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Chronic Inflammatory Demyelinating Polyneuropathy
Chronic Inflammatory Demyelinating Polyneuropathy
Chronic Inflammatory Demyelinating Polyneuropathy
polyneuropathy
From Wikipedia, the free encyclopedia
Chronic inflammatory demyelinating
polyneuropathy
Classification and external resources
ICD-9 357.81
OMIM 139393
DiseasesDB 30084
eMedicine neuro/467
MeSH D020277
Contents
1 Overview
2 Diagnosis
3 Treatment
4 Prognosis
5 See also
6 References
7 External links
Overview
Structure of a typical neuron
Neuron
Dendrite
Soma
Axon
Nucleus
Node of
Ranvier
Axon terminal
Schwann cell
Myelin sheath
Lack of awareness and treatment of 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.[5]
Diagnosis
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challenged and removed. (August 2011)
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 breathing, eye, bowel, bladder 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:
Spinal tap and serum test for anti-ganglioside antibodies. These antibodies are present
in the branch of CIDP diseases comprised by anti-GM1, anti-GD1a, and anti-GQ1b.
Biopsy is considered for those patients in whom the diagnosis is not completely clear, when
other causes of neuropathy (e.g., hereditary, vasculitic) cannot be excluded, or when
profound axonal involvement is observed on EMG.
Treatment
First-line treatment for CIDP includes corticosteroids (e.g. prednisone), plasmapheresis
(plasma exchange) and intravenous immunoglobulin (IVIG) which may be prescribed alone
or in combination with an immunosuppressant drug.[7]
Physical therapy may improve muscle strength, function and mobility, and minimize the
shrinkage of muscles and tendons and distortions of the joints.
Prognosis
As in multiple sclerosis, another demyelinating condition, it is not possible to predict with
certainty how CIDP is going to affect an individual in the future. 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.
See also
Autoimmune diseases
Neurology
References
1. Kissel JT (2003). "The treatment of chronic inflammatory demyelinating
polyradiculoneuropathy". Semin Neurol 23 (2): 16980. doi:10.1055/s-2003-41130.
PMID 12894382.
2. http://www.cidpusa.org/LEWIS%20SUMMER.dwt
3. http://www.beverlyhillsneurology.com/cidp.html
4. Toothaker TB, Brannagan TH (2007). "Chronic inflammatory demyelinating
polyneuropathies: current treatment strategies". Curr Neurol Neurosci Rep 7 (1): 63
70. doi:10.1007/s11910-007-0023-5. PMID 17217856.
5. Latov N (2002). "Diagnosis of CIDP". Neurology 59 (12 Suppl 6): S26.
doi:10.1212/wnl.59.12_suppl_6.s2. PMID 12499464.
6. Azulay JP (2006). "[The diagnosis of chronic axonal polyneuropathy: the poorly
understood chronic polyradiculoneuritides]". Rev. Neurol. (Paris) (in French) 162
(12): 12925. PMID 17151528.
7. Hughes RA (2002). "Systematic reviews of treatment for inflammatory demyelinating
neuropathy". Journal of Anatomy 200 (4): 3319. doi:10.1046/j.1469-
7580.2002.00041.x. PMC 1570692. PMID 12090400.
8. Odaka M, Tatsumoto M, Susuki K, Hirata K, Yuki N (2005). "Intractable chronic
inflammatory demyelinating polyneuropathy treated successfully with ciclosporin". J.
Neurol. Neurosurg. Psychiatr. 76 (8): 111520. doi:10.1136/jnnp.2003.035428.
PMC 1739743. PMID 16024890.
ww.gamunex-c.com/html/hcp-clinical-data-and-disease-information-CIPD-clinical-data-CIDP-
disease-information.htm
Although the exact cause is not known, CIDP is considered an autoimmune disorder.
Symptoms are caused by damage to the myelin sheath in the peripheral nerves.
Signs and symptoms of CIDP
Symptoms of CIDP vary from mild to debilitating, and patients may have atypical presentations.
Signs of CIDP may include4
Causes of CIDP
The cause of CIDP is not known. As a syndrome, different causes and triggers are likely. However, it
is generally accepted that most, if not all, CIDP manifestations are autoimmune, triggered by
infections or toxins in genetically susceptible individuals. The autoimmune nature of CIDP is
suspected because of its occasional association with other immune-mediated diseases, such as
systemic lupus, hepatitis B and C, HIV, and multiple sclerosis (MS), its response to
immunosuppressive and immunomodulatory treatments, and pathologic findings on nerve biopsy.
Other causes of neuropathy that may be difficult to distinguish from CIDP include:
Inherited neuropathy
Systemic inflammatory-autoimmune disorders
Dysproteinemias
Diabetes mellitus
Vasculitis
Other metabolic and toxic neuropathies