Chronic inflammatory demyelinating polyneuropathy is a condition in which there is progressive damage to nerve fibers in the peripheral nervous system. The pathogenesis of this disease is not entirely clear, but it is clearly visible that it is associated with abnormalities in the functioning of the immune system of patients. What is the symptom of chronic inflammatory demyelinating polyneuropathy, on the basis of what tests can it be diagnosed and what are the treatment options for this entity?

Contents:

  1. Chronic Inflammatory Demyelinating Polyneuropathy: Causes
  2. Chronic Inflammatory Demyelinating Polyneuropathy: Symptoms
  3. Chronic Inflammatory Demyelinating Polyneuropathy: Diagnostics
  4. Chronic Inflammatory Demyelinating Polyneuropathy: Treatment
  5. Chronic inflammatory demyelinating polyneuropathy: prognosis

Chronic inflammatory demyelinating polyneuropathy (CIDP) was first mentioned in the medical literature at the end of the 19th century - then, exactly in 1890, the German neurologist Hermann Eichhorst described first cases of this disease.

Disease rarely receives much attention due to the fact that it is relatively rare - it is estimated that CIDP occurs in a few (5-7) out of 100,000 people. In men, the disease occurs even twice as often as in women, typically the first symptoms of chronic inflammatory demyelinating polyneuropathy appear in 4-5. decade of life.

Chronic Inflammatory Demyelinating Polyneuropathy: Causes

The exact cause of chronic inflammatory demyelinating polyneuropathy is unknown. The problem is quite often included in the group of autoimmune diseases - the abnormalities in the functioning of their immune system are noticeable in patients with CIDP. In the course of the disease, for inflammatory infiltrates in the vicinity of nerve fibers, in which macrophages and T lymphocytes are involved.

The effect of the inflammatory reaction in patients with CIDP is the destruction of the myelin sheath surrounding nerve fibers, which is responsible for the occurrence ofcharacteristic symptoms of chronic inflammatory demyelinating polyneuropathy.

In fact, in patients with CIDP, it is quite easy to identify phenomena related to autoimmunity, difficulties arise when trying to find an explanation for this type of reaction. So far, no antibodies that would favor the pathological processes occurring in patients with this unit have been known, and there are no known antigens, contact with which could initiate autoimmune reactions leading to the destruction of the myelin sheaths characteristic of CIDP.

The only thing scientists have found so far is that chronic inflammatory demyelinating polyneuropathy sometimes appears in association with other conditions - diabetes, HIV infection, monoclonal gammapathy of undefined importance are examples of such entities and systemic lupus erythematosus, sarcoidosis and certain neoplastic diseases.

Chronic Inflammatory Demyelinating Polyneuropathy: Symptoms

The primary symptom of chronic inflammatory demyelinating polyneuropathy is muscle weakness in the upper and lower extremities. The disease causes dysfunction of both distal and proximal muscles, it is also characteristic that the symptoms are symmetrical and gradually worsen over time.

Patients mainly suffer from motor dysfunction, and in addition, they also experience various sensory disturbances (such as loss of sensation, but also the feeling of unusual sensations, such as numbness or stinging). In addition, patients with CIDP also experience weakened or even complete loss of tendon reflexes, and may also have disturbances in the sense of vibration.

In addition to the typical symptoms of CIDP listed above, patients may develop other types of ailments, which may include:

  • receiving a duplicate image,
  • feeling of general weakness,
  • imbalance,
  • difficulty swallowing.

Chronic Inflammatory Demyelinating Polyneuropathy: Diagnostics

Initially, a neurological examination is performed in patients whose symptoms may indicate chronic inflammatory demyelinating polyneuropathy. After they have found deviations characteristic of this disease, additional tests are ordered, thanks to which it is possible to confirm CIDP and exclude other possible causes of the patient's ailments.

Among the diseases with which to differentiatechronic inflammatory demyelinating polyneuropathy, mainly multifocal motor neuropathy, polyneuropathy being one of the elements of the POEMS syndrome and Guillain-Barre syndrome.

When chronic inflammatory demyelinating polyneuropathy is suspected, one of the first tests performed in patients is electromyography (EMG). The abnormalities in this study that may confirm the diagnosis of CIDP include slowing down the conduction of impulses in nerve fibers, the existence of a partial conduction block and an increase in the latency of the movement potentials.

In patients with suspected this unit, lumbar puncture is also ordered: in patients with chronic inflammatory demyelinating polyneuropathy of the cerebrospinal fluid, an increased amount of protein is usually found with normal fluid cytosis.

Sometimes a nerve fiber biopsy is performed, thanks to which it is possible to detect inflammatory infiltrates from T lymphocytes and macrophages, but also to notice the processes of demyelination and remyelination of nerve fibers. In some patients, imaging tests are ordered - for example, during MRI, it is possible to observe inflammatory changes within the nerve roots or hyperplasia of the nerve plexuses, which can occur in the course of CIDP.

Chronic Inflammatory Demyelinating Polyneuropathy: Treatment

In the treatment of chronic inflammatory demyelinating polyneuropathy, interactions focused on disorders of the immune system are the most important. It is because of this necessity that the basic preparations prescribed to patients suffering from this condition are glucocorticosteroids and administered in the form of intravenous immunoglobulin infusions.

In patients with severe forms of CIDP, plasmapheresis and other immunosuppressive agents (such as cyclophosphamide, cyclosporine or mycophenolate mofetil) are also used. Regular participation in rehabilitation classes is very important for the patients to maintain their psychomotor fitness for as long as possible. It is also important to treat conditions that may have contributed to the onset of CIDP symptoms, such as diabetes and sarcoidosis.

Chronic inflammatory demyelinating polyneuropathy: prognosis

The course of chronic inflammatory demyelinating polyneuropathy can be very different - unfortunately, this disease has a tendency to proceed with states of remission and relapse. Slow down the progress of the disease or even lead to a complete disappearance of related diseasesHowever, the symptoms can be properly and effectively treated - thanks to it, even in almost half of the patients (on average in 4 out of 10 treated people) the symptoms of chronic inflammatory demyelinating polyneuropathy disappear completely.

About the authorBow. Tomasz NęckiA graduate of the medical faculty at the Medical University of Poznań. An admirer of the Polish sea (most willingly strolling along its shores with headphones in his ears), cats and books. In working with patients, he focuses on always listening to them and spending as much time as they need.

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Category:

Neurology