Superficial siderosis is a rare, disabling degenerative disorder of the brain and spinal cord. It develops when blood leaks into the subarachnoid space, the fluid-filled layer around the central nervous system, slowly and repeatedly over many years. The broken-down blood leaves an insoluble iron-bearing pigment called hemosiderin on the surface of the brain and the pia mater, and the iron released from that layer is toxic to the nerve tissue beneath it. Because the damage builds so gradually, the usual signs of progressive hearing loss in both ears, unsteady balance and gait (ataxia) and spinal cord weakness with pyramidal signs often appear decades after the original bleeding. In many patients no bleeding source is found, but dural tears, spinal surgery, tumors and head or nerve injuries can be the cause. Diagnosis rests on MRI, and treatment aims to find and close any bleeding source and to manage symptoms. It is commonly mistaken for multiple sclerosis or Parkinson's disease.
Facts
Classification
ICD-10 ChapterIX: Diseases of the Circulatory System 2 Communicable or Non-communicable Transmission Route Causative Agent
Causative Agent (category)Idiopathic / Multifactorial 1 First Described Superficial siderosis
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Connections
Treats
Source Superficial siderosis (NORD Rare Disease Database)
Source Superficial siderosis (NORD Rare Disease Database)
Sources
1. Superficial siderosis (NORD Rare Disease Database)
Disease Overview, Summary
Superficial siderosis is a disabling degenerative disorder affecting the brain and spinal cord.
Standard Therapies
A cause is not identified for a large percentage of patients.
Causes
Superficial siderosis is an acquired disorder caused by chronic long-term subarachnoid bleeding.
Treats: Epidural Blood Patch, Standard Therapies [treats]
an epidural blood patch may pull the dura from surrounding bone and immobilize the spinal cord long enough for healing to take place
Treats: Chelation Therapy, Standard Therapies [treats 2]
This removal action must be accomplished through iron chelation.
View the Source2. Orphanet (INSERM): Orphanet: Superficial siderosis (ORPHA:247245)
ExternalReference, ICD-10 mappingQuote, ExternalReference, ICD-10 mapping
I69.0
View the Source 3. Superficial siderosis of the central nervous system: an usual cause of sensorineural hearing loss (PMC)
Introduction, first sentencesQuote, Introduction, first sentences
It was first described in 1908, by Hamill
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