Author(s): Elisabeth Frankini, DO, Rajashree Srinivasan, MD, MBBS, Priya Chitta Nangrani, MD
Originally published: September 20, 2013 Last updated: November 20, 2025
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Disease/Disorder

Definition

Among the acquired immune-mediated polyneuropathies, the most common are acute inflammatory demyelinating polyradiculoneuropathy (AIDP), also referred to as Guillain-Barré syndrome (GBS), and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Most authorities classify GBS as a clinical spectrum encompassing AIDP, acute motor-sensory axonal neuropathy, and acute motor axonal neuropathy (AMAN). A variant of GBS, Fisher (formerly Miller-Fisher) syndrome, will not be addressed in depth.

Etiology

Acquired immune-mediated demyelinating polyneuropathies may present in response to a variety of different clinical scenarios1,2,3,4,8

  • Vaccinations (swine influenza, meningococcus, influenza, hepatitis B, rabies, tetanus toxoid)
  • Autoimmune disorders
  • Systemic lupus erythematosus and other collagen vascular diseases
  • Inflammatory bowel disease
  • Diabetes mellitus
  • Thyrotoxicosis
  • Acquired immunodeficiencies
  • Solid organ and bone marrow transplantation and graft versus host disease
  • Lymphoma and monoclonal gammopathy of undetermined significance (MGUS)
  • POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin changes)
  • Cholangiocarcinoma and melanoma, or as a paraneoplastic complication of carcinoma (small cell lung, pancreas, colon)
  • Toxins (procainamide, cyclosporine, and tacrolimus)
  • Biologic agents (Immune checkpoint inhibitors, TNF-alpha inhibitors)
  • Recent surgery
  • Postpartum state

Antecedent illness, vaccination, or surgery is implicated in 60% to 70% of patients with AIDP.1

Associated pathogens include2,5,6,8

  • Campylobacter jejuni (C. jejuni)
  • Cytomegalovirus (CMV)
  • Epstein-Barr virus (EBV)
  • Mycoplasma pneumoniae
  • Influenza
  • Hepatitis A, B, C and E
  • Human immunodeficiency virus (HIV)
  • Zika Virus
  • Coronavirus (SARS-CoV-2)

A trigger can be identified in <30% of CIDP patients, but respiratory and other infections, vaccinations, surgeries, blood transfusions, and even intra-articular steroid injections have been reported. In addition, pregnancy and infection have been reported to precede 20% to 30% of cases of CIDP exacerbation/relapse.1

Epidemiology including risk factors and primary prevention

AIDP is the leading cause of acute flaccid paralysis in developed countries, with an annual incidence of 0.6 to 2.7 per 100,000 persons. Although the incidence increases with age, the peak incidence is between 50 and 70 years. Males are approximately 1.5 times more likely to be affected by GBS, which is notably atypical for an immunologic condition.8

In contrast, the peak incidence of CIDP occurs in middle age (40s-60s). The relapsing-remitting form presents more often among patients in their 20s, whereas older patients may present with a more chronic, insidiously progressive motor and sensory polyneuropathy. Prevalence varies widely because of variable adherence to diagnostic criteria, from 1 to 9 per 100,000 persons. CIDP presenting in childhood carries a lower incidence of 0.48 per 100,000 persons.7

Patho-anatomy/physiology

The underlying etiology and pathophysiology are immunologic. Molecular mimicry between surface glycolipids of antigenic stimuli (i.e. infectious agents, vaccinations) and myelin epitopes may trigger an immune attack through both cellular and humoral mechanisms. Autoantibodies target gangliosides on the Schwann cell plasmalemma of peripheral nerve and roots, leading to activation of the complement cascade and resultant inflammatory infiltrate (macrophages), and ultimately to vesiculation of myelin.1,2

AIDP and CIDP are primarily demyelinating conditions. However, severe demyelination can lead to axonal damage. Postmortem studies confirm segmental demyelination in large and small motor and sensory nerves and spinal roots with signs of secondary axonal degeneration. By contrast, axonal GBS variants, such as AMAN, are characterized by IgG and complement-mediated attack with macrophage invasion of the nodes of Ranvier with myelin sparing.32

