Information about Chronic Inflammatory Demyelinating Polyneuropathy
Chronic inflammatory demyelinating polyneuropathy (CIDP) is an acquired immune-mediated inflammatory disorder of the peripheral nervous system but often can have central nervous system involvement. The disorder is sometimes called chronic relapsing polyneuropathy. CIDP is closely related to Guillain-Barre syndrome and it is considered the chronic counterpart of that acute disease.
Chronic inflammatory demyelinating polyneuropathy is disease believed to be due to immune cells, cells which normally protect the body, but are now attacking the nerves in the body. As a result, the affected nerves fail to respond, or respond only weakly, to stimuli causing numbing, tingling, pain, progressive muscle weakness, loss of deep tendon reflexes (areflexia), fatigue, and abnormal sensations.. The likelihood of progression of the disease is high.
CIDP is under-recognized and under-treated due to its heterogeneous presentation (both clinical and electrophysiological) and the limitations of clinical, serologic, and electrophysiologic diagnostic criteria. Despite these limitations, early diagnosis and treatment is important in preventing irreversible axonal loss and improving functional recovery.[1]
Under-recognized and under-treated CIDP is also due to limitations of clinical trials. Although there are stringent research criteria for selecting patients to clinical trials, there are no generally agreed-on clinical diagnostic criteria for CIDP due to its different presentations in symptoms and objective data. Application of the present research criteria to routine clinical practice often miss the diagnosis in a majority of patients and patients are often left untreated despite progression of their disease.[2]
In some cases electrophysiological studies fail to show any evidence of demyelination, though conventional electrophysiological diagnostic criteria are not filled the patient may respond to immunomodulatory treatments. In such cases, presence of clinical characteristics suggestive of CIDP are critical justifying fully investigations including sural nerve biopsy.[3]
On examination the patients may have weakness, and loss of deep tendon reflexes (rarely increased or normal). There may be atrophy (shrinkage) of muscles, fasciculations (twitching) and loss of sensation. Patients may have Multi-Focal Motor neuropathy, as they have no sensory loss.
The patient may present with a single cranial nerve or peripheral nerve dysfunction.
Autonomic system dysfunction can occur; in such a case, the patient would complain of orthostatic dizziness, problems with bowel and bladder functions, and cardiac problems.
Most experts consider the necessary duration of symptoms to be greater than 8 weeks for the diagnosis of CIDP to be made.
IVIG 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.
Immunosuppressive drugs are often of the cytotoxic (chemotherapy) class, including Rituximab (Rixutan) 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.[4] Ciclosporin is thought to bind to immunocompetent lymphocytes, especially T-lymphocytes.
Non-cytotoxic immunosuppressive treatments usually include imuran and cellcept.
Anti-thymocyte globulin (ATG), 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.
Physiotherapy may improve muscle strength, function and mobility, and minimize the shrinkage of muscles and tendons and distortions of the joints.
If diagnosed early the disease is usually a treatable cause of acquired neuropathy and initiation of early treatment to prevent loss of nerve axons is recommended. However, many individuals are left with residual numbness, weakness, fatigue and other symptoms which can lead to long-term morbidity and diminished quality of life. [5]
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, the doctor will not be able to give you a definite prognosis.
Although there is not one single overall treatment for CIDP, there is much that your doctor can do to help. Each person responds in different ways to different treatments. A period of experimentation with different treatment regimes is likely to be necessary in order to discover the regime which is most appropriate for you.
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Overview
The pathologic hallmark of the disease is loss of the myelin sheath (the fatty covering that wraps around and protects nerve fibers) of the peripheral nerves.Chronic inflammatory demyelinating polyneuropathy is disease believed to be due to immune cells, cells which normally protect the body, but are now attacking the nerves in the body. As a result, the affected nerves fail to respond, or respond only weakly, to stimuli causing numbing, tingling, pain, progressive muscle weakness, loss of deep tendon reflexes (areflexia), fatigue, and abnormal sensations.. The likelihood of progression of the disease is high.
