How To Diagnose And Treat Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)

September 9, 2023

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a disorder of autoimmunity where an individual's immune system inappropriately attacks the healthy tissues that protect the nerve cells. The myelin sheath is a type of protective fatty insulation that helps shield nerves from damage and allows for the smooth and uninterrupted transmission of impulses from one nerve to the next. The trigger of CIDP in some individuals is not currently known and has no infectious or genetic association. Chronic inflammatory demyelinating polyneuropathy is more common in older adults than it is in any other age group, and it is more prevalent in men than it is in women. Individuals with CIDP experience symptoms including gradual limb weakening, reflex loss, balance loss, numbness, tingling, and difficulty walking. CIDP can be diagnosed using a physical exam, blood tests, urine tests, nerve conduction study, and a lumbar puncture.

There are several ways chronic inflammatory demyelinating polyneuropathy can be diagnosed and treated. Reveal them now.

Diagnostic Tests

It can be difficult for a physician to narrow down a diagnosis of chronic inflammatory demyelinating polyneuropathy in an individual who presents with symptoms characteristic of this rare neurological disorder. An individual who has symptoms of chronic inflammatory demyelinating polyneuropathy may be brushed off until their symptoms become so pronounced that they interfere with everyday activities and responsibilities. The only diagnostic tests used specifically to make a chronic inflammatory demyelinating polyneuropathy diagnosis are a spinal tap, nerve biopsy, and electromyography. A chronic inflammatory demyelinating polyneuropathy patient may exhibit abnormally high levels of certain proteins in their cerebrospinal fluid that can be detected with the use of a spinal tap procedure. Patients will also present with a pattern of multifocal demyelination when they undergo a nerve conduction study or electromyography.

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Plasmapheresis is a treatment method where the patient's blood is removed from the body and plasma is separated from the rest of the blood inside of a specialized machine. The plasma portion of the blood is replaced with a mixture of saline and albumin before it is mixed with the rest of the blood and sent back into the patient's body. The mechanism of this type of blood filtering is similar to what occurs with dialysis. The objective of this treatment is to remove the components of the blood responsible for eliciting the inappropriate attack on the healthy myelin sheathing. Numerous different types of autoantibodies are implicated in chronic inflammatory demyelinating polyneuropathy. The most common autoantibodies identified in affected individuals include antibodies against peripheral myelin protein two or PMP2, peripheral myelin protein 22 or PMP22, and myelin protein zero or MPZ. The regular removal of these antibodies from an affected individual's blood via plasmapheresis can help alleviate symptoms of CIDP.

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Symptoms of chronic inflammatory demyelinating polyneuropathy are caused by an abnormal reaction of the patient's immune system where it attacks and destroys the cells that make up the myelin sheath around the nerves. Corticosteroids are a type of medication that changes mediator functions in the part of the body where the immune system is attacking its own tissues and alters the synthesis of proteins in the cells to suppress the immune responses and inflammation. In the cases of many individuals with autoimmune conditions, corticosteroids are used as the first-line treatment to help the patient's symptoms go into remission. Corticosteroids are known to be effective for the treatment of chronic inflammatory demyelinating polyneuropathy in most individuals, but may not work for everyone. Other treatment methods are often used alongside corticosteroids in patients with severe CIDP. Some of the most common corticosteroids used in individuals with CIDP include intravenous methylprednisolone, oral daily prednisone, and pulsed dexamethasone.

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Intravenous Immunoglobulin

Intravenous immunoglobulin is an infusion composed of small portions of IgM and IgA and a more significant portion of heterogeneous human IgG. In each batch of intravenous immunoglobulin, there are blood components that have been prepared from the serum of between one and fifteen thousand human donors. This type of solution can provide a wide array of antibodies with the capacity to neutralize the components of the immune system causing damage to the affected individual's myelin sheathing. The exact mechanism of action that has proven to show improvements in the symptoms of CIDP is not clear, but it is thought to involve the inhibition of the autoantibody action on the healthy tissues. Intravenous immunoglobulin has been able to promote the remyelination of lacking nerves, which may reverse some of the damage done by the affected individual's rogue immune system.

