Superficial Siderosis. Subpial Siderosis. Infratentorial Superficial Siderosis. No matter how your doctor officially labels your diagnosis odds are no one has the slightest idea of the battle you are about to face.
What is Superficial Siderosis?
Toxic free-iron molecules are curbed by a protein called ferritin, which forms into a layer of hemosiderin in various areas of the brain, brainstem, spinal cord, and cranial nerves. Long-term exposure is toxic to the tissue underneath, resulting in hearing loss, imbalance, dizziness, weakness, numbness, and bowel/bladder dysfunction. Although easily identified by magnetic resonance imaging (MRI), superficial siderosis is often confused for other progressive neurological conditions such as multiple sclerosis, Parkinson’s, or multiple system atrophy.
Due to the severe nature of the disease, in 2018 the Social Security Administration added superficial siderosis to the Compassionate Allowance list of conditions that allows for the fast-tracking of disability claims
Explaining the process requires some big and complicated words. Not big as in real size or number of letters in a word but in the sense of consequences. Yes, it progresses slowly for a good percentage of sufferers, but what if those early years are spent searching for an explanation of random symptoms? Imagine living through a decade of endless testing and frustration with no answers.
How does Superficial Siderosis develop?
As individual blood cells travel through your subarachnoid space, they begin to rupture through a process called Hemolysis. The bursting of cell walls creates a heme overload that triggers the Bergman glia and Microglial cells to fight back by producing the enzyme heme oxygenase-1. This enzyme breaks down the heme and results in the release of free iron molecules, carbon dioxide, and biliverdin. Your body converts biliverdin into bilirubin and routes it out of your body through your liver. Glial cells manufacture ferritin, which binds to the free iron.
Eventually, the ferritin bound iron molecules attach themselves by forming a layer of hemosiderin on the subpial layer that covers the nooks and crannies of your brain, brain stem, spinal cord and surrounding tissue. Gravity comes into play. Laying down or sleeping positions the cerebellum to become a prime target. Walking or sitting upright makes your spine at risk the remainder of the day. Cranial nerve sections that run through your cerebrospinal fluid also become covered in hemosiderin. This long-term exposure to free-iron molecules is toxic. This may result in neural damage, cerebellar atrophy, and neurodegeneration.
What symptoms are Superficial Siderosis related?
It’s frustrating trying to find out if a problem you’re having is related to your Superficial Siderosis or it stems from an unrelated health issue. Many are battling multiple conditions or non-related disease. Care must be taken not to fall into the trap of blaming Superficial Siderosis for everything.
Studies always mention the classic triad symptoms: Ataxia, Sensorineural Hearing Loss, and Myelopathy. Still, if you look carefully into the problems most often reported by Superficial siderosis diagnosees, you find the clinical symptom list is a little more complicated than the classic trio.
Our first posting in 2015 was to list 30 problems thought to be Superficial Siderosis related. Over the past three years, this list has evolved into a searchable glossary. A few of the original symptoms, such as tremors and dental pain are temporarily gone, still awaiting documentation.
Is there new information?
A recent explosion of published studies has documented new clinical symptoms found with superficial siderosis. Most track to one of the three parent categories. A few, such as Depression, Craniospinal Hypotension, and Neurological Reserve, aren’t direct clinical symptoms but have an obvious connection. No one, to our knowledge, has ever experienced all of them. Our glossary continues to be a work-in-progress, ever-evolving and expanding as we review new information.