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Neurology India
Medknow Publications on behalf of the Neurological Society of India
ISSN: 0028-3886 EISSN: 1998-4022
Vol. 58, Num. 3, 2010, pp. 351-360

Neurology India, Vol. 58, No. 3, May-June, 2010, pp. 351-360

Review Article

Management strategies in chronic inflammatory demyelinating polyradiculoneuropathy

1 Department of Neurology and Ophthalmology, Michigan State University, East Lansing, Michigan, USA
2 Michigan State University, East Lansing, Michigan, USA
3 Department Neurology, Barrow Neurological Institute, Phoenix, Arizona, USA
Correspondence Address: Suraj Ashok Muley, Department of Neurology, Barrow Neurological Institute, 500 W Thomas Road, Suite 720, Phoenix, AZ 85013, USA,

Date of Acceptance: 17-Jun-2010

Code Number: ni10097

PMID: 20644261
DOI: 10.4103/0028-3886.65534


Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a chronic, proximal and distal, asymmetrical or symmetrical, motor and sensory demyelinating polyneuropathy with a progressive course for at least 2 months. The accurate diagnosis is crucial as CIDP is amenable to treatment. Recent advances have provided new strategies and options for management of this syndrome. In this article, we review the clinical and diagnostic features as well as discuss recent insights and treatment strategies along with our experience in the management of patients with CIDP.

Keywords: Chronic demyelinating neuropathy, chronic inflammatory demyelinating neuropathy, variants of chronic demyelinating neuropathy


Chronic inflammatory demyelinating polyradiculo-neuropathy (CIDP) is a sensorimotor neuropathy that evolves over at least 2 months with either a progressive or a relapsing remitting course. [1],[2] Variants of CIDP with distinct clinical presentations have been described and their recognition is important because of varied treatment responses. In general, patients with CIDP present with acute to subacute onset of proximal and distal weakness, and sensory symptoms that have a progressive or stepwise course for over 2 months. [1],[2] About 15% of patients have an acute onset that can be confused with Guillain-Barrι syndrome (GBS) and the diagnosis of CIDP is made only in retrospect. [3] Physical examination shows hyporeflexia or areflexia that is out of proportion to the degree of weakness, suggesting that the underlying pathology is that of segmental demyelination. [2],[4]

Various diagnostic criteria have been proposed for CIDP. These criteria vary in their specificity and sensitivity. The American Academy of Neurology (AAN) criteria were initially proposed in 1991 mainly for research purposes and had a specificity of 100% [2],[5] but lacked sensitivity (45.7%) and were found to be inadequate for routine clinical practice. The recently proposed European Federation of Neurological Societies and Peripheral Nerve Society (EFNS/PNS) guidelines on management of CIDP [4] provide diagnostic criteria that may have greater clinical applicability and improved sensitivity of 81.3% and specificity of 96.2% compared with the AAN criteria. The EFNS/PNS guidelines have taken into consideration the cerebrospinal fluid (CSF) changes and response to immunomodulatory treatment. Diagnostic role of enhancing or enlarged nerve roots on neuroimaging were also incorporated, but the role of nerve biopsy remained limited [4] [Table - 1a and Table - 1b]. There are numerous other criteria proposed, however, [6],[7],[8] the discussion of those is beyond the scope of this article. Nevertheless, clinical experience of neurologists world over suggests that there are a significant number of patients who do not satisfy these diagnostic criteria but still respond to treatments that are effective for CIDP. [9] Thus it is prudent to consider the diagnostic possibility of CIDP when the history, physical examination, and electrodiagnostic studies suggest an acquired demyelinating neuropathy.

Chronic inflammatory demyelinating polyradiculoneuropathy-variants

Over the last few decades, variations in the clinical and electrodiagnostic features of CIDP have been described. These diseases have a common denominator in that all are related to focal segmental demyelination but have distinctive treatment responsiveness, suggesting varied underlying pathophysiologic mechanisms.

Distal acquired demyelinating symmetric neuropathy

Patients with distal acquired demyelinating symmetric neuropathy have a slowly evolving, sensory predominant larger fiber neuropathy with relative preservation of motor function that typically presents with sensory ataxia. A significant proportion (66%) of these patients have IgM paraproteinemia and/or antibodies to myelin-associated glycoprotein (MAG). [10] Distally accentuated conduction slowing is the electrophysiologic hallmark of these neuropathies. [11] It is important to rule out myeloproliferative disorders in these patients. Recognition of this disorder is important since response to treatment is different from that in CIDP.

