Idiopathic Chronic Inflammatory Demyelinating Polyneuropathy Research Articles

Effects of oral steroid sparing immunosuppressive drugs in long term maintenance treatment of chronic inflammatory demyelinating polyradiculoneuropathy.

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an acquired treatable autoimmune disorder. Due to limited availability and affordability of IV immunoglobulins and therapeutic plasma exchange in Pakistan, oral immunosuppressive drugs (ISDs) are used despite limited role in literature. The study aimed to determine the response to ISDs in CIDP patients by assessing the frequency of remission, reduction of disability using a neuropathy related disability score called Inflammatory Neuropathy Cause and Treatment score (or INCAT score), as well as reduction in steroid maintenance dose. The retrospective observational study of six months duration (May to October, 2020) was carried out in Aga Khan University Hospital, Karachi, Pakistan. Medical record of all the patients with idiopathic CIDP taking oral ISDs in last five years was selected which included bio-data, clinical signs and symptoms, medication details, and INCAT scores. Descriptive statistics were described i.e. frequency, percentages, mean/standard deviation using Microsoft Excel v.2021. Out of thirteen patients, Azathioprine was used in nine, Mycophenolate mofetil in two and Cyclosporine in two, with remission (INCAT score improvement ≥ 1) achieved in eight, one and zero patients respectively. Duration of ISDs ranged from three to twenty-four months (average 15.8 months). Patients with monoclonal paraproteinemia and prior exposure to ISDs had a poor response to the introduction of subsequent ISDs. The study describes preliminary experience of the potential role of relatively cheaper and more convenient oral ISDs (especially Azathioprine) as an alternative or sparing agent to first line agents for CIDP and sets the stage for larger scale studies and randomized controlled trials.

Red Flags for Chronic Inflammatory Demyelinating Polyradiculoneuropathy Associated with Sarcoidosis or Connective Tissue Diseases.

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a rare autoimmune disorder of the peripheral nervous system. Diagnosis relies on clinical and electrophysiological criteria. Various disorders requiring specific treatment regimens may be associated with CIDP, including sarcoidosis (SAR-CIDP) and connective tissue disease (CTD-CIDP). Therefore, it is important to distinguish between CIDP, SAR-CIDP and CTD-CIDP. In this retrospective monocentric study, we analyzed 16 patients with SAR-CIDP and 11 with CTD-CIDP and compared them with a group of 17 patients with idiopathic CIDP. SAR-CIDP patients had a frequently acute or subacute CIDP onset. CTD-CIDPs were mostly Sjögren's syndrome and lupus, and patients had a chronic onset. An older age at onset (64.5 vs. 54 years, p = 0.04), more atypical presentation (19/25 (76%) vs. 6/17 (35%), p = 0.008), acute/subacute onset of symptoms (15/25 (60%) vs. 1/17 (6%), p = 0.0004) and more frequent weight loss (7/16 (44%) vs. 0/17 (0%), p = 0.017) were identified SAR-CIDP and CTD-CIDP groups. Response to intravenous immunoglobulin therapy was lower in the combined SAR-CIDP and CTD-CIDP group (44% versus 82%, p = 0.005). As sarcoidosis and CTDs might be associated with CIDP and require specific management, the "red flags" mentioned above should be kept in mind by clinicians managing patients with CIDP.

Pathology of Initial Axon Segments in Chronic Inflammatory Demyelinating Polyradiculoneuropathy and Related Disorders.

The diagnosis of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is based on a combination of clinical, electrodiagnostic and laboratory features. The different entities of the disease include chronic immune sensory polyradiculopathy (CISP) and autoimmune nodopathies. It is debatable whether CIDP occurring in the course of other conditions, i.e., monoclonal IgG or IgA gammopathy, should be treated as a separate disease entity from idiopathic CIDP. This study aims to evaluate the molecular differences of the nodes of Ranvier and the initial axon segment (AIS) and juxtaparanode region (JXP) as the potential cause of phenotypic variation of CIDP while also seeking new pathomechanisms since JXP is sequestered behind the paranode and autoantibodies may not access the site easily. The authors initially present the structure of the different parts of the neuron and its functional significance, then discuss the problem of whether damage to the juxtaparanodal region, Schwann cells and axons could cause CIDP or if these damages should be separated as separate disease entities. In particular, AIS's importance for modulating neural excitability and carrying out transport along the axon is highlighted. The disclosure of specific pathomechanisms, including novel target antigens, in the heterogeneous CIDP syndrome is important for diagnosing and treating these patients.