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

AIDP

AIDP presents abruptly and follows a monophasic course with progressive bilateral symmetric weakness. During the acute phase, the first 2 weeks of disease onset, patients are at risk for complications, including respiratory failure. Disease typically progresses for the next two weeks before reaching the plateau phase (typically weeks 4-8 after disease onset). Fifty percent of patients reach the nadir by 2 weeks, 80% by 3 weeks, and 90% by 4 weeks.1 Patients can respond well to treatment, with 77% of patients walking independently in six months. However, up to 16% of patients will relapse with symptoms within eight weeks of treatment.30

CIDP

In contrast, an indistinct onset and progression of signs/symptoms for more than 8 weeks are characteristic of the indolent course of CIDP. Two patterns predominate: relapsing-remitting (20%-65%) and progressive.9 Comparisons with multiple sclerosis (MS) are apropos, with CIDP often coined as the peripheral analog of MS, sharing an immunologic etiology, demyelinating pathophysiology, and similar patterns of progression.

Distinguishing AIDP and CIDP

Clinically, the 8-week temporal delineation is often less clear, as CIDP may present with an acute onset, and AIDP may have sequelae that persist beyond 8 weeks. Posterior column sensory signs (ataxia, vibratory or proprioceptive loss) or relapse/progression may favor CIDP, whereas autonomic involvement, facial weakness, preceding infection, and mechanical ventilation favor AIDP.10

Specific secondary or associated conditions and complications

During the acute phase, patients are at risk of several complications, including respiratory failure, autonomic dysfunction and bulbar weakness. Patients should be monitored closely for these life-threatening complications. During the progressive phase, patients are at risk of indirect complications including aspiration pneumonia, deep vein thrombosis and pulmonary embolism.30

Largely out of the scope of this review, other conditions along the clinical spectrum of CIDP include, but not limited to multifocal motor neuropathy, multifocal acquired demyelinating sensory and motor (MADSAM) neuropathy or Lewis-Sumner syndrome, distal acquired demyelinating symmetric neuropathy (DADS), IgG or IgA monoclonal gammopathy of undetermined significance (MGUS), and IgM monoclonal gammopathy with or without anti-myelin associated glycoprotein (MAG) antibodies.9,11,32 Multiple case reports have reported patients with CIDP who develop symptoms similar to lumbar stenosis and cauda equina syndrome secondary to nerve root hypertrophy at multiple lumbar levels with resultant radiculopathy and/or myelopathy.35,36,37

Essentials of Assessment

History

AIDP

Often, AIDP symptoms are preceded by respiratory infection, fever, diarrhea, vaccination, or surgery, especially to bones or digestive organs, 3 days to 6 weeks prior to the onset of neurologic symptoms.8 Initial symptoms include paresthesia, weakness, and pain, and may be preceded by vague back or neck pain. Over 12 to 28 days, progressive bilateral ascending symmetric weakness develops, then plateaus.2 Weakness begins in the legs 90% of the time, with accompanying paresthesias in the hands and feet 80% of the time. Sixty-six percent of patients report pain, particularly in the back and extremities.33Although weakness often starts in the lower limbs, leading to ascending paralysis, descending or simultaneous upper and lower limb involvement is seen in nearly half of patients and should raise the possibility of AIDP.1 After recovery from motor weakness, patients often complain of residual fatigue.8

CIDP

Typical CIDP presents with progressive, stepwise, or recurrent symmetric proximal and distal weakness and sensory dysfunction of all extremities developing over ≥8 weeks.11 In typical CIDP, motor involvement is greater than sensory and can be accompanied by tremor or cranial nerve/bulbar involvement (oculomotor and facial nerves are more commonly involved10). Though uncommon, typical CIDP can also present with optic disc edema and autonomic dysfunction.8 Variants of CIDP present with predominantly distal (DADS), asymmetric (MADSAM), or focal (localized to plexus or peripheral nerve) weakness, pure motor, or pure sensory patterns.11 Detrusor areflexia and loss of bladder sensation affect micturition in 25% of patients with CIDP.7

Physical examination

Characteristic exam findings in both groups include generalized hyporeflexia or areflexia, although 10% demonstrate normal or brisk reflexes.2 In AIDP, bilateral facial weakness may be present, mimicking stroke/transient ischemic attack. Autonomic dysfunction, including tachy- or bradycardia, urinary retention and hyper- or hypotension, may be present in AIDP.

