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PubMed2003

The treatment of chronic inflammatory demyelinating polyradiculoneuropathy.

Chronic inflammatory demyelinating polyradiculoneuropathy is an inflammatory disorder of nerve that usually presents with slowly progressive weakness and sensory loss and areflexia. It is among the most treatable of the peripheral nerve disorders, and several modalities have been shown to be effective in prospective, randomized controlled trials. Although most patients show a gratifying early response to treatment, in many cases the patients relapse. The cumulative effects of the neuropathic impairments, along with side effects from long-term immunosuppressive treatment, combine to produce significant long-term morbidity and loss of function. This review will cover the epidemiology, clinical features, laboratory findings, and pathogenesis of chronic inflammatory demyelinating polyradiculoneuropathy; the current status of the treatment of this disorder will be reviewed, highlighting those therapies shown to be effective in randomized controlled trials.

Anti-Inflammatory Agents↗

PubMed2001

P0 protein is a target antigen in chronic inflammatory demyelinating polyradiculoneuropathy.

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is widely regarded as an autoimmune disorder, although the autoantigen remains unknown. In this study, the sera of 21 CIDP patients were examined by immunofluorescence for antimyelin activity and by Western blotting for binding to myelin proteins. Six sera contained anti-P0 immunoglobulin G antibodies, and four of these caused conduction block and demyelination following intraneural injection in experimental animals. Absorption with P0 protein eliminated the demyelinating activity. These results show that P0 is an autoantigen in some patients with CIDP. Since P0 possesses powerful adhesion properties and is largely responsible for myelin compaction, the demonstration of demyelination by human anti-P0 antibodies provides new insight into this important and common immunopathological process.

Animals↗

PubMedDate not supplied

Sonographic detection of diffuse peripheral nerve hypertrophy in chronic inflammatory demyelinating polyradiculoneuropathy.

Chronic inflammatory demyelinating polyradiculoneuropathy is an autoimmune disease characterized by recurrent demyelination and remyelination with resultant thickening of the peripheral nerves. We report a case in which sonography was instrumental in demonstrating diffuse peripheral nerve hypertrophy. On sonography, both brachial plexuses were found to be diffusely hypertrophic and hypoechoic. Similar findings were noted for the median, sciatic, and femoral nerves. The brachial plexus findings were confirmed by MRI.

Adult↗

PubMed2005

Antibodies to peripheral nerve myelin proteins in chronic inflammatory demyelinating polyradiculoneuropathy.

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an acquired disorder of the peripheral nervous system with a probable auto-immune pathogenesis. The nature of the responsible autoantigens is unclear in most patients. We used the Western immunoblot technique to seek antibodies to peripheral nerve protein antigens. Sera from eight of 32 (25%) CIDP patients, 12 of 37 (32%) Guillain-Barré syndrome (GBS) patients, zero of 30 (0%) chronic idiopathic axonal polyneuropathy patients and two of 39 (5%) healthy control subjects contained anti-peripheral nerve protein antibodies. The frequency of such antibodies was significantly greater in both CIDP (p = 0.04) and GBS (p = 0.003) patients than in normal control subjects. For CIDP patients, there were non-significant trends for antibodies to be more common in females and in those who responded to treatment with either intravenous immunoglobulin or plasma exchange. The commonest antibodies were directed against a band at 28 kDa, resembling that labelled by a monoclonal antibody against myelin protein zero (P0). Six CIDP and seven GBS patients' sera reacted with this band. These results support the view that antibodies to myelin proteins, and especially P0, are present in the serum of some patients with CIDP and GBS.

Adult↗

PubMed1993

Chronic inflammatory demyelinating polyradiculoneuropathy.

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) represents an important acquired condition characterized by progressive, symmetrical, proximal and distal weakness. Muscle stretch reflexes are depressed and sensory loss is variable. Patients may have mild to severe weakness and may require assisted ambulation or wheelchairs. Rarely do patients require respiratory support. Laboratory features include nerve conduction changes and nerve biopsy pathology indicative of demyelination. Cerebrospinal fluid protein is characteristically elevated without pleocytosis. The condition is responsive to immunosuppressive therapy, especially prednisone and plasmapheresis (plasma exchange). CIDP frequently follows a chronic or relapsing course.

Chronic Disease↗

PubMed1994

A plasma exchange versus immune globulin infusion trial in chronic inflammatory demyelinating polyradiculoneuropathy.