Value of terminal latency index and sensory electrophysiology in idiopathic and diabetic chronic inflammatory demyelinating polyradiculoneuropathy

ObjectivesTo evaluate sensory electrophysiology, terminal latency index (TLI), and treatment response in idiopathic and diabetic chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). MethodsWe performed a retrospective review of 147 patients with CIDP who underwent electrodiagnostic evaluation (January 2000–December 2015). Eighty-nine patients fulfilled electrophysiological criteria described by the Ad hoc Subcommittee of the American Academy of Neurology and Albers et al. Fifty-eight patients were divided into idiopathic (N = 40) and diabetic (N = 18) groups. These groups were compared for age, sex, cerebrospinal fluid protein, response to treatment, sensory response abnormalities, and TLI measurements using chi-square tests for binary and categorical variables and using t-tests and mixed-effects models for continuous variables. ResultsThe difference in abnormal rates of sensory responses was significant for the sural nerve, with the idiopathic group having a lower rate than the diabetic group (80% vs. 100%, p < 0.001). No group differences in the TLI measurements were significant. ConclusionsSural sensory responses may have some value in differentiating idiopathic CIDP from diabetic CIDP. Larger prospective studies are needed to confirm our findings. SignificanceOur study suggests that abnormal sural sensory potentials may have some significance in differentiating idiopathic CIDP from diabetic CIDP.

A comparative study of CIDP in a cohort of HIV-infected and HIV-uninfected patients.

Objective:To investigate differences in clinical presentation, electrodiagnostic measures, CSF changes, and treatment outcome measures in HIV-infected and HIV-uninfected patients with chronic inflammatory demyelinating polyneuropathy (CIDP).Methods:A retrospective analysis of medical records of all patients meeting the European Federation of Neurology diagnostic criteria for idiopathic CIDP was performed in 2 neuromuscular units in Kwa-Zulu Natal between 2003 and 2015.Results:Eighty-four patients were included in the study; 39 were HIV-infected and 45 were HIV-uninfected. Among the HIV-infected patients, the majority were younger, were female, and had a monophasic progressive illness. Eighty-six percent (86%) were corticosteroid-responsive and 76% were in remission within 6–12 months requiring no further treatment. Among the HIV- uninfected patients, the majority were older, were male, and had a relapsing-remitting course. Twenty-seven percent (27%) were corticosteroid-responsive, 95% required combination therapy, and 33% were not in remission by 18 months on therapy.Conclusion:This study shows that HIV-infected patients with CIDP were younger, were more often female, displayed a monophasic progressive course, were highly steroid-responsive, and went into remission within 12 months of corticosteroid initiation.

Incidence and Natural History of Idiopathic Chronic Inflammatory Demyelinating Polyneuropathy: A Population-Based Study in Iceland

Background and Aim: We report a population-based study conducted in Iceland to determine the incidence, clinical characteristics and prognosis of idiopathic chronic inflammatory demyelinating polyneuropathy (CIDP) during a 21-year period. Methods: Cases were identified from the records of all practicing neurologists in the country, the only neurology department in the country and both neurophysiology laboratories. All index cases met the 2010 European Federation of Neurological Societies/Peripheral Nerve Society criteria for CIDP. Results: Nineteen individuals fulfilled the diagnostic criteria during the study period. The average annual incidence was 0.3/100,000 (95% CI 0.04-2.47). There were 14 men (74%) in a gender ratio of 1:2.8. The mean age at diagnosis was 57 (range 19-81 years): women, 36 years and men, 63 years; p = 0.0006. The disease course was remitting-relapsing in 21% and chronic progressive or monophasic in 79%. The average length of follow-up was 6.9 years. The standardized mortality ratio for the 21-year study period was 0.9 (95% CI 0.3-2.2). Conclusion: We believe we have identified all diagnosed with CIDP in Iceland during a 21-year period. Many had no or only limited disease progression over the years and mortality is not increased compared with the general population.

