Chronic Inflammatory Demyelinating Polyneuropathy: Causes, Symptoms & Treatment

Chronic inflammatory demyelinating polyneuropathy is also known as chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). It is a very rare disease of the peripheral nerves.

What is chronic inflammatory demyelinating polyneuropathy?

Chronic inflammatory demyelinating polyneuropathy is a disease of the nerves located outside the central nervous system. The disease is rare, with an incidence of two per 100,000 people. Men are more often affected than women. The disease usually begins at an older age. The exact cause is still unclear, but the inflammation appears to be immunologically mediated. Chronic inflammation damages the myelin layer of the peripheral nerves, which can lead to weakness and sensory disturbances in the arms or legs. The disease is treatable but cannot be cured.

Causes

The exact pathogenesis of CIPD has not yet been determined. It is thought that the immune system perceives the myelin sheath as a foreign substance and attacks it. However, it is unclear what triggers this autoimmune process. In some patients, abnormal proteins could be found in the blood. These could promote the damage to the nerves. Other pathogenetic concepts postulate that the aberrant immune response occurs at the humoral and cellular levels. Antibodies circulating in the blood are directed against the antigens of the peripheral nerves. An immune response involving complement, autoreactive T cells, and macrophages is generated. In contrast to the very similar Guillain-Barré syndrome, chronic inflammatory demyelinating polyneuropathy is very rarely preceded by an infectious disease. However, CIPD often occurs in association with diabetes mellitus, paraproteinemia, lymphoma, osteosclerotic myeloma, or other autoimmune diseases such as lupus erythematosus.

Symptoms, complaints, and signs

Chronic inflammatory demyelinating polyneuropathy develops rather slowly. It peaks about two months after the onset of the first symptoms. CIPD usually manifests as paralysis that begins in the legs and later progresses. The paralyses occur symmetrically and are accompanied by reflex attenuation (hyporeflexia) or reflex loss (areflexia). Sensory disturbances in the form of burning or tingling may also occur. Furthermore, affected patients often complain of feelings of compression in the legs or arms. If the upper extremities are paralyzed, fine motor skills are also severely limited. Incomplete paralysis of the legs results in gait disturbances and difficulties in standing up or climbing stairs. Complete paralysis of arms or legs is rare. An unsteady, wide-legged and swaying gait may occur. In children, this gait ataxia is often even the only symptom. Patients also suffer from severe fatigue. Occasionally, muscle tremors occur. CIPD can occur in different variants. Sensory symptoms and ataxic neuropathies are more frequent in sensory CIPD. The motor nerves are also affected here, so that motor deficits also occur in the course of the disease. Lewis-Sumner syndrome is characterized by an asymmetric distribution. The predominantly sensory symptoms initially occur in the upper extremities. The symptoms of CIDP with monoclonal gammopathy of undetermined significance (MGUS) and the axonal variants of CIPD are similar. However, CIDP with MGUS is characterized by monoclonal IgG and IgA gammopathies. Ganglioside antibodies can be detected in the axonal variants.

Diagnosis and course

When chronic inflammatory demyelinating polyneuropathy is suspected, electroneurography is usually performed. This involves determining the functional status of the peripheral nerves. Among other things, nerve conduction velocities, distribution of conduction velocities, refractory period, and amplitude are recorded. In CIPD, nerve conduction velocity is slowed due to demyelination. It is about 20 percent below normal. Distal latencies are prolonged. At the same time, F-wave loss is observed. In most cases, a cerebrospinal fluid examination is also performed. In this case, the cerebrospinal fluid is examined in the laboratory.There is a nonspecific protein elevation, indicating a barrier disorder. The concentration is less than 10 cells per microliter. This is also referred to as cytoalbuminous dissociation. Magnetic resonance imaging can demonstrate symmetrically distributed inflammatory nerve changes and thickened spinal nerve roots. In some forms of CIPD, the so-called ganglioside antibodies can be detected in the blood serum. If a confirmed diagnosis cannot be made using the above diagnostic methods, a nerve biopsy must be performed. In most cases, a biopsy of the lower leg nerve (sural nerve) is taken for histological examination. Inflammatory demyelinating neuropathies can be demonstrated in the semi-thin section. In addition, segmental demyelination can be seen. Differential diagnosis should always include Guillan-Barré syndrome and other polyneuropathies.

Complications

In most cases, severe paralysis results from this disease. These can occur progressively in different parts of the body, leading to a restriction of the patient’s movements. The possibilities in everyday life are thus severely limited. In most cases, the patient’s reflexes also decrease and various movements are only possible with difficulty. As a result, coordination disorders and walking difficulties can develop, so that the affected person may be dependent on walking aids or on the assistance of other people. It is not uncommon for severe fatigue to occur, which cannot be compensated for by sleep. The muscles tremble even with small efforts. Many people suffer from psychological complaints and depression due to the restrictions in everyday life. Contacts with other people can also be damaged by the disease. Treatment usually takes place with the help of medication and leads to success. However, the therapy can lead to severe bone loss. As a rule, the treatment must be repeated after several months. In older patients, the risk of various damages remaining increases.

When should you see a doctor?

