:
My dad My dad has this disease, the doctors in South Carolina don't know anything about this. And my dad is getting worse by the day. He is having double vision when he looks to the left and was hospitalized for 3 days for that. He keeps having episodes of non-stop vomiting. He has had to retire and can't walk with out a walker or sometimes has to be in a wheel chair. He is losing weight like crazy! The doctors here don't know what to do for him. He had cryptococcal meningitis about 7 years ago and almost died, now this. I want to get him to you if you are seeing patients?. He has blue cross blue shield and they said they will not pay outside our state. I'm afraid he will die if we can't find a doctor that knows about this.
Answer:
This is very frustrating. I have a neurology colleague in South Carolina named Jay Avarasala. Check to see if your father can see him. We communicate on a regular basis.
Question:
My husband's neurologist has told us that that there is a difference regarding the brain and meningeal siderosis, not systemic. He was not sure Ferriprox would be effective or even work in the brain which is not truly an iron overload. I didn't realize there was a difference. Is there nothing we can do for meningeal siderosis?
Answer:
Meningeal siderosis and superficial siderosis are the same thing. They refer to iron overload confined to the central nervous system. There are other iron overload conditions that lead to iron overload in the entire body, called systemic iron overload disorders. Ferriprox along with the other 4-5 iron chelators are all good for systemic iron overload disorders, but Ferriprox is the only one that can get in to the brain so it’s the only one we’ve tried for superficial siderosis.
Question:
My mom was diagnosed with SS over a year so. She is 77 years old and shows few, if any, of the symptoms. She is currently receiving no treatment as there is nothing to treat. My question, should she be taking proactively preventative medication like those I've seen others on this sight post about? Also, the cause of my mom's SS is undetermined as she has never had trauma or surgery. Is there any evidence that the condition is hereditary? My grandmother suffered from dementia which makes me question any connections. I wonder if an MRI would reveal anything for me. Thank you, Ontario, Canada
Answer:
With so few symptoms, I usually do not recommend treatment with Ferriprox unless the patient is young. The reason for the age difference is that young people can tolerate the burden of using Ferriprox and have lots of years to appreciate the potential long term benefit. There is no known case of familial siderosis (yet). We do not know any genetic predispositions except possibly Marfan’s syndrome. I don’t think you need an MRI with this family history.
Question:
My son has recently (six months ago) been diagnosed with SS and displays unsteadiness but no hearing impairment. We are having difficulty here in Australia finding a neurologist that can/will suggest any treatment options at all. Is there someone that you know of in Australia that can help us please? The last nuero we saw we asked about Ferripox but his answer was wait until you're deaf and then it may be worthwhile, obviously we would prefer to start treatment options to try and prevent or delay the onset of more severe symptoms.
Answer:
No, I’m sorry but I don’t have any colleagues in Australia with an interest in SS.
Question:
My zinc levels are a little low, 9.7 this month. How low can they be before it's a concern and any ideas for increasing zinc without increasing iron?
Answer:
When my patient's zinc levels get a little low, I recommend taking a 1 week break from Ferriprox and taking over-the-counter zinc tablets. The zinc level will bump up quickly
Question:
Nutritional yeast. Dr. Levy, I wanted to ask your opinion of nutritional yeast as a dietary supplement for SS patients. My father suffered from Guillain Barre Syndrome in the late 70s that put him in the hospital for 8 weeks. As a part of his recovery, my mother introduced him to nutritional yeast, and he accredits nutritional yeast for the totality and speed of his recovery. It is my understanding that nutritional yeast has a positive effect on the CNS. Are you familiar with nutritional yeast and what is your opinion? Thank you. I look forward to hearing your opinion.
Answer:
Honestly, I do not know if nutritional yeast has any benefits. My first thought is that we consume lots of yeasts from bread products but I am not an expert in nutrition and there may be other factors that I am not aware of. I can certainly say that it’s not harmful to consume.
Question:
Should I be on Ferriprox? I had brain surgery and have cavernoma and have a bleed on the brain. I' waiting to hear back from my neurologist.
Answer:
A one time bleed from a cavernoma may not cause superficial siderosis. Bleeds in the brain from all causes are very common, but superficial siderosis is very rare. We posit that superficial siderosis arises from repeated bleeding over a long period of time.
Question:
Some years ago, through a doctor in Seattle, you diagnosed that I have Superficial Siderosis. Things have gone on like I was told, even being in a wheelchair. But now some of my teeth are turning black, and so far one of them has split in half. Is this happening because of the siderosis?
Answer:
Superficial siderosis doesn’t cause teeth to turn black. I suspect that is unrelated to the siderosis.
Question:
Stroke Dr Levy, is it true that strokes are more likely with SS patients? If so what kind? So far, I’ve seen 4 strokes in my 75 patients with superficial siderosis. All 4 patients were older than 65, but they were all ischaemic strokes (the non-bleeding type) in different parts of the brain.
Answer:
I can’t say for certain that strokes are more common in SS.