Multifocal motor neuropathy

Patients with multifocal motor neuropathy (MMN) present with focal weakness in the distribution of individual peripheral nerves without sensory symptoms. Electrodiagnostic testing reveals partial conduction block related to segmental demyelination with sparing of the sensory responses even through the site of conduction block. [12] In some patients, who are otherwise indistinguishable from the prototypic patients, conduction block may not be demonstrable with routine electrodiagnostic testing. [13],[14] Anti-GM1 antibodies are seen in a proportion of these patients but are not required for the diagnosis. [15] This disorder can be easily confused with motor neuron disease because of the lack of sensory findings, but careful clinical examination that reveals weakness in a peripheral nerve rather than segmental distribution and a high index of suspicion can aid in the diagnosis. Recognition is important since these patients may respond to immunomodulatory treatments.

Multifocal acquired demyelinating sensory and motor neuropathy: Lewis-Sumner syndrome

These patients present with weakness and numbness in the distribution of individual peripheral nerves related to focal demyelinative conduction block. [16] Some of these patients can evolve to develop diffuse confluent involvement that can be indistinguishable from CIDP. [17] Given that response to treatment may be similar to that seen with CIDP, there is some controversy as to whether this is a distinct pathophysiologic entity or whether it belongs to the same spectrum as CIDP.


Diagnosis of CIDP is primarily made on the basis of clinical presentation and electrodiagnostic findings. A clinical picture of progressive weakness and numbness over a duration of 8 weeks with hyporeflexia that is out of proportion to the degree of weakness is characteristic. [2],[4] The presence of proximal weakness in a neuropathy suggests involvement of nerve roots and is common in patients with CIDP. [1]

Electrodiagnostic findings are also important diagnostic tools and show incongruous slowing of conduction velocities related to patchy segmental demyelination and remyelination. [2] Typical findings on nerve conduction studies include at least 20-30% slowing of conduction velocities (compared with lower limit of normal [LLN]) at sites that are not prone to entrapment, 125%-150% (compared with LLN) prolongation of distal latencies and F-wave latencies. [2] Segmental amplitude change of 20-50% in the compound muscle action potential related to partial conduction block and/ or abnormal temporal dispersion (>15% change in duration between proximal and distal sites can also be seen. [2],[4] Conduction block is more common in patients with MMN and Lewis-Sumner syndrome (LSS). [14],[16] Reduction in the motor and sensory amplitudes are also seen and are commonly related to secondary axonal degeneration but can also be related to very distal conduction block. [18] Electromyography though not included in the diagnostic criteria, if performed shows findings that are consistent with acute and/or chronic partial denervation but reduced recruitment that cannot be explained on the basis of axon loss suggests the possibility of conduction block that may be proximal and not easily evident on nerve conduction studies. [19]

CSF analysis can show cytoalbuminogenic dissociation with elevated protein levels without elevation in the white cell count. When elevation of the white cell count is seen human immunodeficiency virus (HIV) infection should be considered. [2] When CSF analysis is done early on in the disease process, elevation of protein level may not be seen. [20] Rarely, patients with idiopathic CIDP can have CSF pleocytosis. [20]

The role of nerve biopsy in the diagnosis of CIDP has been diminishing. Several studies have shown that the nerve biopsy histopathology is not significantly different in patients with axonal neuropathy compared with CIDP. [5],[21] We reserve nerve biopsy only for patients with atypical clinical presentation where the diagnosis is not obvious with routine testing.

Other diagnostic workup to exclude concomitant diseases include complete blood counts and erythrocyte sedimentation rate, hepatic and renal function tests, antinuclear antibody, extractable nuclear antigen autoantibodies, thyroid function tests, glucose tolerance test, serum and urine immunofixation or immunoelectrophoresis, HIV and hepatitis serology, antibodies to Borrelia burgdorferi or Lyme disease, and angiotensin I-convering enzyme levels. [2] Chest radiograph and in patients unresponsive to treatment, a skeletal survey may be repeated. [4] Hereditary neuropathy may be excluded with examination of the family members or performing analysis for PMP22 deletion or duplication.


Goals of treatment

Effective treatment of CIDP is predicated on early diagnosis when the degree of axon loss is relatively mild. Once there is significant secondary axon loss, response to treatment can be incomplete. The goals of treatment are to improve weakness, prevent disability, and induce and sustain a remission. It is important to achieve these goals in a cost-effective manner with minimal treatment-related adverse effects.

General approach

The firstline treatments for CIDP include prednisone, intravenous immunoglobulin (IVIg), and plasma exchange (PE) [Table - 2], [Figure - 1]. These treatments have been shown to be effective, but whether one treatment is better than the others (in terms of improving weakness and inducing a remission) remains unclear. Although IVIg and PE have been shown to be effective in placebo-controlled trials, it remains unclear whether these treatments are effective in inducing a long-term remission. [22] Prednisone has also been shown to be effective in improving weakness but long-term treatment that is often necessary in CIDP results in severe steroids-related adverse effects. Newer treatment regimens that use high-dose pulsed steroids may be better tolerated and perhaps more effective in improving weakness and inducing remission but need further study. [23],[24] Long-term immunosuppressants, such as azathioprine and mycophenolate mofetil, can be used, when the primary treatment is not sufficient and as steroid or IVIg-sparing agents. In intractable cases, cyclophosphamide with or without stem cell rescue has been shown to be effective. [25],[26],[27] These treatment options have been outlined in [Table - 2] and [Table - 3].

