What Is Superficial Siderosis?

Superficial siderosis is a slowly progressive chronic neurodegenerative condition most often caused by past trauma or surgery that introduced bleeding into your central nervous system. The body is designed to rid itself naturally of blood infiltration into the central nervous system (CNS), but in rare cases, this long-term bleeding overwhelms your system. The result is an accumulation of hemosiderin on the pial surfaces of the brain and spinal cord.

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 system through your liver.

Glial cells now manufacture ferritin to capture and store the free-iron. For some unknown reason, the ferritin becomes overwhelmed. Unable to contain all of the free-iron the excess is left floating in spinal fluid throughout the subarachnoid space surrounding your brain and spinal cord. Eventually, the iron molecules attach themselves by forming a layer of hemosiderin on

 

the subpial layer that covers the nooks and crannies of your brain and spinal cord. 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 spinal fluid also become covered in hemosiderin.

This long-term exposure to free-iron molecules and hemosiderin is toxic. The result is neural damage, cerebellar atrophy, and neurodegeneration. Hearing loss, ataxia, and myelopathy related clinical symptoms mysteriously progress over time mimicking more widely recognized diseases. Superficial Siderosis is so rarely seen a correct diagnosis is often not found for years. Improved MRI technology and increased awareness have allowed radiologists to be often the first to report an accurate diagnosis.

There are limited  treatment choices for Superficial Siderosis

You can choose to live with the natural progression. Your physician may be able to locate an active bleed and perform a surgical repair to stop fresh blood infiltration. You can opt to try chelation drug therapy to remove the iron residue.

The only option with a guaranteed result is living with the natural progression. Depending on the severity and area your iron deposits are located the symptoms will continue to progress until your quality of life is affected. If you, or someone

 

you know, has been diagnosed with superficial siderosis the search for answers is beyond frustrating. Learning to cope with this rare disease is a challenge. Finding a support network is essential.

Unexplained symptoms led to ten years of appointments, scans, and testing in a search for answers before a radiologist recognized the distinctive pattern of Superficial Siderosis. We invite you to follow our journey through this diagnosis as we share how we deal with the clinical symptoms, treatments, therapy options, research and general information we’ve collected along the way.

Pathophysiology of Hemosiderin2
Pathophysiology of Hemosiderin

superficial siderosis research

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