Chronic Inflammatory Demyelinating Polyradiculoneuropathy
Disease Details
Family Health Simplified
- Description
- Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP) is a neurological disorder characterized by progressive weakness and impaired sensory function in the legs and arms due to immune system-induced damage to the myelin sheath of peripheral nerves.
- Type
- Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP) is not typically caused by genetic factors; it is generally considered an autoimmune disorder. Therefore, there is no specific type of genetic transmission associated with CIDP.
- Signs And Symptoms
- In its traditional manifestation, chronic inflammatory demyelinating polyneuropathy is characterized by symmetric, progressive limb weakness and sensory loss, which typically starts in the legs. Patients report having trouble getting out of a chair, walking, climbing stairs, and falling. Problems with gripping objects, tying shoe laces, and using utensils can all be brought on by upper limb involvement. Proximal limb weakness is a fundamental clinical characteristic that sets apart chronic inflammatory demyelinating polyneuropathy from the vast majority of distal polyneuropathies, which are far more common. Proprioception impairment, distal paresthesias, loss of feeling, and poor balance are all brought on by sensory involvement. Only a small percentage of cases involve neuropathic pain.Fatigue has been identified as common in CIDP patients, but it is unclear how much this is due to primary (due to the disease action on the body) or secondary effects (impacts on the whole person of being ill with CIDP).Numerous reports have outlined a range of clinical patterns that are thought to be chronic inflammatory demyelinating polyneuropathy variations. Different variations include ataxic, pure motor, and pure sensory patterns; additionally, there are multifocal patterns in which the distributions of specific nerve territories experience weakness and sensory loss.
- Prognosis
- As in multiple sclerosis, another demyelinating condition, it is not possible to predict with certainty how CIDP will affect patients over time. The pattern of relapses and remissions varies greatly with each patient. A period of relapse can be very disturbing, but many patients make significant recoveries.If diagnosed early, initiation of early treatment to prevent loss of nerve axons is recommended. However, many individuals are left with residual numbness, weakness, tremors, fatigue and other symptoms which can lead to long-term morbidity and diminished quality of life.It is important to build a good relationship with doctors, both primary care and specialist. Because of the rarity of the illness, many doctors will not have encountered it before. Each case of CIDP is different, and relapses, if they occur, may bring new symptoms and problems. Because of the variability in severity and progression of the disease, doctors will not be able to give a definite prognosis. A period of experimentation with different treatment regimens is likely to be necessary in order to discover the most appropriate treatment regimen for a given patient.
- Onset
- Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP) typically has a gradual onset. Symptoms often develop over the course of at least 8 weeks, distinguishing it from acute neuropathies like Guillain-Barré Syndrome, which progress more rapidly.
- Prevalence
- Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) has a prevalence of approximately 1 to 8 cases per 100,000 people.
- Epidemiology
- In 1982 Lewis et al. reported a group of patients with a chronic asymmetrical sensorimotor neuropathy mostly affecting the arms with multifocal involvement of peripheral nerves. Also in 1982 Dyck et al reported a response to prednisolone to a condition they referred to as chronic inflammatory demyelinating polyradiculoneuropathy. Parry and Clarke in 1988 described a neuropathy which was later found to be associated with IgM autoantibodies directed against GM1 gangliosides. This latter condition was later termed multifocal motor neuropathy This distinction is important because multifocal motor neuropathy responds to intravenous immunoglobulin alone, while chronic inflammatory demyelinating polyneuropathy responds to intravenous immunoglobulin, steroids and plasma exchange. It has been suggested that multifocal motor neuropathy is distinct from chronic inflammatory demyelinating polyneuropathy and that Lewis-Sumner syndrome is a distinct variant type of chronic inflammatory demyelinating polyneuropathy.The Lewis-Sumner form of this condition is considered a rare disease with only 50 cases reported up to 2004. A total of 90 cases had been reported by 2009.
- Intractability
- Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP) can be challenging to manage, but it is not considered universally intractable. With proper treatment, many patients can achieve significant improvement or remission. Treatment options include corticosteroids, intravenous immunoglobulins (IVIG), plasmapheresis, and immunosuppressive drugs. However, the response to treatment can vary, and some cases may be more resistant, requiring long-term management.
- Disease Severity
- Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP) can vary in severity. Some individuals may experience mild symptoms such as numbness or weakness, while others may suffer from significant motor dysfunction, sensory loss, and disability. The condition can be progressive, relapsing, or monophasic, influencing the overall severity and impact on daily life.
- Healthcare Professionals
- Disease Ontology ID - DOID:5213
- Pathophysiology
- Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP) is an autoimmune disorder where the body's immune system mistakenly attacks the myelin sheath—the protective covering of the peripheral nerves. This leads to inflammation and subsequent demyelination, primarily affecting the nerve roots and proximal nerve segments. The demyelination disrupts normal nerve signal transmission, leading to symptoms such as muscle weakness, sensory disturbances, and impaired motor function. In some cases, axonal damage can also occur, potentially leading to long-term disability.
