Encyclopedia > 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. Autoimmune diseases arise from an overactive immune response of the body against substances and tissues normally present in the body. ...
The peripheral nervous system or PNS, is part of the nervous system, and consists of the nerves and neurons that reside or extend outside the central nervous system (the brain and spinal cord) to serve the limbs and organs, for example. ...
A diagram showing the CNS: 1. ...
Guillain-Barré syndrome (GBS), is an acquired immune-mediated inflammatory disorder of the peripheral nervous system (i. ...
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. In neuroscience, myelin is an electrically insulating phospholipid layer that surrounds the axons of many neurons. ...
Nerves (yellow) Nerves redirects here. ...
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.
Diagnosis CIDP is under-recognized and under-treated due to its heterogeneous presentation (both clincial 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] Look up Heterogeneous in Wiktionary, the free dictionary. ...
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] Electrophysiology is the study of the electrical properties of biological cells and tissues. ...
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 malleolus and the calcaneus. ...
A biopsy (in Greek: bios = life and opsy = look/appearance) is a medical test involving the removal of cells or tissues for examination. ...
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. In physiology, corticosteroids are a class of steroid hormones that are produced in the adrenal cortex. ...
Prednisone is a synthetic corticosteroid drug which is usually taken orally and can be used for a large number of different conditions. ...
Plasmapheresis is the removal of (components of) blood plasma from the circulation. ...
Schematic of antibody binding to an antigen An antibody is a protein complex used by the immune system to identify and neutralize foreign objects like bacteria and viruses. ...
For a list of immunosuppressive drugs, see the transplant rejection page. ...
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 (chemotheraputic) class, including Rituximab (Rixutan) which targets B Cells, cyclophosphamide, a drug which reduces the function of the immune system, and ATG, a protein that kills the immune cells that are thought to be causing your disease. 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. Cytotoxicity is the quality of being poisonous to cells. ...
Rituximab, sold under the trade names Rituxan® and MabThera®, is a monoclonal antibody used in the treatment of B cell non-Hodgkins lymphoma, B cell leukemia, and some autoimmune disorders. ...
B cells are lymphocytes that play a large role in the humoral immune response (as opposed to the cell-mediated immune response). ...
Cyclophosphamide (the generic name for Cytoxan, Neosar) is a nitrogen mustard alkylating agent, used to treat various types of cancer and some autoimmune disorders. ...
ATG may be: In genetics, the triplet for the start codon methionine. ...
Ciclosporin (INN), cyclosporine or cyclosporin (former BAN), is an immunosuppressant drug. ...
A lymphocyte is a type of white blood cell involved in the human bodys immune system. ...
T cells are a subset of lymphocytes that play a large role in the immune response. ...
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. Physical therapy can help restore lost functionality in many people. ...
Prognosis 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. 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] In medicine, epidemiology and actuarial science, the term morbidity can refer to the state of being diseased (from Latin morbidus: sick, unhealthy), the degree or severity of a disease, the prevalence of a disease: the total number of cases in a particular population at a particular point in time, the...
References - ^ [1]Toothaker TB, Brannagan TH 3rd. "Chronic inflammatory demyelinating polyneuropathies: current treatment strategies." Curr Neurol Neurosci Rep. 2007 Jan;7(1):63-70
- ^ [2]Latov N. "Diagnosis of CIDP." Neurology. 2002 Dec 24;59(12 Suppl 6):S2-6.
- ^ [3]Azulay JP. "The diagnosis of chronic axonal polyneuropathy: the poorly understood chronic polyradiculoneuritides". Rev Neurol (Paris). 2006 Dec;162(12):1292-5.
- ^ [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]Kissel JT. "The treatment of chronic inflammatory demyelinating polyradiculoneuropathy. Semin Neurol." 2003 Jun;23(2):169-80.
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