Chronic inflammatory demyelinating polyradiculoneuropathy is a paralytic syndrome, causing considerable disability and even death. In controlled clinical trials, plasma exchange prevented or ameliorated neurological deficits, but the efficacy of immune globulin infusion remains unproved. Also unknown is whether immune globulin infusion is as effective, or more effective, than plasma exchange and what dosages and frequencies are best. In this observer-blinded study, using some objective end points not subject to bias (e.g., summated compound muscle action potential), 20 patients with progressive or static polyneuropathy were randomly assigned to receive either of the two treatments for 6 weeks, followed by a washout period, and then were assigned to receive the other treatment. Plasma exchange (twice a week for 3 weeks then once a week for 3 weeks) and immune globulin infusion (0.4 gm/kg once a week for 3 weeks, then 0.2 gm/kg once a week for the next 3 weeks) were used. End points assessed before and after treatment schedules were neurological disability score; muscle weakness of the neurological disability score; summated compound muscle action potentials of ulnar, median, and peroneal nerves; summated sensory nerve action potentials of ulnar and sural nerves; and vibratory detection threshold of the great toe using CASE IV. Observers were masked as to treatment used. Of 20 patients, 13 received both treatments whereas 4 did not worsen sufficiently to receive the second treatment--1 patient left the study during and 2 after the first treatment to receive unscheduled treatment elsewhere.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

PubMed2006

[Histopathological features of chronic inflammatory demyelinating polyradiculoneuropathy].

Chronic inflammatory demyelinating polyneuropathy (CIDP) was proposed by Dyck et al. in 1975. Diagnosis was based mainly on nerve biopsy features with segmental demyelination, onion bulb formation and inflammatory infiltrates. In many pathological studies, frequencies of these features of CIDP were not observed in the same percentages. Limitations on the nerve biopsy were explained by the study of small, distal, only sensory nerve specimens in the lower limb. In recent years, the usefulness of nerve biopsy has been reconsidered. If electron microscopy and teased-fiber studies are used, the examination can recognize CIDP erroneously classified as chronic idiopathic axonal polyneuropathy. Therapeutic options should be guided by suggestive abnormalities of demyelination and or inflammation on nerve biopsy even in the presence of a electrophysiologic axonal pattern.

Biopsy↗

PubMed2004

Pathogenesis of chronic inflammatory demyelinating polyradiculoneuropathy.

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a term applied to a spectrum of immune-mediated demyelinating neuropathies that are heterogeneous in clinical manifestations and probably in pathogenesis. Although histopathologic studies of CIDP have been complicated by a relapsing course of the inflammatory reaction and its predominance in proximal nerve segments, many clues point to involvement of both cellular and humoral immune factors in the pathogenesis. Uncertainties remain regarding the provoking antigen(s) and location of the initial T cell activation. Breakdown of blood nerve barrier by activated T cells and its cytokines is followed by a local intraneural immune response with recruitment of macrophages and secretion of toxic factors, which cause damage to the myelin and axons. Activated T cells may also induce B cells to produce antibodies against nerve/myelin antigens. This review summarizes our current knowledge of the immunopathogenesis and insight from animal models of CIDP.

Animals↗

PubMed2001

Corticosteroids for chronic inflammatory demyelinating polyradiculoneuropathy.

BACKGROUND: Chronic inflammatory demyelinating polyradiculoneuropathy is an autoimmune peripheral neuropathy and would be expected to benefit from corticosteroids. Non-randomised studies suggest that corticosteroids are often beneficial. OBJECTIVES: To evaluate the efficacy of corticosteroids for treating chronic inflammatory demyelinating polyradiculoneuropathy. SEARCH STRATEGY: Search of the Cochrane Neuromuscular Disease Group register for randomised trials of corticosteroids treatment for chronic inflammatory demyelinating polyradiculoneuropathy and enquiry from subject experts. SELECTION CRITERIA: Types of studies: All randomised or quasi-randomised trials Types of participants: All patients with chronic inflammatory demyelinating polyradiculoneuropathy who were diagnosed by an internationally accepted definition. Types of interventions: Treatment with any form of corticosteroid or adrenocorticotropic hormone. Types of outcome measures: PRIMARY OUTCOME MEASURE: Change in disability 12 weeks after randomisation. SECONDARY OUTCOME MEASURES: 1. Change in impairment 12 weeks after randomisation. 2. Change in maximum motor nerve conduction velocity or compound muscle action potential amplitude after 12 weeks. 3. Side effects of corticosteroids. DATA COLLECTION AND ANALYSIS: One author extracted the data and the other checked them. MAIN RESULTS: We identified one randomised controlled trial, an open study in which 19 corticosteroid treated patients showed more improvement in impairment than 16 untreated controls after 12 weeks. Experience from large non-randomised studies suggests that steroids are beneficial. REVIEWER'S CONCLUSIONS: A single randomised controlled trial with 35 participants provided weak evidence to support the common opinion derived from non-randomised studies that oral corticosteroids reduce impairment in chronic inflammatory demyelinating polyradiculoneuropathy. Corticosteroids are known to have serious long term side effects. The long term risks and benefits have not been adequately studied.