CIDP in a HIV endemic population: A prospective case series from Johannesburg, South Africa

ObjectiveTo describe patients with Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP) in Johannesburg, South Africa, a setting of high HIV prevalence, and to determine the influence, if any, of HIV on CIDP. MethodsPatients were recruited prospectively. The study design was a hospital based case series of unselected consecutive CIDP patients. CIDP was diagnosed according to the European Federation of Neurological Societies/Peripheral Nerve Society criteria for the diagnosis of CIDP (First Revision). Demographic, clinical, laboratory and electrophysiological data were documented. ResultsTwenty three patients with CIDP were recruited over a two year period. Mean age was 38years. The female to male ratio was 3.6:1. Less than half (43%) had a raised cerebrospinal fluid (CSF) protein. All patients had idiopathic CIDP, three had associated diabetes mellitus. Ten patients (43%) were HIV positive. Thirteen patients were HIV negative. Clinical and electrophysiological characteristics were identical in the two groups. In the HIV positive group all the patients were black females. The CD4 counts ranged from 87 to 747cells/mm3. HIV positive status was associated with a progressive disease course and significantly with a CSF lymphocytic pleocytosis (p=0.007). Albuminocytological dissociation was associated with HIV negative status. ConclusionsTesting for HIV in patients with CIDP in a region of high HIV prevalence is recommended.CSF lymphocytic pleocytosis occurs in HIV associated CIDP.

Les polyradiculonévrites inflammatoires démyélinisantes chroniques

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a demyelinating chronic neuropathy of immune origin whose diagnosis is based upon clinical, biological and electrophysiological data; previously critical to the diagnosis the nerve biopsy is now restricted to the rare situations where accurate diagnosis cannot be reached using these data alone. CIDP are mainly idiopathic, but a few associated diseases must be sought for as they require specific attention. Such associated diseases must particularly be discussed when the manifestations are severe or resistant to immunomodulating or immunosuppressive agents. Indeed, idiopathic CIDP are usually responsive to these treatments. The effectiveness of these treatments is limited by the importance of the secondary axonal loss. The dependence or the resistance may sometimes justify the association of several immunomodulating treatments. A single randomized controlled trial support the use of cytotoxic drugs and none with rituximab.

A comparative study of chronic inflammatory demyelinating polyradiculoneuropathy with and without diabetes mellitus

Diabetes mellitus (DM) is occasionally associated with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) raising the question of coexistence or etiological link. The study compares, neurophysiological and outcome of CIDP patients with and without DM. Consecutive CIDP patients were subjected to detailed clinical evaluation, haematology, serum chemistry, vasculitis profile, paraproteins, myeloma screening and cerebrospinal fluid (CSF) examination. Electrodiagnostic (EDx) tests included motor and sensory conduction and F-wave studies. The patients were treated with oral prednisolone 1 mg/kg/day with or without azathioprine 1-2 mg/kg and followed up for 6 months. The clinical and EDx finding in CIDP with and without DM were compared. Thirty-five CIDP patients were included and nine had DM. CIDP with diabetes (CIDP-D) had higher frequency of autonomic dysfunction. In CIDP-D, motor (38.9% vs. 16.7%) and sensory (40.7% vs. 14.1%) nerve conductions were more frequently unrecordable or had reduced compound muscle action potential (CMAP) amplitude. F-waves were also more frequently unrecordable in CIDP-D (28.8% vs. 12.8%) compared with idiopathic CIDP (I-CIDP). The degree of conduction block was more in I-CIDP. At 6-month follow up, I-CIDP patients improved better than CIDP-D. CIDP-D patients present with higher frequency of autonomic dysfunction, electrophysiological evidences of associated axonal loss and had a poorer outcome at 6 months compared with I-CIDP.

Idiopathic chronic inflammatory demyelinating polyneuropathy: an epidemiological study in Italy

Aim:The clinical and epidemiological characteristics of chronic inflammatory demyelinating polyneuropathy (CIDP) in an Italian population were assessed.Subjects and methods:All subjects with a diagnosis of demyelinating neuropathy after 1990 in Piemonte...

Clinical and subclinical autonomic dysfunction in chronic inflammatory demyelinating polyradiculoneuropathy

