What Is Siderosis?

Superficial siderosis is a degenerative disease that impacts the brain and spinal cord. Siderosis develops when chronic bleeding (hemorrhaging) occurs within the subarachnoid space where cerebrospinal fluid (CSF) resides in the brain. This results in a toxic buildup of hemosiderin, which is a crucial component of iron delivery and storage in the body. The most common symptoms of siderosis are hearing loss, movement abnormalities, and motor difficulties.

Siderosis can impact any gender, race, or age. However, it is more common to develop the disease over the age of 50. It develops in males 3 times more than females and impacts 1 out of every 1 million people in the general population.

In many cases, there is no apparent cause for the chronic bleeding that occurs with siderosis. But the most common causes are intracranial tumors and brain injury. Siderosis develops slowly over time as the bleeding continues and there is no cure for it. Treatment can prevent further degeneration or disease progression. Treatment is usually unable to reverse the damage that has happened from the hemosiderin deposits. Siderosis is sometimes misdiagnosed as multiple sclerosis (MS), Parkinson’s disease, or multiple system atrophy.

Signs and Symptoms

There are several symptoms associated with siderosis. The most common symptoms include:

• Progressive hearing loss in both ears
• Progressive loss of coordination (wide-based gait and balance issues)
• Nerve dysfunction along the spine (pain, loss of coordination and balance, overactive reflexes) 

Other symptoms include:

• Uncontrollable eye movement
• Different pupil sizes
• Impaired cognition
• Impaired cognition
• Difficulty swallowing
• Uncontrollable muscle movements
• Coordination and balance problems
• Loss of smell and taste
• Bladder and bowel issues
• Slurred speech
• Abnormal foot reflexology

Causes

Siderosis is caused by chronic bleeding that occurs within the subarachnoid space where cerebrospinal fluid (CSF) resides in the brain. This results in a toxic buildup of hemosiderin, which is a crucial component of iron delivery and storage in the body. In about 35% of the cases of siderosis, there is no known cause. For 65% of the other cases, the most common causes include:

• Intracranial brain tumors
• Traumatic brain or spinal injury
• Arteriovenous malformations (abnormal connection between the brain’s arteries and veins)
• Aneurysms
• Spinal surgical changes
• Cranial surgery
• Subdural hematomas (blood pools in the space between the skull and the brain)
• Amyloid angiopathy (protein buildups on the on walls of the arteries within the brain)
• Meningocele (bulging sacs from the spinal column)
• Vascular abnormalities
• Nerve damage 

Risk Factors

There are certain risk factors that put you at a greater risk of developing siderosis. Risk factors include:

• Aging (your risk continues to increase as you age)
• Being male
• Brain injury
• Intracranial tumors 

Diagnosis

To diagnose siderosis, your doctor will evaluate your symptoms, take a thorough medical history, and perform a physical examination. The most effective way to diagnose siderosis is through magnetic resonance imaging (MRI). Often, siderosis can be misdiagnosed with multiple sclerosis, Parkinson’s disease, or multiple system atrophy. Siderosis can only be diagnosed with an MRI.

Treatment

Standard treatment for siderosis consists of first identifying the source of the brain bleed and taking action to stop the bleeding. Your doctor will discuss the best course of action to stop the brain hemorrhaging with you. There are several ways to stop the bleeding, including using fibrin glue, an epidural blood patch, or a surgical closure.

The next phase of treatment focuses on stopping any hemosiderin deposition from continuing. The recommended form of treatment for this is taking oral chelation medication. This type of medication carries some risk, so it is important to consult with your doctor to discuss whether it is right for you. There is no cure for superficial siderosis.

Although superficial siderosis is not immediately life-threatening, the damage caused by the hemosiderin deposition is irreversible, so it is crucial to begin treatment early in the disease progression. People with mid to late-state disease progression tend to be more neurologically compromised, requiring longer recovery time from surgery or illness. Additionally, people who have shown to have symptoms of cognitive impairment have a 20% greater likelihood of developing dementia.

Prevention

There are no known specific prevention measures to reduce your risk of developing superficial siderosis. However, since one of the known causes of the condition is intracranial tumors, following prevention measures for developing cancer may be beneficial.

Complications

Complications for superficial siderosis include:

• Irreversible neurological damage
• Longer recovery from surgery or illness
• Dementia
• Side effects from medications