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We're really grateful that the expert based at Massachusetts General has been working with us since 2015, and we've built up quite an archive over the past few years. With a little sifting you should find the answer you're looking for. However, we know we haven't covered everything, so if there's something else you need to know, please contact us - we'd be happy to forward your question. It's part of what we do.
The answers on this page cover a wide range of symptoms that couldn't be placed in one particular category (please also see our pages on hearing, balance, bowel & bladder problems). 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 there any reasons why someone with SS could not have a tattoo?
Answer:
If your spouse is against it, I strongly recommend against having a tattoo.
Question:
Brain fog I workout early in the morning. After some workouts I'm finding I get brain fog a couple of hours later where it's hard to think and concentrate. It lasts for a few hours. I don't know the reason. I was wondering if it is just being tired or if I'm causing a bleed or doing any damage. I do have a weak neck from the operation 27 years ago to remove the tumor from my cerebellum.
Answer:
I am not sure why you are getting these transient brain fog episodes after working out. I would look for other associations – medications (especially Ferriprox), sleep, body temperature – that may explain what is going on.
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:
Diagnosis. I’m 25 years old with a history of migraines. I have a subarachnoid cyst that we’ve been watching for 10 years. I recently had an MRI showing a new curvilinear low signal susceptibility artefact along the right great horizontal fissure, suggesting a possible focal area of superficial siderosis. My neurologist assumed it was the result of a brain bleed. He referred me to a local neurosurgeon and he agreed that he thought it was a bleed. My question is can superficial sidersosis present in just a focal area at the beginning? Both doctors told me it would be everywhere on my scans if it was SS. I guess I’m just not sure how similar brain bleeds and SS look. I’m worried if it is SS and I don’t get any sort of early intervention, that symptoms will progress throughout the span of decades until it’s too late to treat.
Answer:
What you are describing is called focal siderosis, rather than classical superficial siderosis. A patch of blood products from a nearby bleed does not lead to superficial siderosis and is almost always fairly harmless. However, if you continue to bleed over and over again, the blood products may deposit on other parts of your brain, and that could lead to superficial siderosis.
Question:
Dr Levy, I have been having terrible pain in my lower back, it seems to be coming from my spine, not tissue damage. Can SS cause this and what should I take if it is SS? I find it hard sitting for too long and standing and walking, and trying to turn over in bed.
Answer:
Thanks for your input. It can due to many things related to your SS including muscle spasms and neuropathy, and even the acute bleed. It’s hard to tell for sure.
Question:
Hallucinations. My husband has SS. This morning he told me that he has been having hallucinations at least 2 to 3 times a week, mostly after he has just woken in the daytime (also after napping) and they last an hour or two. He has lost hearing and most of his eyesight. What causes this to occur? Are there any suggestions of what I could do to help him? Thankfully he is lucid and clear thinking the rest of the time.
Answer:
Auditory and visual hallucinations can occur for a number of reasons. Many of them are benign. For example, people with poor vision from any cause can have visual hallucinations in the dark. Sometimes just making the room brighter will help this issue. For others, certain medications may cause hallucinations especially if associated with confusion at the same time of day after taking a medication– in that case, reducing the dose will help. Auditory hallucinations have also been reported in SS especially in cases with very severe hearing loss. The first step to fixing this issue is trying to figure out what’s causing the hallucinations. First, try to improve the lighting. Then, review the list of medications. And once the common things are ruled out, it may be an SS thing.
Question:
Has anyone also suffered SS and linked thyroid issues? I know I must stop reading about SS on Google and Forums like this as it appears there is little of a positive nature to look forward to! Any help, guidance or encouragement will obviously be appreciated. Equally if there is anything about my diagnosis that help others now or in the future then please let me know.
Answer:
No, I don’t know of any link between SS and thyroid disease. Thyroid disease is so common that is would not surprise me if any SS patient also has thyroid disease.
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 though 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 everyone 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:
Hi I've recently been diagnosed with superficial siderosis. I've searched for five years for an answer to my symptoms. Every time I do too much physical activity, when I sit and rest and then stand up and walk, I get the following:
1. pain in the shoulders and neck
2. brief pain in the tail end of my spine
3. Pressure spots in my eyes like when you press your eyelids with eyes closed
4. Sound like a steam train chuff-chuffing in time with my heartbeat.
Does this mean I'm still bleeding? They can't find a tear in my spine. What should I do or ask for? I'm getting no advice.
Answer:
It certainly sounds like you are bleeding, yes. The only way to find the tear in the spinal column is to do a CT myelogram. That test uses a harmless dye injected into the spinal fluid and a CT scanner (or x-ray machine) to track the flow of the dye up the spinal column. If there is a tear in the lining, the spinal fluid and the dye will leak out and that will be visible on the myelogram. The problem is, if the scar tissue happens to be working at the moment you get the test,
Question:
Hi, Is there a complete list of symptoms somewhere please?
Answer:
Yes, the Silent Bleed Superficial Siderosis symptom page gives a good answer.
Question:
I am diagnosed with SS one year ago. I am from Lithuania, maybe first patient with SS in my country. What are the statistics for SS? How many SS cases are out there per million people? I can't walk without crutches anymore because of poor coordination, also I can constantly "hear" rhythmic noises in my head. Is that some of the symptoms of SS?
Answer:
SS is incredibly rare. The incidence is approximately 1 per million. Your symptoms of balance problems and tinnitus are classic for SS.
Question:
I am experiencing severe pain in my head and neck area every time I move my head. This has been going on for at least two weeks. I am in touch with the web site and realise it is an active bleed, neurologists here don't know what can be done. I am desperate for some direction, could you advise? Applying heat seems to relieve it somewhat temporarily. I was diagnosed with SS in 1993.
Answer:
Neck pain is likely due to an acute bleed. It causes irritation of the lining around the spinal cord. The best things to do in this situation are: 1. Hydration as much as possible. 2. Ibuprofen 600 – 800 mg three times per day. 3. Rest. It should go away in 2-3 days. Also, try to avoid precipitating the bleed if you know what activities trigger it.
Question:
I am much worse in the summer heat; energy and balance. Also having some trouble swallowing all of a sudden, is this due to SS?
Answer:
Swallowing problems are common in SS. It’s usually not a sudden issue though. It typically starts with trouble swallowing liquids and then slowly progresses to solids.
Heat (and cold) can both impact on brain function. It doesn’t cause any damage but it makes the symptoms feel worse. Consider a cooling vest in the summer.
Question:
I am someone formally diagnosed with superficial siderosis, but have had very little research done as to the cause. I have also been diagnosed with mild anaemia - nothing too severe, but RDW (red cell distribution width) and HCT (hematocrit) are usually below the normal levels for a man my age. My question is can mild anaemia be considered a red flag for ongoing bleeding in someone diagnosed with superficial siderosis? Or, can fluctuations in CBC (complete blood count) levels ever be used to help determine whether bleeding associated with SS is increasing or decreasing? Is there any known link between anaemia and possible chronic CNS bleeding?
Answer:
No the amount of blood that leaks into the spinal fluid intermittently to cause superficial siderosis is similar to the amount from a paper cut on to your skin. It's very small. The amount of blood loss would not be expected to be detectable on a blood count and it certainly should not cause anaemia. Unfortunately, nothing about the blood counts provides any clues about the cause, diagnosis or prognosis of superficial siderosis.
As an aside, when you use Ferriprox, blood counts are monitored monthly because iron chelation with Ferriprox causes iron deficiency anaemia as a side effect.
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 vasospsms. 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 suspectible 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 milliliter of spinal fluid.