Autonomic neuropathy, although common in Guillain-Barré syndrome, is considered rare in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and has not been systematically investigated in that disorder. The present study was aimed at determining the prevalence of autonomic dysfunction and investigating the integrity of autonomic nervous system (ANS) reflexes in CIDP. We studied 17 patients with idiopathic CIDP and 20 healthy controls. Six quantitative autonomic function tests (AFTs) were used: Valsalva ratio, 30/15 ratio, and inspiration-expiration difference for parasympathetic function; and tilt test, handgrip test, and sympathetic skin response for sympathetic function. Eleven patients had symptoms of autonomic dysfunction. AFTs were abnormal in 13 patients. Parasympathetic and sympathetic systems were affected with equal frequency. The tilt test was abnormal most frequently, followed by the 30/15 ratio. Three patients developed postural hypotension with loss of consciousness during the tilt test. Abnormality of AFTs did not correlate with the presence of dysautonomic symptoms; duration, severity, and clinical course of the disease; or with age or gender of patients. Our study suggests a higher frequency of clinical and subclinical involvement of the ANS in CIDP than previously estimated. Dysautonomic symptoms are frequent but are mild. However, upon prolonged passive standing, autonomic failure can lead to loss of consciousness. The subclinical involvement of the ANS affects mainly the sympathetic vasomotor and parasympathetic cardiovascular fibers.

Use of intravenous immunoglobulin for treatment of neurologic conditions: a systematic review

Given the increasing use of intravenous immunoglobulin (IVIG) for various neurologic conditions and uncertainty pertaining to its benefits and harms, a systematic review was conducted of randomized controlled trials (RCTs) evaluating IVIG for all neurologic indications for which there was at least one published trial. For this systematic review, a systematic search strategy was applied to MEDLINE (1966-June 2003) and the Cochrane Register of Controlled Trials (June 2003) to identify potentially eligible RCTs comparing IVIG to placebo or an active control. All dosage regimens were considered. Abstracts were excluded, and no restriction was placed on language of publication. Two investigators independently performed data extraction with a standardized form. Measures of effect were calculated for each trial independently, and studies were pooled based on clinical and methodologic judgment as to its appropriateness. Where pooling of trials was inappropriate, a qualitative discussion of findings is provided. Thirty-seven trials representing 14 conditions were identified. IVIG is more effective than placebo for treatment of relapsing-remitting multiple sclerosis and idiopathic chronic inflammatory demyelinating polyneuropathy. There is also potential benefit for treatment of multifocal motor neuropathy, myasthenia gravis, dermatomyositis, stiff-person syndrome, and Lambert-Eaton myasthenic syndrome. There was insufficient evidence to determine whether IVIG therapy was more effective than plasma exchange for Guillain-Barré syndrome. There was also insufficient evidence regarding paraprotein-associated polyneuropathy. No evidence of benefit was observed for secondary progressive multiple sclerosis or inclusion body myositis.

Different types of chronic inflammatory demyelinating polyneuropathy have a different clinical course and response to treatment

Chronic inflammatory demyelinating polyneuropathy (CIDP) can occur in association with other systemic diseases such as diabetes mellitus (DM) and IgG or IgA monoclonal gammopathy of undetermined significance (MGUS). Whether CIDP that is idiopathic (I-CIDP) or associated with diabetes (CIDP-DM) or MGUS (CIDP-MGUS) differ in clinical presentation, laboratory features, response to treatment, and long-term outcome is unclear, as is the relationship between these coexisting diseases and CIDP. In order to clarify this issue, we began a prospective follow-up study. Thirty-one consecutive patients with untreated CIDP, fulfilling the most restrictive diagnostic criteria, were enrolled over 18 months. Among the patients, 16 were diabetic, 7 had a MGUS, and 8 had an idiopathic CIDP. All patients were treated with IVIg, and the responders were treated again if they relapsed. In all three groups, improvement occurred after treatment. At the end of the follow-up, there was no difference in clinical conditions between groups, but a significant difference existed in the number of relapses and of IVIg administrations. CIDP-DM is a more severe disease, but with a significantly better response to IVIg and fewer relapses, than the other types that we studied.

Chronic inflammatory demyelinating polyradiculoneuropathy in diabetic patients.

This retrospective analysis was undertaken to determine whether a subset of diabetic patients with demyelinating polyneuropathy were similar to patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Ten patients meeting the clinical criteria for idiopathic CIDP were compared to nine patients with diabetes and demyelinating polyneuropathy. The diabetic patients with demyelinating polyneuropathy displayed clinical, electrophysiologic, and histologic features that were similar to those in CIDP patients. All six patients with diabetes and demyelinating polyneuropathy who were treated with immunomodulatory therapy showed a favorable response. Our study highlights the importance of investigating diabetic patients with polyneuropathy in an attempt to identify patients with demyelinating polyneuropathy, because of the likelihood of benefit in these patients from immunomodulatory treatment.

Demyelinating neuropathy in diabetes mellitus.

