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Immune-mediated neuropathies are a diverse group of disorders caused by immune-mediated response against antigens in the peripheral nerves. These can range from a fulminant, life-threatening crisis to an asymptomatic, minimally progressive process. Some are part of a larger systemic autoimmune process, and others are an isolated peripheral nerve disorder. The major groupings include

Acute immune-mediated neuropathy (Guillain-Barré Syndromes [GBS] and variants)1

  • Acute inflammatory demyelinating polyradiculoneuropathy (AIDP)
  • Acute motor and sensory axonal neuropathy (AMSAN)
  • Acute motor axonal neuropathy (AMAN)
  • Miller-Fisher syndrome (MFS)
  • Sensory ataxic GBS
  • Other acute variants such as acute pandysautonomic neuropathy

Chronic immune-mediated polyneuropathy1,2

  • Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and variants  
    • Pure motor form or pure sensory form of CIDP
    • Multifocal acquired demyelinating sensory and motor neuropathy (MADSAM), known as Lewis-Summer Syndrome
    • Distal acquired demyelinating symmetrical neuropathy (DADSN)
    • Chronic inflammatory axonal neuropathy
    • Demyelinating neuropathies with monoclonal gammopathy of undetermined significance (MGUS) other than IgM
  • Multifocal motor neuropathy (MMN) with/without conduction block
  • Demyelinating neuropathy associated with monoclonal IgM MGUS with and without anti-MAG (Myelin Associated Glycoprotein) neuropathies
  • Chronic ataxic neuropathy with ophthalmoplegia, monoclonal protein, cold agglutinins and disialosyl antibodies (CANOMAD)
  • Chronic ataxic neuropathy with disialosyl antibodies (CANDA)
  • Other immune-mediated neuropathies related to systemic immune disorders, and rarer types such as paraneoplastic neuropathies associated with anti-Hu antibodies and vasculitic neuropathies, neuropathy associated with POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, M-protein and Skin abnormalities) syndrome

Epidemiology including risk factors

The annual incidence of immune-mediated neuropathies (IN) varies among specific types with the most common being GBS and subtype AIDP as well as CIDP, MMN, and POEMS syndrome.


  • The annual incidence is reported to be 0.81-2.3 per 100,000, the most common cause of acute, flaccid paralysis worldwide. Men are more often affected than women.3 The incidence increases in older age.
  • AIDP is the major occurring subtype of GBS in Europe and North America (60-80%) while axonal forms such as AMAN and AMSAN are found more commonly in China and Japan (50% of cases).
  • GBS is commonly precipitated by an infection with incidence varying depending on geographic locations and infectious outbreaks.
    • Campylobacter jejuni (most common identifiable antecedent infection), Cytomegalovirus (CMV), Epstein-Barr virus (EBV) predominate as infectious pathogens. Other organisms implicated are influenza A, Mycoplasma pneumoniae, Haemophilus influenzae, Hepatitis A, B and C.2,4 Connection between C.jejuni and GBS is strong, particularly the AMAN variant (67%-92%).
    • Zika virus is the recently identified antecedent infection with several-fold increased incidence reported in several countries as of 2016.4
    • The swine flu vaccine was felt to be a potential risk factor in 1976; however, in 2009 the risk in China was lower in those vaccinated than in those who were not.5
    • Coronavirus disease-2019 was reported to be associated with GBS by post-infectious immune-mediated mechanism.6
  • Other risk factors include autoimmune disorders, malignancy and surgery.
    • Immune checkpoint inhibitors as cancer treatment are associated with GBS (about 0.1%) likely by a T-cell-mediated mechanism.7

Chronic acquired demyelinating neuropathies

  • The overall prevalence of around 6 cases per 100,000 individuals, the most common type being CIDP with estimated prevalence rate of 1.0 to 8.9 cases per 100,000.8
  • MMN is a rare motor asymmetric neuropathy affecting no more than 1-2 individuals per 100,000 affecting males more than females by 3 times.8
  • Most chronic demyelinating neuropathies affect males more commonly and typically occur in the middle to old age.8

Etiology and pathogenesis

Immune-mediated neuropathies most commonly occur when immunologic tolerance to key antigenic sites on myelin, axon, nodes of Ranvier or ganglionic neurons is lost.

