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Part of our Dr Levy Q&A pages
For the last 10 years i have been experiencing many debilitating neurological symptoms and some diagnoses such as Craniocervical instability, occult tethered cord syndrome and propable arachnoiditis. I would like to ask who is the most suitable doctor to diagnose an active CSF bleed/leak and confirm/rule out SS. A neuroradiologist or a neurosurgeon.
A neurosurgeon is a good doctor for fixing surgical problems in the spinal column. A neuroradiologist is a good doctor for 1. Identifying leaks based on MRI and myelograms and 2. Injecting glue or blood patches to correct problems that are non-surgical. A neurologist is the doctor who would help coordinate the care, make the diagnosis and initiate chelation therapy.
I have an AVM - Grade 4, inoperable. It bled repeatedly twenty years ago (9 bleeds), but seemed to settle down after Proton Beam Radiation (which did not work, but strengthened the vessels enough that I was relatively well for twenty years). This past year, I've been dealing with increasing dizziness, tingly and painful feet and behaviour changes...felt as though I've been going insane. My first question (I'm sure there will be more...lol)...can SS symptoms be transient/periodic? I have had a few episodes of severe dizziness that lasts three to four days, and then it'll back off to mild feelings of being off balanced or lightheaded, and then I'll have days where I feel well and steady. Same with my feet, some days good, some bad, some in-between. Can the symptoms come and go like that? Also...why would it only show up on MRI after twenty years? Or is it that, in all of the 20 years I've been getting scanned, this was the only time it was seen by someone who knew to look for it?
Superficial siderosis is a progressive condition. It progresses very slowly and while it causes damage to the brain tissue, your brain will try to compensate. It’s the compensation ability that fluctuates, not the siderosis. Compensation ability is bolstered by good sleep, regular exercise, physical therapy, young age. Compensation ability is hampered by heat, stress, poor sleep, illness and older age.
Superficial siderosis worsens radiologically over time. Depending on how much iron/blood there is, regular MRIs may miss it. Special MRI scans called T2*(star), gradient echo imaging, or susceptibility weighted imaging make the iron/blood much more visible and obvious. These scans are being done more routinely these days.
I was recently diagnosed with SS after an MRI ordered because of vertigo. I have a history of iron deficiency anemia and have been working with a hematologist and getting intravenous iron infusions. I read that SS patience have normal iron levels, so I am wondering if the infusions were a bad idea and made the situation worse. Also, would I be a candidate to take ferriprox because my iron levels are already so low?
Iron infusions into the blood stream are not generally harmful. When you bleed into the brain, most of the iron that deposits comes from blood cells. If you have transient higher levels of iron in the blood from the infusions at the time of a bleed, the contribution is probably minimal compared to the amount of iron in red blood cells.
Iron deficiency anemia can make chelation challenging because chelation therapy is not directed to the brain. Chelators remove iron from the whole body. When I treat my iron-deficient patients with chelation therapy, I often will use chelation for 5 days per week and iron supplements (rapidly absorbed iron supplements, not the slow release form) for the other 2 days per week.
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?
I have had two patients with audio hallucinations but delirium is unusual in superficial siderosis. I would look into other potential causes.
Superficial Siderosis charter
Hello Dr Levy Can you please advise if there is a European charter for other doctors to view/use on how to test for and treat Superficial Siderosis. Only a point recently made on the fb forum highlighted that even MRI'S are possibly not being completed correctly. A gent said to identify a leak/bleed you need to have your hips raised during the MRI. Alot of UK doctors are not well informed regarding SS.
Superficial siderosis is very rare. There are no standards or charters that I am aware of. In the UK, Dr. David Werring is very knowledgeable about the condition.
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?
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.
How to access Ferriprox
Dr. Levy, a family member who suffered a major spinal cord injury decades ago has had two MRIs that show SS and has experienced several years of hearing loss and cerebellar ataxia. This family member also has a history of DVT and is on OAT. In your webinar, you suggest that all SS patients should be initiated on Ferriprox, since doing nothing means inevitable progression. As far as I know, my family member's physician and neurologist have not discussed initiation of Ferriprox chelation. How difficult and expensive is it to access Ferriprox? My family member is insured through a state insurance exchange. What can we do to try and access the drug? And is the DVT/OAT a contraindication? Thanks!
Ferriprox is expensive in the US, but it is fairly easy to access online (in the US) and available in many countries. There is also generic versions (deferiprone) and in India it’s called Kelfer that are less expensive. It’s always worth requesting insurance coverage for these medications before paying for them out of pocket.
Using blood thinners can increase the bleeding risk in the brain and spinal cord in superficial siderosis. However, blood thinners are generally life saving for people with blood clots and other disorders. In most cases, even if they worsen the siderosis, I continue to use them in patients who need them. You can use chelating agents while using blood thinners at the same time.
Options for pain reduction?
I have severe pain just above my left hip in my lower back. I have gotten to the point where the pain bothers me enough to keep me from walking or working out. I have tried Cymbalta and it made me very depressed. I have tried a couple others in that category with similar effects including a lot of sleeping. 800mg of ibuprophen was helping but per this site, we SS'ers shouldn't take it, is that correct? I am now using Tylenol which amazingly is helping. Is tylenol ok to use for the every 4 hours. What else can I try that is not is the area of cymbalta if tylenol is not good? I've read on the facebook page some people are using Morphine. Please advise.
Ibuprofen is generally safe to use. It is a weak blood thinner but generally not harmful in superficial siderosis. Tylenol is also safe. I try to avoid narcotics though. One thing that may help is transcutaneous electric nerve stimulators. These are devices that emit a light electrical discharge to the nerves that enter the spinal cord. The brain prioritizes these electrical signals over pain signals. I would recommend these devices over chronic medication use.
Food to eat
I have been sticking to the idea of while on Ferriprox, M-F, no tomato's, vit c, iron, or zinc . Also, if I have beef, I make sure I don't take it for 3 hours afterward. Someone on the facebook page mentioned we shouldn't eat eggs either. Eggs and Milk seem to really help my indigestion. On Ferriprox, should we not be eating eggs within 2 hours of the meds? My wife also has been using Termeric in her smoothies for her foot pain and it has helped. Termeric aids in circulation. Is this a problem for me to take while on Ferriprox or is it ok?
I’m not aware of any specific problems consuming eggs or turmeric while using Ferriprox. Eggs do contain a lot of iron though.
Deferiprone or Ferriprox
I have been advised that Deferiprone or Ferriprox for the treatment of early stage Superficial Siderosis is only available in USA. Are you aware of there any proposed Clinical Trials in Australia, in particular Melbourne ? or a Specialist we can contact regarding these medications?
I don’t know of any clinical trials of deferiprone in Australia but the medication is generally available worldwide including in Hong Kong and other places near you. It’s also available to purchase online. There are also generic versions called deferiprone and Kelfer that are much cheaper.