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{{Infobox medical condition (new)
{{Short description|A neurological disorder characterized by progressive weakness and impaired sensory function in the legs and arms}}
| name            = Chronic inflammatory demyelinating polyneuropathy
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'''Chronic inflammatory demyelinating polyneuropathy''' is an acquired [[autoimmune disorder|immune-mediated inflammatory disorder]] of the [[peripheral nervous system]]. The disorder is sometimes called chronic relapsing polyneuropathy (CRP) or chronic inflammatory demyelinating poly'''radiculo'''neuropathy (because it involves the nerve roots).<ref name=radiculo /> CIDP is closely related to [[Guillain–Barré syndrome]] and it is considered the [[Chronic (medical)|chronic]] counterpart of that [[acute (medicine)|acute]] disease.<ref>{{Cite web|url=http://cidpneuropathysupport.com/glossary/gbs-guillain-barre-syndrome/|title=GBS (Guillain-Barré Syndrome) - CIDP Neuropathy|website=cidpneuropathysupport.com|language=en-US|access-date=2017-12-14}}</ref> Its symptoms are also similar to [[progressive inflammatory neuropathy]].


==Types==
'''Chronic inflammatory demyelinating polyneuropathy''' ('''CIDP''') is a neurological disorder characterized by progressive weakness and impaired sensory function in the legs and arms. It is considered an autoimmune disorder, where the body's immune system mistakenly attacks the myelin sheath, the protective covering of the nerves. CIDP is related to [[Guillain-Barré syndrome]], but it progresses more slowly and lasts longer.
Several variants have been reported. Specially important are:


*An asymmetrical variant of CIDP is known as '''Lewis-Sumner Syndrome'''.<ref>{{Cite web | url=http://www.cidpusa.org/LEWIS%20SUMMER.dwt |title = Lewis summer syndrome GUIDE}}</ref>
==Pathophysiology==
*A variant with [[central nervous system|CNS]] involvement named '''combined central and peripheral demyelination''' (CCPD)<ref name="Kira">Jun-ichi Kira, Ryo Yamasaki, Hidenori Ogata, Anti-neurofascin autoantibody and demyelination, Neurochemistry international, Dec. 2018, doi: https://doi.org/10.1016/j.neuint.2018.12.011</ref>
CIDP is caused by inflammation of the peripheral nerves and nerve roots, leading to the destruction of the [[myelin]] sheath. This demyelination disrupts the normal conduction of electrical impulses along the nerves, resulting in muscle weakness, sensory disturbances, and loss of reflexes. The exact cause of the autoimmune response in CIDP is not fully understood, but it is believed to involve both cellular and humoral immune mechanisms.


Currently there is one special variant in which the CNS is also affected. It is termed "combined central and peripheral demyelination" (CCPD) and is special because it belongs at the same time to the CDIP syndrome and to the multiple sclerosis spectrum.<ref name="Kira"/> These cases seem to be related to the presence of [[anti-neurofascin demyelinating diseases|anti-neurofascin autoantibodies]].
==Symptoms==
The symptoms of CIDP can vary widely among individuals, but they typically include:
* Progressive muscle weakness, particularly in the arms and legs
* Tingling or numbness in the extremities
* Loss of deep tendon reflexes
* Fatigue
* Difficulty walking
* Sensory ataxia


==Signs and Symptoms==
The progression of symptoms can be gradual, occurring over several months, and may fluctuate in severity.
Diagnosis is typically made on the basis of presenting symptoms in tandem with [[Electrodiagnostic medicine|electrodiagnostic testing]] or a nerve biopsy. Doctors may use a lumbar puncture to verify the presence of increased cerebrospinal fluid protein. Symptoms such as [[Hyporeflexia|diminished or absent deep-tendon reflexes]] and [[sensory ataxia]] are common. Other symptoms include proximal and distal muscle weakness in the limbs.{{citation needed|date=October 2018}}


