What is Superficial Siderosis?

This is an attempt by a survivor to bring information in plain understandable english on the ‘rare’ condition called Superficial Siderosis of the Central Nervous System to fellow world-wide survivors, their familes and supporters.

It has come about, because all too many diagnosed survivors, even some long term, appear to be unaware of all of the huge number of side effects the condition may bring, and the author considers someone aware of the possibilities is much better prepared emotionally, when complications do actually strike.
Because of the scarcity of the condition there is little documentation for survivors to study and enlighten themselves on the intricacies of it, so this report is aimed to assist.
Accute, or new, survivors are quite within their rights to be completely ignorant of the condition as most General Practitioners have never heard of it, and even some Neurologists are not well informed on the disease or how to address it.
What actually is Superficial Siderosis ?
Superficial Siderosis of the Central Nervous System is when iron salt deposits accumulate on the brain surface, spinal cord and or cranial nerves. The hard iron salt core is created from chronic bleeding into the subarachnoid space or brain surface, in underneath the three protective membranes. In most cases the source of the bleeding is never located due to a considerable time delay before diagnosis. It is also reported that more than one bleed is required to cause SS.
In the past , the disease was described as rare but this in reality is no longer the case. It has been said that one person per million suffers from it and that does make it rare enough – this being the case there are actually hundreds of thousands of survivors throughout the entire world, however many in not-so-advanced countries will obviously never be clinically diagnosed. Occasionally, the condition may be diagnosed in a reasonably short time, but in general it doesn’t expose itself for many years, often a decade or more since being triggered. In 22 patients under one review, this period lasted from 4 months until 30 years, the average time until diagnosis was 15 years. Today with the advent of Magnetic Resonance Imaging ( MRI ), it can potentially be located very quickly even with little obvious symptoms – often the sudden deterioration of hearing in one or both ears is sufficient to cause concern and investigation.
The cerebellum, about the size of a tennis ball, is located between the ears just inside the skull, and is the organ most effected by Superficial Siderosis. This vital part of the brain is the co-ordination centre rather than the thinking section. People with problems in the cerebellum are mostly clumsy and unsteady in their movements. The hard iron salt casing limits, or may even completely stop, the flow of instruction both to and from the cerebrum, the main section of the brain.
Both the brain and spinal cord are bathed by the same fluid called CSF, such a bleed anywhere in the central nervous system can carry blood to all parts of the brain and spinal cord. It is not clear to the experts why the cerebellum is preferentially affected by the iron overload. It is thought that maybe there are specialised cells in the cerebellum that absorb the iron which is certainly found in highest concentration in the cerebellum compared to the rest of the brain and spinal cord. Blood deposits, and thus iron salt build up, may therefore also present itself around the lower spine which would be expected to be displayed in the same black lines, as seen on the perimeter of the cerebellum during MRI testing.
There are a huge number of functions a person with Superficial Siderosis may have difficulty with, including hearing, ability to smell and taste, balance, co-ordination and weakness of limbs, difficulty with bladder and bowel functions, eye movements, early dementia, bilateral sciatica, pain in lower back and joints. Because of the perplexity of the cerebellum’s workings, other less common side effects connected to Superficial Siderosis have been TIA or mini strokes, loss of ability to hold one’s head up, compression of gullet muscles.
To catch Superficial Siderosis one would most probably have experienced an illness such as meningitis or viral encephalitis, a blow to the head, or had an operation to the Central Nervous System. Many survivors have trouble identifying their trigger – an operation or illness is usually memorable, but a blow to the head may have, at the time, appeared quite minor and insignificant. It is said by some survivors, that their sole accident in earlier life, was so minor that it was never even reported to next-of-kin.
Earlier findings, notably those of Fearnley and others in Brain magazine in 1995, state that age of onset was from 14 to 77 years, age of death from 29 to 78 years and the duration until death excluding premature death from 1 to 38 years. We now know of survivors much younger than 14 y.a. and older than 77 are recorded. We also know that some have gone longer than 38 years since diagnosis. Males are more likely to be diagnosed with SS at a rate of 3:1. The author knows of few females.
The disease has been written up many times as being ‘fatal’ which is possibly an unfair description, especially to a medically uneducated person, as it is definitely not life threatening in the same way as major heart attacks nor inoperable cancer. It is fair to say, however, that SS does shorten lifes, but worrying about the condition is probably worse than the condition itself. Assistance with control of the many sideline effects from specialists is essential.
Another review was carried out in 2006 by Michael Levy and others, incorporating patients from several countries including North America, France, Turkey, Italy, Spain, Finland, Germany and Japan. Altogether 270 cases were looked at, from birth until into their 90’s, most common was in the 50’s and 60’s age bracket. Levy and co. came up with slightly different precentages – hearing loss 81%, ataxia ( loss of coordination and balance) 81%, Myelopathy ( any disease of the spinal cord ) 53%, Urinary problems 14%, headaches 14%, anosmia ( abilty to smell ) 14%, diplopia ( double vision ) 4%, bowel problems 3%, ageusia ( loss of ability to taste ) 2% and cranial nerve palsies 2%. This review favoured males over females 2:1. It reported that treatment with iron chelators had produced only one case report of some success, as did other forms such as lumbar CFS shunting and steroids. They note “ unfortunately, there is no therapy to reverse the damaging effects of hemosiderin deposition in the brain. The natural course of Superficial Siderosis is a progressive neurological decline. “
Currently, some implants directly onto the brainstem are being pioneered and if successful will be most valuable as they by-pass damaged auditory nerves. It may be some time before the success of this can be accurately determined.