Question:
Stuttering and forgetting words
My son has a new symptom he's started stuttering when talking, and he looks confused, he's had SS for 21 years, and is quite advanced is this SS related, he's also had 6 strokes as a child.
Answer:
It’s possible that this could be related to SS. If SS involves the language centers of the brain, I suppose this could happen. It could also be due to damage from the previous strokes. Or maybe a combination. The other possibility to consider is a small seizure related to the previous strokes. The reason I raise that possibility is that after small seizures, people feel confused.
Question:
Treatment of superficial siderosis.
I have a diagnosis of ss based on two MRI's of this past March and June. I have a doctor probing in the dark and still guessing. I know you are an expert on this. How do I treat this? I am in sad shape because of it.
Demyelinating
Is Superficial Siderosis classed as a demyelinating condition? And would immunoglobulin therapy be helpful for the condition as it is used for other demyelinating neurological conditions?
Answer:
Superficial siderosis is not demyelinating, no. It does not destroy myelin. The first cells to die from iron toxicity are supportive cells called astrocytes. They are trying to buffer the excess iron and become overwhelmed and die. They can be replaced but over time, the ability to replace these cells declines. When supportive cells disappear, the brain cells and myelin-producing cells are left unsupported and eventually die out.
Treatments that target the immune system like immunoglobulin therapy are not helpful in superficial siderosis. There is no role for any immunological medications.
Question:
if a stroke patient can be rehabilitated to use their arms and legs or other body parts damaged from a stroke, is there a possibility that we as SS patients can retrain our brains to regain balance and muscle function?
Answer:
Yes! Rehabilitation takes advantage of your brain’s ability to generate new circuits and learn new tricks. Whether your damage is from stroke, trauma or siderosis, this phenomenon holds true. I highly recommend physical therapy, occupational therapy, vestibular therapy or whatever other targeted therapy you need to improve your neurological function.
Question:
Checking our children for Marfan's syndrome. Hello Dr Levy. You may recall I sent you a message asking 'When I was 12 I had a brain aneurysm, my sister had a stroke a year ago, could weak blood vessels be hereditary?’ to which you replied ' Marfan’s syndrome is one such disorder of blood vessel walls that can be leaky and cause aneurysms and superficial siderosis.' As we both have children, should we get them checked for Marfan’s syndrome?
Answer:
Marfan’s syndrome is not a difficult diagnosis to make. Although it’s a genetic illness, there are many physical features of Marfan’s that should be evident on physical exam. There are other genetic causes of ‘weakened’ blood vessel walls but I’m not really an expert in this area. If you are keen to investigate further, I recommend a visit with a medical geneticist.
Question:
Intracranial Hypotension.
I'm currently suffering from both superficial siderosis and intracranial hypotension. Do you know whether there is any link between the two conditions and whether the interaction between them is of any significance?
My main curiosity is that could it be possible having low exacerbates the deposition of Hemosiderin within the CNS?
Answer:
Yes, superficial siderosis and intracranial hypotension are tightly associated. Both conditions are commonly due to a tearing in the lining around the spinal cord. A tear in the lining leads to a leak of spinal fluid. One of the functions of spinal fluid is to provide buoyant support to the brain. If spinal fluid is leaking out, the support is reduced and the brain literally drags along the base of the skull. When you lay down, you should feel better as the brain is no longer dragging. A tear in the lining around the spinal cord is the most common site of bleeding in superficial siderosis. With repeated tearing, the scar tissue becomes friable and bleeds easily. So from a single tear, you can develop intracranial hypotension from the spinal fluid leak and, in the long term, superficial siderosis from the bleeding.
Question:
I recently went for a follow up MRI for arachnoiditis post subarachnoid haemorrhage/intraventricular haemorrhage . My new diagnosis is siderosis of the lumbosacral spine. Have you ever seen arachnoiditis and siderosis occur together in the spine? Are the physical pain symptoms the same?
Answer:
Yes, superficial siderosis and arachnoiditis co-occur. When bleeding occurs in the spinal column, the fresh blood triggers an inflammatory reaction in the lining around the spinal cord. That process is known as arachnoiditis. Symptoms of arachnoiditis all come from dysfunction of nerves trying to pass through the inflamed lining to the lower abdomen, lower back, pelvis and legs. It’s also very painful. Arachnoiditis is treated using anti-inflammatory medications.
In contrast, superficial siderosis is caused by the long term consequences of chronic or intermittent bleeding over a long time. In response to bleeding, the brain and spinal cord produce a protein called ferritin to chelate and neutralize the free iron from the blood breakdown products. This product of ferritin and iron is called hemosiderin and it is insoluble. It is unable to be filtered back to the bloodstream so it collects in the spinal fluid. By gravity, it collects around the spinal cord when you’re awake, in the back of the brain when you sleep on your back and in the front of the brain when you sleep on your tummy. For the most part, hemosiderin is effective at suppressing the damage caused by free iron but over years and decades, the iron can escape from time to time and cause damage to the brain/spine tissue underneath. The areas most vulnerable to iron damage are the nerves going to the ears causing hearing loss, the nerves in the balance center of the brain (cerebellum) and the nerves in the spinal cord causing weakness, numbness and bowel/bladder/sexual dysfunction. We treat superficial siderosis by stopping the bleeding and chelating the iron out of the brain with iron chelating medications.