Question:
I have been taking Ferriprox over 4 yrs now. I had some balance improvement by the end of the first year. In the past 6 months I have been experiencing vertigo/dizziness, pain in my lower back & the back of my head, and sharp pains in my feet, legs, and eyes. Along with all of this, I still have continued loud ear ringing and extreme fatigue. Can all of this be related to Superficial Siderosis? Thanks for all you do! God bless you!
Answer:
Vertigo/dizziness, pain and loud ringing in your ears (tinnitus) are almost certainly due to ongoing issues related to superficial siderosis. The extreme fatigue is probably due to the Ferriprox. To address the fatigue, you can try to reduce the morning dose.
Question:
I have had SS for the last ten years now. Could you tell me how if it can effect the back?
Answer:
Superficial siderosis itself does not cause pain, but the trauma that caused the condition can cause pain. The most common cause of bleeding in superficial siderosis is a tear in the lining around the spinal cord. If that tear affects the nerve roots in the back, it can be painful.
Question:
I have neuropathy pain in my arms, legs and face. Until a few weeks ago I had only burning and numb feet. Since a few days ago I have the burning also in my face, neck, back, hand, arms and legs. My neurologist does not believe that it is a SS symptom, because all measuring (Nerve conduction study, Electromyography, Evoked potential, Somatosensory evoked potential) are normal. Is neuropathy caused by SS measurable?
Answer:
Sometimes. Given your history, all of your neuropathy is most likely due to SS.
Question:
I have obtained a personal copy of my MRI on CD and this is the first time I have seen it. The images are very interesting (I even have two gaps in my skull I never knew about!) well the back one is most likely due to my operation that caused the SS in me but my main question is; how do you identify a bleed on an MRI scan?
Answer:
Depends on the sequence of MRI and when the bleeding occurred. Hemosiderin, the blood product in SS is black on the T2 sequence and much more visible on gradient echo MRI.
Question:
I have superficial siderosis and the dizziness is making it very hard to get out of bed. The feeling like I have been on a merry go round is so bad I need to sit or lie on it. I had a very large brain tumour removed in 1989 and another one that filled my ear canal and spilled out because it was large. I had all my bone drilled out and filled with fat from my tummy. Three years before the superficial siderosis, I was diagnosed with relapsing polychondritis and wonder if the two could be related. The first tumour was by the cerebellum. I am 72 years old and told nothing will be done because of my age and I can not walk without my frame. I wonder if this would shorten my life by much. I live in New Zealand.
Answer:
You are the third person in New Zealand I know with superficial siderosis! I suspect your bleeding is due to the extensive surgeries you have had around the skull and that probably resulted in bleeding scar tissue. Superficial siderosis does not tend to shorten lifespans but it can be very disabling in some.
Question:
I know I have had a bleed, but I am wondering if that bleed can last for two weeks. I had a lumbar puncture and the doctor doing it had to change the needle as the blood was clogging up the smaller needle. She then changed to a larger needle. When she had finished she showed me the vials and they were really red. I have been feeling quite sick for the last two weeks, it was only a week ago that I had lumbar puncture. My head feels like it is going to explode and my neck on the left side is stiff and really sore. I can't turn my head to the right. I have to sleep partly sitting up, also I can't lie on the pillow with my left side. Dr Levy could a bleed last that long, it has made me feel really sick, that I have to sleep 1/2 the day just to get thru the rest of the day. Would like to know your input as to what may be going on with me.
Answer:
This is an acute bleed. See answer below about acute bleed.
Question:
I was diagnosed 5 years ago. I am a paraplegic which has masked the condition for many years. It is 38 years since I was stabbed in the spine. I am getting new symptoms. Dr Levy said the only thing you can't do, is do nothing. I was put on deferiprone for a year and a half which made me feel better, but I became anaemic. No one is sure if this was due to the meds as nothing changed when I came off. I feel that was the only hope for me, but because there was still blood in my lumbar puncture I was never put back on the meds. The scan with dye has not been done. I now have problems with temperature and get sweats and often vomit with no warning. Also bladder problems. Because of my spinal injury, I'm worried that the siderosis is being overlooked or put down to my spinal injury. In the morning my head feels terrible for a few hours. It takes a lot longer to feel ok in my head. My speech is getting worse. I feel desperate to do something before my quality of life is completely gone. I have children and a wife that I'm not ready to give up on. Please can you help me? I am happy to enter a trial.
Answer:
I think you are right that some of your symptoms are likely due to either additional bleeding into the spinal fluid or due to superficial siderosis. In both cases, deferiprone could help and I would try to advocate for that treatment again. I assume cost is the issue with deferiprone which is why you’re asking about a potential trial? I hope to launch another deferiprone trial in 2021 but I don’t have more details on that right now.
Question:
I was diagnosed with immune deficiency around the same time it was found I have SS. I have had immunoglobulin therapy for 1 year now. I feel like when I have an intermittent bleed now my recovery from that episode is a little faster than before the IG. It could be wishful thinking but do think there is any benefit of me taking this for my SS? Do you think there is any connection between people who get SS and being immunocompromised?
Answer:
I cannot think of a link between your immune disorder and superficial siderosis. I also cannot think of how IVIG might be useful in your recovery from an intermittent bleed except that maybe the infusion is keeping you well hydrated.
Question:
I was wondering if swollen leg is an issue? I just seem to have noticed my left thigh/ ankle and foot seems swollen. The toes on my left foot is a little spaced out as well. I am 37 have had SS for many years but only got diagnosed 3 or so years ago.
Answer:
Superficial siderosis doesn’t cause leg swelling per se. I would recommend getting checked out for common causes of leg swelling such as blood clots and mechanical compression. However, if the spinal cord is significantly involved from superficial siderosis, you may have dysautonomia, which may cause some leg swelling and redness. But this is rare and usually involves both legs.
Question:
Is it normal to have Parkinson's like shaking with Superficial Siderosis?
Answer:
No, tremors are not typical with SS neither are Parkinsons symptoms. That doesn’t sound like SS to me.
Question:
Is sugar making my neuropathy-worse?
Answer:
maybe. If you are diabetic or borderline diabetic, sugar can cause neuropathy which will make your SS-neuropathy feel worse.
Question:
Is there anything SS patients should avoid? Any activities that should be avoided? Any foods/medications that should be avoided?
Answer:
Activities to avoid: anything that re-tears the lining around your spinal cord (for most, that is the source of bleeding).
Foods to avoid: anything that will stick to the Ferriprox in your gut: foods that contain high vitamin C, iron or zinc content 2 hours before or after taking the medicine. No pills containing iron, zinc or vitamin C at all.
Question:
It's been suggested to hold a convention for SS which is not practical, financially or geographically. But how about a webinar? Maybe we could set it up through Johns Hopkins University or Johns Hopkins Hospital, but if not we could set it up ourselves. Our group spans such a huge geographic area, UK to California, even Australia, that we would have to be careful with time. I'm sure Jason Roberts would be on board & we could all learn, even ask questions, from the SS expert. What do you think? My tech savvy kids can help if we need!
Answer:
I think that's a great idea. I would be happy to participate although I’m terrible at the logistics, organization and technical issues. But would be great to do!!
N.B. The webinar did take place on 15/07/15, please view it here.
Question:
Mental acuity. I have been diagnosed with SS recently, based on MRI. I have ataxia and back pain, but NO hearing loss. My chief complaint is that I cannot concentrate for a prolonged time because of dizziness and vertigo, basically, even a small head movement causes dizziness spell. Is this dizziness a direct result of SS?
