Latest questions & answers

clenching my teeth

I'm not sure if this could be related to SS, so I thought I would mention it. I noticed recently I'm clenching my teeth a lot,

I had an operation last year which the surgeon said it was worth doing as the wire I had join C1 + C2 had wore through my dura, so he removed the wire and then took a cutting

 of my dura from my skull and put it on on the leak and then used a patch on my skull, and at the start I felt great and feeling very hopeful, but recently I have noticed my balance is getting very bad and I'm clinching my teeth a lot and I'm not sure if it's

 me concentrating on balancing and walking or is it possibly directly linked to SS. Aneurysm was coiled and a shunt fitted later due to hydrocephalus. I was also diagnosed with terson syndrome which was repaired, prosopagnosia and simultagnosia, alexia without

 agraphia.

I have had travel sickness whilst walking, bathing, turning over in bed etc etc since beginning however over time I feel it has got worse. Neurosurgeon and gp haven’t been

 able to really advise why I should still be experiencing this and it can really affect my day to day life. The nausea is much worse if it’s bright outside, busy traffic noise and people. This also affects how fast I can walk and sometimes affects my balance

 similar to being drunk. I wear sunglasses pretty much all the time with exception of when at home and ear plugs as required.

Even on a good day and I feel I am walking quite well I still have this constant internal uneasiness and nausea. This can feel like being spun, just once, then stopped abruptly

 repeatedly.

Is this a symptom of SS or related to my SAH or CVI? Any thoughts or advice you can offer would be greatly appreciated.

>>> Teeth clenching is not an issue I have seen in superficial siderosis before. I suspect this might be unrelated to your superficial siderosis. It’s also hard for me to say what your symptoms of internal

 uneasiness and nausea are due to. It might be a combination of long standing factors including superficial siderosis or it might be someone related to your previous surgeries or perhaps a new issues. Certainly superficial siderosis can be the sole cause of

 these symptoms but given your complicated history, I would check with neurologist to make sure there isn’t something else going on.

SS diagnosis and any value to treatment with Deferoprone

Would you support treatment with Deferaprone?

I have bee trying to get a teleb-appt with you for several weeks. Sadly, I found out on Tuesday, October 12, the Mass Gen is no longer permitted to do them.

I have a march 2020 SS diagnosis. It began in 2013 about a week after my 7th major spinal surgery. My first bleed in 13 eas followed by hearing loss in 14, balance issues

 in 15, memory problems, brain fog and neuropathy in both feet around the same time. Things have gotten much worse during the pandemic.

My recent tests say it is static. My question is this. Would you support treatment with Deferaprone?

Dr. Ku:ar at Mayo says I have the worst spine he ha# ever seen.

Thank you four help

>>> The decision to use deferiprone to treat superficial siderosis depends on many factors including accessibility/cost, side effect profile (mainly fatigue), other co-morbidities. It also depends on your

 age because in many cases of heavy siderosis, it can takes many years for the deferiprone to remove the iron deposits. In my experience using this chelator, it does work to remove iron deposits from the surface of the brain, albeit slowly. However, sometimes

 the damage that was done by the iron toxicity is not easily repaired. Often, I set an expectation that deferiprone will be used to slow progression rather than to improve neurological function.

Steroid therapy

Hello, and thank you so much for your time...

My question is based on my past experiences with what I now consider to be flare-ups of SS. I've sought medical attention for years for head aches, lack of balance, numbness

 in hands, feet, weird behaviour, etc. I always ask for Decadron, because, in past, it has always seemed to clear things up quickly. Some docs will prescribe, some won't, but it's the one thing I always ask for nonetheless. Which leads me to ask...what is the

 role of inflammation in the SS process, and would steroid therapy be a way to help SS victims through their more difficult times?

>>> There is some inflammation after bleeds. Bleeding into the spinal fluid can cause an arachnoiditis which is painful (headache and back ache) and associated with fever and stiffness. Steroids can help for

 that. However, we don’t know if steroids have a long lasting benefit to reduce the iron deposits and reduce superficial siderosis.