Question:
Help in the UK. Hi I was diagnosed recently with superficial siderosis, caused by a bleed following a brain tumour. I am really struggling to see any doctor or consultant who even has the basic knowledge of SS. My current consultant just brushed off any effects I have like loss of balance lower back pain. One year on, I have seen no one and it's a fight to even try to get help.
Answer:
My collaborator in the UK, Dr. David Werring at Queens Square, London, is very knowledgeable about SS. He is also a friend and a good person.
Note from the Silent Bleed Team - there are now a few consultants around the UK who are engaging with treatment and management of superficial siderosis. Members of our Facebook Group (search The Silent Bleed - Superficial Siderosis) are always happy to share their experiences and local knowledge. We've also met David Werring in London and endorse what Dr Levy has to say about him.
Question:
Doctor search. Hello, my mother has recently been diagnosed with superficial siderosis. We are trying to find a doctor that has knowledge of this condition. Do you have any recommendations in the Midwest?
Answer:
Dr. Raj Kumar at the Mayo Clinic in Rochester, MN, is a world expert in superficial siderosis.
Question:
Cerebral Spinal superficial siderosis. Hi, my husband and I have been trying to find help for three years. We have been to countless doctors including the Mayo Clinic in Minnesota, looking for help. He had surgery in December and his most recent MRI shows the surgery was not a success. Looking for any amount of help we can find.
Answer:
Dr. Kumar at the Mayo Clinic in Rochester, MN, and I are the only two neurologists who see patients with superficial siderosis in the US. Dr. Kumar’s expertise is in the surgical approach to finding and stopping bleeds. I can’t imagine a more experienced team. But I also have neurosurgery colleagues at Johns Hopkins and UCLA who I’ve worked with before. My area of expertise is in the non-surgical management of superficial siderosis although I do refer patients to surgery when they really need it.
Question:
My mom was diagnosed with SS over a year so. She is 77 years old and shows few, if any, of the symptoms. She is currently receiving no treatment as there is nothing to treat. My question, should she be taking proactively preventative medication like those I've seen others on this sight post about? Also, the cause of my mom's SS is undetermined as she has never had trauma or surgery. Is there any evidence that the condition is hereditary? My grandmother suffered from dementia which makes me question any connections. I wonder if an MRI would reveal anything for me. Thank you, Ontario, Canada
Answer:
With so few symptoms, I usually do not recommend treatment with Ferriprox unless the patient is young. The reason for the age difference is that young people can tolerate the burden of using Ferriprox and have lots of years to appreciate the potential long term benefit. There is no known case of familial siderosis (yet). We do not know any genetic predispositions except possibly Marfan’s syndrome. I don’t think you need an MRI with this family history.
Question:
Should I be on Ferriprox? I had brain surgery and have cavernoma and have a bleed on the brain. I' waiting to hear back from my neurologist.
Answer:
A one time bleed from a cavernoma may not cause superficial siderosis. Bleeds in the brain from all causes are very common, but superficial siderosis is very rare. We posit that superficial siderosis arises from repeated bleeding over a long period of time.
Question:
Nutritional yeast. Dr. Levy, I wanted to ask your opinion of nutritional yeast as a dietary supplement for SS patients. My father suffered from Guillain Barre Syndrome in the late 70s that put him in the hospital for 8 weeks. As a part of his recovery, my mother introduced him to nutritional yeast, and he accredits nutritional yeast for the totality and speed of his recovery. It is my understanding that nutritional yeast has a positive effect on the CNS. Are you familiar with nutritional yeast and what is your opinion? Thank you. I look forward to hearing your opinion.
Answer:
Honestly, I do not know if nutritional yeast has any benefits. My first thought is that we consume lots of yeasts from bread products but I am not an expert in nutrition and there may be other factors that I am not aware of. I can certainly say that it’s not harmful to consume.
Question:
Stroke Dr Levy, is it true that strokes are more likely with SS patients? If so what kind? So far, I’ve seen 4 strokes in my 75 patients with superficial siderosis. All 4 patients were older than 65, but they were all ischaemic strokes (the non-bleeding type) in different parts of the brain.
Answer:
I can’t say for certain that strokes are more common in SS.
Question:
Hi Dr Levy, I have been to see the neurologist in NZ. They have told me that the front of my brain is also bleeding as well as the cerebellum. The tear I have is in the brain stem and bottom of the spinal cord. They don't seem in a hurry to do anything about it, even tho I sent you my lumbar puncture results, which they have. I would like to be on Ferripox like you suggested, you also suggested that they stop the source of bleeding. Over here they don't see the need to do this. Is there anyway I can ask them for the help I need. I bleed twice a week. Also does every one with SS have aphasia and speech problems? Thanks for your input it is great
Answer:
Aphasia and speech problems is common SS because of siderosis around the brainstem. I’m really not sure how to advise you regarding your country’s healthcare system but it’s worth pushing for your treatment as you are your best advocate.
