Your physician will review your MRI to determine which type of superficial siderosis is present. There are three variations, two of which present a unique set of symptoms. One type may be identified as staining only and will not affect the patient.
What is Superficial Siderosis of the Central Nervous System
Superficial Siderosis (Infratentorial superficial siderosis (iSS)) is a disorder that develops over time when long-term bleeding into your central nervous system (CNS) overwhelms the body’s natural defenses. As a result, when blood cells break down, neuro-toxic iron particles are left traveling throughout your spinal fluid. In the act of self-protection, your system reacts by releasing proteins to trap these iron particles by encasing them.
Hemosiderin is the result of this protein and iron combination. Over time, gravity pulls sticky hemosiderin into a coating layer over surfaces with direct exposure to circulating spinal fluid. However, long-term exposure to free-iron particles, oxidative stress, and inflammation is toxic to the underlying neural tissue.
In the early stage, this disorder’s progressive nature is very slow-moving. However, it’s essential to know, repairing the bleed source remains your first line of defense. Sadly, 50% of diagnosed superficial siderosis patients are not able to have their bleed source located. Another critical point to remember is the intensity of hemosiderin shown on your MRI may not always be consistent with your current symptoms.
While medical opinions remain divided, anecdotal and first-hand evidence over the last six years has demonstrated as long as hemosiderin remains in place, the symptom progression will continue.
Patients diagnosed with Infratentorial superficial siderosis may also have hemosiderin deposition in the supratentorial regions of the brain due to the circulation of cerebrospinal fluid throughout the cisterns of the brain.
Cortical Superficial Siderosis
Cortical superficial siderosis (cSS) is often found with cerebral amyloid angiopathy and is now a credible diagnostic marker. cSS is associated with transient focal neurological episodes and increased intracerebral hemorrhage risk. MRI imaging will show hemosiderin only in the supratentorial area of the brain, on the surface, between the folds, and the uppermost regions of the brain. The spinal cord or the infratentorial region (cerebellum, pons, and brainstem) will not show deposits.
Microbleeds are thought to be the source of hemosiderin build-up in cases of cortical superficial siderosis (cSS). Patients diagnosed with only cortical superficial siderosis will not experience the balance, mobility, or myelopathy symptoms as the patient with infratentorial superficial siderosis (iSS).