  • Demyelinating neuropathies (occurring at myeline) are the most common type.
    • Either isolated demyelination or a combination of axonal injury and demyelination.
  • Current evidence supports the notion that IN is mediated by antibodies directed against myelin antigens, along with autoreactive T cells and macrophages that invade the myelin sheath, axonal membranes or the nodes of Ranvier. In certain disorders the triggering factors have been identified and progress made in the understanding of immunopathologic process and in many others the exact mechanisms remain unclear.9
  • GBS10
    • AIDP – the main target appears to be the myelin
    • AMAN- where primary pathology is in the axon, either due to massive acute demyelination and inflammation or due to a primary attack on axons and nodes of Ranvier mediated by macrophages and antibodies.
    • AMSAN- which is like AMAN but with concurrent involvement of sensory axons.
    • Miller-Fisher syndrome- the presence of IgG antibodies against GQ1b ganglioside.
    • Sensory ataxic GBS- probably occurs due to the involvement of dorsal roots and ganglionic neurons. Some of these patients have also IgG antibodies to GQ1b or GQ1B ganglioside.
    • Acute pandysautonomic neuropathy- where the target antigen is probably in the sympathetic ganglionic neurons.
  • CIDP
    • The pathogenesis is poorly understood. Different patterns of immune responses or different antigen targets underlie the different subtypes.
    • The demyelination in CIDP is multifocal, like the one seen in GBS, affecting roots, plexuses, and proximal nerve trunks, accounting for variable distribution of symptoms.
    • Inflammatory cells such as T cells and macrophages infiltrate nerves throughout the perineurium and endoneurim with disruption of blood-nerve barriers and macrophages splitting and destroying the myelin.
      • Different T cell responses partly explain the clinical heterogeneity of CIDP.
    • Humoral immunity processes such as antibodies and complements play a role in CIDP.11
      • The role of myelin proteins such as P0, myelin P2 protein, PMP22, and connexin as autoantigens in CIDP has not been confirmed.
      • Nodal proteins such as neurofascin 186 (NF 186) and gliomedin, and paranodal proteins (NF 155 and contactin 1 associated with paranodal dissection) are identified and associated with the specific pathology.
      • Antibodies to glycolipids LM1 (ganglioside, frequently with ataxia), GM1, or GD1b were subsequently detected in some patients with CIDP.
    • Several studies suggest that genetically determined factors contribute to the development of CIDP.10
  • MMN
    • IgM antibodies against GM1 were found in 43-85% of patients with MMN.
    • Anti-GM1 antibodies (similar to anti-GM1 IgG Ab in AMAN) bind at the node of Ranvier and activate complement disrupting sodium channel clustering. Higher complement activating capacity is associated with severe axon loss and muscle weakness.
    • Pathological studies of nerves revealed loss of myelinated axons as well as axonal degeneration and regeneration. One biopsy study suggested that axonal loss degeneration and loss predominate vs other studies demonstrated demyelination and onion bulbs.10

Disease progression including natural history, disease phases or stages, disease trajectory (clinical features and presentation over time)

New onset/Acute

  • GBS is a self-limited acute neuropathy characterized by symmetric flaccid paralysis, areflexia and sensory deficits that start 1-3 weeks after infection. Symptoms develop in hours to weeks and reach a nadir within 4 weeks, but often less than 2 weeks.
  • 10% of GBS showed treatment-related fluctuation, typically within 4-8 weeks from onset.
  • Between 4-15% of patients die, and up to 20% are disabled after a year despite modern treatment with worsening prognosis with advanced age.
  • In children, the clinical course is more rapid with likely complete recovery.2,10


  • Subacute neuropathies (i.e., SIDP or subacute inflammatory demyelinating polyradiculoneuropathy) are defined as progressing for 4-8 weeks.3


