Steps For Help
IMPRESSION: Diffusely abnormal decreased signal of the meningeal surfaces more pronounced about the brainstem, cerebellum, and basilar cisterns. There appears to be mild associated cerebellar atrophy. The MR findings and the clinical report of dizziness and hearing loss are consistent with superficial siderosis. The abnormal decreased signal is observed primarily on the T2-weighted series correlating with the pial and ependymal surfaces most pronounced about the brainstem, basilar cisterns, and cerebellum. Findings correlate with hemosiderin deposition in a pattern of superficial siderosis. Etiology for superficial siderosis is presumed to include episodes of subarachnoid hemorrhage, although not always clinically evident. Overview Progressive hearing loss will affect 95% of Superfi... More and cerebellar findings, by a report, often present symptoms and appear to correlate in this case.
Be Prepared To Travel
In our case, there was a 12-month waitlist before a local neurologist would accept a new patient. Six months later, we finally found Gary, a neurologist willing to accept a superficial siderosis patient. Although her office was a two-hour drive from our home, Dr. Allison Hennigan was a kind, and most importantly, interested physician. Finding an interested neurologist is a critical first step. Too often patients are forced to accept care from a doctor who believes there is nothing to be done.
Our Provider Directory is a searchable list of neurologists and neurosurgeons from around the world. Each one has had a superficial siderosis patient under their care. We encourage our SS community to send us their physicians’ information for inclusion in the directory for a very important reason. If a doctor has multiple patients with the same disorder it provides a strong incentive to learn everything they can about this disorder. Our goal is to expand this into a knowledgeable provider network. We can also help your physician connect with a superficial siderosis specialist for advice.
The bottom line is it is challenging to find a neurologist who is both willing to accept a superficial siderosis patient and prepared to research a care plan.
Do You Have An Active Bleed?
Your first step after diagnosis should be to find out if you have an ongoing bleed. If your neurologist suspects your bleed is still active they may perform a lumbar puncture to help determine if there is still blood in your spinal fluid. Be aware it may not work if your bleed is intermittent. Most neurologists will refer you to a neurosurgeon for this step. A CT Myelogram is now the preferred method to locate an active bleed from a dural defect. If a bleed source is located, your neurosurgeon will review your options for closure.
If your MRI report states superficial siderosis is present, then you have hemosiderin deposits present. The Mayo Clinic in Minnesota is considered the most experienced facility with superficial siderosis bleed identification and has excellent results with complex bleed repairs. Dr. Kumar is considered the leading neurosurgical superficial siderosis expert in the United States.
Cedars-Sinai Hospital, Los Angeles CA, and Oschner-LSU Shreveport, LA also has excellent neurosurgical departments that have experience with closing dural defects. An active bleed can be very hard to pinpoint, especially if the source is not obvious. While some SS patients choose to travel for a surgical bleed repair it should be possible to find an experienced neurosurgeon in your local area.
Unfortunately, the next step is where professional opinions diverge. Many neurosurgeons still believe if an active bleed is repaired the progression will stop. While it is extremely important to your care plan to have the repair completed if possible, the existing hemosiderin deposits will continue to be problematic. Neurotoxic to nerve function, patients will continue to decline slowly as long as the hemosiderin remains in place.
We have no idea who first decided to try and remove the hemosiderin deposits. Early chelation trials must have failed. The problem? No drug could cross the blood-brain barrier. Dr. Michael Levy was a member of the Johns Hopkins research team conducting a 90-day pilot study testing the safety and efficacy of Deferiprone (Ferriprox®). This lipid-soluble iron chelator was able to cross the blood-brain barrier on ten patients. The results of this early study were promising enough to expand the research with additional candidates. This comprehensive study was published on December 28, 2017. You may read and download a copy of this study on our website.
“To all SS patients on Ferriprox or thinking about Ferriprox, I want to take this opportunity to clear a misconception about the expectation of this drug. Ferriprox is an iron chelator, a small molecule that binds iron. The unique property of Ferriprox that makes it suitable for SS is its ability to dissolve into the spinal fluid and chelate iron on the surface of the brain and spinal cord. While Ferriprox is chelating the iron in an SS patient, it should not make the patient feel better in any way (except psychologically maybe?). Remember, symptoms from SS are due to damage to the outer layer of the brain due to iron toxicity from the adjacent siderosis layer. As long as the siderosis is present, the damage it causes continues even while the iron is being chelated. SS patients should expect to continue to progress in their SS disease until the iron is gone and healing can start. In my experience, it takes two years in most patients to begin to see reductions in the siderosis extent by MRI. Those brain areas that have been cleared can begin to heal while neighboring siderotic brain tissue is being damaged. Since I’ve started using Ferriprox in 2010 (the majority since 2012 when the drug was FDA approved in the US), only a few patients have managed to clear all or most of their iron, and in those patients, we saw the most clinical improvement.“Dr. Michael Levy, May 19, 2015 post to the Facebook support group
Chelation therapy is still considered a very new treatment option that has strict requirements of its own. Our neurosurgeon suggested we needed a neurologist to oversee treatment. Our PCP was willing to begin chelation once we discovered finding a neurologist was delayed but decided to refer us to a hematologist.
In the end, we found some very engaged physicians. That’s what you need to look for in a doctor. Our hematologist and neurologist both emailed Dr. Michael Levy for advice. Dr. Levy is considered the foremost superficial siderosis neurologist and researcher in the United States. He freely gives his time to answer any physician who contacts him. He also accepts long-distance patients for care at the Superficial Siderosis Clinic and Research Laboratory, Massachusetts General Hospital. Patients are required to travel to Massachusetts at least once for an in-person appointment.
If your doctor tells you there are no options, find a new one. If your doctor says there is nothing to worry about, find a new one. Never settle.