We typically start treatment with corticosteroids as they are proven to be cost effective and clinically efficacious in CIDP treatment, through long-term clinical experience with its use. [28] The authors typically start with pulsed steroids administered in a once-a-week regimen. [24] In patients who have no response to treatment within 1-3 months [28] or unable to tolerate steroid-related adverse effects, we would switch to IVIg. [4] Daily prednisone, IVIg, or PE can also be used as firstline treatments but there is a subset of patients who may not respond to IVIg because of genetic predisposition. [29] In general, about 66% of patients respond to one of the firstline treatments (corticosteroids, IVIg, or PE). [30] Once adequate improvement is accomplished, a slow taper of the primary therapy is instituted with close surveillance to monitor for relapse. We use a 120-point neuromuscular scale to monitor strength and Inflammatory Neuropathy Cause and Treatment (INCAT) disability scale to monitor function. [24] Long-term immunosuppressants can be used if there is difficulty with the taper or if response to treatment is suboptimal. It is important to remember that a lack of complete resolution of weakness may be related to axonal degeneration, which may not respond to more aggressive immunotherapy and may improve over time with neural repair mechanisms.

Individual therapies


The efficacy of corticosteroids for treatment of CIDP has been described in only one randomized study. [31] In this study, some improvement in strength and other objective measures was observed compared with patients who received no treatment. Subsequently, other studies have compared corticosteroids with other treatment modalities or have used steroids in conjunction with other agents. [7]

Forms and dosing: The randomized study [32] used prednisone in the dose of 120 mg every other day, with subsequent tapering of the dose to 0 mg over 12 weeks, and there was improvement in 86% of treated patients. The study by Hughes et al., [7] used prednisone 60 mg/day with tapering of the dose to 10 mg over six weeks. [7] Other studies have typically used prednisone in doses of 40-100 mg for 2-4 weeks, followed by a gradual or alternate day taper. [32],[33]

Molenaar and colleagues [34] used pulsed high-dose dexamethasone, 40 mg daily for 4 days every 28 days for 6 months, in CIDP and had shown a 70% response rate with fewer adverse effects. However, pulsed high-dose dexamethasone treatment did not induce remission more often than prednisone treatment in pulsed high-dose dexamethasone vs standard prednisone treatment for CIDP (PREDICT) study. High-dose dexamethasone could be considered as induction therapy in CIDP, but comparison with IVIg treatment is needed. [23] A retrospective study showed that intravenous methylprednisone in doses of 1000 mg IV daily for 3-5 days, followed by once a week for 1 month, and later once every 2-12 weeks was as effective as IVIg and oral prednisone, but with fewer side effects from corticosteroids compared with oral prednisone. [35] Oral methylprednisone at a dose of 500 mg once a week had shown improvement in the majority of patients with a remission rate of 60% that was sustained over several years; the treatment was well tolerated in most patients with a syndrome of unpleasant taste and insomnia lasting for up to 2 days after each dose. [24] Corticosteroids are not useful in certain forms of CIDP, such as purely motor CIDP and MMN, [36],[37] and may actually worsen the weakness.

Intravenous immunoglobulin

IVIg was approved for treatment of CIDP in the United States in 2008. There have been 7 randomized controlled trials, involving 287 patients, that have proven the efficacy of IVIg. [22],[38],[39],[40],[41],[42] The IVIg for CIDP (ICE) trial [39] was a double-blinded randomized, placebo-controlled trial involving 117 patients with a crossover design. The study showed that 54% of patients treated with IVIg as opposed to 21% of patients receiving placebo showed improvement in adjusted INCAT disability score that was maintained during the crossover time.

There is a 0-3% risk of thromboembolic events, such as stroke, myocardial infarction, pulmonary embolism, in patients receiving IVIg. [43] Renal failure may occur as a complication of older preparations containing high osmolarity sucrose solvent. [44] Patients with preexisting renal disease, diabetes mellitus, hypovolumia, sepsis, concomitant use of nephrotoxic drugs, and age more than 65 years have been identified as risk factors for developing nephrotoxicity. [45] Checking renal function prior to infusion and thereafter has been recommended by the Center for Disease Control and Prevention (CDC). [45] However, this adverse effect has been minimized with use of newer sucrose-free preparations. Reversible vasospasm, headaches, rash, congestive heart failure, transient drop in blood cell counts, [46] and the theoretic risk of transmission of infectious diseases are some of the other rare adverse events reported with the use of IVIg.