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As we know, chronic inflammatory demyelinating polyneuropathy is an illness known to cause progressive muscle weakness. This malfunction can make it difficult for a patient to perform everyday tasks like walking. Once the illness is under control, the atrophy or muscle wasting that occurs in the individual can be reversed as long as there is no permanent damage to the nerves. Even individuals who do have permanent nerve damage due to CIDP may choose to undergo physiotherapy to help maximize their movement potential and improve their quality of life. Physiotherapy is the utilization of movement and exercises to help re-strengthen muscles, improve mobility, increase range of motion, and rehabilitate impaired muscles. Physiotherapy is merely a part of a patient's treatment regimen, as it does not provide any curative qualities to the autoimmune disorder itself. Physiotherapy can help alleviate symptoms and prevent some complications of CIDP, but it does not stop the patient's immune system from attacking the protective myelin around the nerves.

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If corticosteroids are not an option for a chronic inflammatory demyelinating polyneuropathy patient, immunotherapy may be added to help mediate their symptoms. Immunotherapy is a treatment typically used to slow down the actions of an individual's immune system. This mechanism is difficult to achieve because it involves leaving some parts of the patient's immune system active and functioning, while selectively deactivating the parts of the immune system causing the CIDP symptoms. Other methods in immunotherapy used to treat autoimmune disorders like CIDP are not specific to the particular parts of the immune system that cause the specific disorder. This non-specificity results in adverse side effects some patients may not be able to handle. Immunotherapy is the manipulation of the individual's immune system responses to treat the imbalance without causing significant adverse side effects.

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Stem Cell Transplant

Some individuals affected by chronic inflammatory demyelinating polyneuropathy may benefit from the use of a hematopoietic stem cell transplant. A hematopoietic stem cell transplant is a procedure where stem cells are used to rebuild a patient's immune system. First, high doses of ATG and cyclophosphamide are used to eradicate all of the existing cells in the immune system of a chronic inflammatory demyelinating polyneuropathy patient. Next, stem cells are introduced to the affected individual's body. These stem cells essentially repopulate and rebuild the patient's immune system with the hope it will no longer attack healthy tissues in the body. A new and healthy immune system that results from a stem cell transplant has proven to cure chronic inflammatory demyelinating polyneuropathy in some patients who had no success with other methods of treatment.

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A chronic inflammatory demyelinating polyneuropathy patient may need to take immunosuppressants as part of their treatment regimen to relieve symptoms. An immunosuppressant is a type of medication used to lower or decrease the activity of an affected individual's immune system. Disorders caused by the inappropriate and abnormal attack of the immune system on the healthy tissues of an individual's body can sometimes be mediated when the immune system is downregulated in its activities. Immunosuppressants work by reducing the overall activity of the patient's immune system. It is important to consider an individual becomes more vulnerable to infections and abnormal cell growth in their body when they take immunosuppressants. In some cases, a patient may be treated with an immunosuppressant in conjunction with other medications, like corticosteroids. Azathioprine, methotrexate, cyclophosphamide, mycophenolate, and cyclosporine are the most common immunosuppressants utilized in the treatment of chronic inflammatory demyelinating polyneuropathy.

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Patient Prognosis

The prognosis of a chronic inflammatory demyelinating polyneuropathy patient is difficult to determine because it presents with a variety of different clinical evolutions and patterns. A young patient who has less pain at the onset of their chronic inflammatory demyelinating polyneuropathy will have a better long term outcome than an older patient who experiences more pain. Poor functional outcome in an individual affected by chronic inflammatory demyelinating polyneuropathy is associated with the finding of axonal loss on a nerve biopsy. Individuals affected by the chronic progressive course of chronic inflammatory demyelinating polyneuropathy are known to have a worse prognosis than patients affected by the relapsing course of chronic inflammatory demyelinating polyneuropathy. Chronic inflammatory demyelinating polyneuropathy patients respond in various ways to different treatment methods. The efficacy of treatment is highly dependent upon how progressive the disorder is at the time of diagnosis.

Learn about chronic inflammatory demyelinating polyneuropathy's connection to Guillain-Barre syndrome next.

Connection To Guillain-Barre Syndrome

Guillain-Barre syndrome is a condition where an individual's peripheral nerves develop an acute inflammatory disease characterized by symptoms that develop suddenly and progress rapidly. This syndrome is caused by an inappropriate and abnormal reaction by an individual's immune system, where it attacks the protective myelin sheathing around the nerves. Most individuals affected by Guillain-Barre syndrome recover without a problem and do not experience long-term or persistent symptoms. However, some Guillain-Barre syndrome patients may experience a permanent alteration of their immune system, where it continues to destroy myelin sheaths slowly and progressively. This alteration that occurs in an affected individual's immune system causes long-term symptoms that can be characterized as chronic inflammatory demyelinating polyneuropathy.

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