Question:
Dr Levy, my neurologist in Auckland NZ rang today and asked me how do you find the source of the brain bleed? They did an MRI and told me 2 years ago that it was coming from my brain stem. That sounds right to me as when I am going to have a bleed it burns and is really sore in that area. Can you help me? Thanks a lot for all your help.
Answer:
If the bleed is most likely in the brainstem, a CT myelogram will not help because the dye only goes up to the neck and not the brainstem. There is no test that can identify a bleed in the brainstem. It’s probably not worth an exploratory surgery of the brainstem unless it’s very obvious that is the source of the bleed for example because of a previous surgery in that area.
Question:
My son has recently (six months ago) been diagnosed with SS and displays unsteadiness but no hearing impairment. We are having difficulty here in Australia finding a neurologist that can/will suggest any treatment options at all. Is there someone that you know of in Australia that can help us please? The last nuero we saw we asked about Ferripox but his answer was wait until you're deaf and then it may be worthwhile, obviously we would prefer to start treatment options to try and prevent or delay the onset of more severe symptoms.
Answer:
No, I’m sorry but I don’t have any colleagues in Australia with an interest in SS.
Question:
I had yet another lumbar puncture, two in a row. The first one showed lots of blood in the vials, the second one showed large amounts of blood, the doctor doing it had to change the needle as it was clogging. The results showed no evidence of bilirubin, despite the large amounts of blood. I am writing what my neurologist wrote. She has discussed this with a Neuradiologist. The plan is to do another CSF again under the guidance of this doctor. Given it is the third attempt to answer questions regarding presence of persisting intracranial hypotension and presence of blood. I asked if I could have a myleogram and they aren't interested in finding the cause. I have written to you before and told you how my neck gets so stiff I can't turn my head and my neck stem burns and is very sore. you suggested that the tear maybe in my brain stem. I am now also experiencing the same pain down my whole spine and bad down the lower spine. My neurologist says given the stability of the findings on neuroimaging, that she would need to have more reason to pursue this. She has decided to take one step at a time and not do the myleogram. She has asked for a CSF pressure measurement and sequential tube collection for red blood cells and the presence of xanthochromia performed at the radiology. I don't understand the last paragraph, what is that. What are your thought on this letter and how would you approach it? I sometimes feel that my neurologist is lost and doesn't know what to do. In the meantime, my health is deteriorating. I haven't go a lot of energy and sweat a lot even when it cold. I have developed angina as well. I am not doing very well at all.
Answer:
I suspect they did not actually insert the needle into the lumbar space where the CSF is. That’s probably why your neurologist wants to do this with neuroimaging to guide the needle. Bilirubin is the iron product from red blood cells that break down in the CSF. Xanthochromia is the “yellowness” from bilirubin.
Question:
I had a big bleed four weeks ago. I bled for two and a half weeks, through the bleeding I had a lumbar puncture which showed large amount of blood, these are the neurologist's words. I told the doctor doing it that I would have lots of blood owing to knowing I was bleeding. My neurologist wants another Lumbar puncture done again, the third with blood in it as she feels that I was pricked. I asked her if I could have a myelogram and if the source of bleeding could be found so as they could repair it. She has declined my request and just wants to know if another lumbar puncture will have blood in it. I am sure it will as I have a bleed every week to ten days. I know because my neck is so stiff I can't turn my head, my headache feels like it is going to explode and my balance and speech go out the door. I have to just sleep. Please can I have an input to as to where I should go and what I should say to the neurologist. I would like the bleeds slowed down as they really drain me.
Answer:
You can tell your neurologist that a CT myelogram starts with insertion of a needle into the lumbar space. She can obtain spinal fluid first. And then proceed with the myelogram. Ultimately, you are probably right about your condition. I think you are bleeding every few days as you say, and that’s causing your symptoms. You might have to find a new neurologist who will proceed with the CT myelogram.
Question:
Knee surgery and superficial siderosis. My mother has recently been diagnosed with ss. She also is due to have a knee replacement in the next month. Can the knee surgery have any complications with the SS and vice versa? We have not been given any information apart from told to give the MRI to the consultant who is operating which we have. Just a bit wary of going ahead with surgery if it's going to make the SS worse.
Answer:
If my SS patients need surgery, I generally approve it. The only issue is that anaesthesia can take a lot longer to wear off and balance can be worse for several times after surgery. However, the anaesthesia will eventually wear off.
Question:
Is there likely to be CSF leak at the location of the bleed as I am currently suffering from low ICP which was confirmed by intracranial pressure monitoring?
Answer:
Yes, absolutely. The site of bleeding in the spinal column is usually a dural tear where spinal fluid leaks out too.