CIDP is under-recognized and under-treated due to its heterogeneous presentation (both clinical and electrophysiological) and the limitations of clinical, serologic, and electrophysiologic diagnostic criteria. Despite these limitations, early diagnosis and treatment is important in preventing irreversible axonal loss and improving functional recovery.[1]
Under-recognized and under-treated CIDP is also due to limitations of clinical trials. Although there are stringent research criteria for selecting patients to clinical trials, there are no generally agreed-on clinical diagnostic criteria for CIDP due to its different presentations in symptoms and objective data. Application of the present research criteria to routine clinical practice often miss the diagnosis in a majority of patients and patients are often left untreated despite progression of their disease.[2]
In some cases electrophysiological studies fail to show any evidence of demyelination, though conventional electrophysiological diagnostic criteria are not filled the patient may respond to immunomodulatory treatments. In such cases, presence of clinical characteristics suggestive of CIDP are critical justifying fully investigations including sural nerve biopsy.[3]
Diagnosis
- Clinical neurological examination
On examination the patients may have weakness, and loss of deep tendon reflexes (rarely increased or normal). There may be atrophy (shrinkage) of muscles, fasciculations (twitching) and loss of sensation. Patients may have Multi-Focal Motor neuropathy, as they have no sensory loss.
The patient may present with a single cranial nerve or peripheral nerve dysfunction.
Autonomic system dysfunction can occur; in such a case, the patient would complain of orthostatic dizziness, problems with bowel and bladder functions, and cardiac problems.
Most experts consider the necessary duration of symptoms to be greater than 8 weeks for the diagnosis of CIDP to be made.
- Electrodiagnostics - electromyography (EMG) and nerve conduction study (NCS).
Treatment
First line treatment for CIDP includes corticosteroids such as prednisone, plasmapheresis (plasma exchange) and intravenous immunoglobulin (IVIg) which may be prescribed alone or in combination with an immunosuppressant drug.IVIG 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.
Immunosuppressive drugs are often of the cytotoxic (chemotherapy) class, including Rituximab (Rixutan) 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.[4] Ciclosporin is thought to bind to immunocompetent lymphocytes, especially T-lymphocytes.
Non-cytotoxic immunosuppressive treatments usually include imuran and cellcept.
Anti-thymocyte globulin (ATG), 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.
Physiotherapy may improve muscle strength, function and mobility, and minimize the shrinkage of muscles and tendons and distortions of the joints.
Prognosis
It is not possible to predict with certainty how CIDP is going to affect an individual in the future just like as in Multiple Sclerosis or any other autoimmune disease. The pattern of relapses and remissions varies greatly from person to person. A period of relapse can be very disturbing but many patients make a good recovery. The course of CIDP varies widely among individuals. Some may have a bout of CIDP followed by spontaneous recovery, while others may have many bouts with partial recovery in between relapses.If diagnosed early the disease is usually a treatable cause of acquired neuropathy and initiation of early treatment to prevent loss of nerve axons is recommended. However, many individuals are left with residual numbness, weakness, fatigue and other symptoms which can lead to long-term morbidity and diminished quality of life. [5]
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, the doctor will not be able to give you a definite prognosis.
Although there is not one single overall treatment for CIDP, there is much that your doctor can do to help. Each person responds in different ways to different treatments. A period of experimentation with different treatment regimes is likely to be necessary in order to discover the regime which is most appropriate for you.
References
1. ^ [1]Toothaker TB, Brannagan TH 3rd. "Chronic inflammatory demyelinating polyneuropathies: current treatment strategies." Curr Neurol Neurosci Rep. 2007 Jan;7(1):63-70
2. ^ [2]Latov N. "Diagnosis of CIDP." Neurology. 2002 Dec 24;59(12 Suppl 6):S2-6.
3. ^ [3]Azulay JP. "The diagnosis of chronic axonal polyneuropathy: the poorly understood chronic polyradiculoneuritides". Rev Neurol (Paris). 2006 Dec;162(12):1292-5.