Anyone who notices symptoms such as muscle tremors, severe fatigue, or paralysis in the legs that slowly spread to the upper regions of the body should see a doctor. Sensory disturbances such as burning or tingling also indicate chronic inflammatory demyelinating polyneuropathy. If walking difficulties persist, the emergency medical services should be called. The same is recommended if there is an accident or fall due to unexpected paralysis or if the symptoms suddenly increase. If psychological complaints are added, a psychologist can be consulted in consultation with the general practitioner. Very rarely, chronic inflammatory demyelinating polyneuropathy is preceded by an infectious disease. More commonly, it occurs in association with diabetes mellitus, paraaproteinemia, lymphoma, and various autoimmune diseases. Anyone suffering from these pre-existing conditions should speak with their primary care physician immediately if symptoms are mentioned. Other contacts are the neurologist or a specialist in polyneuropathies. With children showing signs of CIPD, the pediatrician is best consulted. In the event of a medical emergency, contact emergency medical services.

Treatment and therapy

If symptoms are mild, prednisone is administered. Prednisone is a steroid hormone in the glucocorticoid class. It has immunosuppressive and anti-inflammatory effects. Since prednisone can result in osteoporosis, osteoporosis prophylaxis should also be considered. The side effects of prolonged glucocorticoid therapy can be severe. To keep the dose small, additive immunosuppressive agents such as cyclophosphamide, cyclosporine, methotrexate, and rituximab are administered. Intravenous administration of immunoglobulins and plasmapheresis are also possible therapeutic options. With plasmapheresis, it must be noted that symptoms can also worsen again after an initial improvement. Moreover, therapy with immunoglobulins and plasmapheresis must be repeated every one to three months. About two thirds of all patients benefit from this therapy combination. The age of manifestation seems to have an impact on the course of the disease. Patients who were younger than 20 years at onset of the disease show a remitting course with good regression.If patients are older than 45 years, neurologic deficits usually remain.

Outlook and prognosis

The prognosis of chronic inflammatory demyelinating polyneuropathy is related to the age of the patient as well as the time of diagnosis. The more advanced the development of the disease at the time of initial diagnosis, the less favorable the future course of the disease. An older age of the patient at the onset of polyneuropathy also has a decisive influence on the prognosis. Motor impairment can be observed more frequently in patients under 20 years of age. Physicians refer to these cases as motor-affected neuropathy with subacute progression. At the same time, these patients more often experience a good regression of the symptoms that have occurred. If the initial manifestation of the polyneuropathy takes place at an age over 60 years, persistent neurological deficits develop more frequently. Patients suffer more from chronic sensorimotor disorders of the peripheral nervous system. In addition, the prospects of recovery are often hampered by other existing diseases. This represents a considerable restriction in everyday life and reduces well-being. At the same time, reduced health and little prospect of improvement increase the risk of suffering further mental disorders. Approximately 10% of sufferers die as a result of polyneuropathy. One in three patients experiences periods of remission. Periods of freedom from symptoms may span several months or years. Permanent recovery is considered unlikely.

Prevention

Because the exact pathomechanisms of CIDP are unclear, no effective prevention is currently known.

Follow-up

Very few, if any, specific options and measures of direct aftercare are available to the affected person for this disease. The affected person is primarily dependent on a quick and, above all, an early diagnosis, so that it does not come to further complications or to further complaints. The earlier a doctor is consulted, the better the further course of the disease usually is. Therefore, it is recommended to contact a doctor at the first symptoms and signs of the disease. Self-cure cannot occur with this disease. The treatment of the disease is usually done by taking various medications. The affected person should always consult or contact a doctor first in case of side effects or interactions. Likewise, it is important to ensure that the medication is taken regularly and in the correct dosage in order to alleviate the symptoms properly. As a rule, those affected are also dependent on the help and support of their own family. This can help to prevent psychological upsets or even depression. Whether it comes through the disease to a reduced life expectancy of the affected person, can not be universally predicted thereby.

What you can do yourself

In chronic inflammatory demyelinating polyneuropathy, symmetrical paralysis occurs that affects the extremities. This creates many hurdles in daily life that cannot always be overcome by self-help. As long as the syndrome manifests itself through sensory disturbances and states of exhaustion, affected persons should avoid stress and extreme physical overexertion, both professionally and in their private lives. Relaxation exercises can be learned within a therapy. Gentle sports such as yoga and swimming support and strengthen the muscles. As the disease progresses, symptoms of paralysis and coordination problems increase. If the gait is unsteady, a walking aid is a safe way of still being able to cope with everyday life independently as far as possible. If motor and cognitive skills become increasingly impaired as the disease progresses, assisted living is a good alternative. Affected persons should consistently apply preventive self-help measures at an early stage in order to be able to maintain their level of living in the long term. Likewise, breaking bad habits such as regular alcohol consumption, smoking and drug abuse, is advisable. Since the symptom is often associated with diabetes mellitus, autoimmune diseases as well as osteosclerotic myeloma, it is important to adjust one’s diet. Especially since the administration of medication to alleviate the syndrome attacks the bones. A balanced diet rich in vitamin D and calcium is recommended, as well as foods rich in vitamins and omega-3 fatty acids.Depression and pain episodes can be counteracted through support groups as well as artistic pursuits.