Plasma exchange

PE is effective in CIDP and this has been demonstrated in 2 randomized controlled trials involving 47 patients. [47],[48] Adverse effects, such as infection from indwelling catheter, pneumothorax, vessel perforation, hypotension, electrolyte imbalance, citrate-induced hypocalcemia, thrombosis, and bleeding are seen in 3-17% of patients. [49] Patients with MMN may show deterioration with PE. [50]

Long-term immunosuppressants

Steroid sparing agents commonly used to maintain remission include azathioprine and mycophenolate mofetil. Mycophenolate mofetil was shown to be effective in reducing pain or showing minimal improvement in disability scales in an open-label study. [51] Another retrospective study found modest benefit in about 20% of patients and stabilized patient condition, allowing reduction of steroid or IVIg therapy. [52]

There have been small numbers of nonrandomized, open-label studies showing efficacy of other agents, such as cyclosporine, fludarabine, interferons, and etanercept. Intractable and refractory cases may respond to cyclophosphamide and rituximab. However, there is only one double-blinded placebo-controlled study assessing the efficacy of rituximab in patients with anti-MAG demyelinating neuropathy. There was 34% reduction in IgM, 50% reduction in anti-MAG titers, and improvement in INCAT scores in 4 of the 13 patients receiving rituximab. [53] Rituximab may also be effective in CIDP patients associated with thrombocytopenia, [54] diabetes, [55] anti-SGPG IgM antibody, [56] and Evans syndrome. [57] Similarly, there has been a small study reporting the use of cyclophosphamide in conjunction with PE, to be effective in patients with MMN [58] and anti-MAG [59] neuropathy.

In a single patient with IVIg-dependent relapsing CIDP, unresponsive to steroids or conventional immunosuppressive agents, remission was achieved following treatment with alemtuzumab. [60]

Treatment of specific forms of CIDP

MMN: IVIg is the firstline treatment for patients with MMN. [61],[62] Plasmapheresis and corticosteroids have been associated with worsening in these patients, and should be avoided. [35],[36] For patients resistant to IVIg, secondline treatments, such as chemotherapeutic agents are used.

Anti-MAG neuropathy: Anti-MAG neuropathy has a relatively benign course, and treatment is not warranted for most of the patients. However, when treatment is warranted, rituximab is the treatment of choice as it reduces the levels of IgM. [53]

LSS: Patients with LSS may respond to both steroids and IVIg. We typically start treatment with steroids and reserve IVIg for patients who are either unresponsive to treatment or develop intolerable adverse effects.

Sensory variant of CIDP: The treatment for CIDP with sensory predominance is same as in CIDP. However, considering the risks involved in CIDP treatment, we defer treatment only if there is sensory ataxia, or the weakness is severe enough to impair activities of daily living.

Acute presentation of CIDP: About 15% of CIDP patients present with rapidly progressive symptoms. [3] During this time, the clinical picture is indistinguishable from GBS, and the diagnosis is often made only in retrospect. The symptoms continue to progress over 8 weeks in CIDP, whereas they tend to plateau by 4 weeks in GBS. During the acute phase, management consists IVIg and PE.

CIDP with central nervous system (CNS) involvement: There have been several reports suggesting subclinical involvement of the CNS in patients with CIDP. [63],[64],[65],[66],[67] CNS involvement is evidenced by MRI findings and evoked potential testing. Typical lesions of multiple sclerosis (MS) are infrequent in patients with CIDP. [67] The treatment is no different with or without CNS involvement. There is a suggestion that CIDP in patients with CNS involvement is more demyelinating, and hence more responsive to immunotherapy compared with CIDP without CNS involvement that can have axonal damage as well. [64] If clinical presentation warrants, MS or other inflammatory causes of white matter lesions need to be ruled out.

Diabetes and CIDP: There has been suggestion that CIDP is more prevalent in patients with diabetes, [68] although a recent study suggested that the incidence of diabetes was lower in patients with CIDP than in healthy controls. [69] The reported higher incidence of CIDP in diabetes has been attributed to misdiagnosis since conduction slowing is commonly seen in diabetes and overreliance on electrodiagnostic features can be misleading. In our experience, there are rare instances of the coexistence of diabetes and CIDP and in these cases the pathogenic relation between CIDP and diabetes remains unclear. We would consider a diagnosis of CIDP in patients with diabetes if there is progressive proximal and distal weakness, abnormal temporal dispersion, or conduction block at nonentrapment sites on electrophysiologic studies and markedly elevated CSF protein levels. It has been shown that CIDP patients with diabetes have a higher frequency of autonomic dysfunction, unrecordable motor, sensory nerve conductions and F-waves, and reduced compound muscle action potential. Also, the degree of conduction block is higher in CIDP patients without diabetes, than those with CIDP and diabetes. [70] In most instances, definitive and objective response to immunotherapy is used to solidify the diagnosis. We typically start treatment with pulsed oral prednisone. In our experience, despite elevation of blood glucose levels for 1-2 days after each dose, the treatment is well tolerated. In patients who develop intolerable adverse effects with this regimen or in patients who do not respond to treatment, we use IVIg. Close monitoring for renal failure is necessary since diabetics commonly have subclinical nephropathy and IVIg can precipitate renal failure in these patients. Long-term treatment strategies are similar to those for idiopathic CIDP. There has been one study that used SQIg in 2 patients with CIDP, and showed benefit with IVIg. The use of SCIg was well tolerated, and led to stabilization of their disease course. [71] Further controlled studies are required to prove the efficacy of this form of treatment, as it would be ideal for diabetic patients who develop CIDP.