  • CIDP is characterized by the occurrence of chronic progressive, relapsing or monophasic symmetrical weakness in both proximal and distal muscles, impaired sensation and paresthesia due to distal fiber sensory loss and absent or diminished reflexes which progress over 2 months.8,10
    • CIDP has an unpredictable course, although the majority of patients have severe/disabling motor, sensory, or sensorimotor deficits after several years. In a recent population-based study, the nadir overall neuropathy limitation scale score was 5, and 58 % of patients were unable to walk independently at some points of their illness.12,13
  • MMN presents with asymmetric weakness often related to the distribution of individual nerves. Arms are usually affected earlier and more severely than legs, with more than 80% of patients initially affected in forearm or hand muscles along with a patchy reduction of reflexes or sometimes even brisk reflexes (often confused with motor-neuron disease).2,10

Essentials of Assessment

History and Physical Examination

  • Acute (GBS)
    • Progressive bilateral weakness in GBS with difficulty in climbing stairs getting out of chair is most notable 1-2 days after paresthesias.2
    • Cranial nerves including facial, bulbar, and ocular motor nerves are commonly involved in GBS (especially in MFS).
    • MFS may present with ophthalmoplegia, ataxia, and areflexia.2
    • Respiratory muscles are commonly affected, and 25% of patients may need artificial ventilation. Autonomic dysfunction such as cardiac arrhythmias, arterial hypertension or hypotension, abnormal sweating, GI dysmotility may occur in two-thirds of patients
    • Bowel or bladder complaints are rare.
    • Autonomic complaints are common in GBS and variants.
  • Chronic14
    • Weakness is proximal and distal in typical CIDP.
    • Fasciculations and cramps common in MMN (approximately 50%).
    • The typical presentation of anti-MAG neuropathy is that of distal, predominantly sensory large fiber ataxic neuropathy, like DADSN. Some patients may also exhibit a neurogenic tremor in the arms.10
  • Muscle stretch reflexes are diminished in GBS and CIDP by definition, except for the AMAN variant, which can have increased reflexes in the recovery phase.

Differential diagnosis

  • GBS: transverse myelitis, myasthenia gravis, botulism, carcinomatous or lymphomatous meningitis, toxin-related neuropathy, vasculitis-related neuropathy.
  • CIDP
    • IgA, IgM, or IgG monoclonal gammopathies
    • Neuropathy related to human immunodeficiency virus (HIV), hepatitis C, Sjogren’s disease, lymphoma, ulcerative colitis and Crohn disease, melanoma and diabetes.
    • Secondary immune-mediated neuropathies are associated with vasculitic diseases such as polyarteritis nodosa (PAN), Wegener granulomatosis, Churg-Strauss syndrome (CSS), microscopic polyangiitis, temporal arteritis, drug-induced vasculitis, nonsystemic vasculitis neuropathy), connective tissue disease, rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), Sjogren syndrome, systemic sclerosis, cryoglobulinemia), sarcoidosis, or malignancies.2