Recent studies have reported that patients with diabetes mellitus (DM) have a predisposition to develop chronic inflammatory demyelinating polyneuropathy (CIDP). To determine whether patients with DM have a polyneuropathy fulfilling electrophysiologic criteria for CIDP, and whether CIDP is more frequent in patients with type 1 than in patients with type 2 DM. We prospectively studied the frequency of electrophysiologic changes meeting the criteria for CIDP in patients with DM seen in our electrophysiology laboratory during a 51-month period (period 1). To evaluate the relationship between DM and CIDP, we prospectively determined during a 14-month period (period 2) the frequency of DM in patients seen in our electrophysiology laboratory with other neuromuscular diseases, and the frequency of idiopathic CIDP. During period 1, 120 patients with DM met the electrophysiologic criteria for CIDP (DM-CIDP). The most frequent clinical features of DM-CIDP were those of a predominantly large-fiber sensorimotor neuropathy, with recent motor deterioration and a moderately increased cerebrospinal fluid protein concentration. Twenty-six of the 120 patients were given intravenous immunoglobulin (400 mg/kg per day for 5 days), and 21 patients (80.8%) had significant improvement in the neurologic deficit at the end of 4 weeks of therapy. The DM-CIDP occurred equally in type 1 and type 2 DM. During period 2, 1127 patients were seen. Of these, 189 (16.8%) had DM with various neurologic disorders, including 32 patients (16.9%) with DM-CIDP. Among the remaining 938 patients without DM, 17 (1.8%) had idiopathic CIDP. The odds of occurrence of DM-CIDP was 11 times higher among diabetic than nondiabetic patients (P<.001). Demyelinating neuropathy meeting the electrophysiologic criteria for CIDP occurred in both types of DM, and its occurrence was significantly higher in diabetic than in nondiabetic patients.

Diabetic Lumbosacral Polyradiculoneuropathies.

The pathogenic basis and treatment of diabetic polyradiculoneuropathy is a source of recent controversy as there may be two or more distinct forms of diabetic polyradiculoplexopathy. We believe that the following two categories of diabetic polyradiculoneuropathy can be made on the basis of clinically differences: 1) the more common asymmetric, painful polyradiculoneuropathy; and 2) the rare symmetric, painless, polyradiculoneuropathy. The asymmetric, painful form (also known as diabetic amyotrophy) may have an autoimmune basis, but the etiology is not clear. The natural history for diabetic amyotrophy is spontaneous improvement. Nevertheless, various immunotherapies (eg, corticosteroids and intravenous immunoglobulin (IVIg) have been tried with subsequent improvement in symptoms. Treatment is reserved only for patients with severe ongoing pain, given the significant side effects of these medications in those patients with diabetes. Prednisone and IVIg may help alleviate the pain associated with diabetic amyotrophy. Relief of pain can help patients begin physical therapy earlier, however, there are no prospective, blinded, controlled studies that demonstrate that these treatments lead to an earlier and better recovery of muscle strength compared with the natural history of the disorder. The symmetric, painless form of diabetic polyradiculoneuropathy may in fact represent chronic inflammatory demyelinating polyneuropathy (CIDP) occurring in a patient with diabetes mellitus (DM). Patients with idiopathic CIDP may improve various immunomodulating therapies, including corticosteroid treatment, plasma exchange (PE), and IVIg. In this regard, patients with the symmetric, painless, proximal diabetic polyradiculoneuropathy may also respond to corticosteroids, plasma exchange, IVIg, azathioprine, or cyclophosphamide. However, as with diabetic amyotrophy, some patients improve spontaneously without treatment. In still other patients, the neuropathy appears unresponsive to immunotherapy. In such patients, this polyradiculoneuropathy might be caused by metabolic dysfunction associated with DM. Unfortunately, from a clinical, laboratory, and electrophysiologic standpoint, it is impossible to distinguish the patients with a symmetric, painless diabetic polyradiculoneuropathy who might respond to therapy. A trial of PE can be useful in identifying patients who might have a polyradiculoplexopathy that is responsive to immunotherapy. If patients respond to PE, they may continue to receive intermittent exchanges or be switched over to prednisone or IVIg.