Answer:
Yes, it can do. SS can affect the nerve going from the inner to the brain. While it usually causes hearing loss, this nerve also carries position information that could explain your symptoms.
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 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 thining 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 brother had a brain tumor removed last October and it wasn't until January that they told us his pre surgery MRI showed signs of SS. He lost a lot of his eye sight, cognitive skills, and coordination after the surgery. But he was recovering over time with lots of therapy and family support. He came to see you with his wife in the spring. It seemed he was in the bottom 10% of symptoms and was doing okay. He was regaining a lot of deficits until he had a seizure in June. At that point he lost a lot of his hearing, eyesight, balance and is in a huge amount of pain. They found what possibly might be a new tumor growing in a new location but decided it wasn't significant enough at the moment to take out.
Question 1: are seizures related to SS?
Question 2: Could these new symptoms be related to SS, even though they were symptoms he had post-surgery and he had previously been regaining back? In other words do SS symptoms fluctuate back and forth? Thank you for your time.
Answer:
Seizures are not related to SS. They are related to brain tumors though. SS lowers the “neurological reserve” so that any metabolic stressor (seizures, medication change, surgery, infection) makes recovery a lot slower.
Question:
My father has hearing loss and bladder problems that have been attributed to age and prostate conditions but I feel may be associated to the SS. Aside from these, his primary symptom is a feeling of tightness around the head and of being incredibly hot despite his temperature being normal. The hotness is proving incredibly distressing - he is so hot he wants to be in an ice bath. I can see no other reports of this as a symptom and I wonder if you have heard of it as a symptom and, if so, do you have any tips on managing it? Second query is that the cause of my father's SS has been given as amyloid angiopathy and I wondered if this makes any difference in terms of trying iron chelation as a therapy. Can we still approach the SS in the same way as one would approach it when it is caused by trauma? Many thanks indeed for your efforts and dedication to this site. I will be checking in and passing it onto my parents for their info.
Answer:
I don’t know if the banding sensation around his head and the hotness are due to superficial siderosis. That kind of problem generally localizes to the top of the spinal cord at level C2 which can be impacted by many things such as a dural tear that causes SS but also other problems like disc disease. I would get a scan of the new to see what we’re dealing with at that C2 level.
Regarding the urinary symptoms, it’s probably a combination of age (prostate) and siderosis.
Amyloid angiopathy is an additional problem that causes superficial siderosis. Amyloid angiopathy is due to friable blood vessels within the brain that routinely bleed and leak out in the spinal cord fluid. I suspect the amyloid angiopathy will be a bigger concern than the superficial siderosis over the next few years. Ferriprox has never been tested in amyloid angiopathy or in superficial siderosis due to amyloid angiopathy.
Question:
My hearing and eye sight have deteriorated. My ophthalmologist feels that the problem is coming from the cerebellum as my eye sight is terrible. I have double vision also and experience what looks like black clouds going across my eyes. Can you shed some light on this? Have others had this problem and can it be corrected? Thank you Dr Levy for your help and input.
Answer:
The cerebellum is involved in maintaining sharp eye movements. The cerebellum is a common target of SS so eye movements are affected. The way this manifests is nystagmus, which is eye shaking. And that causes blurriness of vision.
Question:
My legs and hips are giving me fits every day. I'm on 10mg Baclofen 4 times a day. Does not seem to be helping. They tense up mainly in the thigh area and it gets really painful in my right hip where I have to take a very hot shower to hope it calms down. Exercise seems to make it worse. What do you suggest? It bothers my sleeping, too. I've been on Ropinirole for restless leg syndrome but that did not help either. I've been on Ferriprox for 3 years now. I've started losing a lot of hair and my fingernails are dry, splitting and peeling. Is this due to something the Ferriprox is doing? What should I do? Desperately needing advice.
Answer:
The spasms are probably due to hemosiderosis of the spine. That’s part of the Superficial Siderosis process. In addition to Baclofen, I often use anti-seizure medications like Gabapentin or Carbamazepine. You can ask your doctor for a trial (or me if I’m your doctor!). Your hair loss and brittle nails may be due to iron deficiency if you have no other explanation for them. One approach to long term treatment with Ferriprox is to try to use the Ferriprox every other month. In the off months, take iron pills to boost your body’s iron levels. It will presumably take longer to treat the siderosis but it may be more tolerable in the long run.
Question:
My mom had brain operation done 20 years back, due to blood clots found in brain. She has suffered migraines for 7 years now and recently, her headaches have become worse. Now her MRI report gives provisional diagnosis of superficial siderosis, though there are no clinical findings as yet. Please help me in this.
Answer:
An MRI that shows superficial siderosis without clinical symptoms is just early superficial siderosis. Eventually, the iron deposits will cause symptoms. It usually takes between 5-20 years. The headaches and migraines may be due to intermittent bleeds.
Superficial siderosis is both an MRI finding, and a condition. As an MRI finding, it may or may not be associated with symptoms. It may be that everyone with MRI findings of superficial siderosis will develop symptoms or it may be possible that some people are more resistant to the iron overload toxicity. The condition of superficial siderosis presents with hearing loss and balance problems, which occurs years after the first bleeding. Your mom may be someone whose MRI was done after the first bleeding, but before the first symptom. It may be that your mom’s symptoms may come on later in life. We cannot tell at this point.
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 once thick, beautiful hair - that I was constantly getting compliments on - is getting very thin. I run my hand through it and out comes a few strands every time. Especially when I wash it! I also color it to cover the grey (yes, it's true, I confess). I am finding now that the coloring isn't covering all my hair. Is the Ferriprox and lack of iron causing this? Is there something I can do about it?
Answer:
Iron deficiency can cause hair loss, yes. If you’re iron deficient (by lab testing, you should be anemic), you should stop the Ferriprox and take iron pills to recover. If you’re not iron deficient, it’s possible the hair loss is a side effect of the Ferriprox. The only way to know would be to stop the Ferriprox for a few weeks and see if the hair loss continues or not. If it’s not the Ferriprox, there are many other causes of hair loss you should investigate including hormonal and thyroid issues.
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 son has what he calls a bouncing eye so when he reads he closes one eye, is this related to SS?
Answer:
Very likely yes. This problem is caused nystagmus and is due to SS. There are other treatments available such as medications and special prism glasses but typically, nystagmus is difficult to treat.
Question:
New trial of antioxidant. I was diagnosed with SS 2.5years ago confirmed by 3 MRI's. If possible I wish very much to be included in the antioxidant group for testing or find out more about this. I live in Canada. My neurologist has offered me nothing not even hope. He knows nothing about SS, never had a patient and does not seem interested. Just live your life! I would also like very much to try the drug for the iron. My GP retired and I feel I have no one in my corner.
Answer:
Canada is where Ferriprox is made. And it is approved there, as well as antioxidants that may be protective. If your doctor is not helpful, perhaps seek out a vascular neurologist who is more interested in this condition.
Question:
Pain in both shoulders down to my elbows has been going on since mid December I have only had two days without pain. Could this be neuropathy caused by SS. Or am I looking at rheumatoid arthritis?
Answer:
Hard to tell. There can be many reasons for pain in the shoulders, arm and elbows. It could be neuropathy due to SS, but I would rule out the easily reversible causes first.