Adrenal Cortex Hormones↗

PubMed2003

Cytotoxic drugs and interferons for chronic inflammatory demyelinating polyradiculoneuropathy.

BACKGROUND: Chronic inflammatory demyelinating polyradiculoneuropathy is a disease causing progressive or relapsing and remitting weakness and numbness. It is probably due to an autoimmune inflammatory process. Immunosuppressive or immunomodulatory drugs would be expected to be beneficial. OBJECTIVES: We aimed to review systematically the evidence from randomised trials concerning cytotoxic drugs and interferons for chronic inflammatory demyelinating polyradiculoneuropathy. SEARCH STRATEGY: We searched the Cochrane Neuromuscular Disease Group trials register (searched December 2001), MEDLINE (searched January 1977 to December 2001), EMBASE (January 1980 to December 2001), CINAHL (searched January 1982 to December 2001) and LILACS (searched January 1982 to December 2001). We contacted the authors of the trials identified and other disease experts seeking other published and unpublished trials. SELECTION CRITERIA: We sought randomised and quasi-randomised trials of all immunosuppressive agents such as azathioprine, cyclophosphamide, methotrexate, cyclosporin A, mycophenolate mofetil, and rituximab and all immunomodulatory agents such as alpha interferon and beta interferon in participants fulfilling standard diagnostic criteria for chronic inflammatory demyelinating polyradiculoneuropathy. DATA COLLECTION AND ANALYSIS: Two of us independently selected the trials which met our criteria, judged their methodological quality and extracted the data onto specially designed forms. We wanted to measure the change in disability after one year as our primary outcome measure. MAIN RESULTS: We found one parallel group open trial of azathioprine for nine months involving 27 participants and another of interferon beta involving 10 participants in a double blind crossover trial with each treatment period lasting 12 weeks. Neither trial provided our primary outcome measure and neither showed a significant beneficial effect on any of the outcome measures selected by the authors or ourselves in the protocol for this review. REVIEWER'S CONCLUSIONS: The evidence is inadequate to decide whether azathioprine, interferon beta or any other immunosuppressive drug or interferon is beneficial in chronic inflammatory demyelinating polyradiculoneuropathy.

Anti-Inflammatory Agents↗

PubMed2004

Cytotoxic drugs and interferons for chronic inflammatory demyelinating polyradiculoneuropathy.

BACKGROUND: Chronic inflammatory demyelinating polyradiculoneuropathy is a disease causing progressive or relapsing and remitting weakness and numbness. It is probably due to an autoimmune inflammatory process. Immunosuppressive or immunomodulatory drugs would be expected to be beneficial. OBJECTIVES: We aimed to review systematically the evidence from randomised trials concerning cytotoxic drugs and interferons for chronic inflammatory demyelinating polyradiculoneuropathy. SEARCH STRATEGY: We searched the Cochrane Neuromuscular Disease Group trials register (searched May 2004), MEDLINE (searched January 1977 to May 2004), EMBASE (January 1980 to May 2004), CINAHL (searched January 1982 to December 2001) and LILACS (searched January 1982 to December 2001). We contacted the authors of the trials identified and other disease experts seeking other published and unpublished trials. SELECTION CRITERIA: We sought randomised and quasi-randomised trials of all immunosuppressive agents such as azathioprine, cyclophosphamide, methotrexate, cyclosporin A, mycophenolate mofetil, and rituximab and all immunomodulatory agents such as alpha interferon and beta interferon in participants fulfilling standard diagnostic criteria for chronic inflammatory demyelinating polyradiculoneuropathy. DATA COLLECTION AND ANALYSIS: Two of us independently selected the trials which met our criteria, judged their methodological quality and extracted the data onto specially designed forms. We wanted to measure the change in disability after one year as our primary outcome measure. MAIN RESULTS: We found one parallel group open trial of azathioprine for nine months involving 27 participants and another of interferon beta involving 10 participants in a double blind crossover trial with each treatment period lasting 12 weeks. Neither trial provided our primary outcome measure and neither showed a significant beneficial effect on any of the outcome measures selected by the authors or ourselves in the protocol for this review. REVIEWERS' CONCLUSIONS: The evidence is inadequate to decide whether azathioprine, interferon beta or any other immunosuppressive drug or interferon is beneficial in chronic inflammatory demyelinating polyradiculoneuropathy.