  • Electrodiagnostics (EDX) is an important clinical tool for distinguishing demyelinating and axonal types.10
    • Electrodiagnostic criteria for demyelination
      • Partial motor conduction block and temporal dispersion reduced motor conduction velocity (60% of the normal mean),10 prolonged motor latencies, and F wave latencies2
      • Conduction block10
    • Typical EDX findings in GBS
      • Test results can be normal early in acute disease.
      • Prolonged F wave or loss of F wave and H reflex may be the only finding if the patient is tested early.4
      • In AIDP, electrodiagnostic performed within 2-15 days after onset may shows motor conduction blocks (CB) in approximately 60% of patients. Conduction block resolves with the appearance of CMAPs with slow initial components and increased duration on stimulation proximal to the site of block, consistent with the remyelinating process.3 The other abnormalities are prolonged motor distal latencies and decreased amplitudes.15
      • Serial NCS lead to reclassification of GBS subtypes in as many as 40% of patients, especially from AIDP to an axonal form. Thus, the classification may differ among studies that employ single or repeated NCS.3
      • AMAN: decreased distal CMAPs, prolonged distal motor latency (DML), CB, and conduction slowing in forearm segments in the first week.10
    • CIDP16
      •  American Academy of Neurology Ad Hoc Subcommittee Criteria in 1991 (requiring 3 of 4 following criteria)
        • Reduced conduction velocities (CVs; e.g. <80% of the lower limit of normal [LLN] if the distal motor amplitude is normal, and <70% of LLN if the amplitude is substantially reduced)
        • Prolonged DMLs
        • Prolonged F-wave latencies (FLs; e.g.>125% of the upper limit of normal [ULN] if the distal motor amplitude is normal, and >150% of ULN if the amplitude is reduced for distal latencies and F-waves)
        • CB/temporal dispersion (TD); e.g. CB is >50% reduction of proximal/distal [p/d] amplitude and abnormal temporal dispersion is >130% increase of p/d duration)
      • European Federation of Neurological Societies/Peripheral Nerve Society Guideline in 2010
        • Definite: at least one of the following:
          • At least 50% prolongation of DML above ULN in two nerves
          • At least 30% reduction of motor CV below LLN in two nerves
          • At least 20% prolongation of F-wave latency above ULN in two nerves (>50% if amplitude of distal negative peak CMAP <80% of LLN)
          • Abnormal temporal dispersion (>30% duration increase between the proximal and distal negative peak CMAP) in at least two nerves
          • Absence of F-waves in two nerves if these nerves have amplitudes of distal negative peak CMAPs at least 20% of LLN + at least one other demyelinating parameter in at least one other nerve
          • Partial motor CB: at least 50% amplitude reduction of the proximal negative peak CMAP relative to distal, if distal negative peak CMAP at least 20% of LLN, in two nerves, or in one nerve + at least one other demyelinating parameter in at least one other nerve, or
          • Distal CMAP duration of at least 9 ms in at least one nerve + at least one other demyelinating parameter in at least one other nerve
        • Probable
          • At least 30% amplitude reduction of the proximal negative peak CMAP relative to distal, excluding the posterior tibial nerve, if distal negative peak CMAP at least 20% of LLN, in two nerves, or in one nerve + at least one other demyelinating parameter in at least one other nerve
        • Possible
          • As in ‘I’ but in only one nerve
    • MMN
      • Presence of motor CB with weakness in 2 or more nerves outside common entrapment sites,
      • Absence of upper motor neuron signs or significant sensory loss, and with normal sensory nerve conduction in typical cases.1
    • In ant-MAG neuropathy, patients often have a disproportionate prolongation of distal latencies compared with patients who have CIDP.1

Laboratory studies and supplementary tests

  • Autoantibodies are variably associated, immune-mediated clinical syndromes; the highest associations are:
    • Anti-GQ1b and GT1a IgG Ab in MFS
    • Anti-GM1 and GD1a IgG Ab in AMSAN and AMAN
    • Anti-GM1 IgM Ab in MMN
    • Anti-MAG in the DADSN
    • IgM monoclonal gammopathies may be associated with anti-MAG neuropathies10
    • Anti-Contactin 1 IgG Ab in CIDP (aggressive onset and initial axonal involvement with poor response to IVIG)
    • Anti- NF155 IgG Ab in CIDP (tremor, ataxia, and distal motor involvement)
    • Anti- NF 186 and NF 140 IgG Ab in CIDP (subacute onset, sensory ataxia, and conduction block with good response to IVIG and steroids)
    • Anti- GD3, GD1b, GT1b and GQ1b IgM Ab in CANOMAD and CANDA
  • Campylobacter jejuni serology is positive in 44%-88% of GBS cases.1
  • Elevated CSF protein and normal cell count are seen particularly in GBS, CIDP, and variants (except MMN).
  • Approximately 90% of patients with anti-MAG neuropathy have elevated CSF protein concentration with normal CSF cell counts.10
  • Nerve biopsy can be useful to confirm vasculitic neuropathies. In patients with equivocal electrodiagnostic findings, nerve biopsies can help distinguish between primary demyelinating and axonal neuropathy.
    • Motor nerve biopsy can help distinguish between MMN and motor neuron disease
    • Sensory nerve biopsy can aid diagnosis of CIDP, anti-MAG neuropathy or POEMS syndrome8
  • Autonomic testing can confirm autonomic involvement.
  • Pulmonary function tests and telemetry are necessary in severe cases that have autonomic and diaphragmatic involvement, particularly in GBS.


  • Imaging is useful primarily to exclude other diagnoses.
  • An MRI can show enhancement and enlargement of nerve roots and peripheral nerves in AIDP and CIDP.

