Patient, family & Dr. Levy with giant check
Brain imaging before and after deferiprone therapy. Click to enlarge image.

The human body has a limited capacity to rid itself of blood products in the subarachnoid space, but in ultra-rare cases, chronic or intermittent bleeding over the years leads to overwhelming amounts of toxic iron molecules there.

The internal bleeding, or hemorrhaging, that results in Superficial Siderosis can be caused by tumors, vascular abnormalities, injuries and defects in the membranes surrounding the brain. Trauma is the most common cause, which often occurs decades before symptoms begin.

Long-term exposure to the iron deposits is toxic to the brain tissue underneath resulting in:

  • Sensorineural hearing loss: Hearing loss caused by damage to the inner ear or the nerve from the ear to the brain. This will affect some 95% of SS patients at some point in their lives.
  • Ataxia: Impaired balance and coordination
  • Myelopathy: Severe compression of the spinal cord

The neural damage from Superficial Siderosis may be permanent, and disability will continue to progress even if an active bleed source is repaired.

Although easily identified by magnetic resonance imaging (MRI), Superficial Siderosis is often confused with other progressive neurological conditions such as Multiple Sclerosis, Parkinson’s, or Multiple Systems Atrophy. In 2014, there were only 100 known cases of Superficial Siderosis in the United States. With more precise MRI testing and the standardized diagnostic protocol, that number will rise both here and abroad.

There is currently no cure for Superficial Siderosis. The only medications currently available to treat SS are oral chelation drugs, which can cross the blood-brain barrier. The best known of these medications is deferiprone (Ferriprox). Oral chelation therapy carries risks and may not be advisable for all patients. We have been testing the efficacy of deferiprone, as well as developing new MRI techniques that can assess changes in iron content in the brain.

For further information, please contact our clinic or the Superficial Siderosis Research Alliance.