== Causes ==
==Diagnosis==
{{Neuron map|Neuron}}
Diagnosing CIDP involves a combination of clinical evaluation, laboratory tests, and electrophysiological studies. Key diagnostic criteria include:
Chronic inflammatory demyelinating polyneuropathy (or polyradiculoneuropathy) is considered an autoimmune disorder destroying myelin, the protective covering of the nerves. Typical early symptoms are "tingling" (sort of electrified vibration or paresthesia) or numbness in the extremities, frequent (night) leg cramps, loss of reflexes (in knees), muscle fasciculations, "vibration" feelings, loss of balance, general muscle cramping and nerve pain.<ref>{{cite web|url=http://cidplog.com/ |title=C.I.D.P. Log|website=cidplog.com|language=en|access-date=2018-09-27}}</ref><ref>{{cite journal |last1=Latov |first1=Norman |title=Diagnosis and treatment of chronic acquired demyelinating polyneuropathies |journal=Nature Reviews Neurology |volume=10 |issue=8 |pages=435–446 |date=2014-07-01 |doi= 10.1038/nrneurol.2014.117 |pmid=24980070 }}</ref>  CIDP is extremely rare but 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 favoured in preventing irreversible axonal loss and improving functional recovery.<ref>{{cite journal |vauthors=Toothaker TB, Brannagan TH |title=Chronic inflammatory demyelinating polyneuropathies: current treatment strategies |journal=Current Neurology and Neuroscience Reports |volume=7 |issue=1 |pages=63–70 |year=2007 |pmid=17217856 |doi= 10.1007/s11910-007-0023-5|url=}}</ref>
* Clinical presentation of progressive weakness and sensory dysfunction
* Electromyography (EMG) and nerve conduction studies showing demyelination
* Elevated protein levels in the cerebrospinal fluid (CSF) without a significant increase in white blood cells


There is a lack of awareness and treatment of CIDP.  Although there are stringent research criteria for selecting patients for clinical trials, there are no generally agreed-upon 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 misses the diagnosis in a majority of patients, and patients are often left untreated despite progression of their disease.<ref>{{cite journal |last1=Latov |first1=Norman |title=Diagnosis of CIDP |journal=Neurology |volume=59 |issue=12 Suppl 6 |pages=S2–6 |year=2002 |pmid=12499464 |doi= 10.1212/wnl.59.12_suppl_6.s2}}</ref>
A nerve biopsy may be performed in some cases to confirm the diagnosis by demonstrating demyelination and inflammation.


CIDP has been associated with [[diabetes mellitus]], [[HIV]] infection, and [[paraproteinemia]]s.
==Treatment==
The primary goal of treatment for CIDP is to reduce inflammation and suppress the immune response. Common treatment options include:
* [[Corticosteroids]] such as prednisone
* Intravenous immunoglobulin (IVIG)
* Plasmapheresis (plasma exchange)
* Immunosuppressive drugs such as azathioprine or rituximab


===Variants with paranodal autoantibodies===
Physical therapy and occupational therapy are also important components of managing CIDP, helping to maintain muscle strength and improve functional abilities.
{{main|Anti-neurofascin demyelinating diseases}}


Some variants of CIDP present autoimmunity against proteins of the [[node of Ranvier]]. These variants comprise a subgroup of inflammatory neuropathies with IgG4 autoantibodies against the paranodal proteins [[neurofascin]]-155, [[contactin]]-1 and [[caspr]]-1.<ref name="Doppler">Kathrin Doppler, Claudia Sommer, The New Entity of Paranodopathies: A Target Structure with Therapeutic Consequences, Neurology International, April 2017; 01(01): E56-E60, DOI: 10.1055/s-0043-102455</ref>
==Prognosis==
The prognosis for individuals with CIDP varies. Some patients experience significant improvement with treatment, while others may have a more chronic course with relapses and remissions. Early diagnosis and treatment are crucial for improving outcomes and preventing long-term disability.


