Types of Peripheral Neuropathy - Inflammatory

Idiopathic | Pre-diabetic/Diabetic | Hereditary | Toxic/Secondary to Drugs | Inflammatory | Systemic/Metabolic |
Compression | Other Types of PN

POEMS syndrome is a rare multisystem disorder caused by the improper growth of bone marrow cells, resulting in an abnormal accumulation of proteins (immunoglobulin) in tissues and organs. "POEMS" is an acronym for the five most common symptoms of this syndrome:

P - Peripheral neuropathy, affecting many nerves

O - Organomegaly (i.e., abnormal enlargement of organs, such as the spleen and liver)

E - Endocrinopathy (i.e., abnormal hormone levels that may inhibit normal growth, sexual development, metabolic functions)

M - Monoclonal gammopathy or M protein (an immunoglobulin that results from a collection of bone marrow cells that have abnormally proliferated)

S - Skin changes, darkening of skin pigment, thickening of skin, increased body hair

The cause of POEMS is unknown, but it is usually associated with plasma cell disorders such as myeloma. It is neither contagious nor inherited. To receive a diagnosis of POEMS, a patient need not have all five symptoms. The disorder is more common in people in their 50s, with men being slightly more at risk than women. It occurs more frequently in Japan, where it is known as Crow-Fukase syndrome.

SYMPTOMS

(Not all symptoms and signs may be present.)

In addition to the characteristics described by the POEMS acronym above, such as peripheral neuropathy, patients may experience:

• Changes in vision
• Easy fatigue
• Enlarged breast area tissue (in both women and men)
• Failure of ovaries and testes to function
• Fluid retention in the abdomen
• Swollen ankles and legs
• Weakness in the feet, legs, hands and arms caused by nerve damage

EVALUATION AND TESTS

(Not all evaluation and tests may be necessary.)

• Neurological exam
• Electromyography
• Nerve conduction velocity test
• Blood tests (e.g., immunoglobulin electrophoresis, hormone studies)
• Urine tests
• X-ray of bones
• Biopsy of bone marrow

TREATMENT AND THERAPY

(Not all treatments and therapies may be indicated.)

Treatment focuses on alleviating the worst symptom of POEMS, which is the nerve damage that causes progressive weakness in the body's extremities. Treatment also attempts to stop the production of the bone marrow cells that create problems in other parts of the body.

• Physical therapy
• Radiation therapy
• Corticosteroids
• Chemotherapy
• Hormone replacement, if necessary

For peripheral neuropathy induced by POEMS:

• Discontinue drugs that cause peripheral neuropathy
• Institute non-drug treatments to reduce pain, such as:
• Avoiding extended periods of standing or walking
• Wearing looser shoes
• Pain medication
• Take safety measures to compensate for loss of sensation
• Ask your doctor about special therapeutic shoes (which may be covered by Medicare and other insurance)