Chronic inflammatory demyelinating

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

Chronic inflammatory demyelinating polyneuropathy (CIDP) is an acquired immune-

mediated inflammatory disorder of the peripheral nervous system. The disorder is sometimes
called chronic relapsing polyneuropathy (CRP) or chronic inflammatory demyelinating
polyradiculoneuropathy (because it involves the nerve roots).[1] CIDP is closely related to
Guillain-Barr syndrome and it is considered the chronic counterpart of that acute disease. Its
symptoms are also similar to progressive inflammatory neuropathy. An asymmetrical variant
of CIDP is known as Lewis-Sumner syndrome.[2]

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

Chronic inflammatory demyelinating polyneuropathy[3] is believed to be due to immune cells,

cells which normally protect the body from foreign infection, but here begin incorrectly
attacking the nerves in the body instead. 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.[4]

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]

In some cases electrophysiological studies fail to show any evidence of demyelination.

Though conventional electrophysiological diagnostic criteria are not met, the patient may still
respond to immunomodulatory treatments. In such cases, presence of clinical characteristics
suggestive of CIDP are critical, justifying full investigations, including sural nerve biopsy.[6]

Diagnosis
 This section does not cite any references or sources. Please help improve this
section by adding citations to reliable sources. Unsourced material may be
challenged and removed. (August 2011)

Diagnosis is usually made through a clinical neurological examination. 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 be intermittent.

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.

Electrodiagnostics - electromyography (EMG) and nerve conduction study (NCS).

In usual CIDP, the nerve conduction studies show demyelination. These findings include:

1. a reduction in nerve conduction velocities;

2. the presence of conduction block or abnormal temporal dispersion in at least one
motor nerve;
3. prolonged distal latencies in at least two nerves;
4. absent F waves or prolonged minimum F wave latencies in at least two motor nerves.
(In some case EMG/NCV can be normal).

Serum test to exclude other autoimmune diseases.

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.

Sural nerve biopsy

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]

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.
IVIG is probably the first-line CIDP treatment, but is extremely expensive (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). Gamunex brand
IVIG is the only U.S. FDA approved treatment for CIDP, as in 2008 Talecris, the maker of
Gamunex, received orphan drug status for this drug for the treatment of CIDP.

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. Cyclosporin has also been used in CIDP but with less
frequency as it is a newer approach.[8] Cyclosporin 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 as "off-label" treatments for CIDP, meaning that their use here is accepted by
the FDA, but that CIDP treatment is not explicitly indicated or approved in the drug
literature. Before azathioprine is used, the patient should first have a blood test that ensures
that azathioprine can safely be used.

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.

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.

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.[1]
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
regimes is likely to be necessary in order to discover the most appropriate treatment regimen
for a given patient.

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

Chronic Inflammatory Demyelinating Polyneuropathy CIDP Disease Information

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CIDP is an autoimmune disorder whereby

autoantibodies attack and destroy the
myelin sheath of the peripheral nerves1
Chronic inflammatory demyelinating polyneuropathy (or polyradiculoneuropathy),
abbreviated as CIDP, is the most commonly acquired demyelinating neuropathy. Since the
etiology remains unknown, CIDP remains a syndrome with several variants, primarily
defined by differences in clinical presentation. The classification is still debated, but virtually
all neurologists define the disorder as being chronic (>8 weeks progression), demyelinating,
and inflammatory or immune-mediated.

CIDP is considered to be an autoimmune disorder whereby autoantibodies attack and destroy

the myelin sheath of the peripheral nerves.1 It can commonly present either as a continuously
progressive affliction or, less often, as a recurrent disorder in which episodes (relapsing and
remittent) in individual patients may be separated by months or years.

Incidence and prevalence of CIDP

Chronic inflammatory demyelinating polyneuropathy (CIDP) is the most commonly acquired
demyelinating neuropathy. The exact prevalence of CIDP is unclear but, in studies performed
worldwide, it has been estimated to range from 1.9 per 100,000 to 7.7 per 100,000.2,3

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

Symmetrical proximal and distal motor and sensory loss

Loss of deep tendon reflexes in affected extremities
Clinical course progressing for more than 2 months
Fatigue and pain in extremities

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