These cases are special not only because of their pathology, but also because they are non-responsive to the standard treatment. They are responsive to [[Rituximab]] instead.<ref name="Doppler"/>
==Related pages==
 
* [[Guillain-Barré syndrome]]
Also some cases of [[#combined central and peripheral demyelination|combined central and peripheral demyelination]] (CCPD) could be produced by neurofascins.<ref>J. Ciron et al. The coexistence of recurrent cerebral tumefactive demyelinating lesions with longitudinally extensive transverse myelitis and demyelinating neuropathy, Multiple Sclerosis and Related Disorders, Volume 27, January 2019, Pages 223-225</ref>
* [[Peripheral neuropathy]]
 
===Autoantibodies of the IgG3 Subclass in CIDP===
 
Autoantibodies to components of the Ranvier nodes, specially autoantibodies the Contactin-associated protein 1 ([[CASPR]]), cause a form of CIDP with an acute "[[Guillain-Barre]]-like" phase, followed by a chronic phase with progressive symptoms. Different IgG subclasses are associated with the different phases of the disease. IgG3 Caspr autoantibodies were found during the acute GBS-like phase, while IgG4 Caspr autoantibodies were present during the chronic phase of disease.<ref>Christiane S. Hampe, Significance of Autoantibodies, Neuroimmune Diseases pp 109-142, August 2019</ref>
 
== Diagnosis ==
 
There are several types of immune-mediated neuropathies recognised.<ref name=Finsterer2005>Finsterer J (2005) Treatment of immune-mediated, dysimmune neuropathies. Acta Neurologica Scandinavica. 112(2):115-125</ref><ref name=Ensrud2001>Ensrud ER, Krivickas LS (2001) Acquired inflammatory demyelinating neuropathies. Physical Medicine and Rehabilitation Clinics of North America. 12(2):321-334</ref> These include
 
* Chronic inflammatory demyelinating polyneuropathy (CIDP) with subtypes:
** Classical CIDP
** CIDP with [[diabetes]]
** CIDP/[[monoclonal gammopathy]] of undetermined significance
** Sensory CIDP
** [[Multifocal motor neuropathy]]
** Multifocal acquired demyelinating sensory and motor neuropathy ([[Lewis-Sumner syndrome]])
** Multifocal acquired sensory and motor neuropathy
** Distal acquired demyelinating sensory neuropathy
* [[Guillain–Barré syndrome]] with subtypes:
** Acute inflammatory demyelinating [[polyradiculoneuropathy]]
**[[Acute motor axonal neuropathy]]
** Acute motor and sensory axonal neuropathy
** Acute pandysautonomia
** Miller Fisher syndrome
* [[Immunoglobulin M|IgM]] monoclonal gammopathies with subtypes:
** [[Waldenström's macroglobulinemia]]
** Mixed [[cryoglobulinemia]], [[gait ataxia]], late-onset [[polyneuropathy]] syndrome
** [[Myelin-associated glycoprotein]]-associated [[Gamma globulin|gammopathy]], polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes syndrome ([[POEMS syndrome|POEMS]])
 
For this reason a diagnosis of chronic inflammatory demyelinating polyneuropathy needs further investigations.
 
The diagnosis is usually provisionally 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.
 
[[Autonomic nervous system|Autonomic]] system dysfunction can occur; in such a case, the patient would complain of [[orthostatic]] dizziness, problems [http://cidplog.com/cidp-symptoms.shtml breathing, eye, bowel, bladder]  and [[cardiac]] problems. The patient may also present with a single [[cranial nerve]] or peripheral nerve dysfunction.
 
On examination the patients may have weakness, and loss of deep tendon reflexes (rarely increased or normal). There may be [[atrophy]] (shrinkage) of muscles, [[fasciculation]]s (twitching) and loss of sensation. Patients may have [[Multifocal motor neuropathy|multi-focal motor neuropathy]], as they have no sensory loss.
 
Most experts consider the necessary duration of symptoms to be greater than 8 weeks for the diagnosis of CIDP to be made.
 