History
SS was first discovered in a case presented to the Chicago Neurological Society in 1908. In Fearnley and others’ extensive 1995 review they found reference to 87 cases world-wide of which 63 had sufficient information to be reviewed.
Many of these cases were diagnosed during autopsy as the first diagnosis of living sufferers was not made until 1965. Not being a notifiable disease, most cases are never heard about.
Hearing
Hearing is the most affected symptom of Superficial Siderosis. It is locatable in up to 95% of all cases. Nearly all cases progress bilaterally, or both sides, over a period of 1 to 15 years. Once the hearing deficit has displayed itself, it is usually quite speedy, leaving the sufferer totally deaf, or with only a very small band of low tone of little to no value.
The single biggest complaint of survivors is the way they are treated by Audiologists and often prescribed with more than one set of expensive multi functional hearing aids in a short time, when in reality a good strong basic pair would have been suffice and lasted much longer. It has been reported by survivors that they paid for up to three sets of aids in just 18 months, only to find that all became superfluous within 2 plus years. Audiologists therefore need to fully understand the disease before going down the track and thus prescribe appliances accordingly, not just be eager to obtain their patient’s money for what is usually short term usage of the prescription item/s. A survivor attending an Audiologist for the first time should initially fully discuss the condition and ask for quotes on costings and strength of the appliance/s.
In association with the lack of hearing comes tinnitus, the ringing noises, or other similar noises in the ear or ears. Tinnitus is liable to alter its sound in different situations – anything from the sound of rushing water or air, insects calling, musical type tones, to pieces of metal rubbing themselves against one another. Sounds generally vary from left to right also.
The author believes from personal experience that not only does the hearing decline but the brain looses its ability to perform. He regularly finds that as the day progresses the brain breaks down from continual working. Particularly noticable is a failure to decipher other than his own nationality’s speech.
Cochlear Implanting
It is accepted that the cochlear and the cerebella cortex are particularly vulnerable when Superficial Siderosis strikes. Because of this, many Otolaryngologists will not entertain the question of an SS survivor being fitted with Cochlear Implants because they firmly believe the two do not go together. There is, however, definite proof of some SS survivors having perfectly successful implants and in some cases lasting over several years. This gives the distinct impression that SS is not a total canvas, but is very much spasmodic and non-conformist in where it can attack.
Balance