1.Dyck PJ, Lais AC, Ohta M, Bastron JA, Okazaki H, Groover RV. Chronic inflammatory polyradiculoneuropathy. Mayo Clin Proc 1975;50:621-51.  Back to cited text no. 1    
2.Research criteria for diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP): Report from an Ad Hoc Subcommittee of the American Academy of Neurology AIDS Task Force. Neurology 1991;41:617-8.  Back to cited text no. 2    
3.McCombe PA, Pollard JD, Mcleod JG. Chronic inflammatory demyelinating polyradiculoneuropathy-a clinical and electrophysiological study of 92 cases. Brain 1987;110:1617-30.  Back to cited text no. 3    
4.Hughes RA, Bouche P, Cornblath DR, Evers E, Hadden RD, Hahn A, et al. European federation of Neurological Societies/peripheral Nerve Society guideline on management of chronic inflammatory demyelinating polyradiculoneuropathy: Report of a joint task force of the European Federation of Neurological Societies and the Peripheral nerve society. Eur J Neurol 2006;13:326-32.  Back to cited text no. 4    
5.Rajabally YA, Nicolas G, Pieret F, Bouche P, Van den Bergh PY. Validity of diagnostic criteria for chronic inflammatory demyelinating polyneuropathy: A multicenter European study. J Neurol Neurosurg Psychiatry 2009;8012:1364-8.  Back to cited text no. 5    
6.Koski CL, Baumgarten M, Magder LS, Barohn RJ, Goldstein J, Graves M, et al. Derivation and validation of diagnostic criteria for chronic inflammatory demyelinating polyneuropathy. J Neurol Sci 2009;277:1-8.  Back to cited text no. 6    
7.Hughes R, Bensa S, Willison H, Van den Bergh P, Comi G, Illa I, et al. Randomized controlled trial of intravenous immunoglobulin versus oral prednisolone in chronic inflammatory demyelinating polyradiculoneuropathy. Ann Neurol 2001;50:195-201.  Back to cited text no. 7    
8.Saperstein DS, Katz JS, Amato AA, Barohn RJ. Clinical spectrum of chronic acquired demyelinating polyneuropathies. Muscle Nerve 2001;24:311-24.  Back to cited text no. 8    
9.Sander HW, Latov N. Research criteria for defining patients with CIDP. Neurology 2003;60:S9-15.  Back to cited text no. 9    
10.Katz JS, Saperstein DS, Gronseth G, Amato AA, Barohn RJ. Distal acquired demyelinating symmetric neuropathy. Neurology 2000;54:615-20.  Back to cited text no. 10    
11.Kaku DA, England JD, Sumner AJ. Distal accentuation of conduction slowing in polyneuropathy associated with antibodies to myelin-associated glycoprotein and sulphated glucuronyl paragloboside. Brain 1994;117:941-7.  Back to cited text no. 11    
12.Parry GJ, Clarke S. Multifocal acquired demyelinating neuropathy masquerading as motor neuron disease. Muscle Nerve 1988;11:103-7.  Back to cited text no. 12    
13.Pakiam AS, Parry GJ. Multifocal motor neuropathy without overt conduction block. Muscle Nerve 1998;21:243-5.  Back to cited text no. 13    
14.Katz JS, Wolfe GI, Bryan WW, Jackson CE, Amato AA, Barohn RJ. Electrophysiologic findings in multifocal motor neuropathy. Neurology 1997;48:700-7.  Back to cited text no. 14    
15.Pestronk A, Cornblath DR, IIyas AA, Baba h, Quarles RH, Griffin JW, et al. A treatable multifocal motor neuropathy with antibodies to GM1 ganglioside. Ann Neurol 1988;24:73-8.  Back to cited text no. 15    
16.Lewis RA, Sumner AJ, Brown AJ, Asbury AK. Multifocal demyelinating neuropathy with persistent conduction block. Neurology 1982;32:958-64.  Back to cited text no. 16    
17.Oh SJ, Claussen GC, Kim DS. Motor and sensory demyelinating mononeuropathy multiplex (multifocal motor and sensory demyelinating neuropathy): A separate entity or a variant of chronic inflammatory demyelinating polyneuropathy? J Peripher Nerv Syst 1997;294:363-9.  Back to cited text no. 17    
18.Harbo T, Andersen H, Jakobsen J. Length-dependent weakness and electrophysiological signs of secondary axonal loss in chronic inflammatory demyelinating polyradiculoneuropathy. Muscle Nerve 2008;38:1036-45.  Back to cited text no. 18    