Typical diagnostic tests include:
 
* Electrodiagnostics – [[electromyography]] (EMG) and [[nerve conduction study]] (NCS). In usual CIDP, the nerve conduction studies show [[demyelination]]. These findings include:
*# a reduction in nerve conduction velocities;
*# the presence of conduction block or abnormal temporal dispersion in at least one motor nerve;
*# prolonged distal latencies in at least two nerves;
*# absent [[F wave]]s 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.
* [[Lumbar puncture]] 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.
* [[Ultrasound]] of the peripheral nerves may show swelling of the affected nerves<ref name=Herraets2017>Herraets IJT, Goedee HS, Telleman JA, van Asseldonk JH, Visser LH, van der Pol WL, van den Berg LH (2017) High-resolution ultrasound in patients with Wartenberg's migrant sensory neuritis, a case-control study. Clinical Neurophysiology. 129(1):232-237. doi: 10.1016/j.clinph.2017.10.040</ref><ref name=Goedee2017>Goedee HS, van der Pol WL, van Asseldonk JH, Franssen H, Notermans NC, Vrancken AJ, van Es MA, Nikolakopoulos S, Visser LH, van den Berg LH (2017) Diagnostic value of sonography in treatment-naive chronic inflammatory neuropathies. Neurology 88(2):143-151. doi: 10.1212/WNL.0000000000003483</ref><ref name="Décard2017">Décard BF, Pham M, Grimm A (2017) Ultrasound and MRI of nerves for monitoring disease activity and treatment effects in chronic dysimmune neuropathies - Current concepts and future directions. Clinical Neurophysiology. 129(1):155-167. doi: 10.1016/j.clinph.2017.10.028</ref>
* [[Magnetic resonance imaging]] can also be used in the diagnostic workup<ref name=Shibuya2015>Shibuya K1, Sugiyama A, Ito S, Misawa S, Sekiguchi Y, Mitsuma S, Iwai Y, Watanabe K, Shimada H, Kawaguchi H, Suhara T, Yokota H, Matsumoto H, Kuwabara S (2015) Reconstruction magnetic resonance neurography in chronic inflammatory demyelinating polyneuropathy. Annals of Neurology. 77(2):333-337. doi: 10.1002/ana.24314</ref><ref name=Rajabally2014>Rajabally YA, Knopp MJ, Martin-Lamb D, Morlese J (2014) Diagnostic value of MR imaging in the Lewis-Sumner syndrome: a case series. Journal of Neurological Science. 342(1-2):182-185. doi: 10.1016/j.jns.2014.04.033</ref>
 
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.<ref>{{cite journal |author=Azulay JP |title=[The diagnosis of chronic axonal polyneuropathy: the poorly understood chronic polyradiculoneuritides] |language=French |journal=Revue Neurologique (Paris) |volume=162 |issue=12 |pages=1292–5 |year=2006 |pmid=17151528 |doi= 10.1016/S0035-3787(06)75150-5|url=http://www.masson.fr/masson/MDOI-RN-12-2006-162-12-0035-3787-101019-200607904}}</ref>
 
===Differential diagnosis===
* [[Bickerstaff brainstem encephalitis]]
* [[Fisher syndrome]]
* [[Guillain–Barré syndrome]]
 
== Treatment ==
First-line treatment for CIDP is currently [[intravenous immunoglobulin]] and other treatments include [[corticosteroids]] (e.g. [[prednisone]]), and [[plasmapheresis]] (plasma exchange) which may be prescribed alone or in combination with an [[immunosuppressive drug|immunosuppressant drug]].<ref>{{cite journal |author=Hughes RA |title=Systematic reviews of treatment for inflammatory demyelinating neuropathy |journal=Journal of Anatomy |volume=200 |issue=4 |pages=331–9 |year=2002 |pmid=12090400 |doi=10.1046/j.1469-7580.2002.00041.x |pmc=1570692 }}</ref> Recent controlled studies show subcutaneous immunoglobulin appears to be as effective for CIDP treatment as intravenous immunoglobulin in most patients, and with fewer systemic side effects.<ref>{{Cite journal|last=Hadden|first=Robert D. M.|last2=Marreno|first2=Fabrizio|date=2016-12-28|title=Switch from intravenous to subcutaneous immunoglobulin in CIDP and MMN: improved tolerability and patient satisfaction|journal=Therapeutic Advances in Neurological Disorders|volume=8|issue=1|pages=14–19|doi=10.1177/1756285614563056|issn=1756-2856|pmc=4286942|pmid=25584070}}</ref>
 
Intravenous immunoglobulin 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. Intravenous immunoglobulin is probably the first-line CIDP treatment, but is extremely expensive. For example, 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.
 