4. ^ [4]Odaka M, Tatsumoto M, Susuki K, Hirata K, Yuki N. "Intractable chronic inflammatory demyelinating polyneuropathy treated successfully with ciclosporin"J Neurol Neurosurg Psychiatry. 2005 Aug;76(8):1115-20.
5. ^ [5]Kissel JT. "The treatment of chronic inflammatory demyelinating polyradiculoneuropathy. Semin Neurol." 2003 Jun;23(2):169-80.
2. ^ [2]Latov N. "Diagnosis of CIDP." Neurology. 2002 Dec 24;59(12 Suppl 6):S2-6.
3. ^ [3]Azulay JP. "The diagnosis of chronic axonal polyneuropathy: the poorly understood chronic polyradiculoneuritides". Rev Neurol (Paris). 2006 Dec;162(12):1292-5.
4. ^ [4]Odaka M, Tatsumoto M, Susuki K, Hirata K, Yuki N. "Intractable chronic inflammatory demyelinating polyneuropathy treated successfully with ciclosporin"J Neurol Neurosurg Psychiatry. 2005 Aug;76(8):1115-20.
5. ^ [5]Kissel JT. "The treatment of chronic inflammatory demyelinating polyradiculoneuropathy. Semin Neurol." 2003 Jun;23(2):169-80.
External Links
- National Institute of Health (USA) government fact sheet on CIDP
- CIDP eMedicine.com
- CIDP Discussion Forum
- GBS Organisation of the UK, with online CIDP booklets and information
- The Neuropathy Association
See Also
MeSH D001327 Autoimmunity is the failure of an organism to recognize its own constituent parts (down to the sub-molecular levels) as "self", which results in an immune response against its own cells and tissues.
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The Peripheral nervous system resides or extends outside the "CNS" central nervous system (the brain and spinal cord) to serve the limbs and organs. Unlike the central nervous system, however, the PNS is not protected by bone, leaving it exposed to toxins and mechanical injuries.
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The central nervous system (CNS) represents the largest part of the nervous system, including the brain and the spinal cord. Together with the peripheral nervous system, it has a fundamental role in the control of behavior.
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Guillain-Barré syndrome
Classification & external resources
ICD-10
ICD-9
DiseasesDB 5465
eMedicine emerg/222
MeSH D020275
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Classification & external resources
ICD-10
ICD-9
DiseasesDB 5465
eMedicine emerg/222
MeSH D020275
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Myelin is an electrically insulating phospholipid layer that surrounds the axons of many neurons. It is an outgrowth of glial cells: Schwann cells supply the myelin for peripheral neurons while oligodendrocytes supply it to those of the central nervous system.
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A nerve is an enclosed, cable-like bundle of axons (the long, slender projection of a neuron). Neurons are sometimes called nerve cells, though this term is technically imprecise since many neurons do not form nerves, and nerves also include the glial cells that
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Heterogeneous (American English)) means that something (an object or system) consists of a diverse range of different items. It is the antonym of , which means that an object or system consists of many identical items.
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Electrophysiology is the study of the electrical properties of biological cells and tissues. It involves measurements of voltage change or electrical current flow on a wide variety of scales from single ion channel proteins to whole tissues like the heart.
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The neurological examination is the physical examination of the nervous system. It attempts to identify or exclude signs of nervous system disease, and - if these signs are present - to produce a likely anatomical or physiological explanation that can be tested through medical
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Radiculitis
Classification & external resources
ICD-10 M 54.1
ICD-9 729.2
DiseasesDB 29521
MeSH D011843 Radicular Pain, or Radiculitis
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Classification & external resources
ICD-10 M 54.1
ICD-9 729.2
DiseasesDB 29521
MeSH D011843 Radicular Pain, or Radiculitis
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For the American thrash metal band, see Atrophy (band)
Atrophy is the partial or complete wasting away of a part of the body. Causes of atrophy include poor nourishment, poor circulation, loss of hormonal support, loss of nerve supply to the target organ,..... Click the link for more information.
MeSH D005207 A fasciculation (or "muscle twitch") is a small, local, involuntary muscle contraction (twitching) visible under the skin arising from the spontaneous discharge of a bundle of skeletal muscle fibers.