Inosine

Inosine is available OTC. What would you advise people like me who are tempted to order and try it out on their own?

>>> Inosine hexaphosphate (IP6) has two properties that are appealing for superficial siderosis patients. First, the inosine compound is a neuroprotective agent that was shown to be helpful in other neurodegenerative

 conditions like Parkinsons disease. Second, the phosphates provide a limited measure of iron chelation; it’s considered a weak iron chelator. Otherwise, IP6 appears to be harmless at the recommended dose (see instructions on the bottle).

Blood brain barrier question

https://www.nature.com/articles/s41467-019-12426-9

There are current studies being done on using ultrasound waves to get meds past the BBB to treat other diseases such as ALS, MS, etc. Is this new technology a future possibility

 for SS sufferers? Could Ferriprox possibly ever be injected straight into the brain, to more effectively target the bad iron in the brain as opposed to the good iron in the blood and body?

>>> Ferriprox (deferiprone) has no trouble getting into the brain. That’s why we use it for superficial siderosis. Within 7 minutes of absorption in the gut, deferiprone is detectable in the spinal fluid.

 So this technology will not help in this regard. 

However, there are other iron chelators that cannot get into the brain. We don’t currently use them for superficial siderosis for that reason. But if there were a way to get them in – perhaps with devices

 like these – it might be useful.

Prochlorperazine

My GP has prescribed Prochlorperazine tablets for my spinning head.

It has had no effect so far, in fact, I continue to get worse!

Can they be effective in controlling the Ataxia or can you suggest an alternative?

>>> We don’t have any treatments for ataxia. I wish we did. The only thing I found that helps is vestibular rehab – physical therapy that focuses on balance.  If anyone in the Facebook community has suggestions

 for medications, supplements or techniques that would help, please do share.

Life expectancy

Does superficial siderosis shorten life expectancy?

>>> No, not that we know of. 

Superficial hemersiderosis

I have been diagnosed with SS and am waiting for a lumber punch to be done I have had an MRI scan. I am due to fly to Jersey in the channel islands from England is this OK

 to do . 1 hour 5mins in the air .

>>> There is no problem generally in flying with superficial siderosis. You may have additional factors that influence your decision but you should check with your doctor about them.

Ferriprox after bleed is sealed

Do you still see a case for use of Ferriprox/deferiprone if: 1. A Siderosis bleed has been sealed surgically; and 2. The patient does not have ongoing ferritin in their spinal

 fluid (if that was confirmed by lumber puncture)? In the above 1and 2 scenario do you believe that Ferriprox/deferiprone would still be a viable treatment to attempt to chelate the historical existing deposits on the brain and spine or, based on research,

 do you see the value in its use only for patients who have ongoing bleeds as evidenced post surgery by ferritin in spinal fluid? Thanks

>>> Ferriprox (deferiprone) can do two things: 1. Remove acute blood products. It does this easily. For those with ongoing bleeding, this medication can help to prevent additional iron deposits from accumulating.

 2. Remove old blood products. This is harder to do once the iron is bound to the surface of the brain. But eventually, after several years, deferiprone will start to remove those old iron blood products.

Nausea

I was diagnosed with SS about 8 months ago. I had an MRI and an MRA and both showed that I have SS. I've been experiencing some of the symptoms for about 3 years....dizzy,

 balance issues, nausea. I have had several falls in the past 3 years and I think most of them have been caused by my balance problem. My neurologist prescribed a medication to take for the nausea which didn't help. I've been taking Nortriptyline for about

 2 yrs. and that seems to help the nausea. I'm wondering if I need to try to make an appt. with Dr. Levy or some other neurologist in the NY/NJ area. My GP suggested that I go to Columbia Presbyterian in NYC. Please advise. Thank you.

>>> It would be a good idea to get specific expertise in superficial siderosis from me (Michael Levy) or Dr. Kumar (Mayo Clinic) at least once every few years. You can use your local physicians as part of the team to handle day to day issues.