Question:
My dad My dad has this disease, the doctors in South Carolina don't know anything about this. And my dad is getting worse by the day. He is having double vision when he looks to the left and was hospitalized for 3 days for that. He keeps having episodes of non-stop vomiting. He has had to retire and can't walk with out a walker or sometimes has to be in a wheel chair. He is losing weight like crazy! The doctors here don't know what to do for him. He had cryptococcal meningitis about 7 years ago and almost died, now this. I want to get him to you if you are seeing patients?. He has blue cross blue shield and they said they will not pay outside our state. I'm afraid he will die if we can't find a doctor that knows about this.
Answer:
This is very frustrating. I have a neurology colleague in South Carolina named Jay Avarasala. Check to see if your father can see him. We communicate on a regular basis.
Question:
Are you aware of a specialists in Australia or particularly in Brisbane who might specialise in SS?
Answer:
Unfortunately, no. I do not know any neurologists in Australia who specialize in superficial siderosis. I have been in communication with a Dr. Compton in Epping about a superficial siderosis patient but I don't know if this is his specialty.
Question:
Do you think there is a genetic component? My friend's mother has SS. Her neurologist was unable to answer the question about whether this can be genetic. Please can you advise?
Answer:
We are actively engaged in a study to determine if there is a genetic component to superficial siderosis. This project is being spearheaded by a group in London, UK, and we are participating.
Generally I'd say there is no known genetic component. There are some conditions that can increase the risk of superficial siderosis like Marfan's disease but the chance that any two related people would both sustain an injury that leads to chronic bleeded into the spinal fluid is exceedingly unlikely.
Question:
I'm helping a loved one to find a neurologist who has experience with treating superficial siderosis near VA and NC line. Area from Roanoke VA to Greensboro NC line, any advice?
Answer:
Superficial siderosis is incredibly rare. The only two doctors I know of who routinely treat superficial siderosis are me and Dr. Kumar at the Mayo Clinic. However, any neurologist can learn about superficial siderosis and consult us for advice.
Question:
Can you see the difference between new iron deposits and old iron deposits on an MRI scan?
Answer:
Unfortunately, no.
Question:
Can an intermittent bleed stop on its own?
Answer:
Yes. But in superficial siderosis, the bleeding occurs over years to decades so there is no reason to expect that the bleeding will stop after all that time.
Question:
I have mild superficial siderosis and have been on Ferriprox for three years. I have just received my latest scan results and it shows no change. I do a lot of exercise, mainly free weights and low impact cardio and I walk my dog every day. Could the frequent change in blood pressure close an intermittent bleed? Could my workouts increase a bleed and could the Ferriprox be just working on new deposits?
Answer:
To answer your first question, it depends on the source of the bleeding. Exercise and exertion may cause re-bleeding if there is shearing pressure on the bleeding site, but I don't think it's due to changes in blood pressure. In answer to the second, Ferriprox is very good at chelating fresh blood. I think there is plenty of Ferriprox to chelate both the fresh blood and the continue to work on old iron deposits.
Question:
My boyfriends sister is 31 and has been diagnosed with iron on the brain and early onset Parkinson's. She has a beautiful one year old little boy and her symptoms are memory loss, falling or tripping and tiredness - she is deteriorating quickly. Is there anyone out there that has this very rare condition and can give us advice on how to treat her illness or give us advice on what we can do? She has seen consultants at Salford Royal and Liverpool hospitals as well as being seen privately but it is so rare there is very little known about it and all different kinds of medication.
Answer:
It's unusual for someone as young as 31 to have such severe symptoms of superficial siderosis unless the iron deposits have been present since birth. For example, there are some babies who require brain surgery and develop chronic bleeds a result. Unless your boyfriend's sister falls into this category of patient, I am a little suspicious of the diagnosis. I wonder if there are other issues that may be contributing to her neurological symptoms.
Question:
If ferritin levels were very high, it would tell if I had an active bleed, is that right?
Answer:
No, not at all. Ferritin is a measure of total body iron. It is measured in the blood. If you have a bleed in the brain, it will not be reflected on the level of ferritin in the blood. The other thing to know about ferritin is that when you have an infection or acute metabolic problem (pancreatitis, for example), your body makes a lot of ferritin. The ferritin is produced to bind up all of the free iron and deprive bacteria of any. So sometimes if you check your ferritin level, you may notice that it suddenly increased markedly. For the purpose of monitoring ferritin as measure of total body iron, just disregard those occasional increased values.
Question:
Is the ferritin levels in CSF different from blood levels, if so, how?
Answer:
Ferritin is not measured in the CSF. Normally, there should be no ferritin in the CSF because there should be no iron in the CSF. In siderosis patients, ferritin is made and secreted into the CSF to sop up the free iron released by blood cells. We don’t usually measure the level of ferritin so I would not have a sense of normal or abnormal values if we did.
Question:
How are SS and ALS similar yet different?