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. [[Ciclosporin]] has also been used in CIDP but with less frequency as it is a newer approach.<ref>{{cite journal |vauthors=Odaka M, Tatsumoto M, Susuki K, Hirata K, Yuki N |title=Intractable chronic inflammatory demyelinating polyneuropathy treated successfully with ciclosporin |journal=Journal of Neurology, Neurosurgery, and Psychiatry |volume=76 |issue=8 |pages=1115–20 |year=2005 |pmid=16024890 |pmc=1739743 |doi=10.1136/jnnp.2003.035428 |url=}}</ref> Ciclosporin 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 "off-label", meaning that they do not have an indication for the treatment of CIDP in their package inserts. Before azathioprine is used, the patient should first have a blood test that ensures that azathioprine can safely be used.
 
[[Anti-thymocyte globulin]], 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.
 
A review of several treatments found that azathioprine, [[interferon alpha]] and [[methotrexate]] were not effective.<ref name=Rajabally2017>{{cite journal|last1=Rajabally|first1=Yusuf A.|title=Unconventional treatments for chronic inflammatory demyelinating polyneuropathy|journal=Neurodegenerative Disease Management|volume=7|issue=5|pages=331–342|doi=10.2217/nmt-2017-0017|pmid=29043889|year=2017}}</ref> Cyclophosphamide and rituximab seem to have some response. Mycophenolate mofetil may be of use in milder cases. Immunoglobulin and steroids are the first line choices for treatment.
 
In severe cases of CIDP, when second-line immunomodulatory drugs are not efficient, autologous [[hematopoietic stem cell transplantation]] is sometimes performed. The treatment may induce long-term remission even in severe treatment-refractory cases of CIDP. To improve outcome, it has been suggested that it should be initiated before irreversible axonal damage has occurred. However, a precise estimation of its clinical efficacy for CIDP is not available, as [[randomized controlled trial]]s have not been performed.<ref>{{Cite journal|last=Burman|first=Joachim|last2=Tolf|first2=Andreas|last3=Hägglund|first3=Hans|last4=Askmark|first4=Håkan|date=2018-02-01|title=Autologous haematopoietic stem cell transplantation for neurological diseases|url=http://jnnp.bmj.com/content/89/2/147|journal=Journal of Neurology, Neurosurgery, and Psychiatry|language=en|volume=89|issue=2|pages=147–155|doi=10.1136/jnnp-2017-316271|issn=0022-3050|pmid=28866625|pmc=5800332}}</ref>
 
[[Physical therapy]] and [[occupational therapy]] may improve muscle strength, [[activities of daily living]], 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 will affect patients over time. 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]].<ref name=radiculo>{{cite journal |author=Kissel JT |title=The treatment of chronic inflammatory demyelinating polyradiculoneuropathy |journal=Seminars in Neurology |volume=23 |issue=2 |pages=169–80 |year=2003 |pmid=12894382 |doi=10.1055/s-2003-41130 }}</ref>
 
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 regimens is likely to be necessary in order to discover the most appropriate treatment regimen for a given patient.{{Citation needed|reason=whole paragraph appears speculative and/or opinion, e.g. "many doctors will not have encountered it before" |date=January 2017}}
 