Ataxia, or the loss of co-ordination and balance is the second most characteristic symptom with Superficial Siderosis, 88% of sufferers are affected. In Fearnley’s review they found that of these cases, 56% involved were both limbs and gait, while 44% were predominantly gait.
Noticeable were stiffness, jerkiness and a weakness with difficulty in moving and spinal myoclonus or muscle spasms with alternating muscular contractions and relaxations. Some survivors report shakiness of legs, arms but more especially hands.
The general advice at the start is to use a walking pole, stick or cane as Americans call them, advancing to one in each hand when required. From this, the next step in usually two crutches or else a wheeled walking frame and then the normal next advancement is to a motorised wheelchair.
It is important that some form of brace or reliant be used. There are huge variants when balance and co-ordination is a problem. Some people have problems negotiating rises, others downhill. A common problem are steps or stairs. Your author very early in the piece fell several times going down steps, yet could freely run at speed up them. Now, a few years into SS, any direction on stairs are a problem to him.
As a result of altering one’s gait to counteract for poor balance, this alone has the possibility to create problems indirectly attributable to SS. A perfect example if that many survivors take up walking with their feet wider apart to improve stability, this having the potential to effect muscles and skeleton.
Bed Bound
27% of survivors are permanently confined to bed as a result of ataxia, the loss of co-ordination and balance or because of myelopathic ( any disease of the spinal cord) syndrome, or both.
Dementia
Early dementia is a problem coming into the lesser degree category. It is rated at 24% in survivors. This problem in one where medication can be prescribed to help lessen the chance of catching it. Folic Acid and assorted Vitamin B supplements are the usual medication.
Bladder & Bowels
Also rated at 24% possibility, is the loss of bladder functions. Normal treatment for this is either self catheterization or a permanent catheter being fitted.
Hand in hand with this, although nowhere are references giving percentages, is the loss of bowel function where incontinence underwear is usually required.
Anosmia
Anosmia, or the lack of ability to smell, is listed conservatively at 17%. It is considered that it possibly is often not checked out by specialists and should probably rate higher. One, or both nostrils, may be effected.
Sensory Signs
The failure of the senses is rated at 13%.
Anisocoria
Anisocoria, is when one pupil of the eye is larger than the other and is rated at least 10%, and once again it often isn’t checked by specialists.
Other effects
Following these are many more sideline effects which rate without percentages. They include bilateral sciatica pain down the backs of both legs from the sciatica nerve in the lower spine. This is possibly connected in some way to lower back pain, another complaint. Also tensioned neck and extra – ocular motor palsies ( eye movements and cross eyeness ). Other complaints have showed up including a TIA or mini stroke in an otherwise perfectly healthy man and quite a variety of eye problems from a complete lack of vision in one eye to speedy deterioration of short, and or, long distance vision.
Headaches are another definite further problem. These may be temple located or alternatively may be at the rear and base of the skull in the proximity of the cerebellum. Coupled with this, many sufferers develop migraines.
A common side effect of SS is the complete change of sleeping patterns which may show up as irregular or interrupted sleep, or may be the opposite – long periods of sound sleep. Some survivors report being able to always soundly sleep up to 13 or 14 hours every night. Many say they need midday naps but this generally effects night time sleeping habits in some way and therefore mid day breaks are best if only a rest is taken, without sleeping.
The author finds that dusk and nightime are the worst periods for balance. It is particularly important to make maximum use of lights to navigate during hours of darkness, especially in narrow passageways and doorways. Outside, over irregular terrain, without a light is a definite no-no.
Treatment
Surgery has only rarely been described as being used in SS patients because of the usual time delay before diagnosis. If sufficiently early in the diagnosis, then ablating or plugging the cause of bleeding may help restrict further complications. The bleeds may even be attributed to complications in the spine such as tumours or other similar problems.
Some other forms of medication such as iron chelators have been tried but have not definitely proven to be beneficial.
It is considered worthwhile to treat SS survivors against the possibility of getting dementia. Folic Acid in tablet form plus forms of Vitamin B, either in daily tablet form or monthly injected, are those most commonly administered.
To lessen the chance of a return of TIA’s or strokes various forms of medication are prescribed such as blood thinners and the traditional half an Aspirin a day.
For those experiencing permanent headaches sometimes anti-depressants in mild doses are found to provide long term relief, along with common medication such as Panadol.

