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.
In about 35% of cases, there is no determined reason for the bleeding.
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For this reason, it may take as many as 10 or more years for early symptoms to manifest.
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.
Superficial siderosis itself has no cure after hemosiderin deposits have caused damage.
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.
However, the disease is progressive and requires medical care.
About 20% of people diagnosed with progressive cognitive impairment may also eventually develop neurodegenerativedementia.
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.
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.
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.
Various treatment options are available.
Discuss any concerns you have about the disease and your prognosis with your healthcare provider.
The estimated prevalence of cases in the United States is 1 in 1 million.
Superficial siderosis is a progressive disease that may be both disabling or fatal.
Current research does not point to genetics as being a major contributor to superficial siderosis.
However, one much older study pointed to superficial siderosis as a potential contributor to genetic and nongenetic deafness.
National Organization for Rare Diseases.Superficial siderosis.
Vyas S, Giragani S, Singh P, Bansali A, Khandelwal N.Superficial siderosis.Ann Indian Acad Neurol.
2017;48(12):3210-3214. doi.org/10.1161/STROKEAHA.117.018974
Massachusetts General Hospital.The superficial siderosis clinic and research laboratory.
2004 Sep 15;130A(1):22-5. doi: 10.1002/ajmg.a.30050.
PMID: 15368490. doi: 10.1002/ajmg.a.30050
