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.
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. Long-term exposure to free-iron particles escaping the hemosiderin layer 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.
Cortical Superficial Siderosis
Cortical superficial siderosis (cSS) is considered a diagnostic marker. MRI imaging will show hemosiderin on the surface, between the folds, and the uppermost areas of the brain. However, the cortical superficial siderosis patient will not develop hemosiderin on the surfaces of the cerebellum, brain stem, or spine.