==Epidemiology==
 
In 1982 Lewis et al. reported a group of patients with a chronic asymmetrical sensorimotor neuropathy mostly affecting the arms with multifocal involvement of peripheral nerves.<ref name=Lewis2005>Lewis RA, Sumner AJ, Brown MJ, Asbury AK (1982) Multifocal demyelinating neuropathy with persistent conduction block. Neurology 32: 958–964</ref> Also in 1982 Dyck ''et al'' reported a response to prednisolone to a condition they referred to as chronic inflammatory demyelinating polyradiculoneuropathy.<ref name=Dyck1982>Dyck PJ, O'Brien PC, Oviatt KF, Dinapoli RP, Daube JR, Bartleson JD, ''et al''. Prednisone improves chronic inflammatory demyelinating polyradiculoneuropathy more than no treatment. Annals of Neurology. 11: 136–141</ref> Parry and Clarke in 1988 described a neuropathy which was later found to be associated with IgM autoantibodies directed against GM1 gangliosides.<ref name=Parry1988>Parry GJ, Clarke S. Multifocal acquired demyelinating neuropathy masquerading as motor neuron disease. Muscle and Nerve. 11: 103–107</ref><ref name=Pestronk1988>Pestronk A, Cornblath DR, Ilyas AA, Baba H, Quarles RH, Griffin JW, et al. A treatable multifocal motor neuropathy with antibodies to GM1 ganglioside. Annals of Neurology. 24: 73–78</ref> This latter condition was later termed [[multifocal motor neuropathy]]<ref name=Nobile-Orazio2001>Nobile-Orazio E. Multifocal motor neuropathy. Journal of Neuroimmunology 115: 4–18</ref> This distinction is important because multifocal motor neuropathy responds to intravenous globulin alone while chronic inflammatory demyelinating polyneuropathy responds to intravenous globulin, steroids and plasma exchanges.<ref name=VanDoorn2002>Van Doorn PA, Garssen MP (2002) Treatment of immune neuropathies. Current Opinion in Neurology. 15: 623–631</ref> It has been suggested that multifocal motor neuropathy is distinct from chronic inflammatory demyelinating polyneuropathy and that Lewis-Sumner syndrome is a distinct variant type of chronic inflammatory demyelinating polyneuropathy.<ref name=Lewis2007>Lewis RA (2007) Neuropathies associated with conduction block. Current Opinion in Neurology. 20(5):525-530</ref>
 
The Lewis-Sumner form of this condition is considered a rare disease with only 50 cases reported up to 2004.<ref name=Viala2004>Viala K, Renié L, Maisonobe T, Béhin A, Neil J, Léger JM, Bouche P (2004) Follow-up study and response to treatment in 23 patients with Lewis-Sumner syndrome. Brain 127(Pt 9):2010-2017</ref> A total of 90 cases had been reported by 2009.<ref name=Rajabally2009>Rajabally YA, Chavada G (2009) Lewis-Sumner syndrome of pure upper-limb onset: diagnostic, prognostic, and therapeutic features. Muscle and Nerve. 39(2):206-220. doi: 10.1002/mus.21199</ref>
 
== Vaccine Injury Compensation for CIDP ==
The National Vaccine Injury Compensation Program has awarded money damages to patients who came down with CIDP after receiving one of the childhood vaccines listed on the Federal Government's [[National Vaccine Injury Compensation Program|vaccine injury table]]. These Vaccine Court awards often come with language stating that the Court denies that the specific vaccine "caused petitioner to suffer CIDP or any other injury. Nevertheless, the parties agree to the joint stipulation, attached hereto as Appendix A. The undersigned finds said stipulation reasonable and adopts it as the decision of the Court in awarding damages, on the terms set forth therein."<ref>{{Cite web|url=https://ecf.cofc.uscourts.gov/cgi-bin/show_public_doc?2016vv0262-86-0|title=Riley v. Secretary of Health and Human Services, Case No. 16-262V|last=|first=|date=July 30, 2019|website=United States Court of Federal Claims|url-status=live|archive-url=|archive-date=|access-date=}}</ref>  A keyword search on the Court of Federal Claims "Opinions/Orders" database  for the term "CIDP" returns 202 opinions related to CIDP and vaccine injury compensation.<ref>{{Cite web|url=https://www.uscfc.uscourts.gov/aggregator/sources/7|title=United States Court of Federal Claims Opinions/Orders|last=|first=|date=October 24, 2019|website=United States Court of Federal Claims|url-status=live|archive-url=|archive-date=|access-date=October 24, 2019}}</ref>
 
