Chronic inflammatory demyelinating polyneuropathy

Revision as of 14:51, 6 June 2016 by Luke Rusowicz-Orazem (talk | contribs)
(diff) ← Older revision | Latest revision (diff) | Newer revision → (diff)
Jump to navigation Jump to search

WikiDoc Resources for Chronic inflammatory demyelinating polyneuropathy

Articles

Most recent articles on Chronic inflammatory demyelinating polyneuropathy

Most cited articles on Chronic inflammatory demyelinating polyneuropathy

Review articles on Chronic inflammatory demyelinating polyneuropathy

Articles on Chronic inflammatory demyelinating polyneuropathy in N Eng J Med, Lancet, BMJ

Media

Powerpoint slides on Chronic inflammatory demyelinating polyneuropathy

Images of Chronic inflammatory demyelinating polyneuropathy

Photos of Chronic inflammatory demyelinating polyneuropathy

Podcasts & MP3s on Chronic inflammatory demyelinating polyneuropathy

Videos on Chronic inflammatory demyelinating polyneuropathy

Evidence Based Medicine

Cochrane Collaboration on Chronic inflammatory demyelinating polyneuropathy

Bandolier on Chronic inflammatory demyelinating polyneuropathy

TRIP on Chronic inflammatory demyelinating polyneuropathy

Clinical Trials

Ongoing Trials on Chronic inflammatory demyelinating polyneuropathy at Clinical Trials.gov

Trial results on Chronic inflammatory demyelinating polyneuropathy

Clinical Trials on Chronic inflammatory demyelinating polyneuropathy at Google

Guidelines / Policies / Govt

US National Guidelines Clearinghouse on Chronic inflammatory demyelinating polyneuropathy

NICE Guidance on Chronic inflammatory demyelinating polyneuropathy

NHS PRODIGY Guidance

FDA on Chronic inflammatory demyelinating polyneuropathy

CDC on Chronic inflammatory demyelinating polyneuropathy

Books

Books on Chronic inflammatory demyelinating polyneuropathy

News

Chronic inflammatory demyelinating polyneuropathy in the news

Be alerted to news on Chronic inflammatory demyelinating polyneuropathy

News trends on Chronic inflammatory demyelinating polyneuropathy

Commentary

Blogs on Chronic inflammatory demyelinating polyneuropathy

Definitions

Definitions of Chronic inflammatory demyelinating polyneuropathy

Patient Resources / Community

Patient resources on Chronic inflammatory demyelinating polyneuropathy

Discussion groups on Chronic inflammatory demyelinating polyneuropathy

Patient Handouts on Chronic inflammatory demyelinating polyneuropathy

Directions to Hospitals Treating Chronic inflammatory demyelinating polyneuropathy

Risk calculators and risk factors for Chronic inflammatory demyelinating polyneuropathy

Healthcare Provider Resources

Symptoms of Chronic inflammatory demyelinating polyneuropathy

Causes & Risk Factors for Chronic inflammatory demyelinating polyneuropathy

Diagnostic studies for Chronic inflammatory demyelinating polyneuropathy

Treatment of Chronic inflammatory demyelinating polyneuropathy

Continuing Medical Education (CME)

CME Programs on Chronic inflammatory demyelinating polyneuropathy

International

Chronic inflammatory demyelinating polyneuropathy en Espanol

Chronic inflammatory demyelinating polyneuropathy en Francais

Business

Chronic inflammatory demyelinating polyneuropathy in the Marketplace

Patents on Chronic inflammatory demyelinating polyneuropathy

Experimental / Informatics

List of terms related to Chronic inflammatory demyelinating polyneuropathy

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [6]

Synonyms and keywords:

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.

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

Patients usually present with a history of weakness, numbness, tingling, pain and difficulty in walking. They may additionally present with fainting spells while standing up or burning pain in extremities. Some patients may have sudden onset of back pain or neck pain radiating down the extremities, usually diagnosed as radicular pain. These symptoms are usually progressive and may come and go.

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.

In usual CIDP, the nerve conduction studies show demyelination . These findings include: (a) a reduction in nerve conduction velocities b; (b) the presence of conduction block or abnormal temporal dispersion in at least one motor nerve; (c) prolonged distal latencies in at least two nerves; (d) absent F waves or prolonged minimum F wave latencies in at least two motor nerves. (In some case EMG/NCV can be normal).

Biopsy is considered for those patients in whom the diagnosis is not completely clear, when other causes of neuropathy (eg, hereditary, vasculitic) cannot be excluded, or when profound axonal involvement is observed on EMG.

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.

External Links

See Also