Treatment Options For Superficial Siderosis: Causes & Symptoms

Superficial siderosis is a disorder of the brain which develops due to chronic iron deposition in the neuronal tissues related to the cerebrospinal fluid. Hemosiderin gets deposited in the neuronal tissue, and causes neuronal loss, gliosis and demylination of the neuronal cells.

Superficial siderosis is a very rare disease, with less than 270 cases reported in scientific narrative as of 2006. It afflicts people of all age groups, with men being affected 3 times more frequently than women. Recently, the number of reported cases of superficial siderosis has increased due to diagnosis via MRI; nonetheless, it still remains an uncommon disease.

Symptoms Of Superficial Siderosis

The symptoms of superficial siderosis are due to symptoms resulting from brain damage

  • Sensorineural loss of hearing: Is the commonest symptom of superficial siderosis. Usually, the highest pitches are lost first, and within 1 to 12 years, the hearing loss progresses to complete deafness.
  • Ataxia: The impairment of gait is a presenting feature.
  • Anosmia: Loss of smell.
  • Pyramidal signs: Indications of a disorder of the pyramidal tracts.
  • Bladder disturbances.
  • Unequal sized pupils.
  • Dementia is an important manifestation.

Causes Of Superficial Siderosis

The chief underlying etiological factor of superficial siderosis is continual bleeding in to the sub-arachnoid space of the brain; this releases RBCs in to the cerebrospinal fluid.

The chronic bleeding may be due to -cavernous malformation, arteriovenous malformation, myxopapillary ependymoma of the spinal cord, from a ventricular shunt, sub-dural hematoma or sub-arachnoid hemorrhage.

Chronic bleeding may also be because of past brain surgery trauma to the brain, with superficial siderosis becoming apparent several years later. In a large chunk of cases, the source of the bleeding wasn’t established.

Treatment Options For Superficial Siderosis

There is no cure for superficial siderosis; treatments are done to help allay the symptoms and to prevent the development of any more symptoms.

  • In case the source of bleeding is established (sources are by and large not recognized), then bleeding source is corrected via surgery; this helps stop the development of further symptoms in some cases but has no effect on symptoms that have already developed.
  • Doctors prescribe deferiprone, a lipid-soluble iron chelator, to manage most cases of superficial siderosis, given that, this drug can chelate iron in the central nervous system. When you are on to this medication, you require frequent blood tests, usually weekly to monitor the blood levels since this medication reduces levels of certain components of blood, such as the neutrophils. It is vital for you to understand that, while it is ok if these levels reduce in a healthy individual, if they diminish in a patient having superficial siderosis and on to chelation therapy and taking Deferiprone Ferriprox, it can cause life threatening infections which can even cause death.
  • Dealing with the most common symptom, hearing loss, is largely successful through the use of cochlear implants. Some people may not perceive a major improvement after cochlear implantation, which is because of damage that has occurred to the vestibulocochlear nerve and not the cochlea itself. Others fare better, and may have a complete return to near normal hearing, however, it is difficult to assess how well a person will respond to the treatment.