What Is Superficial Siderosis?

Superficial siderosis is a rare and often unrecognized degenerative disorder that affects the brain and spinal cord. People with this condition usually present with symptoms such as hearing loss, motor issues, and movement abnormalities.

This condition stems from recurring or chronic bleeding into the subarachnoid space, where cerebrospinal fluid (CSF) resides in the brain. The long-term bleeding results in a buildup of hemosiderin—a component of iron storage and delivery—on the brain from circulating CSF.

This article will provide an overview of the signs, symptoms, causes, risk factors, diagnosis, and treatment of superficial siderosis.

Signs and Symptoms of Superficial Siderosis

Several symptoms are associated with superficial siderosis that affect the central nervous system (CNS). The most common symptoms of this condition include:

• Progressive hearing impairment in both ears (95% of patients)
• Progressive loss of coordination, a wide-based gait (how you walk), and balance issues (88% of patients)
• Nerve dysfunction along the spine that results in pain, loss of coordination and balance, and overactive reflexes (76% of patients)

Other common signs and symptoms of superficial siderosis include:

• Impaired cognition
• Uncontrollable eye movements
• Different pupil sizes
• Difficulty swallowing
• Uncontrollable muscle movements
• Loss of coordination and motor function
• Loss of smell and taste
• Slurred speech
• Bladder or bowel issues
• Abnormal foot reflexology

Superficial Siderosis Causes

Superficial siderosis stems from slow and repeated bleeding in the subarachnoid space in the brain. The blood is circulated along with CSF, resulting in hemosiderin—a component of iron—depositing on layers of the brain and spinal cord. In about 35% of cases, there is no determined reason for the bleeding.

Risk Factors

One 2017 study noted that older age may increase your risk for superficial siderosis. Out of 1,412 study participants, 0.21% of people between 50 and 80 years old and 1.43% of people over 69 years old were discovered to have had superficial siderosis.

Superficial siderosis can affect people of every age, gender, and race, although males appear to have the condition 3 times more often than females.

Diagnosis

Superficial siderosis develops over many years due to the slow but progressive bleeding into the subarachnoid space. For this reason, it may take as many as 10 or more years for early symptoms to manifest.

A healthcare provider's review of a person presenting with symptoms may find a history of aneurysm, trauma, or other CNS-related incidents that occurred decades earlier.

Magnetic resonance imaging (MRI) is needed to diagnose this condition. An MRI is a screening tool that uses radiofrequency and magnets to make images within the body.

Treatment

The first step is to determine if an active bleed is present. If so, action is usually needed to stop the bleeding with fibrin glue, an epidural blood patch, or surgical closure.

Superficial siderosis itself has no cure after hemosiderin deposits have caused damage. However, there are some medications used to treat the condition, including oral chelation drugs, such as Ferriprox (deferiprone). All medicines carry some risks, so it is essential to discuss the risks and benefits of taking any medications to treat superficial siderosis.

Prognosis

Prognosis depends widely on the person, their overall health, and how progressive the disease is.

People with mid to late-stage progression are generally more neurologically compromised than people with early-stage disease. Individuals farther in disease progression may also require more recovery time from surgery or illness.

Although the symptoms and side effects of the disease can become moderate to severe, in general, the condition is not immediately life-threatening. However, the disease is progressive and requires medical care. About 20% of people diagnosed with progressive cognitive impairment may also eventually develop neurodegenerative dementia.

Summary

Superficial siderosis is a rare and often unrecognized degenerative disorder that affects the brain and spinal cord. Patients with this condition usually present with symptoms such as hearing loss, motor issues, and movement abnormalities.

Superficial siderosis stems from slow and repeated bleeding in the subarachnoid space in the brain. The blood is circulated along with CSF, resulting in hemosiderin (a component of iron) depositing on layers of the brain and spinal cord.

In many cases, there is no apparent cause for the bleeding. However, other common causes include trauma or intracranial tumors. Superficial siderosis develops over many years due to the slow but progressive bleeding into the subarachnoid space. An MRI is needed to make a diagnosis.

Superficial siderosis itself has no cure. After years of damage due to hemosiderin deposits, treatment can prevent further disease progression but generally not reverse it. Oral chelation drugs are the most common drug treatment for this condition.

A Word From Verywell

Receiving a superficial siderosis diagnosis may feel overwhelming and frightening. But the earlier you get diagnosed, the sooner you can start treatment to stop the bleeding and possibly prevent further disease progression. Various treatment options are available. Discuss any concerns you have about the disease and your prognosis with your healthcare provider.