In CIDP, motor findings predominate, with symmetric distal > proximal > facial weakness. Large-fiber nerve function (vibration and position sense) is notably impaired, with lesser losses in small-fiber function (touch, pinprick, and temperature). Imbalance and ataxic gait may be evident. Enlarged peripheral nerves can be palpated in 11%.1

Functional assessment

Sensorimotor deficits result in difficulty with ambulation, gait instability and falls, and (more so in CIDP) difficulty rising from a chair. Impaired dexterity and fine motor skills create challenges with handwriting, personal hygiene, and dressing. Functional motor gain and recovery are measured using the traditional Functional Independence Measure (FIM) motor scores at admission and discharge. FIM is a sensitive tool that can assess disability and changes during rehabilitation in GBS patients34. Given the high variability in presentation of each patient with autoimmune neuropathies, it is best to develop additional personalized goals after discussion with the patient.

Laboratory studies

Laboratory workup for AIDP may include serologies for EBV, CMV, C. jejuni, HIV, hepatitis, and West Nile virus. Lumbar puncture is performed if GBS is suspected. Cerebrospinal fluid (CSF) protein levels are elevated in 50% to 66% of GBS cases during week 1, increasing to 75% by week 3.2 A cytoalbuminemic dissociation with elevated CSF protein and normal white blood cell count is classically seen in GBS and CIDP.

Although anti-GQ1b and anti-GT1a immunoglobulin IgG autoantibodies are highly sensitive for the Fisher variant,2 glycolipid antibody testing has limited clinical utility in GBS.

Suggested laboratory workup for CIDP can include complete blood count, renal function, liver function, fasting blood sugar, C-reactive protein, antinuclear antibodies, infectious serologies, serum and urine monoclonal proteins evaluations, anti-MAG antibodies and nodal and paranodal protein antibodies (i.e. anti-neurofascin 155).11 It is suggested to consider testing for nodal and paranodal antibodies in patients with are resistant to standard therapies with IVIG and corticosteroids.11,29

It is not recommended to perform CSF analysis if diagnostic criteria are already met for CIDP. Though if warranted, elevated CSF protein >45 mg/dL10 with a lymphocyte count of < 10/mm3 is supportive for CIDP.7,11

Peripheral nerve biopsies may be useful in exceptional cases of CIDP to evaluate for other potential causes such as vasculitis, when CIDP cannot be confirmed with alternative methods.7,11 Typical findings in CIDP include onion bulbs, endoneurial edema, inflammation, and lymphocytic infiltrate. Segmental demyelination is seen on teased fiber preparations.

Imaging

While imaging is not necessary to make a diagnosis of GBS, there are findings that could be useful in confirming the diagnosis. Lumbar magnetic resonance imaging (MRI) with gadolinium may demonstrate hypertrophy and enhancement of nerve roots in CIDP. Though, MRI is not typically used for the diagnosis of GBS as enhancement of the nerve roots may not be present at the onset of symptoms. Peripheral nerve enlargement can be detected with neuromuscular ultrasound, characterized by increased nerve cross-sectional area (CSA), which correlates with conduction block, disease severity, and functional disability.12 Changes in nerve echogenicity, fascicle size, nerve vascularity and epineurium thickness can aid in the diagnosis of CIDP. Nerve enlargement has been found in 69-100% of patients with CIDP. Enlargement of the median nerve and brachial plexus at two or more proximal sites is considered the most reliable parameter for differentiating CIDP from axonal neuropathy.29