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Cranial nerves are nerves that emerge directly from the brain in contrast to spinal nerves which emerge from segments of the spinal cord. Although thirteen cranial nerves in humans fit this description, twelve are conventionally recognized.
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'''Autonomic can refer to several things, including:
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- Autonomic Nervous System
- Autonomic Computing
- Autonomic Networking
- Autonomic Systems
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Standing is a human position in which the body constantly is in an orthostatic state.
Although quiet standing appears to be static, modern instrumentation shows it to be a process of rocking from the ankle in the sagittal plane.
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Although quiet standing appears to be static, modern instrumentation shows it to be a process of rocking from the ankle in the sagittal plane.
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heart is a muscular organ responsible for pumping blood through the blood vessels by repeated, rhythmic contractions, or a similar structure in the annelids, mollusks, and arthropods.
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Electromyography (EMG) is a technique for evaluating and recording physiologic properties of muscles at rest and while contracting. EMG is performed using an instrument called an electromyograph, to produce a record called an electromyogram.
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A nerve conduction study (NCS) is a test commonly used to evaluate the function, especially the ability of electrical conduction, of the motor and sensory nerves of the human body. Nerve conduction velocity (NCV) is a common measurement made during this test.
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Myelin is an electrically insulating phospholipid layer that surrounds the axons of many neurons. It is an outgrowth of glial cells: Schwann cells supply the myelin for peripheral neurons while oligodendrocytes supply it to those of the central nervous system.
..... Click the link for more information.
..... Click the link for more information.
The sural nerve (short saphenous nerve), formed by the junction of the medial sural cutaneous with the peroneal anastomotic branch, passes downward near the lateral margin of the tendo calcaneus, lying close to the small saphenous vein, to the interval between the lateral
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A biopsy (in Greek: bios = life and opsy = look/appearance) is a medical test involving the removal of cells or tissues for examination. The tissue is generally examined under a microscope by a pathologist, and can also be analyzed chemically (for example, using
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axon or nerve fiber, is a long, slender projection of a nerve cell, or neuron, that conducts electrical impulses away from the neuron's cell body or soma.
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Anatomy
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Corticosteroids are a class of steroid hormones that are produced in the adrenal cortex. Corticosteroids are involved in a wide range of physiologic systems such as stress response, immune response and regulation of inflammation, carbohydrate metabolism, protein catabolism, blood
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Prednisone is a synthetic corticosteroid drug which is usually taken orally but can be delivered by intramuscular injection and can be used for a great number of different conditions. It has a mainly glucocorticoid effect.
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Plasmapheresis (from the Greek plasma, something molded, and apheresis, taking away) is the removal, treatment, and return of (components of) blood plasma from blood circulation. It is thus an extracorporeal therapy.
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Antibodies (also known as immunoglobulins) are proteins that are found in blood or other bodily fluids of vertebrates, and are used by the immune system to identify and neutralize foreign objects, such as bacteria and viruses.
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Immunosuppressive drugs or immunosuppressants are drugs that are used in immunosuppressive therapy to inhibit or prevent activity of the immune system. Clinically they are used to:
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- prevent the rejection of transplanted organs and tissues (e.g.
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Cytotoxicity is the quality of being toxic to cells. Examples of toxic agents are a chemical substance or an immune cell.
Cytotoxicity can be measured by the MTT assay, Trypan blue (TB) assay, Sulforhodamine B (SRB) assay, WST assay and clonogenic assay.
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Cytotoxicity can be measured by the MTT assay, Trypan blue (TB) assay, Sulforhodamine B (SRB) assay, WST assay and clonogenic assay.
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Rituximab, sold under the trade names Rituxan® and MabThera®, is a chimeric monoclonal antibody used in the treatment of B cell non-Hodgkin's lymphoma, B cell leukemia, and some autoimmune disorders.
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B cells are lymphocytes that play a large role in the humoral immune response as opposed to the cell-mediated immune response that is governed by T cells. The principal function of B cells is to make antibodies against soluble antigens.
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