Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP)

February 7, 2017

Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP) is a rare neurological disorder characterized by gradually increasing sensory loss and weakness associated with loss of reflexes. It is an inflammatory disease of the peripheral nerves wherein the body’s immune system attacks its own nerves.

During infancy the body’s immune system learns to differentiate between self and non-self, i.e. normally the body learns not to attack itself and knows to attack any foreign objects like viruses or bacteria that has entered and needs to be destroyed. In an autoimmune disease, the recognition of self and non self is lost, and body attacks itself. In CIPD, myelin is attacked, resulting in demyelination. Myelin is an important part of the peripheral nervous system, which wraps around the nerve axon (the long, wire-like part of a nerve cell) just like insulation around an electrical wire. The nerves extend from the spinal cord to the rest of the body, stimulating muscle contraction and transmitting sensory information back to the nervous system from receptors in the skin and joints. This insulation (myelin) allows electrical impulses to efficiently travel along the nerve axon. When myelin is damaged or removed, these electrical impulses are slowed or lost, and messages transmitted from the brain are disrupted and may never make it to their final destination. If this occurs acutely then the illness is called Guillain-Barre and if it becomes chronic then it is called Chronic Inflammatory Demyelinating Polyneuropathy or CIDP.

CIDP is usually classified by these three types:

  • Progressive :The disease continues to worsen over time
  • Recurrent : Episodes of symptoms that stop and start
  • Monophasic : One bout of the disease that lasts one to three years and doesn’t recur

CIPD is a rare disorder that may affect any age group and the onset of the disorder may begin during any decade of life. It affects males twice as often as females and the average onset age is 50 years. At any one time, between 5,000 and 10,000 people in the United States are affected by it. CIDP, by definition has ongoing symptoms for over 8 weeks and usually does not improve unless ongoing treatment is given.

Causes

Chronic inflammatory poly neuropathy is one cause of damage to nerves outside the brain or spinal cord (peripheral neuropathy). Polyneuropathy means several nerves are involved. It usually affects both sides of the body equally.

Chronic inflammatory demyelinating polyneuropathy (CIDP) is the most common chronic neuropathy caused by an abnormal immune response. CIDP occurs when the immune system attacks the myelin cover of the nerves. The cause of chronic inflammatory polyneuropathy is an abnormal immune response. The specific triggers vary. In many cases, the cause cannot be identified.

It may occur with other conditions, such as:

  • Idiopathic Neuropathy

Sometimes CIDP seems to happen for no particular reason. Doctors call this disorder “idiopathic”, which means “of unknown cause.” Typically, idiopathic CIDP occurs in people over 60 years old; progresses slowly (or doesn’t progress at all after the initial onset); and it can be very disruptive to someone’s normal life and lifestyle.

  • Diabetes

Diabetic neuropathy, or nerve damage caused by diabetes, is one of the most common known causes of neuropathy. It is one of many complications associated with diabetes, with nearly 60 percent of diabetics having some form of nerve damage. It is a progressive disease that can involve loss of sensation, as well as pain and weakness, in the feet and sometimes in the hands.

  • Inflammatory Bowel Disease

Inflammatory bowel disease (IBD) involves chronic inflammation of all or part of your digestive tract.

  • Toxic Chemicals

Toxins, poisons and chemicals can cause peripheral neuropathy, which may cause CIDP. This can happen through drug or chemical abuse or through exposure to industrial chemicals in the workplace or in the environment. Common causes include: exposure to lead, mercury, arsenic and thalium.

It may occur with following diseases as well: HIV, Chronic hepatitis, Systemic lupus erythematosus, Lymphoma, Paraneoplastic syndrome, Thyrotoxicosis, Side effects of medicines to treat cancer or HIV, Vasculitis.

Symptoms

The symptoms of Chronic Inflammatory Demyelinating Polyneuropathy include numbness, tingling, burning and weakness, generally occurring in the feet first and they gradually ascend to involve other parts of the body.  One of the first signs that could develop is a foot drop, where patients develop weakness in their ankles and as a result tend to trip over their feet, or they could develop numbness and tingling in the feet and toes which gradually spreads to the other parts of the body.

The symptoms of CIDP are as follows:

  • Loss of strength and sensation on the limbs
  • Muscle weakness
  • Numbness, pins and needles sensation
  • Poor Balance on Body
  • Trembling of the hands when reaching anything

At times, related physical symptoms also include:

  • Signs of cranial nerve (CN) involvement
  • Gait abnormalities
  • Motor deficits
  • Diminished or absent deep tendon reflexes
  • Sensory deficits
  • Impaired coordination

Treatment

Improvement in functional status and maintenance of long term remission are the main goals of treatment of CIDP. Early intervention is necessary to avoid the permanent weakness, sensory loss, pain and imbalance that are commonly caused by axonal loss. Corticosteroids, IVIg and plasmapherisis are the three conventional therapies that remain as a standard treatment for CIDP.

