POEMS
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.
0.3/100000
POEMS syndrome is a rare multisystem disorder with an estimated prevalence of 0.3 cases per 100,000 people. In the United States, there are an estimated 5,000 cases of POEMS syndrome
85%
85–100% of patients with POEMS syndrome have peripheral neuropathy at the time of diagnosis
Sensorimotor Neuropathy
Painful sensorimotor neuropathy is common, and is usually most severe in patients without CD
“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 & Signs
(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 & 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 & 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
- Pain medication
- Institute non-drug treatments to reduce pain, such as:
- Avoiding extended periods of standing or walking
- Wearing looser shoes
- 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)
Today, doctors are exploring a link between pre-diabetes (also known as impaired glucose tolerance or IGT) and peripheral neuropathy. Approximately 10% of adults in America have what is being called “pre-diabetes” or “borderline diabetes”—a condition where the body has higher than normal blood sugar levels, but not high enough to be diagnosed as true diabetes. If left untreated, people with pre-diabetes are at risk of developing type 2 diabetes, heart disease, and nerve damage (which could result in peripheral neuropathy.)
People with pre-diabetes or IGT can significantly reduce their risk of developing type 2 diabetes through diet, exercise and learning to control their blood sugar levels.
Symptoms
(Not all symptoms and signs may be present)
People with IGT often have no symptoms. People who actually have diabetes—and who therefore are at greater risk of developing peripheral neuropathy—often don’t realize it because the symptoms of diabetes come on so gradually. Pre Diabetic symptoms and its complications include:
- Frequent urination
- Blurred vision
- Constant thirst
- Fatigue
- Frequent infections
- Cuts and bruises that heal slowly
- Tingling or numbness in the hands or feet
Tests
(Not all evaluation and tests may be necessary)
To test for pre-diabetes:
- Blood test
- Oral glucose tolerance test
Treatments
(Not all treatments and therapies may be indicated)
- Over-the-counter pain medication for mild pain
- 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).
The first sign of diabetic neuropathy is usually numbness, tingling or pain in the feet, legs or hands.
Over a period of several years, the neuropathy may lead to muscle weakness in the feet and a loss of reflexes, especially around the ankle.
As the nerve damage increases, the loss of sensation in the feet can reduce a person’s ability to detect temperature or to notice pain. Because the person can no longer notice when his/her feet become injured, people with diabetic neuropathy are more likely to develop foot problems such as skin lesions and ulcers that may become infected.
Diabetic neuropathy may suddenly flare up and affect a specific nerve or group of nerves. When this occurs, the result may be weakness and muscle atrophy in various parts of the body, such as involvement of the eye muscles or eyelid (e.g., causing double vision or a drooping eyelid) or thigh muscles. Alternatively, neuropathy caused by diabetes may slowly progress over time. It also can interfere with the normal functioning of the digestive system and sexual organs.
Symptoms
(Not all symptoms and signs may be present)
- Numbness, tingling, or pain in the toes, feet, legs, hands, arms, and fingers
- Indigestion, nausea, or vomiting
- Diarrhea or constipation
- Dizziness or faintness due to a drop in blood pressure especially when rising to a standing position
- Problems with urination
- Erectile dysfunction (impotence) or vaginal dryness
Tests
(Not all evaluation and tests may be necessary)
- Comprehensive foot exam
- Physical examination
- Neurological exam
- Electromyography
- Nerve conduction velocity test
- Quantitive sensory testing (QST)
- Nerve or skin biopsy
- Blood studies (to verify diabetes (e.g., HbA1C) and to rule out other potential causes)
Treatments
(Not all treatments and therapies may be indicated)
The goal of treatment for diabetic neuropathy is to relieve discomfort and to prevent further tissue damage. The first step is to bring blood sugar levels under control by diet and medication. Another important part of treatment involves taking special care of the feet.
- Over-the-counter pain medication for mild pain
- For severe pain, take over-the-counter pain medication or prescription drugs used for peripheral neuropathy, on a regular basis—rather than waiting until nighttime when symptoms can become more severe
- Keep blood sugar levels in normal range
- Get regular exercise
- Maintain a healthy weight
- Antidepressants (for pain relief)
- Foot care: inspect your feet daily for injuries
- Special Therapeutic shoes (which may be covered by Medicare and other insurance)
- Take safety measures to compensate for loss of sensation
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