Ultrasonography in CIDP

  • Typical findings with prognostic implications
    • Combining nerve/fascicle size with echo intensity and histology at baseline, nerves showed hypoechoic enlargement, reflecting active inflammation and onion bulbs or showed nerve enlargement with additional hyperechogenic fascicles/perifascicular tissue, possibly reflecting axonal degeneration and some showing almost no enlargement, reflecting “burned-out” or “cured” disease without active inflammation.
    • Based on nerve echogenicity through Ultrasound, nerves were classified into 3 classes
      • Class 1: large nerves (increased cross-sectional area [CSA]), with hypoechoic segments. It correlates with good responses to treatment.
      • Class 2: large nerve, with segments with increased nerve CSA and coexistence of hyper- and hypoechogenic enlarged (or normal) fascicles (fascicles with hyperechoic structure and other fascicles with hypoechoic structure).
      • Class 3: normal nerve size, but changes in echogenicity (nerve appears hyperechoic with reduced CSA, as in class 1 or 2). The hyperechoic rim of the epineurium is not clearly identified (blurred boundaries). Fascicles are not well visualized.
    • The results of the above-reported classification were correlated with clinical data, namely, age of patient, duration of disease, muscle strength, and lower and upper limbs disability score.
    • Increase in nerve vascularization; uncertain clinical implication.17
  • Ultrasonography in differential diagnosis;
    • “Bochum ultrasound score” (BUS) included evaluation of cross-sectional area (CSA) in the a) ulnar nerve in the Guyon canal b) ulnar nerve in the upper arm c) radial nerve in the spiral groove and d) sural nerve between the lateral and medial heads of gastrocnemius muscle. 1 point allotted to each anatomical site18
    • Typical BUS finding suggestive of CIDP was pathological CSA enlargement at all 4 anatomical sites compared to reference values. CIDP was highly suggestive in BUS score ≥ 2.
    • For BUS score ≤ 2, other anatomical sites such as median and ulnar nerves in the forearm and tibial nerve at ankle were evaluated. If the patient had pathological enlargement in at least 1 of the aforementioned sites then MMN is diagnosed. If the above 3 sites were normal and BUS was ≤ 2, then median nerve in carpal tunnel and ulnar nerve at the elbow were evaluated. CSA enlargement in at least 1 of these sites was highly suggestive of MADSAM.
    • The study showed 88% sensitivity and 84.6% specificity in distinguishing CIDP from other diagnoses of symmetrical demyelinating polyneuropathies. MMN and MADSAM could both be diagnosed with a sensitivity of 100% and specificity of 83.3%.19

Early predictions of outcomes


  • Early predictors of bad prognosis: older age (over 50), rapid onset of profound weakness, mechanical ventilation, distal compound muscle action potential (CMAP) amplitude less than 10% of normal4
  • Long-term outcomes are worse in AMAN, better in MFS than classic AIDP. Disability remains in 10% of patients.4


  • Early predictors of bad prognosis: Mode of onset, progression time from onset to nadir, asymmetrical symptoms, and upper limb predominant weakness, involvement of distal and intermediate nerve segments20