19 responses to “What is Superficial Siderosis?

  1. Hi I’m Michael from Dublin, Ireland. I’ve been diagnosed for 12 years at this stage. With hinsight it has effected me progressively formuch longer than that

    The article is a very good summary. There is a distinct lack of research on the subject and it is a brilliant of you to foster an online association

    I make 2 comments on your layman’s descrition of SS

    In my case I get chronically tired. We have put it down mainly to the ataxia and need to continuously lip read. Many of my friends would notice this exhaustion far quicker than my hearing disability or clumsiness

    I think it is important not to read the worst into the figures. So for example there is a 14% chance you will lose your ability to smell. By definition that means there is an 86% chance you will not. I suppose what I’m sayig is that we need to manage our emotions a little

    Michael 20 June 2007

  2. This site will be useful for both SS survivors and health professionals. Congratulations on a great job.
    Liz (GP)

  3. It is absolutely wonderful to find a place to meet others dealing with SS. For 3 years I went from specialist to specialist only to leave with them scratcing their heads, or telling me I’m not as young as I used to be (I was 50). Those 3 years cost me a lot. Finally, the Mayo Clinic diagnosed it and I am in the process of looking for a bleeding source. I’ve had hearing loss and progressively worse ataxia. Now numbness, double vision, cognitive problems, phantom sounds and smells, and darned if my feet will go where I want them to. But I thought I was alone. So my heart goes out to everyone of you out there, and we’ll make it thru this.

  4. I would like more information on the physician who is maintaining this site. I would also like to know what hospitals/doctors are now more actively involved in ss? Finally, what can I do to help spread the word about ss to the medical community?

  5. paula newton

    hi, my uncle who is 52 has recentley been diagonosed with ss although he has suffered since he had a brain tumour at the age of 10 . just wondered wat everyone can tell me about this condition as we are still trying to understand it . many thanks my uncle is almost fully deaf, has lost his balance and now walks with a frame

  6. Missy Patterson

    Just found out that my Dad has SS. I would love to know where to get more info. I haven’t been able to find much info out there. Thanks

  7. I have been living with the diagnosis of primary progressive ms for almost 2 years. I was just seen at NIH in the US and they have found the iron ring in my MRI and red cells in my CSF. I am one giant meeting away from the diagnosis of SS. I do have noises in my ears, but no real hearing loss. I have numbness in my legs and spasms all over my body…even in my mouth and around my head. The doctors say I have a “mild” case. Is there such a thing? They told me the iron will go away by itself. I was also told the condition is rare, so they don’t know much. Should I be scared?

    • Answer from Dave:

      —–

      O,

      I’m sorry to be so slow in replying but things have been very trying of
      late. I’ve been almost living at doctors rooms and hospitals. I firmly
      believe it is a collection of neurological powered medical ailments, in
      other words a major let down in performance by the brain.
      Collectively these ailments have really traumatized the brain into not
      performing. My Cochlear Implant is failing to deliver for one.

      One thing not to do is to get wound up and scared. Your coating of
      iron will NOT go away by itself, unless you make medical history.
      Not all of us loose our hearing. A very lucky 5% don’t, but obviously
      they are few and far between. I have only met one SS person with
      perfect hearing. I assume you have tinnitus which is quite common in
      SS.

      Funny you should mention numbness in the legs. Its something I’ve been
      getting myself this past few weeks. Numbness from the knees down.
      Again, not uncommon to SS sufferers. I also experience numbness in
      parts of the head.

      Yes, the terms ‘mild’ and ‘severe’ have started appearing this last
      couple of years. Those in our group termed ‘mild’ are not hugely
      affected by SS – they still work, they can run, they go tramping, they
      play golf, they ride a bike.

      I suggest you contact me on for a more
      personal discussion.

      Dave Hill

  8. My mother died of SS Feb 2009. I am happy to see this cite. It is a lonely thing to lose someone and not know anyone else who has such an experience.