19.Asahina M, Kuwabara S, Nakajima M, Yamada T. Demyelinating polyneuropathy with preferentially proximal involvement. Clin Neurol Neurosurg 1998;100:53-5.  Back to cited text no. 19    
20.Rotta FT, Sussman AT, Bradley WG, Ram Ayyar D, Sharma KR, Shebert RT. The spectrum of chronic inflammatory demyelinating polyneuropathy. J Neurol Sci 200015;173:129-39.  Back to cited text no. 20    
21.Vallat JM, Sommer C, Magy L. Chronic inflammatory demyelinating polyradiculoneuropathy: Diagnostic and therapeutic challenges for a treatable condition. Lancet Neurol 2010;9:402-12.  Back to cited text no. 21    
22.Dyck PJ, Litchy WJ, Kratz KM, Suarez GA, Low PA, Pineda AA, et al. A plasma exchange versus immune globulin infusion trial in chronic inflammatory demyelinating polyradiculoneuropathy. Ann Neurol 1994;36:838-45.  Back to cited text no. 22    
23.van Schaik IN, Eftimov F, van Doorn PA, Brusse E, van den Berg LH, van der Pol WL, et al. Pulsed high-dose dexamethasone versus standard prednisolone treatment for chronic inflammatory demyelinating polyradiculoneuropathy (PREDICT study): A double-blind, randomised, controlled trial. Lancet Neurol 2010;9:245-53.  Back to cited text no. 23    
24.Muley SA, Kelkar P, Parry GJ. Treatment of chronic inflammatory demyelinating polyneuropathy with pulsed oral steroids. Arch Neurol 2008;65:1460-4.  Back to cited text no. 24    
25.Brannagan TH, Pradhan A, Heiman-Patterson T, Winkelman AC, Styler MJ, Topolsky DL, et al. High-dose cyclophosphamide without stem-cell rescue for refractory CIDP. Neurology 2002;58:1856-8.   Back to cited text no. 25    
26.Gladstone DE, Golightly MG, Brannagan TH 3rd. High dose cyclophosphamide preferentially targets naive T (CD45/CD4/RA+) cells in CIDP and MS patients. J Neuroimmunol 2007;190:121-6.  Back to cited text no. 26    
27.Gladstone DE, Prestrud AA, Brannagan TH 3rd. High-dose cyclophosphamide results in long-term disease remission with restoration of a normal quality of life in patients with severe refractory chronic inflammatory demyelinating poly-neuropathy. J Peripher Nerv Syst 2005;10:11-6.  Back to cited text no. 27    
28.Mehndiratta MM, Hughes RA. Corticosteroids for chronic inflammatory demyelinating polyradiculoneuropathy. Cochrane Database Syst Rev 2002;1:CD002062.  Back to cited text no. 28    
29.Iijima M, Tomita M, Morozumi S, Kawagashira Y, Nakamura T, Koike H, et al. Single nucleotide polymorphism of TAG-1 influences IVIg responsiveness of Japanese patients with CIDP. Neurology 2009;73:1348-52.  Back to cited text no. 29    
30.Mehndiratta MM, Hughes RA, Agarwal P. Plasma exchange for chronic inflammatory demyelinating polyradiculoneuropathy. Cochrane Database Syst Rev 2004;CD003906.  Back to cited text no. 30    
31.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. Ann Neurol 1982;11:136-41.  Back to cited text no. 31    
32.Barohn RJ, Kissel JT, Warmolts JR, Mendell JR. Chronic inflammatory demyelinating polyradiculoneuropathy-clinical characteristics, course, and recommendations for diagnostic criteria. Arch Neurol 1989;46:878-84.  Back to cited text no. 32    
33.Dalakas MC, Engel WK. Chronic relapsing (dysimmune) polyneuropathy- pathogenesis and treatment. Ann Neurol 1981;9:134-45.  Back to cited text no. 33    
34.Molenaar DS, van Doorn PA, Vermeulen M. Pulsed high dose dexamethasone treatment in chronic inflammatory demyelinating polyneuropathy: A pilot study. J Neurol Neurosurg Psychiatry 1997;62:388-90.  Back to cited text no. 34    
35.Lopate G, Pestronk A, Al Lozi M. Treatment of chronic inflammatory demyelinating polyneuropathy with high-dose intermittent intravenous methylprednisolone. Arch Neurol 2005;62:249-54.  Back to cited text no. 35    
36.Donaghy M, Mills KR, Boniface SJ, Simmons J, Wright I, Gregson N, et al. Pure motor demyelinating neuropathy-deterioration after steroid treatment and improvement with intravenous immunoglobulin. J Neurology Neurosurg Psychiatry 1994;57:778-83.  Back to cited text no. 36    
