ALTERNATE NAMES

Superficial Siderosis of the CNS; Superficial Hemosiderosis of the CNS; Superficial Hemosiderosis of the Central Nervous System

DESCRIPTION

Superficial Siderosis of the Central Nervous System (CNS) is a progressive disease of the central nervous system caused by the accumulation of hemosiderin (iron salt) deposits on the brain surface, spinal cord, or cranial nerves.

The hard iron salt deposits are created from chronic bleeding into the subarachnoid space or brain surface, underneath the three protective membranes. In most cases, the source of the bleeding is never located due to a considerable time delay before diagnosis. More than one bleed is required to cause superficial siderosis.

DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CM CODING

Diagnostic testing: An MRI of the head, spinal cord or cranial nerves is needed to make the diagnosis of this disease. CT myelogram may assist with identifying leakage from a dural defect.

Physical findings: Individuals with Superficial Siderosis of the CNS may have difficulty with:

• Hearing;

• Ability to smell and taste;

• Balance;

• Co-ordination and weakness of limbs;

• Difficulty with bladder and bowel functions;

• Eye movements;

• Early dementia;

• Bilateral sciatica; and

• Pain in lower back and joints.

Other less common side effects include:

• Transient ischemic accidents (TIA) or mini strokes;

• Orthostatic headache;

• Cognitive difficulties;

• Loss of ability to hold one’s head up; and

• Compression of gullet muscles.

ICD-9: 331.0

PROGRESSION

Superficial Siderosis of the CNS progresses slowly over the course of decades. This disease affects people of a wide range of ages with men being diagnosed approximately three times more frequently than women. The number of reported cases of superficial siderosis has increased with advances in MRI technology, but it remains a rare disease.

TREATMENT

Treatment varies based on the underlying cause and severity of the condition and may include surgery and medications. If identified early in the diagnosis, ablating or plugging the cause of bleeding may help restrict further complications. The bleeds may be attributed to complications in the spine such as tumors or other similar problems.

Some other forms of medication such as iron chelators have been tried but have not definitely proven to be beneficial.

Some individuals may be at risk of developing dementia and are treated with folic acid in tablet form, or vitamin B, in daily tablet form or monthly injection.

To lessen the chance of a return of TIA’s or strokes various forms of medication are prescribed such as blood thinners and the traditional half of an aspirin a day.

For those experiencing permanent headaches, anti-depressants in mild doses are found to provide long-term relief, along with common medication such as Panadol.

SUGGESTED PROGRAMMATIC ASSESSMENT*

Suggested MER for evaluation:

• Clinical history and examination that describes the diagnostic features of the impairment.

• Results of MRI/CT scan of the brain surface, spinal cord, or cranial nerves.

Suggested Listings for Evaluation:

DETERMINATION

LISTING

REMARKS

Meets

11.17