    Is there a genetic component to this? Should I be fearful?

    Tessathulien@yahoo.com

  9. John Ridnell

    I was diagnosed 6 months ago by a large group of neurologists as having this disorder. It follows bilateral sub dural haematoma’s in 1990, the symptoms are typical and have been experienced for about 6yrs.
    I would of course like to arrest the development of the condition, with any treatment available and possibly reverse the symptoms should stem cell, or some other treatment be effective in the future.
    Do you know of any promising treatments? and where these may be found, I am currently in Australia but am able to relocate if neccessary.

  10. Hi all

    its late …But ty For sharing…hope to share in Much detail tomorrow…Nice to meet you Guys…

  11. Tessa..I was Going to bed..but to ease your worries.. your QUITE safe, SS is due to headTrauma, I dont believe its genetic,,,my case History is Much Longer then Folk On this forum have shared… plus more…

    Sleep well

  12. My neurologist needs to read the up-to-date info .and ideas from “survivors”. He diagnosed my S.S. two years ago from my second NMR scan and then found it had passed unrecognised on an earlier scan. I think I am his first and possibly only patient with S.S. So far he has not recommended any treatment except keeping as fit as possible to maximise the “mild” period and sending me for cognitive assessment at Southampton Medical School to monitor any decline.
    After reading your subscriber-comments I am now going to take supplementary B vitamins daily, but would like more
    information on dose and research as to benefits. Useful refs please?
    The chelation of heavy metals (using chemicals to extract unwanted metal so that is excreted from a tissues) is very specialised as many metals are essential, including iron.
    I suspect that there are many more ss sufferers than is realised and greater numbers identified will mean greater medical and pharmaceutical interest.
    I wonder if a retrospective search of a large sample NMR scans by a neurologically-trained technician would produce more unexpected diagnoses ? Would the Alzheimer Researchers take us on as a functionally linked condition?
    jj (ataxis and deafness + symptoms symptoms mentioned by others above which I had not recognised as cerebrospinal previously)

  13. My father-in-law has ever side-effect listed above, even the uncommon ones. I think he was diagnosed about 20 years ago, it was caused by a bleed on the brain from removing a tumour as a child.

  14. I was diagnosed yesterday with superficial siderosis. I was relieved when I was told that there is no active bleeding at this time, or I should say they cannot find the source of the blood products present, and was told there is no anuerysm. I think my most annoying symptom is very loud shrill ringing in my ears. I have a constant low grade headache and dizzy most of the time. I have had an MRI, MRA-MRV in the past two weeks. I have a meningioma that the neurologist is monitoring with an MRI every other year. My ear ringing started the day after rotator cuff surgery. It is constant, but much worse when it is quiet. I know that I am blessed. It could have been a much worse diagnosis. I’m thanking God for giving me the strength to cope with the symptoms and what lies ahead. I trust in Him! :)

  15. Hi All

    Just throwing this out there-

    Im SS survivor – I have long history of other Chronic illnessess too- One would care to ask– along with the Norm, as with Constant headaches to the temple region and the base of the Neck-, loss of hearing, sensitivity to odours- eye wobbles , loss of hearing etc– My biggest issue are my LEGS combined with complete Fatigue— they very weak, the aches ,tightness, the trembles etc[ although they compounded by Polycythemia] – is there anyone else who suffers this ?
    — This Exhaustion really is depressing–
    Rgeards Graham[ Gloucester, England]

  16. Hi, Thanks so much for the infor. My daughter had 2 brain tumours one cerebral, and the other temporal, she left with SS for 9 years now. Doctors never gave us any info at all about the side effects from this disease, She has bad dementia, and wheelchair bound. so much appreciate the read.
    June

    • Hi June,
      Be sure and stay in touch by reading the monthly newsletter written by Dave Hill. They are very informative. How old is your daughter? When did she begin having signs of dimentia? I am 56, and was diagnosed in April 2011.
      Although i have many issues going on with this dreaded disease, my biggest fear is possible early dimentia.
      I hope you and your family have a blessed Christmas season.
      Peace and all good,
      Annie :)