Question:
Recently diagnosed SS sufferer. Very interested to read all of the emails and especially to watch your broadcast. I was diagnosed last month having referred myself to a local Private Neurologist. For many years I have suffered with back pain, balance issues and headaches. Sadly this was treated by my GP surgery as being due to me being overweight!!! Having undergone many of the normal balance tests etc I was sent for two MRI scans. These highlighted a prolapsed disc at T8 and evidence of bleeding along the spine and around my brain. This was shown to me as if someone had taken a black highlighter pen and drawn around my spine and brain! I was asked about previous trauma to establish why the disc and why the bleed. My only knowledge is that I was diagnosed with viral encephalitis at age 5. I will be 50 later this year. As a result of the encephalitis I was left with total nerve deafness in my left ear which has never recovered. I was referred to a neuro surgeon who initially responded by stating he felt the risk of operating on T8 was too high for the potential rewards. The Neurologist who referred me to him stated that the risk of not operating was even higher. I am still, over 3 weeks later in a stalemate situation, awaiting a decision on the way forward .The issue it seems is whether the bleed/s will be found. Meanwhile my balance issues have worsened considerably and the constant back pain is becoming unbearable. Whilst I suffer also from fatigue and concentration issues as yet I haven't noticed any decline in my hearing, although I was left deaf in my left ear as a result of the encephalitis at age 5. I am slightly concerned of bowel and bladder issues having suffered a couple of problems in the last few months. Just before this issue I was advised that I also suffered with an under active thyroid however another GP said this was wrong. Has anyone also suffered SS and linked thyroid issues? I know I must stop reading about SS on Google and forums like this as it appears there is little of a positive nature to look forward to! Any help, guidance or encouragement will obviously be appreciated. Equally if there is anything about my diagnosis that help others now or in the future then please let me know.
Answer:
I think the best reason to consider surgery is to treat the back pain. That is likely due to the disc and not the siderosis. Your balance issues may be due to both, but it’s hard to know how much each is contributing unless you get the surgery and see how things turn out. Thyroid issues are not related to siderosis but they are very common in the general population.
Question:
Restless legs It's my left side is mainly affected but having said that, at night while trying to get to sleep my left leg quad muscles keep wanting to be flexed (if this makes sense). For all intents and purposes, its restless leg syndrome. Sometimes I'm getting it during the day while resting on my bed. I have to take a Valium at night. I'm not sure if I fall asleep from that first, or it calms my leg down. Either way, this is concerning to me. Will it get worse? Is it SS related? What should I do? (I take Neurontin 600 mgs 3 x day. I take B12 500 mgs (?) 2 x day and Magnesium 500 mgs x 2.)
Answer:
Yes, restless legs is very common in SS. I don’t know if it will get worse—it sounds pretty bad already. There are several approved medications and devices to help with restless legs syndrome that you can try.
Question:
SS in temporal lobes bilaterally. Dear Doctor, according to my neurosurgeon, I sustained an acute subdural hematoma along the right frontal, temporal and parietal lobes with traumatic subarachnoid haemorrhage in the right Sylvian fissure; and a haemorrhagic contusion of the left occipital lobe last May. MRI brain done this June shows that I’ve SS in temporal lobes bilaterally. However, my neurosurgeons are not sure of possible future complications. Currently, I am experiencing mainly some memory and attention issues, hyposmia and hypogeusia. What would be the possible symptoms I would be experiencing due to my SS? How long would the symptoms take to manifest?
Answer:
It sounds like you have superficial siderosis by MRI, but not the condition of superficial siderosis. You likely sustained a one-time bleed that deposited lots of iron around the brain. Most of these structures are unlikely to be affected by the iron deposition so I suspect you won’t have any symptoms over time. I suspect that your symptoms are mostly related to the head trauma.
Question:
Seizures Dr. Levy, I was diagnosed, one year ago with superficial siderosis by MRI. I am 67 years old and live in Burnaby, B.C. Canada. My neurologist specialises in MS and is humbly not 'up' on SS. Symptoms began with partial seizures and during the past year I have had two seizures where I went from that 'terrible feeling' for a couple minutes to 15-30 seconds of unconsciousness. Other than that, I have had somewhat mild symptoms such as short term memory, hearing problems, emotions, etc. I have the liberty to wait longer or start on an AED (antiepileptic drug). My doctor has suggested either Tegretol or Keppra. I have had two heart surgeries to replace the Mitral Valve and had a hybrid form of hemochromatosis. The bleed cannot be seen on the MRI. I am scheduled for a second one soon and an EEG. Any suggestions? Thanking you for your time in answering.
Answer:
Regarding the seizure medications, they are both good options. I don’t have a favorite between them. Regarding the diagnosis, if you don’t have SS by MRI, you certainly don’t have SS. Is that what you’re asking?
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:
Someone suggested trying Parkinson's medications for tremors. What are your thoughts about this?
Answer:
It’s possible that medications developed for other purposes like Parkinson’s disease may work to ease symptoms in superficial siderosis. Tremor is not a typical symptom in superficial siderosis but if that’s part of your disease course, please let the rest of the group know what medications work for your tremor.
Question:
Superficial siderosis expertise in Queensland. Please my husband I feel is in a bad way and I feel the NHS don’t want to know. And I’m becoming very worried. My husband recently collapsed with severe dizziness, vomiting and profuse sweating. He was admitted to hospital for 3 days where he was re-hydrated and extensive tests were done. The MRI results show siderosis with "cerebral amyloid angiopathy a consideration". Our GP has been less than helpful, unsympathetic and dismissive. What can we do? SE Queensland, Australia.
Answer:
I cannot be sure without viewing the MRI myself, but I can explain what I know about the link between amyloid angiopathy and superficial siderosis. Amyloid angiopathy is a disease of the blood vessels within the brain. The vessel walls because weakened and are prone to break and bleed. If the bleeds leak blood into the spinal fluid, it can cause superficial siderosis. There is no treatment for amyloid angiopathy. The underlying process is similar to Alzheimer’s disease and we have no treatment for that either. Regarding the episode of dizziness, vomiting and sweating, I am not sure what that was. It sounds like it could have been dehydration related to a stomach virus and perhaps unrelated to the neurological issue? I am not sure.
Question:
Superficial siderosis in right-brain due to cerebral venous sinus thrombosis (CVST) haemorrhage. I have recovered from CVST but due to haemorrhage, ss is active. I am taking levetiracetam is this reducing my siderosis problem? At the moment I’m only experiencing dizziness but I have superficial siderosis on my mind. Can it be cured permanently?
Answer:
If you have a one-time cyst haemorrhage, it’s unlikely to cause superficial siderosis. You may have blood products deposited on the brain after the haemorrhage, but usually, the brain has the capacity to remove those over time. It’s only from repeated bleeds that occur over many years that the removal process is overwhelmed and that leads to superficial siderosis neurodegeneration.
We are trying to cure SS by using several parallel approaches: 1. Stop the bleeding, 2. Remove the iron deposits and 3. Prevent neural damage from the old iron deposits.
Question:
Superficial siderosis symptoms. My husband has recently been diagnosed with superficial siderosis. He has had uncontrollable sweating and intense itching under the skin for 5 or more years. Recently, he has times when the top of his head gets very hot. Are these all symptoms of siderosis?
Answer:
If I had to connect these symptoms to a neurological issue, I can imagine that perhaps the nerves that innervate the scalp might be involved. Those nerves exit the top of the spinal cord and course around the back of the head towards the face – one on each side. If that distribution fits his symptoms, perhaps there is a problem in this area of the spinal cord (C2 level).
Question:
The neuropathy in my feet has gotten so much worse in the last month or two I can hardly stand it. It used to be bad at night and I'd put on hospital socks so I could sleep. Now I have to double up the socks at night and wear them all day. I often have to fold an afghan over and bury my feet in it to try to warm them up. My feet are freezing and burning more than ever before. I'm taking Neurontin - 600 mgs three times a day. It's barely helping now. My feet, toes, and ankles tingle like they're falling asleep and the numbness is creeping up to my left ankle. HELP. what do I do?