37.Feldman EL, Bromberg MB, Albers JW, Pestronk A. Immunosuppressive treatment in multifocal motor neuropathy. Ann Neurol 1991;30:397-401.  Back to cited text no. 37    
38.Hahn AF, Bolton CF, Zochodne D, Feasby TE. Intravenous immunoglobulin treatment in chronic inflammatory demyelinating polyneuropathy: A double blind, placebo-controlled, crossover study. Brain 1996;119:1067-77.  Back to cited text no. 38    
39.Hughes RA, Donofrio P, Bril V, Dalakas MC, Deng C, Hanna K, et al. Intravenous immune globulin (10% caprylate-chromatography purified) for the treatment of chronic inflammatory demyelinating polyradiculoneuropathy (ICE study): A randomised placebo-controlled trial. Lancet Neurol 2008;7:136-44.  Back to cited text no. 39    
40.Mendell JR, Barohn RJ, Freimer ML, Kissel JT, King W, Nagaraja HN, et al. Randomized controlled trial of IVIg in untreated chronic inflammatory demyelinating polyradiculoneuropathy. Neurology 2001;56:445-9.  Back to cited text no. 40    
41.Thompson N, Choudhary P, Hughes RA, Quinlivan RM. A novel trial design to study the effect of intravenous immunoglobulin in chronic inflammatory demyelinating polyradiculoneuropathy. J Neurol 1996;243:280-5.  Back to cited text no. 41    
42.Vermeulen M, van Doorn PA, Brand A, Strengers PF, Jennekens FG, Busch HF. Intravenous immunoglobulin treatment in patients with chronic inflammatory demyelinating polyneuropathy: A double blind, placebo controlled study J Neurol Neurosurg Psychiatry 1993;56:36-9.   Back to cited text no. 42    
43.Brannagan TH. Intravenous gammaglobulin (IVIg) for treatment of CIDP and related immune-mediated neuropathies. Neurology 2002;59:S33-40.  Back to cited text no. 43    
44.Brannagan TH, Nagle KJ, Lange DJ, Rowland LP. Complications of intravenous immune globulin treatment in neurologic disease. Neurology 1996;47:674-7.  Back to cited text no. 44    
45.Center for Disease Control and Prevention. Renal insufficiency and failure associated with immune globulin intravenous therapy-United States, 1985-1998. MMWR Morb Mortal Wkly Rep1999;48:518-21.  Back to cited text no. 45    
46.Voltz R, Rosen FV, Yousry T, Beck J, Hohlfeld R. Reversible encephalopathy with cerebral vasospasm in a Guillain-Barrι΄ syndrome patient treated with intravenous immunoglobulin. Neurology 1996;46:250-1.  Back to cited text no. 46    
47.Dyck PJ, Daube J, O'Brien P, Pineda A, Low PA, Windebank AJ, et al. Plasma-exchange in chronic inflammatory demyelinating polyradiculoneuropathy. N Engl J Med 1986;314:461-5.  Back to cited text no. 47    
48.Hahn AF, Bolton CF, Pillay N, Chalk C, Benstead T, Bril V, et al. Plasma-exchange therapy in chronic inflammatory demyelinating polyneuropathy: A double-blind, sham-controlled, cross-over study. Brain 1996;119:1055-66.  Back to cited text no. 48    
49.Mehndiratta MM, Hughes RA, Agarwal P. Plasma exchange for chronic inflammatory demyelinating polyradiculoneuropathy. Cochrane Database Syst Rev 2004;CD003906.  Back to cited text no. 49    
50.Carpo M, Cappellari A, Mora G, Pedotti R, Barbieri S, Scarlato G, et al. Deterioration of multifocal motor neuropathy after plasma exchange. Neurology 1998;50:1480-2.  Back to cited text no. 50    
51.Radziwill AJ, Schweikert K, Kuntzer T, Fuhr P, Steck AJ. Mycophenolate mofetil for chronic inflammatory demyelinating polyradiculoneuropathy: An open-label study. Eur Neurol 2006;56:37-8.  Back to cited text no. 51    
52.Gorson KC, Amato AA, Ropper AH. Efficacy of mycophenolate mofetil in patients with chronic immune demyelinating polyneuropathy. Neurology 2004;63:715-7.  Back to cited text no. 52    
53.Dalakas MC, Rakocevic G, Salajegheh MK, Dambrosia JM, Hahn AF, Raju R, et al. A double- blind, placebo-controlled study of rituximab in patients with anti-MAG antibody-demyelinating polyneuropathy (A-MAG-DP). Ann Neurol 2009;65:286-93.  Back to cited text no. 53    
54.Benedetti L, Franciotta D, Beronio A, Cadenotti L, Gobbi M, Mancardi GL, et al. Rituximab efficacy in CIDP associated with idiopathic thrombocytopenic purpura. Muscle Nerve 2008;38:1076-7.  Back to cited text no. 54    