Answer:
Superficial Siderosis is a condition of brain and spinal cord iron overload that causes toxicity to tissue exposed to the iron. The most common areas affected are the nerves going to the ears causing hearing loss, the cerebellum causing balance problems, and the spinal cord causing weakness, numbness and bowel/bladder problems. ALS is a neurodegenerative condition that selectively targets the motor nerves in the brain and spinal cord. About 10% of cases are familial (genetic). It causes weakness and death within a few years. They are very different diseases in my view
Note from the Silent Bleed team: ALS or Amyotrophic lateral sclerosis is also known as motor neurone disease (MND).
Question:
Dear Dr Levy, I am glad I found this site and the possibility to contact you. My husband, 51 years old, became the diagnosis Superficial Siderosis 2 years ago. This is his story. My husband has a history of headache for decades. He's had many scans, MRI's and examinations during this period and 2 years ago, after a scan of his head because of hearing loss, by coincidence, a radiologist found iron on his small brains and brainstem. They took a scan of his back and the conclusion was a paraganglioma in his back, between the third and fourth lumbar vertebrae. (it am not sure about this, it could also be between the fourth or fifth). In September 2014, the tumor was removed at UMC Utrecht and it was a benign paraganglioma. During the operation, the surgeon made by accident a hole in the dural sac. Because of this my husband had water in his head but according the surgeon this was not severe and would be gone within a week. A week after the surgery, my husband was already at home, he developed such serious headache and we returned to the UMC. They found nothing and sent my husband home. This was the beginning of the nightmare. Every 2 weeks my husband is ill because of headache attacks and lies in bed for 3 or 4 days. Besides heavy headache he's sick, with vomiting and has a strange pressure in his head. Between the attacks he has headaches, but is able to go to his work. He now works only 4 hours a day and my husband was always busy with his hobby, cycling and now he just goes to his work but at home he is lying on the couch because he is exhausted. Our lives have changed dramatically and the world is getting smaller for us. The neurologist said to us that the paraganglioma caused the bleeding in the subarachnoid space, but the neurosurgeon denies this. What should we believe? Other symptoms of my husband: bad balance, bladder problems with blood in his urine, intestinal problems, dizziness, extreme tiredness, hearing loss, extreme sweating and high heartbeats. The neurologist of UMC Utrecht did not know what to do anymore, and referred us to the neurologist of the Radboud UMC Nijmegen for a second opinion. The conclusion there was clear: superficial siderosis. But it added nothing for us. There was no solution.
My husband tried so much medication, nothing works. The last medicine, Depakine, does also not work and last Friday my husband visited the neurologist at the Radboud and she sent him home and told him that she could not help him anymore. We are very desperate and frustrated about this. Since yesterday my husband is ill again. Besides the SS, my husband is under treatment by a endocrinologist at Radboud UMC, because he had a paraganglioma they are looking for other tumors (feochromocytom). His upper body is clean of tumors, the adrenaline and noradrenaline are ok and now we are waiting for the result of the MRI from his shoulders till his ears. There is planned a genetic research (exome sequencing) , this endocrinologist investigates the case of my husband very thoroughly. Concerning the SS, I have read your site and have some questions. 1 What is the prognosis of SS? 2. Is surgery possible to remove iron from brains? According the doctors in Holland this is not possible 3. I have heard about the medicine Ferriprox, but the doctors in Holland are negative about this medicine. What is the result of this medicine? 4. Is cannabis or weed treatment positive for the severe headaches? 5. Do you know what happened with in the body that my husband constantly has this headache attacks? Thank you that I can tell my story and hope that you are able to give a reaction to us.
Answer:
Prognosis of superficial siderosis depends on many factors including the extent and degree of iron deposition, age, and vulnerability of the underlying brain tissue. Generally, progression is linear – which means you will likely continue to worsen at the same rate over time.
It is not possible to surgically remove iron deposits off the surface of the brain.
We are still working to demonstrate a benefit using Ferriprox but what I can tell you is that some people respond well to it and show reduced iron deposits on the brain by MRI. The key unanswered question is, does that translate to improve neurological function? That’s what we're trying to answer.
Cannabis is useful for treatment of many forms of pain. It's probably not too harmful to try it for headaches.
I suspect the paraganglioma is responsible for the superficial siderosis but the headaches are not due to siderosis. They are due to either bleeding or CSF leak. Bleeding headaches are usually not-positional (it hurts in any position), associated with fever and stiff neck. CSF leak headaches are classically positional which get worse the longer you stand and get better the longer you lie down.
Question:
Can a person who is diagnosed Superficial Siderosis and also has Marfan's Syndrome take Ferriprox safely?
Answer:
As far as I know, yes. I have had two other Superficial Siderosis patients with Marfan’s Syndrome.