Anti-Inflammatory Agents↗

PubMed2004

Plasma exchange for chronic inflammatory demyelinating polyradiculoneuropathy.

BACKGROUND: Chronic inflammatory demyelinating polyradiculoneuropathy is an uncommon progressive or relapsing paralysing disease caused by inflammation of the peripheral nerves. If the hypothesis that it is due to autoimmunity is correct, removal of autoantibodies in the blood by plasma exchange should be beneficial. OBJECTIVES: To evaluate the efficacy of plasma exchange in chronic inflammatory demyelinating polyradiculoneuropathy. SEARCH STRATEGY: We searched the Neuromuscular Disease Group Register (December 2003), and MEDLINE (January 1966 to January 2003), EMBASE (January 1980 to January 2003), CINAHL (January 1982 to December 2002) and LILACS (January 1982 to January 2003). We also scrutinised the bibliographies of the trials, and contacted the trial authors and other disease experts. SELECTION CRITERIA: Randomised or quasi-randomised controlled trials in participants of any age comparing plasma exchange with sham treatment or no treatment. A trial showing no significant difference in the benefit from plasma exchange with intravenous immunoglobulin has been included in a separate Cochrane review. DATA COLLECTION AND ANALYSIS: Two authors selected the trials, extracted the data and assessed methodological quality independently. Where possible data were combined according to the methods of the Cochrane Neuromuscular Disease Review Group. MAIN RESULTS PRIMARY OUTCOME MEASURE: one crossover trial including 18 participants showed 2 (95% confidence interval (CI) 0.8 to 3.0) points more improvement after four weeks in an 11-point disability scale with plasma exchange (10 exchanges over four weeks) than with sham exchange. Rapid deterioration after plasma exchange occurred in eight of 12 who had improved. SECONDARY OUTCOME MEASURES: when the results of this trial and another with 29 participants treated in a parallel group design trial were combined, there were 31 points (95% CI 16 to 45) more improvement in an impairment scale after plasma exchange (six exchanges over three weeks) than after sham exchange. There were significant improvements in both trials in an electrophysiological measure, the proximally evoked compound muscle action potential, after three or four weeks. Non-randomised evidence indicates that plasma exchange induces adverse events in 3% to 17% of procedures. These are sometimes serious. REVIEWERS' CONCLUSIONS: Evidence from two small trials showed that plasma exchange provides significant short-term benefit in about two-thirds of patients with chronic inflammatory demyelinating polyradiculoneuropathy but rapid deterioration may occur afterwards. Adverse events related to difficulty with venous access, use of citrate and haemodynamic changes are not uncommon. More research is needed to identify agents which will prolong the beneficial action of plasma exchange.

Humans↗

PubMed2002

Intravenous immunoglobulin for chronic inflammatory demyelinating polyradiculoneuropathy.