55.Mόnch C, Anagnostou P, Meyer R, Haas J. Rituximab in chronic inflammatory demyelinating polyneuropathy associated with diabetes mellitus. J Neurol Sci 2007;256:100-2.  Back to cited text no. 55    
56.Gono T, Matsuda M, Shimojima Y, Ishii W, Yamamoto K, Morita H, et al. Rituximab therapy in chronic inflammatory demyelinating polyradiculoneuropathy with anti-SGPG IgM antibody. J Clin Neurosci 2006;13:683-7.  Back to cited text no. 56    
57.Knecht H, Baumberger M, Tobςn A, Steck A. Sustained remission of CIDP associated with Evans syndrome. Neurology 2004;63:730-2.  Back to cited text no. 57    
58.Pestronk A, Lopate G, Kornberg AJ, Elliott JL, Blume G, Yee WC, et al. Distal lower motor-neuron syndrome with high-titer serum IgM anti-GM1antibodies-improvement following immunotherapy with monthly plasma-exchange and intravenous cyclophosphamide. Neurology 1994;44:2027-31.  Back to cited text no. 58    
59.Blume G, Pestronk A, Goodnough LT. Anti-MAG antibody-associated polyneuropathies-improvement following immunotherapy with monthly plasma-exchange and IV-cyclophosphamide. Neurology 1995;45:1577-80.  Back to cited text no. 59    
60.Hirst C, Raasch S, Llewelyn G, Robertson N. Remission of chronic inflammatory demyelinating polyneuropathy after alemtuzumab (Campath 1H). J Neurol Neurosurg Psychiatry 2006;77:800-2.  Back to cited text no. 60    
61.van Schaik IN, Bouche P, Illa I, Lιger JM, Van den Bergh P, Cornblath DR, et al. European Federation of Neurological Societies/Peripheral Nerve Society guide- line on management of multifocal motor neuropathy. Eur J Neurol 2006;13:802-8.  Back to cited text no. 61    
62.van Schaik, I, van den Berg LH, de HR, de Haan R, Vermeulen M. Intravenous immunoglobulin for multifocal motor neuropathy. Cochrane Database Syst Rev 2005;CD004429.   Back to cited text no. 62    
63.Zιphir H, Stojkovic T, Latour P, Lacour A, de Seze J, Outteryck O, et al. Relapsing demyelinating disease affecting both the central and peripheral nervous systems. J Neurol Neurosurg Psychiatry. 2008;79:1032-9.   Back to cited text no. 63    
64.Pineda AA, Ogata K, Osoegawa M, Murai H, Shigeto H, Yoshiura T, et al. A distinct subgroup of chronic inflammatory demyelinating polyneuropathy with CNS demyelination and a favorable response to immunotherapy. J Neurol Sci 2007;255:1-6.  Back to cited text no. 64    
65.Fee DB, Fleming JO. Resolution of chronic inflammatory demyelinating polyneuropathy-associated central nervous system lesions after treatment with intravenous immunoglobulin. J Peripher Nerv Syst 2003;8:155-8.  Back to cited text no. 65    
66.Ormerod IE, Waddy HM, Kermode AG, Murray NM, Thomas PK. Involvement of the central nervous system in chronic inflammatory demyelinating polyneuropathy: A clinical, electrophysiological and magnetic resonance imaging study. J Neurol Neurosurg Psychiatry 1990;53:789-93.  Back to cited text no. 66    
67.Feasby TE, Hahn AF, Koopman WJ, Lee DH. Central lesions in chronic inflammatory demyelinating polyneuropathy: An MRI study. Neurology 1990;40:476-8.  Back to cited text no. 67    
68.Ayyar DR, Sharma KR. Chronic inflammatory demyelinating polyradiculoneuropathy in diabetes mellitus. Curr Diab Rep 2004;4:409-12.  Back to cited text no. 68    
69.Laughlin RS, Dyck PJ, Melton LJ 3rd, Leibson C, Ransom J, Dyck PJ. Incidence and prevalence of CIDP and the association of diabetes mellitus. Neurology 2009;73:39-45.  Back to cited text no. 69    
70.Kalita J, Misra UK, Yadav RK. A comparative study of chronic inflammatory demyelinating polyradiculoneuropathy with and without diabetes mellitus. Eur J Neurol 2007;14:638-43.  Back to cited text no. 70    
71.Lee DH, Linker RA, Paulus W, Schneider-Gold C, Chan A, Gold R. Subcutaneous immunoglobulin infusion: A new therapeutic option in chronic inflammatory demyelinating polyneuropathy. Muscle Nerve 2008;37:406-9.  Back to cited text no. 71    

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