Answer:
Neuropathy from superficial siderosis is treated the same way as neuropathy from any other cause like diabetes. Neurontin is a popular option, but there are many. They come in three classes: anti-seizure drugs like Neurontin, anti-depressants and anti-inflammatories. It’s trial and error for everyone to see which ones work and which ones don’t.
Question:
Trying to find a new doctor who wants to help. Thought you might have a colleague at IU Med in Indianapolis Ind who will work with me on my superficial siderosis. The Dr I seen brushed me off. I need one who want to help.
Answer:
The only other doctor with lots of expertise in superficial siderosis in the US is Dr. Raj Kumar at the Mayo Clinic in Rochester, MN.
Question:
Vagus Nerve. Excessive sweating. Someone mentioned that this could be due to damage to the vagus nerve. Have you come across this as a symptom of SS? Could the vagus nerve also be the cause of acid reflux? I suffer from both! Is there a connection? Many thanks.
Answer:
The vagus nerve is generally spared in superficial siderosis. Only 1-2 mm of this nerve is exposed to the spinal fluid and the iron particles within it. However, there are other nerves in the spinal cord that assist with autonomic function including swallowing, blood vessel control, sweating, etc. Dysfunction of these spinal cord nerves can be due to SS.
Question:
Vibrations. Hi My mum complains of a feeling of vibrations. She feels it quite often and feels like she can’t get away from it. Is this someone that is related to SS?
Answer:
It could be related to siderosis. A sensation of vibrations is a form a neuropathy which can be due to siderosis of the nerves entering the spinal cord. Sometimes patients benefit from gabapentin or carbamazepine.
Question:
When I brush my teeth, my right ring finger becomes tingly and numb. When putting on makeup or holding my cellphone in my left hand, all my left hand fingers start to tingle and go numb. Then my right hand fingers join in. I can't hold my arms up for more than 2 minutes before my fingers start to tingle with pins and needles, and go numb. And very lately the tingling travels up my left wrist and into my forearm. Does this mean my SS is getting worse ? This is relatively new - the last 6 months or so.
My skin gets very itchy also. Is this related to that neuropathy?
One more thing (promise) If I turn my head to look left, I totally lose my balance. Not good when checking for oncoming cars while crossing a parking lot. So I don't. I just hope people see this girl with the cane and politely stop for me to cross. Is this SS related?
Okay. I know. Lots of questions. I thank you kindly.
Answer:
The tingling in your hands and arms could be siderosis, but it could also be carpal tunnel and degenerative disc disease. Usually neuropathy from siderosis is not triggered by movement – that’s more suggestive of degenerative disc disease. Itching could be a sign of neuropathy too, yes.
Losing your balance while turning your head is absolutely due to siderosis. It involves the same nerve as the one you use for hearing and that nerve is very susceptible to damage from siderosis.
Question:
Would magnets worn on the head help shift the iron deposits?
Answer:
That's thinking outside the box! Hemosiderin (the protein-iron complex on your brain) is paramagnetic at best. That is, it is just weakly attracted to magnetic fields.
Question:
Yesterday it was a very hot day and I got sunburnt despite putting sun cream on.. I put it on my facebook and a friend asked if I took warfarin (I used to take it) I said no, I stopped ur years ago however with the fact I now take ferriprox, can it cause the same thing (make you more prone to getting sunburnt) as ferriprox is to get rid of the iron.. Both blood related so just curious. Also as a last note, I don't normally get sunburnt as I have 'olive skin' I have always been told hat.
Answer:
This is the first time an SS patient has reported sun sensitivity due to Ferriprox to me. However, it must be common enough in other conditions because the Ferriprox label includes a warning about sunburns.
Question:
You have mentioned that all patients will have a continuing bleed if it has not been stopped. My MRI s have shown I do not have an active bleed, but do I have an intermittent bleed?
Answer:
MRIs do not show active bleeds. The only way to see active bleeds is to obtain a lumbar puncture (spinal tap). If you bled within the last 2 days, you can detect red blood cells. If you bled within the last 2 months, you will detect blood products. If you bled more than 2 months ago, the spinal fluid may appear normal. If you have an active bleed by lumbar puncture, you should pursue finding the leak. CT myelogram is the best test to find a tear of the dura in the spinal cord (most common cause of SS). 70/ How is an intermittent bleed recognised on an MRI or can it only be identified surgically? Answer: MRIs over time can show a buildup and expansion in the iron deposition. If there is more iron depositing over time, the presumption is that you’re bleeding either continuously or intermittently. MRIs change very slowly. Do not expect any changes (worse or better) for 2-3 years at least. Don’t be reassured if your MRI next year is no different from this year’s. One year is too short a time period to see a change when the bleeding is ongoing unless it’s really bleeding a lot. You won’t find intermittent bleeding surgically. Surgical options are available to stop the bleeding if found. There are two ways to detect intermittent bleeds:
#1: Random check-- you can randomly look as many of you have done. You might catch it.
#2: Wait until you think you're bleeding-- some people have episodes of severe headache, neck stiffness and mild fever during a bleed (if there is enough blood). The earlier in the disease course, the more likely you can sense the bleeding. It's best to get the test done on the day of the symptom onset, if possible.
Question:
Shortness of Breath. Dr Levy, could shortness of Breath be related to superficial siderosis? I have been checked by my hospital in London for this and they think it could be due to my SS as all there tests on my heart have been fine. I was diagnosed with SS last year. I have Marfan’s. One flight of stairs and I am fighting for breath.
Answer:
I would be very reluctant to blame superficial siderosis for shortness of breath on exertion. I can’t think of a direct mechanism whereby SS would do that. If SS is on your doctor’s list, it should be near the bottom. I would be much more worried about cardiovascular disease, lung issues, blood clots, etc.
Question:
Trying to find a new doctor who wants to help. Thought you might have a colleague at IU Med in Indianapolis Ind who will work with me on my superficial siderosis. The Dr I seen brushed me off. I need one who want to help.
Answer:
The only other doctor with lots of expertise in superficial siderosis in the US is Dr. Raj Kumar at the Mayo Clinic in Rochester, MN.
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:
Hallucinations. My husband has SS. This morning he told me that he has been having hallucinations at least 2 to 3 times a week, mostly after he has just woken in the daytime (also after napping) and they last an hour or two. He has lost hearing and most of his eyesight. What causes this to occur? Are there any suggestions of what I could do to help him? Thankfully he is lucid and clear thinking the rest of the time.
Answer:
Auditory and visual hallucinations can occur for a number of reasons. Many of them are benign. For example, people with poor vision from any cause can have visual hallucinations in the dark. Sometimes just making the room brighter will help this issue. For others, certain medications may cause hallucinations especially if associated with confusion at the same time of day after taking a medication– in that case, reducing the dose will help. Auditory hallucinations have also been reported in SS especially in cases with very severe hearing loss. The first step to fixing this issue is trying to figure out what’s causing the hallucinations. First, try to improve the lighting. Then, review the list of medications. And once the common things are ruled out, it may be an SS thing.
Question:
I know I have had a bleed, but I am wondering if that bleed can last for two weeks. I had a lumbar puncture and the doctor doing it had to change the needle as the blood was clogging up the smaller needle. She then changed to a larger needle. When she had finished she showed me the vials and they were really red. I have been feeling quite sick for the last two weeks, it was only a week ago that I had lumbar puncture. My head feels like it is going to explode and my neck on the left side is stiff and really sore. I can't turn my head to the right. I have to sleep partly sitting up, also I can't lie on the pillow with my left side. Dr Levy could a bleed last that long, it has made me feel really sick, that I have to sleep 1/2 the day just to get thru the rest of the day. Would like to know your input as to what may be going on with me.