Paraproteinemic neuropathy

Few prospective, randomized, placebo-controlled trials have been performed to guide clinicians in the management of neuropathies seen in the setting of monoclonal gammopathies (paraproteins). Recommendations must be made on the basis of clinical experience and information gleaned from various uncontrolled and open-label trials. In every instance, decisions concerning therapy must be based on the clinical setting in which the paraprotein occurs. Treatment of paraproteinemic neuropathies associated with multiple myeloma, amyloidosis, and Waldenström's macroglobulinemia should be directed at the treatment of the underlying disease. These neuropathies often remain recalcitrant to therapy. If the paraprotein results from cryoglobulinemia due to hepatitis C virus infection, interferon-alpha (with or without ribavirin) provides optimal subjective and objective relief from symptoms. For neuropathy associated with osteosclerotic myeloma (POEMS syndrome) and solitary bone lesions, radiation therapy is the most effective and least toxic initial therapy. In those patients with monoclonal gammopathies of undetermined significance (MGUS), consideration of the clinical syndrome may be very helpful in selecting appropriate treatment. Patients who fulfill diagnostic criteria for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) are best treated in a manner similar to that used for idiopathic CIDP (ie, with intravenous immunoglobulin, plasma exchange, and corticosteroids). Class I evidence documents plasma exchange to be effective in peripheral neuropathies associated with MGUS of the IgG and IgA, but not IgM, types. The most difficult cases to treat are those with peripheral neuropathies associated with IgM monoclonal gammopathies, with or without reactivity to myelin-associated glycoprotein (MAG). A number of published case series propose therapeutic regimens for these conditions, yet optimal treatment remains to be established. In many cases, mildly symptomatic patients should not be subjected to the morbidity associated with current treatment regimens. In those patients requiring treatment, this author initially tries plasma exchange, followed by a course of chlorambucil if the symptoms and signs are predominantly sensory. For cases with rapid progression or significant disability, a regimen of monthly pulses with prednisone and cyclophosphamide is recommended. If improvement does not ensue, a trial of a newer agent, such as rituximab, is recommended. Supportive treatment with physical therapy, orthotics, and ambulatory aids enhances patient independence at a relatively low cost.

Diagnostic criteria for demyelinating polyneuropathy associated with monoclonal gammopathy

In order to define diagnostic criteria for the demyelinating polyneuropathy associated with monoclonal gammopathy of undetermined significance (MGUS), we compared 30 patients with idiopathic chronic inflammatory demyelinating polyneuropathy (CIDP) without a monoclonal gammopathy, with 29 patients with polyneuropathy associated with MGUS. All 59 patients fulfilled research criteria for CIDP. In the patients with MGUS, sensory symptoms and signs predominated, there was usually no cranial nerve involvement, and the neuropathy was symmetrical with a slowly progressive course. On electrophysiological examination, an abnormal median nerve sensory action potential in combination with a normal sural nerve action potential (AMNS) was not found. In idiopathic CIDP patients, a preceding infection was frequent, motor features predominated, there was often cranial nerve involvement, the neuropathy could be asymmetrical, and AMNS was frequently found. Diagnostic criteria for demyelinating polyneuropathy associated with MGUS are presented. © 2000 John Wiley & Sons, Inc. Muscle Nerve 23: 73–79, 2000

Treatment of chronic inflammatory demyelinating polyneuropathy.

The management of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is the main topic of this review. A few comments will also be made about treatment of the demyelinating form of paraproteinaemic demyelinating polyneuropathy (PDN) and of multifocal motor neuropathy (MMN). The review briefly describes the main characteristics of these neuropathies, and examines case series and trials which evaluated the principal therapeutic strategies for CIDP, PDN and MMN, such as intravenous immunoglobulin (IVIg) therapy, steroid treatment, plasma exchange and immunosuppressor administration. Controlled trials demonstrated that IVIg, steroid treatment and plasma exchange are effective in CIDP. For PDN the therapeutic strategies are the same as for idiopathic CIDP, but usually the clinical response is poorer. For MMN, IVIg therapy is definitely the first choice treatment.

Interferon beta normalizes suppressor cell function in dysimmune neuropathies

We found a defective suppressor cell function in vitro both in idiopathic chronic inflammatory demyelinating polyneuropathy and in paraproteinemic neuropathy with antibodies to sulfated glucuronyl paragloboside. In the presence of interferon β, suppressor cell function was normalized. Our results suggest that a decreased suppressor cell function plays a pathogenetic role in dysimmune neuropathies. Interferon β might represent an adjunctive therapy in CIDP both acting on a defective blood–nerve barrier and normalizing an otherwise defective suppressor cell function.