BACKGROUND: Chronic inflammatory demyelinating polyradiculoneuropathy is an immune mediated disorder characterised by progressive or relapsing symmetrical motor or sensory symptoms and signs in more than one limb, developing over at least two months. It may cause prolonged periods of disability and even death. Several uncontrolled studies have suggested a beneficial effect of intravenous immunoglobulin. OBJECTIVES: To review systematically the evidence from randomised controlled trials concerning the efficacy and safety of intravenous immunoglobulin in chronic inflammatory demyelinating polyradiculoneuropathy. SEARCH STRATEGY: We used the Search Strategy of the Cochrane Neuromuscular Disease Review Group to search the Disease Group register and other databases for randomised controlled trials from 1985 onwards. SELECTION CRITERIA: Randomised controlled studies examining the effects of any dose of intravenous immunoglobulin versus placebo, plasma exchange or corticosteroids in patients with definite or probable chronic inflammatory demyelinating polyradiculoneuropathy. Outcome measures had to include one of the following: a disability score, the Medical Research Council sum score, electrophysiological data or walking distance. Studies which reported the frequency of adverse effects were used to assess the safety of treatment. DATA COLLECTION AND ANALYSIS: Two reviewers independently reviewed literature searches to identify potentially relevant trials, scored their quality and extracted data independently. For dichotomous data, we calculated relative risks, and for continuous data, effect sizes (for definition see statistical analysis section) and weighted pooled effect sizes. Statistical uncertainty was expressed in 95% confidence intervals. Sensitivity analysis excluding studies with quality scores below A 0.50 and below B 0.75 was planned but not performed as all studies had quality scores above 0.75. MAIN RESULTS: Six randomised controlled trials were considered eligible including 170 patients. Four studies on 113 patients compared intravenous immunoglobulin against placebo. One trial with 17 patients compared intravenous immunoglobulin with plasma exchange in a cross-over design and one trial compared intravenous immunoglobulin with prednisolone in 32 patients. A significantly higher proportion of patients improved in disability within one month after the onset of intravenous immunoglobulin treatment as compared with placebo (relative risk 3.17, 95% confidence interval 1.74 to 5.75). Whether all these improvements are equally clinically relevant cannot be deduced from this analysis because each trial used a different disability scale with a unique definition of a significant improvement. To overcome this problem an attempt was made to transform the various disability scales to the modified Rankin score. In three trials including 87 patients this transformation could be carried out. A significantly higher proportion of patients improved one point after intravenous immunoglobulin treatment compared to placebo (relative risk 2.47, 95% confidence interval 1.02 to 6.01). The effect size for change of mean disability score at six weeks comparing intravenous immunoglobulin with plasma exchange revealed no difference between the two therapies (effect size -0.07, 95% confidence interval -0.76 to 0.63.) The proportion of patients with a significant improvement did not differ significantly between prednisolone and intravenous immunoglobulin (relative risk of 0.91 (95% CI 0.50 to 1.68). Also, no difference in mean improvement on the disability scale was found at two weeks (effect size -0.12, 95% confidence interval -0.68 to 0.45) or six weeks (effect size -0.07, 95% confidence interval -0.63 to 0.50) between prednisolone and intravenous immunoglobulin. There were no statistically significant differences in frequencies of side effects between the three types of treatment. REVIEWER'S CONCLUSIONS: The evidence from randomised controlled trials shows that intravenous immunoglobulin improves disability for at least two to six weeks compared with placebo, with a number needed to treat of three. During this period it has similar efficacy to plasma exchange and oral prednisolone. Since intravenous immunoglobulin, plasma exchange and prednisolone seem to be equally effective, it is currently uncertain which of these treatments should be the first choice. Cost, side effects, duration of treatment, dependency on regular hospital visits and ease of administration all have to be considered before such a decision can be made.

Humans↗

PubMed2002

Chronic inflammatory demyelinating polyradiculoneuropathy.

In chronic inflammatory demyelinating polyradiculopathy differing clinical subtypes are beginning to emerge as has already occurred with the Guillain-Barré syndrome. However, neither pathogenic correlates nor particular therapeutic approaches have yet been defined for these subgroups. The neurophysiological techniques of terminal latency index and of modified F ratio help differentiate chronic inflammatory demyelinating polyradiculopathy from IgM paraproteinaemic neuropathy. Diagnosis may be assisted by magnetic resonance imaging studies in which enlarged nerve roots and plexuses and gadolinium enhancement may be evident. Further insight into pathogenesis has come from studies showing pathogenic antibodies in a small percentage of patients. Immunohistological studies examining the presence of adhesion, co-stimulatory and antigen presenting molecules in nerve biopsies have shown that T cell activation can be initiated and perpetuated within nerve and that Schwann cells possess the necessary markers to function as antigen presenting cells. Recent clinical trials have confirmed the therapeutic short term efficacy of intravenous immunoglobulin and Prednisone.

Autoantibodies↗

PubMed2005

A review of the medical management of chronic inflammatory demyelinating polyradiculoneuropathy.