Answer:
This is an acute bleed. See answer below about acute bleed.
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 though 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 everyone 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, I have been having terrible pain in my lower back, it seems to be coming from my spine, not tissue damage. Can SS cause this and what should I take if it is SS? I find it hard sitting for too long and standing and walking, and trying to turn over in bed.
Answer:
Thanks for your input. It can due to many things related to your SS including muscle spasms and neuropathy, and even the acute bleed. It’s hard to tell for sure.
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 have neuropathy pain in my arms, legs and face. Until a few weeks ago I had only burning and numb feet. Since a few days ago I have the burning also in my face, neck, back, hand, arms and legs. My neurologist does not believe that it is a SS symptom, because all measuring (Nerve conduction study, Electromyography, Evoked potential, Somatosensory evoked potential) are normal. Is neuropathy caused by SS measurable?
Answer:
Sometimes. Given your history, all of your neuropathy is most likely due to SS.
Question:
I am experiencing severe pain in my head and neck area every time I move my head. This has been going on for at least two weeks. I am in touch with the web site and realise it is an active bleed, neurologists here don't know what can be done. I am desperate for some direction, could you advise? Applying heat seems to relieve it somewhat temporarily. I was diagnosed with SS in 1993.
Answer:
Neck pain is likely due to an acute bleed. It causes irritation of the lining around the spinal cord. The best things to do in this situation are: 1. Hydration as much as possible. 2. Ibuprofen 600 – 800 mg three times per day. 3. Rest. It should go away in 2-3 days. Also, try to avoid precipitating the bleed if you know what activities trigger it.
Question:
My hearing and eye sight have deteriorated. My ophthalmologist feels that the problem is coming from the cerebellum as my eye sight is terrible. I have double vision also and experience what looks like black clouds going across my eyes. Can you shed some light on this? Have others had this problem and can it be corrected? Thank you Dr Levy for your help and input.
Answer:
The cerebellum is involved in maintaining sharp eye movements. The cerebellum is a common target of SS so eye movements are affected. The way this manifests is nystagmus, which is eye shaking. And that causes blurriness of vision.
Question:
Seizures Dr. Levy, I was diagnosed, one year ago with superficial siderosis by MRI. I am 67 years old and live in Burnaby, B.C. Canada. My neurologist specialises in MS and is humbly not 'up' on SS. Symptoms began with partial seizures and during the past year I have had two seizures where I went from that 'terrible feeling' for a couple minutes to 15-30 seconds of unconsciousness. Other than that, I have had somewhat mild symptoms such as short term memory, hearing problems, emotions, etc. I have the liberty to wait longer or start on an AED (antiepileptic drug). My doctor has suggested either Tegretol or Keppra. I have had two heart surgeries to replace the Mitral Valve and had a hybrid form of hemochromatosis. The bleed cannot be seen on the MRI. I am scheduled for a second one soon and an EEG. Any suggestions? Thanking you for your time in answering.
Answer:
Regarding the seizure medications, they are both good options. I don’t have a favorite between them. Regarding the diagnosis, if you don’t have SS by MRI, you certainly don’t have SS. Is that what you’re asking?
Question:
SS in temporal lobes bilaterally. Dear Doctor, according to my neurosurgeon, I sustained an acute subdural hematoma along the right frontal, temporal and parietal lobes with traumatic subarachnoid haemorrhage in the right Sylvian fissure; and a haemorrhagic contusion of the left occipital lobe last May. MRI brain done this June shows that I’ve SS in temporal lobes bilaterally. However, my neurosurgeons are not sure of possible future complications. Currently, I am experiencing mainly some memory and attention issues, hyposmia and hypogeusia. What would be the possible symptoms I would be experiencing due to my SS? How long would the symptoms take to manifest?
Answer:
It sounds like you have superficial siderosis by MRI, but not the condition of superficial siderosis. You likely sustained a one-time bleed that deposited lots of iron around the brain. Most of these structures are unlikely to be affected by the iron deposition so I suspect you won’t have any symptoms over time. I suspect that your symptoms are mostly related to the head trauma.
Question:
Has anyone also suffered SS and linked thyroid issues? I know I must stop reading about SS on Google and Forums like this as it appears there is little of a positive nature to look forward to! Any help, guidance or encouragement will obviously be appreciated. Equally if there is anything about my diagnosis that help others now or in the future then please let me know.
Answer:
No, I don’t know of any link between SS and thyroid disease. Thyroid disease is so common that is would not surprise me if any SS patient also has thyroid disease.
Question:
My father has hearing loss and bladder problems that have been attributed to age and prostate conditions but I feel may be associated to the SS. Aside from these, his primary symptom is a feeling of tightness around the head and of being incredibly hot despite his temperature being normal. The hotness is proving incredibly distressing - he is so hot he wants to be in an ice bath. I can see no other reports of this as a symptom and I wonder if you have heard of it as a symptom and, if so, do you have any tips on managing it? Second query is that the cause of my father's SS has been given as amyloid angiopathy and I wondered if this makes any difference in terms of trying iron chelation as a therapy. Can we still approach the SS in the same way as one would approach it when it is caused by trauma? Many thanks indeed for your efforts and dedication to this site. I will be checking in and passing it onto my parents for their info.
Answer:
I don’t know if the banding sensation around his head and the hotness are due to superficial siderosis. That kind of problem generally localizes to the top of the spinal cord at level C2 which can be impacted by many things such as a dural tear that causes SS but also other problems like disc disease. I would get a scan of the new to see what we’re dealing with at that C2 level.
Regarding the urinary symptoms, it’s probably a combination of age (prostate) and siderosis.
Amyloid angiopathy is an additional problem that causes superficial siderosis. Amyloid angiopathy is due to friable blood vessels within the brain that routinely bleed and leak out in the spinal cord fluid. I suspect the amyloid angiopathy will be a bigger concern than the superficial siderosis over the next few years. Ferriprox has never been tested in amyloid angiopathy or in superficial siderosis due to amyloid angiopathy.
Question:
Restless legs It's my left side is mainly affected but having said that, at night while trying to get to sleep my left leg quad muscles keep wanting to be flexed (if this makes sense). For all intents and purposes, its restless leg syndrome. Sometimes I'm getting it during the day while resting on my bed. I have to take a Valium at night. I'm not sure if I fall asleep from that first, or it calms my leg down. Either way, this is concerning to me. Will it get worse? Is it SS related? What should I do? (I take Neurontin 600 mgs 3 x day. I take B12 500 mgs (?) 2 x day and Magnesium 500 mgs x 2.)
Answer:
Yes, restless legs is very common in SS. I don’t know if it will get worse—it sounds pretty bad already. There are several approved medications and devices to help with restless legs syndrome that you can try.
Question:
I have been taking Ferriprox over 4 yrs now. I had some balance improvement by the end of the first year. In the past 6 months I have been experiencing vertigo/dizziness, pain in my lower back & the back of my head, and sharp pains in my feet, legs, and eyes. Along with all of this, I still have continued loud ear ringing and extreme fatigue. Can all of this be related to Superficial Siderosis? Thanks for all you do! God bless you!
Answer:
Vertigo/dizziness, pain and loud ringing in your ears (tinnitus) are almost certainly due to ongoing issues related to superficial siderosis. The extreme fatigue is probably due to the Ferriprox. To address the fatigue, you can try to reduce the morning dose.