- Carrier Status
- Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP) is not typically associated with a genetic carrier status. It is an acquired autoimmune disorder rather than a hereditary condition. The immune system mistakenly attacks the myelin sheath of peripheral nerves, leading to weakness, sensory loss, and impaired motor function. Since it is not inherited, there is no carrier status relevant to CIDP.
- Mechanism
- In the local tissue compartment of peripheral nerves, the immune system is carefully regulated by a normal, balanced collection of immunocompetent cells as well as soluble factors, maintaining the integrity of the system. Maintaining self-tolerance requires defense against immune reactions to autoantigens. Chronic inflammatory demyelinating polyneuropathy disrupts self-tolerance and activates autoreactive T and B cells, which are normally suppressed immune cells. This leads to the organ-specific damage typical of autoimmune disease. Molecular mimicry may be particularly relevant to the tolerance breakdown linked to autoimmune neuropathies. The process known as "molecular mimicry" occurs when an infectious organism that shares epitopes from its host's afflicted tissue triggers an immune response in the host. However, only a small number of convincingly identified specific targets for such a response have been found in chronic inflammatory demyelinating polyneuropathy.Individuals with chronic inflammatory demyelinating polyneuropathy have evidence of activation of T cells in the systemic immune compartment; however, antigen specificity is still largely unknown.It was proposed more than 20 years ago that autoantibodies play a role in the development of chronic inflammatory demyelinating polyneuropathy. This was supported by the detection of oligoclonal IgG bands in the cerebrospinal fluid and immunoglobulin as well as complement deposition on myelinated nerve fibers.Target antigens may also include gangliosides and related glycolipids. There is serologic evidence of recent Campylobacter jejuni infection in a small number of individuals with chronic inflammatory demyelinating polyneuropathy. Because carbohydrate epitopes are expressed in both microbial lipopolysaccharides and nerve glycolipids, this discovery may, in rare cases, point to molecular mimicry as the root cause of chronic inflammatory demyelinating polyneuropathy.Apart from myelin-directed antibodies, other serum components that can cause demyelination as well as conduction block include complement, cytokines, and other inflammatory mediators. Individuals with chronic inflammatory demyelinating polyneuropathy have a low frequency of specific antibodies, which suggests that different antibodies and different mechanisms are involved in each patient.
- Treatment
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First-line treatment for CIDP is currently intravenous immunoglobulin and other treatments include corticosteroids (e.g., prednisone), and plasmapheresis (plasma exchange) which may be prescribed alone or in combination with an immunosuppressant drug. Recent controlled studies show subcutaneous immunoglobulin appears to be as effective for CIDP treatment as intravenous immunoglobulin in most patients, and with fewer systemic side effects.Intravenous immunoglobulin and plasmapheresis have proven benefit in randomized, double-blind, placebo-controlled trials. Despite less definitive published evidence of efficacy, corticosteroids are considered standard therapies because of their long history of use and cost effectiveness. Intravenous immunoglobulin is probably the first-line CIDP treatment, but is extremely expensive. For example, in the U.S., a single 65 g dose of Gamunex brand in 2010 might be billed at the rate of $8,000 just for the immunoglobulin—not including other charges such as nurse administration.Immunosuppressive drugs are often of the cytotoxic (chemotherapy) class, including rituximab (Rituxan) which targets B cells, and cyclophosphamide, a drug which reduces the function of the immune system. Ciclosporin has also been used in CIDP but with less frequency as it is a newer approach. Ciclosporin is thought to bind to immunocompetent lymphocytes, especially T-lymphocytes.Non-cytotoxic immunosuppressive treatments usually include the anti-rejection transplant drugs azathioprine (Imuran/Azoran) and mycophenolate mofetil (Cellcept). In the U.S., these drugs are used "off-label", meaning that they do not have an indication for the treatment of CIDP in their package inserts. Before azathioprine is used, the patient should first have a blood test that ensures that azathioprine can safely be used.Anti-thymocyte globulin, an immunosuppressive agent that selectively destroys T lymphocytes is being studied for use in CIDP. Anti-thymocyte globulin is the gamma globulin fraction of antiserum from animals that have been immunized against human thymocytes. It is a polyclonal antibody. Although chemotherapeutic and immunosuppressive agents have shown to be effective in treating CIDP, significant evidence is lacking, mostly due to the heterogeneous nature of the disease in the patient population in addition to the lack of controlled trials.A review of several treatments found that azathioprine, interferon alpha and methotrexate were not effective. Cyclophosphamide and rituximab seem to have some response. Mycophenolate mofetil may be of use in milder cases. Immunoglobulin and steroids are the first line choices for treatment.In severe cases of CIDP, when second-line immunomodulatory drugs are not efficient, autologous hematopoietic stem cell transplantation (HSCT) is sometimes performed. The treatment may induce long-term remission even in severe treatment-refractory cases of CIDP. To improve outcome, it has been suggested that it should be initiated before irreversible axonal damage has occurred. However, a precise estimation of its clinical efficacy for CIDP is not available, as randomized controlled trials (RCT) have not been performed. (In MS, the ASTIMS RCT provides evidence for superior effect of HSCT to the then-best practice for treatment of aggressive MS. The more recent MIST RCT confirmed its superiority in MS.)