Medical Management and Rehabilitation Strategies

Current treatment guidelines

At different disease stages

  • New onset/Acute demyelinating neuropathies
    • In GBS, treatment should begin within two weeks if possible.
      • Immunomodulatory treatment consists of plasma exchange and IVIG.
      • The single course of IVIG is usually sufficient with GBS with a classical course. Further trials for optimal doses are needed for severe cases or relapse within 8 weeks. Adverse effects of IVIG are rare, including hypersensitivity reactions, aseptic meningitis, thromboembolism, and hemolytic anemias.11
      • Plasma exchange (PE) is recommended in non-ambulant patients within four weeks of treatment (level A, class 2) or ambulant within two weeks (level B, limited class 2). Four PE every other day was superior to two PE 2 days apart.21,11 Adverse effects of PE include hypocalcemia and metabolic acidosis related to the use of citrate, and hemodynamic changes.
      • PE and IVIG are equivalent (class 1) and steroids are ineffective (level A, class 1).8
    • Multidisciplinary care for the prevention of potentially fatal complications such as respiratory failure, cardiac arrhythmias, infections, and thromboembolism.
    • Autonomic instability may necessitate admission to an intensive care unit (ICU) and telemetry; impending respiratory distress requires pulmonary function monitoring and mechanical ventilation.
  • Chronic/stable
    • Primary treatments for CIDP are corticosteroids, IVIG, and PE (Level A).21,22
      • Given the logistic challenges, PE is considered the second line.
      • Subcutaneous IG can be an alternative to IVIG for maintenance therapy and some studies showed the benefits as initial therapy.23
      • Steroid-sparing agents such as azathioprine and mycophenolate mofetil can be used to facilitate the tapering of corticosteroids. High-dose IV cyclophosphamide can be tried for refractory patients.24
      • Monoclonal antibodies such as rituximab, alemtuzumab, natalizumab. Rituximab can be beneficial in CIDP patients with Ab against node of Ranvier proteins such as contactin-1, contactin-associated protein 1, and NF-155
    • Others
      • Anti-MAG neuropathy: rituximab, or combination with fludarabine are often first-line treatments
      • MMN: IVIG is first-line therapy (level A).


  • At least a third of people with immune-mediated neuropathies experience long-term activity limitations. Residual symptoms may be present many years after recovery from GBS syndromes or CIDP. Persistent residual disability and fatigue are the most common long-term consequences which adversely affect ADLs, IADLs, social and family activities.
  • Goal is to stabilize, prevent complications related to immobilization such as venous thromboembolism, contracture, etc. and optimize function and prepare for further interventions at later disease stages.
  • Inpatient rehabilitation may be necessary for patients with functional deficits. Aerobic conditioning, resistance exercises, gait training, transfers, and activities of daily living are emphasized in physical and occupational therapy. Tilt table training may be necessary for orthostasis. Speech and language therapy is prescribed as necessary. Orthotics are often necessary, particularly ankle-foot orthotics16
  • Symptomatic medications are available to offer relief of neuropathic and mechanical pain, fatigue, and alleviate depression and anxiety, but none have been studied in a rigorous fashion in patients with CIDP.16
  • Several Studies including a large multicenter trial in UK demonstrated significant functional gains with in-patient rehabilitation among patients with acute (GBS) and chronic inflammatory neuropathies with both physical and cognitive disabilities. The study also demonstrated significant reduction in on-going care-costs, especially for highly dependent patients, thus supporting new evidence for cost efficiency of rehabilitation in this group of patients.21

Coordination of care

  • The interdisciplinary approach involves neurology, physical medicine and rehabilitation, hospitalists and intensivists, as well as other specialists and disciplines (physical and occupational therapy, speech and language therapy, respiratory therapy, nursing, orthotists).

Patient & family education

  • Patients and families should be educated on the generally good prognosis of GBS and the potentially recurrent nature of CIDP.
  • Educate regarding complications of treatment such as prednisone and other immunomodulating treatments.
  • Prevention of the complications of immobilization in the outpatient setting will largely fall on caregivers.
  • Patients and families should be educated on the of proper Assistive Devices (AD) and orthotic devices.

Translation into practice: practice “pearls”/performance improvement in practice (PIPs)/changes in clinical practice behaviors and skills

  • Identifying that neuropathy is immune-mediated is important because of the potential for treatment.
  • Early diagnosis of GBS is complicated by its variable presentation and the lack of reliable, objective testing within the first week of treatment (In the first week of the disease, CSF protein is normal in one-third of patients; 50% have normal CMAP amplitude). GBS is best treated within two weeks of the onset of symptoms, sometimes necessitating empiric treatment.
  • Of the several idiopathic neuropathies referred to tertiary centers, 10%-33% are later felt to be CIDP. CIDP should be in the differential of unexplained neuropathies.