  • Corticosteroids – Similar to naturally occurring anti-inflammatory hormones made by the body, and can be used as an initial treatment. Corticosteroids often improve strength, are conveniently taken by mouth, and are inexpensive. Side effects however can limit long-term use. These include osteoporosis (thinning of bones), cataracts, diabetes, hypertension (raised blood pressure), obesity and myopathy (muscle weakness).
  • Immunosuppressive drugs – Clinical experience suggests that immunosuppressive drugs help. These include azathioprine, cyclophosphamide and cyclosporin. Azathioprine is the most widely used in the treatment of CIDP. The use of these drugs carries the theoretical side effect of increased risk of developing cancer, but in practice this increased risk is very small.
  • Intravenous Immune Globulins (IVIG) – The only drug that has FDA, Canadian, and European approval for treatment of CIDP. IVIG contains naturally occurring antibodies obtained from healthy volunteers. IVIG is given through a vein over the course of several hours. Newer preparations of higher concentrations that can be given under the skin (subcutaneous) are currently being tested in controlled trials in CIDP patients. The most common side effects are headache, nausea, chills, flushing, myalgia, hypotension, hypertension, chest discomfort, and fatigue. Infrequent adverse reactions include thromboembolic events, skin reactions, aseptic meningitis, renal tubular necrosis, and severe anaphylactic reaction.
  • Plasma Exchange (PE) – A process by which some of the patient’s blood is removed and the blood cells returned without the liquid plasma portion of the patient’s blood. It may work by removing harmful antibodies contained in the plasma.
  • Physiotherapy – Physiotherapy has an important role to play in the assessment and management of CIDP. It helps to maximize a patient’s physical potential, particularly where weakness is the main problem.

The aims of physiotherapy are to –

  • maximise muscle strength and minimise muscle wastage by exercise using strengthening techniques;
  • minimise the development of contractures (or stiffness) around joints; a physiotherapist can advise on passive stretching techniques to help maintain full range movement at joints;
  • facilitate mobility and function; sometimes, if muscles are very weak, function can be improved by the use of splints and
  • provide a physical assessment which may help in planning future management.

Alternative Treatment

Alternative medicine involves treatment of underlying conditions can help control neuropathy pain. Alternative medicine may also play a role in reducing the symptoms of peripheral neuropathy.

Acids

Studies suggest that supplementing essential acids called ALA (alpha-Lipoic acid) and GLA (gamma linolenic acid) and omega-3 fatty acids may all have a beneficial effect on diabetic peripheral neuropathy, which may be an underlying cause. Over the course of long-term treatment a reduction in symptoms and better blood flow is seen in the patients.

L-Carnitine

L-carnitine is a substance that the body makes and stores in various organs, including the liver and the brain. It helps to regain regular sensation in their limbs when they increase their consumption of a type of carnitine called acetyl-L-carnitine. Red meat, peanut butter and dairy products are good dietary sources of the nutrient, but supplements are also widely available at health food stores and pharmacies.

Vitamins and Minerals

Studies show that vitamin deficiencies may result in peripheral neuropathy conditions in some people. Replenishing vitamins B1, B12 and E may lead to a decrease in symptoms. Recommended dosages are 300mg daily of vitamin E.

Traditional Chinese Medicine

Acupuncture, a form of traditional Chinese medicine, may be an effective way to manage CIPD. Some herbal supplement taken orally may reduce the pain that is consistent with neuropathy. Topical creams containing capsaicin, an anti-inflammatory substance found in chili peppers, can reduce the burning sensation in some people who experience this painful symptom. Tai chi and yoga help align the body and mind, encourage relaxation and may distract people from the pain, even if the measure is temporary.

Herbal Supplements

Several herbal remedies may be alternatives to explore when treating peripheral neuropathy. Some herbal supplement taken orally may reduce the pain that is consistent with neuropathy. Topical creams containing capsaicin, an anti-inflammatory substance found in chili peppers, can reduce the burning sensation in some people who experience this painful symptom.

Treatment does not only mean doctors and physicians making single efforts for the patient, the patient should also be actively interested in getting cured. It always is a team effort…..

Posted in NEUROVASCULAR