Question:
Hello, and thank you for offering to take questions. I can only imagine how busy you must be. I wrote once before, but that question may have been a bit too long and rambling, so I'd like to try something more specific. Please feel free to edit as needed for your use. I've just recently been diagnosed, via brain MRI, as having Superficial Siderosis along the left cerebellopontine angle. My current neurosurgeon read that report, looked at the images and shrugged it off, saying it was very minor and "nothing to worry about". While I've had at least 5 Brain MRIs in the last ten years, this is the first to receive this diagnosis. But, my question is this: when I received this diagnosis, my first thought was that it must be due to a brain bleed I had right after having a Posterior Fossa Meningioma removed. I had a heart attack while in recovery, and treatment for that contributed to the cause of the bleed. BUT and this is where I don't understand, during my first follow up with one of the two neurosurgeons who did my surgery, I asked why it was called a bleed and not a stroke. He replied that a true stroke takes place within the brain, beyond the Meninges and Arachnoid spaces/layers. My bleed had been outside the protective layers around the brain, and placed great pressure on the brain - hence my EVD, hydrocephalus, Tonsillar herniation, etc. So, what I don't understand then is, how would I get SS within these protective layers, if the only known bleed I've had had been outside these layers? My only thought is that this would suggest that there are/have been other bleeds of some sort, inside the protective layers around the brain. Is this logical thinking, or is there some medical/anatomical answer that I may not have the education to consider on my own? I hope this is a valid question for you and look forward to your reply.
Answer:
You can think of three layers of the central nervous system. There is the brain tissue itself. Then there is a lining around the brain, which is made up of three individual linings: the pia, the arachnoid and the dura. And then there is the skull. A bleed in the space between the skull and the dura is called an epidural bleed. A bleed in the space between the dura and the arachnoid is called a subdural bleed. A bleed in the space between the arachnoid and the pia is called a subarachnoid haemorrhage. This is where the bleed is in patients with superficial siderosis. There is no space between the pia and the brain – they are stuck together. A bleed inside the brain is called a haemorrhagic stroke.
Question:
I recently viewed your webinar and learned a lot more. Can you explain a bit more about neurological reserve and the effect that SS can have on that. My OF (old fella) is often running on what I call a flat battery.
Answer:
“Neurological reserve” is your brain’s capacity to deal with stressors, including metabolic and psychological stressors. Everyone has a breaking point where the neurological reserve is depleted and dysfunction ensues. In the young and elderly, the neurological reserve is low so even an infection can cause them to become delirious. In SS patients, the neurological reserve is low too. So when considering surgery and anaesthesia, which are significant metabolic stressors, expect to have a prolonged recovery phase. Other conditions that depend on neurological reserve in SS patients are infections, change in medications, poor circulation and diabetes.
Question:
I had an MRI and Lumbar puncture on Monday 18th August. The results haven't been printed yet, but the Drs who were watching the MRI said they hadn't seen blood in the cerebellum ever like I have got it. They did a head to tail bone MRI and the bottom of my spine has shown something is not good down there. Can SS affect the spine as well? As I have a lot of pain down my back and also my bowel is leaking and my bladder is either going all the time or only empting 6%. This seems to happen when I have had a bleed and get the vasospasms. Is this all connected to the bleeds and SS? I had a brain bleed while having the lumbar puncture, they thought it was at first from the lumbar puncture, but it may have caused something. They kept me in hospital and my speech went completely like a stroke victim, that is what happens every time I get a bleed. Unable to understand my speech at all. Would love your input into this as I am waiting to go to Auckland City Hospital Neurologist for help.
Answer:
All superficial siderosis patients are susceptible to spinal cord involvement. The spinal fluid is made deep in the brain and circulates around the brain and the entire spine. Bleeding into the spinal fluid allows the iron (from inside blood cells) to circulate around both the brain and spinal cord. When you bleed into the spinal fluid, the fresh blood irritates both the lining of the brain which causes neck stiffness and fever, and the blood vessels around the brain which causes neurological dysfunction similar to stroke. To achieve this level of irritation, there usually has to be quite a bit of blood – on the order of 20,000 red blood cells per millilitre of spinal fluid.
Question:
Are superficial siderosis and hemosiderosis the same thing? They were both mentioned on my MRI
Answer:
No. Hemosiderosis is simply “deposition of iron complex” seen by MRI. You can have hemosiderosis in the heart, and other organs. In contrast, superficial siderosis is a specific hemosiderosis of the outside of the brain and spinal cord.
Question:
Do you know of a neurologist in Britain that specializes in SS?
Answer:
I don’t know any that have a particular interest in SS.
NOTE FROM THE SILENT BLEED TEAM: We are in contact with Dr Werring at University College Hospital , London who is involved with research into Superficial Siderosis. Dr Wren based at St George's in Tooting and Frimley Park hospital also has an interest.
Question:
My 40 year old son has just been diagnosed with SS. He is totally deaf, manages a little bit of walking with a frame and has no smell or taste. He also has bladder and bowel incontinence problems. Short term memory loss and he has a dural ecasia which had made him very uncomfortable. He has Marfan's Syndrome and we think the bleed on the brain was caused by taking Warfarin. He had a rod fitted to his back for his curved spine when a teenager. This rod recently has caused his thinning skin to bleed and has become infected. He was rushed to theatre last week and the rod was removed. i am wondering about the dural sac at the base of his spine. How would it affect this? He says he can taste a few things now, could some of his symptoms have been from damaged nerves to the spine?