Cutting Edge/Emerging and Unique Concepts and Practice

Emerging/unique interventions  

  • Advances have been made in surrogate markers in patients with immune-mediated neuropathy with correlation with certain clinical phenotypes or response to specific treatment in CIDP.
  • Personalized medicine is coming for innovative treatment tailored to the specific subgroups of patients.
  • Emerging therapeutic agents in the form of new biologic agents, monoclonal antibodies or fusion proteins offer target-specific therapy and are currently used in other autoimmune disorders.
  • R. Sendhilkumar et al demonstrated significant improvement in quality of sleep with yogic relaxation, pranayama, and meditation among GBS patients25
  • Quality study for neurorehabilitation specific for CIDP was not existing. Integration of individual rehabilitation treatment such as strengthening exercise (moderate evidence in patients with neuropathy) can be applied with pharmacological treatment.26


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  2. Eldar, A.H. and J. Chapman, Guillain Barre syndrome and other immune mediated neuropathies: diagnosis and classification. Autoimmun Rev, 2014. 13(4-5): p. 525-30.
  3. Dimachkie, M.M. and R.J. Barohn, Guillain-Barre syndrome and variants. Neurol Clin, 2013. 31(2): p. 491-510.
  4. Wijdicks, E.F. and C.J. Klein, Guillain-Barre Syndrome. Mayo Clin Proc, 2017. 92(3): p. 467-479.
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  6. Abu-Rumeileh, S., et al., Guillain-Barré syndrome spectrum associated with COVID-19: an up-to-date systematic review of 73 cases. Journal of neurology, 2021. 268(4): p. 1133-1170.
  7. Shahrizaila, N., H.C. Lehmann, and S. Kuwabara, Guillain-Barré syndrome. Lancet, 2021. 397(10280): p. 1214-1228.
  8. Latov, N., Diagnosis and treatment of chronic acquired demyelinating polyneuropathies. Nat Rev Neurol, 2014. 10(8): p. 435-46.
  9. Franssen, H. and D.C. Straver, Pathophysiology of immune-mediated demyelinating neuropathies-part I: neuroscience. Muscle Nerve, 2013. 48(6): p. 851-64.
  10. Franssen, H. and D.C. Straver, Pathophysiology of immune-mediated demyelinating neuropathies–Part II: Neurology. Muscle Nerve, 2014. 49(1): p. 4-20.
  11. Kieseier, B.C., et al., Immune-mediated neuropathies. Nat Rev Dis Primers, 2018. 4(1): p. 31.
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  13. Leger, J.M., R. Guimaraes-Costa, and C. Muntean, Immunotherapy in Peripheral Neuropathies. Neurotherapeutics, 2016. 13(1): p. 96-107.
  14. Bunschoten, C., et al., Progress in diagnosis and treatment of chronic inflammatory demyelinating polyradiculoneuropathy. Lancet Neurol, 2019. 18(8): p. 784-794.
  15. Rajabally, Y.A. and F.L. Hiew, Optimizing electrodiagnosis for Guillain-Barre syndrome: Clues from clinical practice. Muscle Nerve, 2017. 55(5): p. 748-751.
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  17. Padua, L., et al., Heterogeneity of root and nerve ultrasound pattern in CIDP patients. Clin Neurophysiol, 2014. 125(1): p. 160-5.
  18. Herraets, I.J., et al., Nerve ultrasound improves detection of treatment-responsive chronic inflammatory neuropathies. Neurology, 2020. 94(14): p. e1470-e1479.
  19. Kerasnoudis, A., et al., Nerve ultrasound protocol in differentiating chronic immune-mediated neuropathies. Muscle Nerve, 2016. 54(5): p. 864-871.
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  22. Cortese, I., et al., Evidence-based guideline update: Plasmapheresis in neurologic disorders: report of the Therapeutics and Technology Assessment Subcommittee of the American Academy of Neurology. Neurology, 2011. 76(3): p. 294-300.
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  26. Oaklander, A.L. and F. Gimigliano, Are the treatments for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) effective and safe?-A Cochrane Overview summary with commentary. NeuroRehabilitation, 2019. 44(4): p. 609-612.

Original Version of the Topic

Shawn Jorgensen, MD. Immune Mediated Neuropathies. 12/27/2012

Previous Revision(s) of the Topic

Samuel S. Murala, MD, Se Won Lee, MD. Immune Mediated Neuropathies. 3/6/2018

Author Disclosure

Se Won Lee, MD
Nothing to Disclose