Question:
Brain fog I workout early in the morning. After some workouts I'm finding I get brain fog a couple of hours later where it's hard to think and concentrate. It lasts for a few hours. I don't know the reason. I was wondering if it is just being tired or if I'm causing a bleed or doing any damage. I do have a weak neck from the operation 27 years ago to remove the tumor from my cerebellum.
Answer:
I am not sure why you are getting these transient brain fog episodes after working out. I would look for other associations – medications (especially Ferriprox), sleep, body temperature – that may explain what is going on.
Question:
My mom had brain operation done 20 years back, due to blood clots found in brain. She has suffered migraines for 7 years now and recently, her headaches have become worse. Now her MRI report gives provisional diagnosis of superficial siderosis, though there are no clinical findings as yet. Please help me in this.
Answer:
An MRI that shows superficial siderosis without clinical symptoms is just early superficial siderosis. Eventually, the iron deposits will cause symptoms. It usually takes between 5-20 years. The headaches and migraines may be due to intermittent bleeds.
Superficial siderosis is both an MRI finding, and a condition. As an MRI finding, it may or may not be associated with symptoms. It may be that everyone with MRI findings of superficial siderosis will develop symptoms or it may be possible that some people are more resistant to the iron overload toxicity. The condition of superficial siderosis presents with hearing loss and balance problems, which occurs years after the first bleeding. Your mom may be someone whose MRI was done after the first bleeding, but before the first symptom. It may be that your mom’s symptoms may come on later in life. We cannot tell at this point.
Question:
I was wondering if swollen leg is an issue? I just seem to have noticed my left thigh/ ankle and foot seems swollen. The toes on my left foot is a little spaced out as well. I am 37 have had SS for many years but only got diagnosed 3 or so years ago.
Answer:
Superficial siderosis doesn’t cause leg swelling per se. I would recommend getting checked out for common causes of leg swelling such as blood clots and mechanical compression. However, if the spinal cord is significantly involved from superficial siderosis, you may have dysautonomia, which may cause some leg swelling and redness. But this is rare and usually involves both legs.
Question:
I was diagnosed with immune deficiency around the same time it was found I have SS. I have had immunoglobulin therapy for 1 year now. I feel like when I have an intermittent bleed now my recovery from that episode is a little faster than before the IG. It could be wishful thinking but do think there is any benefit of me taking this for my SS? Do you think there is any connection between people who get SS and being immunocompromised?
Answer:
I cannot think of a link between your immune disorder and superficial siderosis. I also cannot think of how IVIG might be useful in your recovery from an intermittent bleed except that maybe the infusion is keeping you well hydrated.
Question:
Someone suggested trying Parkinson's medications for tremors. What are your thoughts about this?
Answer:
It’s possible that medications developed for other purposes like Parkinson’s disease may work to ease symptoms in superficial siderosis. Tremor is not a typical symptom in superficial siderosis but if that’s part of your disease course, please let the rest of the group know what medications work for your tremor.
Question:
Hi, Is there a complete list of symptoms somewhere please?
Answer:
Yes, the Silent Bleed Superficial Siderosis symptom page gives a good answer.
Question:
My legs and hips are giving me fits every day. I'm on 10mg Baclofen 4 times a day. Does not seem to be helping. They tense up mainly in the thigh area and it gets really painful in my right hip where I have to take a very hot shower to hope it calms down. Exercise seems to make it worse. What do you suggest? It bothers my sleeping, too. I've been on Ropinirole for restless leg syndrome but that did not help either. I've been on Ferriprox for 3 years now. I've started losing a lot of hair and my fingernails are dry, splitting and peeling. Is this due to something the Ferriprox is doing? What should I do? Desperately needing advice.
Answer:
The spasms are probably due to hemosiderosis of the spine. That’s part of the Superficial Siderosis process. In addition to Baclofen, I often use anti-seizure medications like Gabapentin or Carbamazepine. You can ask your doctor for a trial (or me if I’m your doctor!). Your hair loss and brittle nails may be due to iron deficiency if you have no other explanation for them. One approach to long term treatment with Ferriprox is to try to use the Ferriprox every other month. In the off months, take iron pills to boost your body’s iron levels. It will presumably take longer to treat the siderosis but it may be more tolerable in the long run.
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 vasospsms. 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 suspectible 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 milliliter of spinal fluid.
Question:
Is it normal to have Parkinson's like shaking with Superficial Siderosis?
Answer:
No, tremors are not typical with SS neither are Parkinsons symptoms. That doesn’t sound like SS to me.
Question:
My son has what he calls a bouncing eye so when he reads he closes one eye, is this related to SS?
Answer:
Very likely yes. This problem is caused nystagmus and is due to SS. There are other treatments available such as medications and special prism glasses but typically, nystagmus is difficult to treat.
Question:
I am much worse in the summer heat; energy and balance. Also having some trouble swallowing all of a sudden, is this due to SS?
Answer:
Swallowing problems are common in SS. It’s usually not a sudden issue though. It typically starts with trouble swallowing liquids and then slowly progresses to solids.
Heat (and cold) can both impact on brain function. It doesn’t cause any damage but it makes the symptoms feel worse. Consider a cooling vest in the summer.
Question:
My brother had a brain tumor removed last October and it wasn't until January that they told us his pre surgery MRI showed signs of SS. He lost a lot of his eye sight, cognitive skills, and coordination after the surgery. But he was recovering over time with lots of therapy and family support. He came to see you with his wife in the spring. It seemed he was in the bottom 10% of symptoms and was doing okay. He was regaining a lot of deficits until he had a seizure in June. At that point he lost a lot of his hearing, eyesight, balance and is in a huge amount of pain. They found what possibly might be a new tumor growing in a new location but decided it wasn't significant enough at the moment to take out.
Question 1: are seizures related to SS?
Question 2: Could these new symptoms be related to SS, even though they were symptoms he had post-surgery and he had previously been regaining back? In other words do SS symptoms fluctuate back and forth? Thank you for your time.
Answer:
Seizures are not related to SS. They are related to brain tumors though. SS lowers the “neurological reserve” so that any metabolic stressor (seizures, medication change, surgery, infection) makes recovery a lot slower.
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 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 thining 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:
When I brush my teeth, my right ring finger becomes tingly and numb. When putting on makeup or holding my cellphone in my left hand, all my left hand fingers start to tingle and go numb. Then my right hand fingers join in. I can't hold my arms up for more than 2 minutes before my fingers start to tingle with pins and needles, and go numb. And very lately the tingling travels up my left wrist and into my forearm. Does this mean my SS is getting worse ? This is relatively new - the last 6 months or so.
My skin gets very itchy also. Is this related to that neuropathy?
One more thing (promise) If I turn my head to look left, I totally lose my balance. Not good when checking for oncoming cars while crossing a parking lot. So I don't. I just hope people see this girl with the cane and politely stop for me to cross. Is this SS related?
Okay. I know. Lots of questions. I thank you kindly.
Answer:
The tingling in your hands and arms could be siderosis, but it could also be carpal tunnel and degenerative disc disease. Usually neuropathy from siderosis is not triggered by movement – that’s more suggestive of degenerative disc disease. Itching could be a sign of neuropathy too, yes.
Losing your balance while turning your head is absolutely due to siderosis. It involves the same nerve as the one you use for hearing and that nerve is very susceptible to damage from siderosis.