Chronic idiopathic demyelinating polyradiculoneuropathy (CIDP) is a rare condition, but merits consideration due to its disabling consequences for patients and the growing existence of efficacious therapies during the last few decades. The first step is to characterise this neuropathy among the chronic dysimmune polyneuropathies, according to clinical, electrophysiologicalal and sometimes pathologicalal and immunochemical criteria. Typical CIDP is currently defined by criteria which have progressively improved since the first attempt made by an Ad Hoc Subcommittee of the American Academy of Neurology in 1991. However, CIDP may be associated with several concurrent diseases, and other chronic demyelinating polyneuropathies may be considered as either subtypes of CIDP, such as sensory CIDP and multifocal acquired sensory and motor neuropathy, or frontiers of CIDP, such as multi-focal motor neuropathy and polyneuropathy associated with monoclonal gammopathy. These considerations are helpful in the choice of treatments, as the response to immunomodulatory agents is different according to the type of the dysimmune neuropathy. CIDP is considered to be an immune-mediated disorder and may respond dramatically to numerous short-term therapies, such as corticosteroids, plasma exchanges, or intravenous immunoglobulin. The aim of this review is both to summarise the main results of the published open and randomised controlled trials for CIDP, and to provide some information about randomised controlled trials currently in progress. The objectives of the current and future trials are firstly, to choose the best regimen for short-term treatments, and secondly, to test new immunosuppressants in long-term therapy, if the neurological condition requires it.

Clinical Trials as Topic↗

PubMed2003

Diffuse spinal and intercostal nerve involvement in chronic inflammatory demyelinating polyradiculoneuropathy: MRI findings.

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an uncommon demyelinating disorder with a relapsing and remitting or continuously progressive course. Hypertrophic nerve roots, sometimes associated with gadolinium enhancement, has been reported more commonly in lumbar spine and less commonly in the brachial plexus and cervical roots; however, diffuse involvement of intercostal nerves bilaterally has never been reported previously. We present MRI findings which include diffuse enlargement and mild enhancement of roots and extraforaminal segments of nerves in all segments except a short segment between T12-L2 as well as all the intercostal nerves in a case of CIPD with a 10-year history.

Biopsy, Needle↗

PubMed1988

Absence of antimyelin antibodies and serum demyelinating factors in most patients with chronic inflammatory demyelinating polyradiculoneuropathy.

Patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) often respond to plasma exchange. One explanation for this is that circulating factors, such as antibodies or toxic factors, are removed from the serum. We studied CIDP nerves and CIDP sera for the presence of antibodies to myelin, Schwann cells and galactocerebroside, using immunofluorescence and enzyme-linked immunosorbent assays. On 1 of 28 CIDP nerve biopsies there was evidence of an IgG antibody bound to myelin. One of 57 CIDP sera had evidence of an antimyelin antibody, compared with 12 of 68 Guillain-Barré syndrome sera. We failed to find evidence of antibodies to Schwann cells or galactocerebroside in CIDP. The serum from only 1 of 11 patients showed demyelinating activity after direct intraneural injection. Thus these techniques do not demonstrate the presence of humoral factors to the tested antigens in the majority of CIDP patients. However, more sensitive methods may demonstrate antibodies to these antigens. Alternatively, CIDP may be associated with antibodies or factors toxic to other antigens, or else the response to plasma exchange may not be due to removal of a humoral factor.

Autoantibodies↗

PubMed2002

Proposed revised electrophysiological criteria for chronic inflammatory demyelinating polyradiculoneuropathy.

Electrophysiological criteria for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) were proposed by an Ad Hoc Subcommittee of the American Academy of Neurology (AAN) in 1991. Only 60% of CIDP patients fulfilled these criteria, which therefore appear poorly sensitive. We therefore sought to revise the electrophysiological criteria. We selected 40 CIDP patients and compared them with 35 patients with axonal polyneuropathy, 116 patients with Charcot-Marie-Tooth type 1A (CMT1A) disease, and 66 patients with immunoglobulin M (IgM) monoclonal gammopathy. The proposed electrophysiological criteria identified 90% of the CIDP patients, although 3% of patients with axonal polyneuropathy were falsely identified. For the CIDP patients, sensitivity and specificity were 90% and 97%, respectively. Of the patients with IgM monoclonal gammaglobulin of undetermined significance (MGUS) and CMT1A, 100% fulfilled these new criteria, whereas 90% and 97%, respectively, fulfilled the AAN criteria. These results suggest that the AAN criteria are more appropriate for IgM MGUS and CMT1A patients than for CIDP patients. We therefore propose new electrophysiological criteria for CIDP that appear to have better sensitivity.

Adult↗