One study used ultrasound to distinguish AIDP from CIDP. In contrast to CIDP, AIDP demonstrated enlarged vagal or cervical nerve roots with sparing of sensory nerves.13

Supplemental assessment tools

Diagnosis of AIDP and CIDP hinges on clinical features, electrodiagnosis, and spinal fluid examination. Common to the electrodiagnostic workup of both AIDP and CIDP is the demonstration of primary demyelination, adhering to the following published criteria: (1) reduced motor nerve conduction velocity (NCV), (2) motor conduction block and/or abnormal temporal dispersion, (3) prolonged distal motor latency (DML), and (4) prolonged or absent F-wave responses. Criteria differ quantitatively between the 2 conditions in the percentage of change from normal values and the required number of abnormal parameters.13

No less than 12 sets of criteria have been published for CIDP,11,13-17 including the Asbury and Cornblath electrodiagnostic criteria for demyelination11 and the American Academy of Neurology research criteria for CIDP. The Joint Task Force of the European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) developed electrodiagnostic guidelines to assist in the diagnosis of typical CIDP and its variants, as they strongly recommend electrodiagnosis to support a clinical diagnosis

EFNS/PNS electrodiagnostic criteria for CIDP11

  • Strongly supportive of demyelination
    • Prolongation of the DML ≥50% above the upper limit of normal (ULN) in 2 nerves, excluding median neuropathy from carpel tunnel syndrome
    • Reduction in motor NCV ≥30% below the lower limit of normal (LLN) in 2 nerves.
    • Prolongation of F-wave minimal latency ≥ 20% above ULN in 2 nerves (≥50% above the ULN if the distal compound muscle action potential [CMAP] amplitude is <80% of the LLN).
    • Absence of F-waves in 2 nerves if their distal CMAP amplitudes are ≥20% of the LLN AND ≥1 other demyelinating parameter in≥1 other nerve.
    • Partial motor conduction block in 2 nerves: ≥30% amplitude reduction of the proximal relative to the distal negative peak CMAP, excluding the tibial nerve, and the distal negative peak CMAP amplitude ≥20% of the LLN, or in 1 nerve and ≥1 other demyelinating parameter in at least 1 other nerve.
    • Abnormal temporal dispersion in ≥2 nerves: >30% duration increase between the proximal and distal CMAP.
    • Prolonged distal CMAP duration in ≥1 nerve and ≥1 other demyelinating parameter in ≥1 other nerve.
    • Sensory conduction abnormalities, such as prolonged distal latency or reduced sensory nerve action potential amplitude (SNAP) or slowed conduction velocity outside of normal limits, in 2 nerves
  • Weakly supportive of demyelination
    • As in the strongly supportive category, but in only 1 nerve.
    • Sensory CIDP with normal motor nerve conduction studies need to fulfill
      • Sensory nerve conduction velocity <80% LLN for SNAP amplitude >80% of LLN or 70% LLN for SNAP amplitude <80% of LLN in at least 2 nerves (median, ulnar, radial, sural nerve) OR
      • Sparing pattern in sural nerve distribution (abnormal median or radial SNAP amplitude with normal sural nerve SNAP amplitude)

Strict compliance with published evidence-based diagnostic criteria is paramount, since one-third of CIDP patients in the US have reportedly been incorrectly diagnosed and possibly receiving inappropriate treatment.19

The Inflammatory Neuropathy Cause and Treatment (INCAT) overall disability sum score (ODSS) is a validated tool to quantify arm and leg disability using a score ranging from 0 (“no disability”) to 12 (“most severe disability score”) that can be useful to track the course of AIDP/CIDP and provide an objective measure of therapeutic response before and after various treatments.20