Physical therapy and occupational therapy may improve muscle strength, activities of daily living, mobility, and minimize the shrinkage of muscles and tendons and distortions of the joints. - Compassionate Use Treatment
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Compassionate use treatment and off-label or experimental treatments for Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP) may include the following:
1. **Compassionate Use Treatments**:
- **Plasma Exchange (Plasmapheresis)**: Sometimes used when standard treatments are ineffective.
- **Intravenous Immunoglobulin (IVIg)**: Though a standard treatment, it can be used compassionately if other treatments fail.
2. **Off-label or Experimental Treatments**:
- **Rituximab**: Normally used to treat certain cancers and autoimmune diseases, it has shown promise in some CIDP cases.
- **Eculizumab**: A monoclonal antibody that has been used in limited cases.
- **Alemtuzumab**: Another monoclonal antibody, typically used for leukemia, explored in some CIDP patients.
- **Stem Cell Transplantation**: Experimental approach involving autologous stem cell transplants.
It is crucial to consult healthcare professionals for case-specific recommendations and to discuss potential risks and benefits. - Lifestyle Recommendations
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For chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), appropriate lifestyle recommendations are important alongside medical treatments to help manage symptoms and improve quality of life:
1. **Physical Therapy:** Engage in regular physical therapy to maintain muscle strength and flexibility. This can help prevent muscle atrophy and improve mobility.
2. **Balanced Diet:** Maintain a balanced diet rich in fruits, vegetables, whole grains, and lean proteins to support overall health and immune function.
3. **Exercise:** Incorporate low-impact exercises such as swimming, walking, or cycling to enhance cardiovascular health without putting excessive strain on the body.
4. **Rest and Sleep:** Ensure adequate rest and quality sleep to help the body recover and manage fatigue, which is a common symptom of CIDP.
5. **Stress Management:** Practice stress-reducing techniques such as meditation, yoga, or deep breathing exercises to help manage stress levels, which can sometimes exacerbate symptoms.
6. **Assistive Devices:** Use canes, walkers, or other assistive devices if needed to enhance mobility and safety.
7. **Avoid Triggers:** Identify and avoid potential triggers that may worsen symptoms, which can include certain infections or overexertion.
8. **Regular Medical Follow-Up:** Maintain regular appointments with healthcare providers to monitor the disease progression and adjust treatments as necessary.
Implementing these lifestyle changes can help manage CIDP more effectively in conjunction with medical therapies. - Medication
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Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP) is often treated using several types of medication. The mainstays of treatment include:
1. **Corticosteroids**: Prednisone is commonly used to reduce inflammation and immune response.
2. **Immunosuppressive drugs**: Medications like azathioprine, mycophenolate mofetil, and cyclophosphamide may be prescribed to suppress the immune system.
3. **Intravenous Immunoglobulin (IVIg)**: This treatment provides antibodies to help modulate the immune system.
4. **Plasma exchange (plasmapheresis)**: Not a medication, but a procedure used to remove and replace the blood plasma.
The choice of treatment depends on the patient's specific condition and response to therapy. Always consult a healthcare professional for personalized medical advice. - Repurposable Drugs
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For chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), several drugs initially developed for other conditions have been found potentially beneficial. These include:
1. **Intravenous Immunoglobulin (IVIg)** - Originally used for immunodeficiency disorders.
2. **Corticosteroids** - Such as prednisone, commonly used for various inflammatory and autoimmune conditions.
3. **Plasma Exchange (Plasmapheresis)** - Used in other autoimmune disorders.
4. **Immunosuppressive Drugs** - Such as azathioprine or mycophenolate mofetil, initially used for organ transplants to prevent rejection. - Metabolites
- For Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP), no specific unique metabolites have been identified as definitive biomarkers for the disease. Diagnosis typically relies on clinical presentation, electrophysiological testing, and sometimes cerebrospinal fluid analysis showing elevated protein levels. Research is ongoing to better understand metabolomic profiles in CIDP patients.
- Nutraceuticals
- Nutraceuticals have been explored as adjunctive treatments for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) to support overall health and potentially modulate the immune response. However, the primary treatment for CIDP typically involves immunotherapy such as corticosteroids, intravenous immunoglobulin (IVIG), or plasmapheresis. Nutraceuticals like omega-3 fatty acids, curcumin, and vitamin D might offer some benefits due to their anti-inflammatory properties, but their role in CIDP management is not well-established and should complement, not replace, conventional treatments. Always consult a healthcare provider for personalized medical advice.
- Peptides
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For Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP), peptides and nanotechnology are both areas of investigation in potential treatments and diagnostic tools:
1. **Peptides:** There are ongoing studies into the role peptides could play in modulating the immune system or promoting nerve repair in CIDP. Specific therapeutic peptides could help alter the immune response or help in remyelination.
2. **Nanotechnology:** Advances in nanotechnology hold promise for CIDP in terms of targeted drug delivery systems and improved diagnostic techniques. Nanoparticles could potentially deliver drugs directly to affected nerves, reducing side effects and increasing effectiveness. Nano-based imaging could also assist in earlier and more precise diagnosis.
Both these areas are still largely experimental but represent future directions in the management of CIDP.