Answer:
This sounds more complicated than the usual case of superficial siderosis because we’re also dealing with Marfan’s and previous neurosurgical procedures.
One thing I can tell you is that nerves in the nose that connect directly to the brain can regenerate. So often my patients will regain their ability to smell when the iron deposition decreases.
Question:
If I have an intermittent bleed is there anything I should not be doing? I work out doing weights, shadow boxing, low impact cardio, Zumba and yoga but have done since before I started Ferriprox and my MRIs have shown no deterioration.
Answer:
Your MRI will not show deterioration because the timescale is too short. You would have to compare an MRI 4-5 years from now to see a deterioration. And you would not necessarily be able to correlate a change in that MRI to a few things you are doing now. So it’s not useful to use MRI as a measure of whether something is harmful.
The best way to know if these activities are leading to bleeding is to do the activity vigorous a few times and then get a lumbar puncture. If you see fresh blood in your spinal fluid, you have your answer. If your spinal fluid is clear, you’re probably OK doing those activities.
Question:
I have been taking Ferriprox for twenty months and my last MRI showed no change. Would an intermittent bleed need to be found? Is it likely the bleed could become a bigger bleed?
Answer:
Iron from active bleeding is much easier for Ferriprox to chelate than iron bound to hemosiderin already stuck to your brain. If you are seeing no progress by MRI, my only suggestion is to keep trying it. I’ve seen only a rare few cases that did not improve by MRI eventually (4 years or more in some cases).
Your bleed hasn’t changed in 15-40 years. It’s unlikely to change now unless you do something traumatic or surgical.
Question:
My husband's neurologist has told us that that there is a difference regarding the brain and meningeal siderosis, not systemic. He was not sure Ferriprox would be effective or even work in the brain which is not truly an iron overload. I didn't realize there was a difference. Is there nothing we can do for meningeal siderosis?
Answer:
Meningeal siderosis and superficial siderosis are the same thing. They refer to iron overload confined to the central nervous system. There are other iron overload conditions that lead to iron overload in the entire body, called systemic iron overload disorders. Ferriprox along with the other 4-5 iron chelators are all good for systemic iron overload disorders, but Ferriprox is the only one that can get in to the brain so it’s the only one we’ve tried for superficial siderosis.
Question:
if a stroke patient can be rehabilitated to use their arms and legs or other body parts damaged from a stroke, is there a possibility that we as SS patients can retrain our brains to regain balance and muscle function?
Answer:
Yes! Rehabilitation takes advantage of your brain’s ability to generate new circuits and learn new tricks. Whether your damage is from stroke, trauma or siderosis, this phenomenon holds true. I highly recommend physical therapy, occupational therapy, vestibular therapy or whatever other targeted therapy you need to improve your neurological function.
Question:
My zinc levels are a little low, 9.7 this month. How low can they be before it's a concern and any ideas for increasing zinc without increasing iron?
Answer:
When my patient's zinc levels get a little low, I recommend taking a 1 week break from Ferriprox and taking over-the-counter zinc tablets. The zinc level will bump up quickly
Question:
Can you explain the relationship between iron and ferritin? My iron levels are staying between 10 and 30 and my ferritin is 15. Is that good?
Answer:
Ferritin is a protein that binds to and carries iron around the body. A ferritin of 15 is fairly low and indicates that your circulating iron reserves are low. Maybe due to Ferriprox, if you're taking Ferriprox. That would be expected as Ferriprox depletes total body iron stores as well as depleting the iron salts from the surface of the brain and spinal cord. We follow ferritin levels to make sure that over time it continues to decline indicating effective chelation.
Question:
I watched the NBC Dateline program on Sunday (6/7/15) which was about the use of cannabis oil. The show focused primarily on children with epilepsy and neurological conditions. It is believed cannibis has helped or could help if it were more readily available. A neurologist stated that it was her opinion that cannabis could help in people with MS. Since SS mimicks MS in some areas. What is your opinion about it's use in SS? How difficult is it to get for medical use? Is Johns Hopkins planning any research on the use of cannibis oill?
Answer:
Cannabis would be expected to help with pain (neuropathy) and spasticity. For those SS patients with neuropathy and spasticity, cannabis would be expected to help. Unfortunately, no, we have no plans for testing cannabis at Hopkins.
Question:
I watched the NBC Dateline program on Sunday (6/7/15) which was about the use of cannabis oil. The show focused primarily on children with epilepsy and neurological conditions. It is believed cannibis has helped or could help if it were more readily available. A neurologist stated that it was her opinion that cannabis could help in people with MS. Since SS mimicks MS in some areas. What is your opinion about it's use in SS? How difficult is it to get for medical use? Is Johns Hopkins planning any research on the use of cannibis oill?
Answer:
Cannabis would be expected to help with pain (neuropathy) and spasticity. For those SS patients with neuropathy and spasticity, cannabis would be expected to help. Unfortunately, no, we have no plans for testing cannabis at Hopkins.
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