== See also ==
* [[Autoimmune disease]]
* [[Autoimmune disease]]
* [[Neurology]]


== References ==
[[Category:Neurological disorders]]
 
{{Reflist}}
 
== External links ==
{{Medical resources
|  DiseasesDB    = 30084
|  ICD10          = {{ICD10|G61.8}}
|  ICD9          = {{ICD9|357.81}}
|  ICDO          =
|  OMIM          = 139393
|  MedlinePlus    =
|  eMedicineSubj  = neuro
|  eMedicineTopic = 467
|  MeshID        = D020277
}}
 
* {{NINDS|CIDP}}
* [https://www.gbs-cidp.org/cidp/ CIDP at GBS/CIDP Foundation International]
 
{{PNS diseases of the nervous system}}
 
{{DEFAULTSORT:Chronic Inflammatory Demyelinating Polyneuropathy}}
[[Category:Autoimmune diseases]]
[[Category:Autoimmune diseases]]
[[Category:Neurological disorders]]
{{dictionary-stub1}}
<gallery>
File:CIDP_Histopathology_Teased_fibre.jpg|Histopathological image of teased nerve fibers in CIDP
File:Neuron.svg|Diagram of a neuron
</gallery>

Revision as of 19:15, 22 March 2025

A neurological disorder characterized by progressive weakness and impaired sensory function in the legs and arms


Chronic inflammatory demyelinating polyneuropathy (CIDP) is a neurological disorder characterized by progressive weakness and impaired sensory function in the legs and arms. It is considered an autoimmune disorder, where the body's immune system mistakenly attacks the myelin sheath, the protective covering of the nerves. CIDP is related to Guillain-Barré syndrome, but it progresses more slowly and lasts longer.

Pathophysiology

CIDP is caused by inflammation of the peripheral nerves and nerve roots, leading to the destruction of the myelin sheath. This demyelination disrupts the normal conduction of electrical impulses along the nerves, resulting in muscle weakness, sensory disturbances, and loss of reflexes. The exact cause of the autoimmune response in CIDP is not fully understood, but it is believed to involve both cellular and humoral immune mechanisms.

Symptoms

The symptoms of CIDP can vary widely among individuals, but they typically include:

  • Progressive muscle weakness, particularly in the arms and legs
  • Tingling or numbness in the extremities
  • Loss of deep tendon reflexes
  • Fatigue
  • Difficulty walking
  • Sensory ataxia

The progression of symptoms can be gradual, occurring over several months, and may fluctuate in severity.

Diagnosis

Diagnosing CIDP involves a combination of clinical evaluation, laboratory tests, and electrophysiological studies. Key diagnostic criteria include:

  • Clinical presentation of progressive weakness and sensory dysfunction
  • Electromyography (EMG) and nerve conduction studies showing demyelination
  • Elevated protein levels in the cerebrospinal fluid (CSF) without a significant increase in white blood cells

A nerve biopsy may be performed in some cases to confirm the diagnosis by demonstrating demyelination and inflammation.

Treatment

The primary goal of treatment for CIDP is to reduce inflammation and suppress the immune response. Common treatment options include:

  • Corticosteroids such as prednisone
  • Intravenous immunoglobulin (IVIG)
  • Plasmapheresis (plasma exchange)
  • Immunosuppressive drugs such as azathioprine or rituximab

Physical therapy and occupational therapy are also important components of managing CIDP, helping to maintain muscle strength and improve functional abilities.

Prognosis

The prognosis for individuals with CIDP varies. Some patients experience significant improvement with treatment, while others may have a more chronic course with relapses and remissions. Early diagnosis and treatment are crucial for improving outcomes and preventing long-term disability.

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