Who is Affected

Who is affected by Superficial Siderosis

Infratentorial superficial siderosis (iSS) 

Superficial siderosis of the central nervous system is an ultra-rare disorder with an estimated incidence of 1 million individuals. While superficial siderosis of the central nervous system affects all races and age groups, males are affected three times more often than females. Very recently, an eight-month-old baby was diagnosed with SS.

You may often trace your development of Infratentorial superficial siderosis to a single triggering event. However, it might have been as long as 30 years past, depending on the nature of your bleed; brain bleeds or spinal surgery dural tears resulting in pseudomeningoceles, head injury, or traumatic accident. Other known causes include root avulsions, epidural cyst removal, intradural cranial surgery, and brain tumors. In addition, your doctor should investigate vascular abnormalities, stroke, fragile capillary regrowth after brain surgery, or nerve damage (brachial plexus injury)—any event which results in long-term chronic bleeding into your central nervous system.

In the U.S., the estimated case count has now risen past 300 patients. This updated case count almost doubles the number of confirmed diagnoses from 2014. Two critical milestones have contributed to this increase in identifying patients; improved MRI techniques and increased awareness of the disorder by neuroradiologists and audiologists.

The actual occurrence of superficial siderosis of the central nervous system affects a far greater population than is currently diagnosed.

Cortical Superficial Siderosis (cSS)

Cortical superficial siderosis is almost but not always diagnosed in older patients. The bleed source contributing to cSS hemosiderin development has been linked to age-related cerebral small vessel disorder or micro-bleeds on the brain’s surface. Sporadic cerebral amyloid angiopathy (CAA) is a common cerebral small-vessel disease and an identified contributor to cognitive dysfunction in the elderly. The current opinion is cSS is formed from recurring blood leaking episodes from brittle CAA-affected vessels.