Question:
My once thick, beautiful hair - that I was constantly getting compliments on - is getting very thin. I run my hand through it and out comes a few strands every time. Especially when I wash it! I also color it to cover the grey (yes, it's true, I confess). I am finding now that the coloring isn't covering all my hair. Is the Ferriprox and lack of iron causing this? Is there something I can do about it?
Answer:
Iron deficiency can cause hair loss, yes. If you’re iron deficient (by lab testing, you should be anemic), you should stop the Ferriprox and take iron pills to recover. If you’re not iron deficient, it’s possible the hair loss is a side effect of the Ferriprox. The only way to know would be to stop the Ferriprox for a few weeks and see if the hair loss continues or not. If it’s not the Ferriprox, there are many other causes of hair loss you should investigate including hormonal and thyroid issues.
Question:
The neuropathy in my feet has gotten so much worse in the last month or two I can hardly stand it. It used to be bad at night and I'd put on hospital socks so I could sleep. Now I have to double up the socks at night and wear them all day. I often have to fold an afghan over and bury my feet in it to try to warm them up. My feet are freezing and burning more than ever before. I'm taking Neurontin - 600 mgs three times a day. It's barely helping now. My feet, toes, and ankles tingle like they're falling asleep and the numbness is creeping up to my left ankle. HELP. what do I do?
Answer:
Neuropathy from superficial siderosis is treated the same way as neuropathy from any other cause like diabetes. Neurontin is a popular option, but there are many. They come in three classes: anti-seizure drugs like Neurontin, anti-depressants and anti-inflammatories. It’s trial and error for everyone to see which ones work and which ones don’t.
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 have obtained a personal copy of my MRI on CD and this is the first time I have seen it. The images are very interesting (I even have two gaps in my skull I never knew about!) well the back one is most likely due to my operation that caused the SS in me but my main question is; how do you identify a bleed on an MRI scan?
Answer:
depends on the sequence of MRI and when the bleeding occurred. Hemosiderin, the blood product in SS is black on the T2 sequence and much more visible on gradient echo MRI.
Question:
I am diagnosed with SS one year ago. I am from Lithuania, maybe first patient with SS in my country. What are the statistics for SS? How many SS cases are out there per million people? I can't walk without crutches anymore because of poor coordination, also I can constantly "hear" rhythmic noises in my head. Is that some of the symptoms of SS?
Answer:
SS is incredibly rare. The incidence is approximately 1 per million. Your symptoms of balance problems and tinnitus are classic for SS.
Question:
Are there any reasons why someone with SS could not have a tattoo?
Answer:
If your spouse is against it, I strongly recommend against having a tattoo.
Question:
Is there anything SS patients should avoid? Any activities that should be avoided? Any foods/medications that should be avoided?
Answer:
Activities to avoid: anything that re-tears the lining around your spinal cord (for most, that is the source of bleeding).
Foods to avoid: anything that will stick to the Ferriprox in your gut: foods that contain high vitamin C, iron or zinc content 2 hours before or after taking the medicine. No pills containing iron, zinc or vitamin C at all.
Question:
Would magnets worn on the head help shift the iron deposits?
Answer:
That's thinking outside the box! Hemosiderin (the protein-iron complex on your brain) is paramagnetic at best. That is, it is just weakly attracted to magnetic fields.
Question:
It's been suggested to hold a convention for SS which is not practical, financially or geographically. But how about a webinar? Maybe we could set it up through Johns Hopkins University or Johns Hopkins Hospital, but if not we could set it up ourselves. Our group spans such a huge geographic area, UK to California, even Australia, that we would have to be careful with time. I'm sure Jason Roberts would be on board & we could all learn, even ask questions, from the SS expert. What do you think? My tech savvy kids can help if we need!
Answer:
I think that's a great idea. I would be happy to participate although I’m terrible at the logistics, organization and technical issues. But would be great to do!!
N.B. The webinar did take place on 15/07/15, please view it here.
Question:
Yesterday it was a very hot day and I got sunburnt despite putting sun cream on.. I put it on my facebook and a friend asked if I took warfarin (I used to take it) I said no, I stopped ur years ago however with the fact I now take ferriprox, can it cause the same thing (make you more prone to getting sunburnt) as ferriprox is to get rid of the iron.. Both blood related so just curious. Also as a last note, I don't normally get sunburnt as I have 'olive skin' I have always been told hat.
Answer:
This is the first time an SS patient has reported sun sensitivity due to Ferriprox to me. However, it must be common enough in other conditions because the Ferriprox label includes a warning about sunburns.
Question:
You have mentioned that all patients will have a continuing bleed if it has not been stopped. My MRI s have shown I do not have an active bleed, but do I have an intermittent bleed?
Answer:
MRIs do not show active bleeds. The only way to see active bleeds is to obtain a lumbar puncture (spinal tap). If you bled within the last 2 days, you can detect red blood cells. If you bled within the last 2 months, you will detect blood products. If you bled more than 2 months ago, the spinal fluid may appear normal. If you have an active bleed by lumbar puncture, you should pursue finding the leak. CT myelogram is the best test to find a tear of the dura in the spinal cord (most common cause of SS). 70/ How is an intermittent bleed recognised on an MRI or can it only be identified surgically? Answer: MRIs over time can show a buildup and expansion in the iron deposition. If there is more iron depositing over time, the presumption is that you’re bleeding either continuously or intermittently. MRIs change very slowly. Do not expect any changes (worse or better) for 2-3 years at least. Don’t be reassured if your MRI next year is no different from this year’s. One year is too short a time period to see a change when the bleeding is ongoing unless it’s really bleeding a lot. You won’t find intermittent bleeding surgically. Surgical options are available to stop the bleeding if found. There are two ways to detect intermittent bleeds:
#1: Random check-- you can randomly look as many of you have done. You might catch it.
#2: Wait until you think you're bleeding-- some people have episodes of severe headache, neck stiffness and mild fever during a bleed (if there is enough blood). The earlier in the disease course, the more likely you can sense the bleeding. It's best to get the test done on the day of the symptom onset, if possible.
Question:
Is sugar making my neuropathy-worse?
Answer:
maybe. If you are diabetic or borderline diabetic, sugar can cause neuropathy which will make your SS-neuropathy feel worse.
Question:
Vagus Nerve. Excessive sweating. Someone mentioned that this could be due to damage to the vagus nerve. Have you come across this as a symptom of SS? Could the vagus nerve also be the cause of acid reflux? I suffer from both! Is there a connection? Many thanks.
Answer:
The vagus nerve is generally spared in superficial siderosis. Only 1-2 mm of this nerve is exposed to the spinal fluid and the iron particles within it. However, there are other nerves in the spinal cord that assist with autonomic function including swallowing, blood vessel control, sweating, etc. Dysfunction of these spinal cord nerves can be due to SS.
Question:
New trial of antioxidant. I was diagnosed with SS 2.5years ago confirmed by 3 MRI's. If possible I wish very much to be included in the antioxidant group for testing or find out more about this. I live in Canada. My neurologist has offered me nothing not even hope. He knows nothing about SS, never had a patient and does not seem interested. Just live your life! I would also like very much to try the drug for the iron. My GP retired and I feel I have no one in my corner.
Answer:
Canada is where Ferriprox is made. And it is approved there, as well as antioxidants that may be protective. If your doctor is not helpful, perhaps seek out a vascular neurologist who is more interested in this condition.
Question:
Pain in both shoulders down to my elbows has been going on since mid December I have only had two days without pain. Could this be neuropathy caused by SS. Or am I looking at rheumatoid arthritis?
Answer:
Hard to tell. There can be many reasons for pain in the shoulders, arm and elbows. It could be neuropathy due to SS, but I would rule out the easily reversible causes first.
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