Early predictions of outcomes

Risk factors for poorer prognosis in AIDP include older age of onset (>50-60y), abrupt onset of profound weakness, mechanical ventilation, and distal CMAP amplitudes < 10% to 20% of the LLN,2 antecedent C jejuni infection, and axonal subset of GBS.21 The risk of mechanical ventilation can be quantified using the modified Eramsus GBS Respiratory Insufficiency Score (mEGRIS). Mechanical ventilation is more likely if there is rapid disease progression, bulbar palsy, weaker neck flexion and bilateral hip flexion and inability to cough.8

In 2007, a new scoring system called the Eramus GBS outcome score was validated. It is typically used in the acute phase and helps determine outcomes at 6 months. The scoring system utilizes three clinical characteristics, including age, preceding diarrhea, and the GBS Disability Score at 2 weeks after illness. The model offers physicians a useful means to discuss progression with patients, identify those at higher risk and inform future treatment strategies.31 GBS Disability Score31

  • 0: A healthy state
  • 1: Minor symptoms and capable of running
  • 2: Able to walk ≥10meters without assistance, but unable to run
  • 3: Able to walk 10m with assistance across an open space
  • 4: Bedridden/chairbound
  • 5: Require assisted ventilation for at least part of the day
  • 6: Dead

Two-thirds of GBS patients are unable to walk independently at the nadir of weakness. Respiratory insufficiency occurs in 25%, contributing to an overall mortality rate of 5%.1,2 However, overall prognosis is favorable with many patients making a good recovery. Following immunotherapy, roughly 77% of patients are ambulatory after 6 months, and 82% are ambulatory at one year.21 In CIDP, favorable response to treatment correlates with a lack of associated conditions, absence of monoclonal proteins, and highly elevated CSF protein. Predominantly distal weakness portends a poorer prognosis.9 Children with CIDP tend to respond well to immunotherapy.

Among severe cases, 20% of patients affected by GBS remain non-ambulatory 6 months after onset.2 Only about 15% of patients are free of residual deficit at 1 to 2 years. Five to ten percent have persistent disabling sensory or motor symptoms. Seven percent experience recurrence.2

Environmental

Similar to MS, there is geographic variation among subtypes of GBS. Although AIDP is the most common presentation in Europe and North America, AMAN is the most common subtype in Asia, South America and Central America.32 AMAN and AIDP occur with equal incidence in India.22 Whereas the most common subtype of GBS is sensorimotor in Europe and North America, pure motor is most common in Bangladesh.8

Professional issues

Although GBS was linked with mass immunizations for H1N1 influenza in 1976, no such association with influenza has subsequently been found.2,24 Risk of contracting GBS because of seasonal influenza vaccination is 1 to 2 in 1,000,000 persons.24 Whether or not to vaccinate patients previously afflicted with GBS remains controversial; however, recent Centers for Disease Control and Prevention guidelines suggest minimal risk of recurrence to be weighed against the sizeable burden of illness for influenza.24,25 Two large studies of over 20 million adolescents vaccinated against meningococcus found no increased risk of GBS.26 In additional studies, there is an estimated less than one case of GBS per million people who have had the influenza, MMR, HPV, or meningococcal vaccines.27 Ongoing surveillance continues through the Vaccine Adverse Event Reporting System.24

Rehabilitation Management and Treatments

See AIDP/CIDP Part 2: Treatment

Cutting Edge/ Emerging and Unique Concepts and Practice

See AIDP/CIDP Part 2: Treatment

Gaps in the Evidence-Based Knowledge

See AIDP/CIDP Part 2: Treatment

References

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Original Version of the Topic

Michele Arnold, MD. AIDP/CIDP Part 1: Evaluation and Diagnosis. 9/20/2013

Previous Revision of the Topic

Michele Arnold, MD. AIDP/CIDP Part 1: Evaluation and Diagnosis. 2/14/2018

Rajashree Srinivasan, MD, MBBS, Amanda Ly, MD. AIDP/CIDP Part 1: Evaluation and Diagnosis. 6/1/2022

Author Disclosure

Elisabeth Frankini, DO
Nothing to Disclose

Rajashree Srinivasan, MD, MBBS
Nothing to Disclose

Priya Chitta Nangrani, MD
Nothing to Disclose