What is superficial siderosis of the central nervous system?
Superficial siderosis (SS) of the central nervous system (CNS) is a chronic condition consisting of hemosiderin deposition in the subpial layers of the brain (and spinal cord) due to chronic or intermittent low-grade extravasation of blood into the subarachnoid space.
Is there a cure for superficial siderosis?
There is currently no cure for Superficial Siderosis. The only medications currently available to treat SS are oral chelation drugs, which can cross the blood-brain barrier. The best known of these medications is deferiprone (Ferriprox). Oral chelation therapy carries risks and may not be advisable for all patients.
What are the symptoms of superficial siderosis?
Patients will present with one or more of the classic triad of symptoms: hearing loss, movement abnormalities (ataxia), and motor difficulties due to suspected spinal cord injury (myelopathy) with pyramidal signs. Proper recognition and timely early diagnosis of superficial siderosis allow for early care planning.
How common is superficial siderosis?
Superficial siderosis is largely considered a rare disease, with less than 270 total reported cases in scientific literature as of 2006, and affects people of a wide range of ages with men being approximately three times more frequently affected than women.
Is superficial siderosis progressive?
Superficial siderosis (SS) is an extremely rare central nervous system (CNS) condition in which hemosiderin (a product of the breakdown of blood) is deposited in the leptomeninges, subpial layer, ependymal surface, and other parts of the CNS and results in progressive neurological dysfunction [1].
Does superficial siderosis cause dementia?
SS is a rare cause of dementia and memory disorders which can be easily diagnosed with MRI.
What is superficial siderosis?
Superficial siderosis is an acquired disorder caused by chronic long-term subarachnoid bleeding. A large percentage of the identifiable bleed sources are abnormalities resulting from recurring brain bleeds (chronic suboccipital hematomas), sacs protruding from the spinal column (meningoceles) or spinal surgery tears resulting in pseudomeningoceles.
How is superficial siderosis (SS) diagnosed?
MRI sequencing is required to confirm the diagnosis. Superficial siderosis will be characterized as a rim of low signal covering the subpial surfaces of the brain, brainstem, and spinal cord, particularly on the gradient-echo (GRE) or susceptibility-weighted (SWI) sequences.
How long does it take to diagnose siderosis?
The average time from the clinical manifestation of early symptoms such as ringing of the ears (tinnitus), complaints of dizziness, or reports of phantom odors to the diagnosis of superficial siderosis may be as long as ten years, partly due to the slow progression of the disease.
What are the symptoms of amyloidosis and siderosis?
Symptoms of amyloid angiopathy include transient focal neurological episodes, cognitive impairment, generalized seizures, headaches, and increased intracerebral hemorrhage risk. Superficial siderosis is an acquired disorder caused by chronic long-term subarachnoid bleeding.