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Emergency: requires immediate attention
Acute inflammatory demyelinating polyneuropathy
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Emergency: requires immediate attention

Acute inflammatory demyelinating polyneuropathy

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Contributors: Jamie Adams MD, Richard L. Barbano MD, PhD
Other Resources UpToDate PubMed

Synopsis

Guillain-Barré syndrome (GBS) represents a spectrum of rare, acute, immune-related polyneuropathies with varying features and presentations. It is divided into 2 main subtypes: demyelinating and axonal. Demyelinating forms include acute inflammatory demyelinating polyneuropathy (AIDP) and the clinical variant Miller Fisher syndrome (MFS). Axonal forms include acute motor axonal neuropathy (AMAN) and acute motor-sensory axonal neuropathy (AMSAN).

The exact etiology is unclear, but GBS typically presents days to weeks after an infection, most commonly Campylobacter jejuni. Other reported triggers include cytomegalovirus (CMV), influenza, Epstein-Barr virus, and human immunodeficiency virus. Of note, GBS has been reported in patients with probable Zika virus infection in French Polynesia, Brazil, and Colombia. Rarely, GBS has been associated with surgery or immunization.

AIDP is the most common form of GBS reported in the United States. It is a rapidly progressive autoimmune disorder of the peripheral nervous system characterized by limb paresthesias, areflexia, and generalized symmetrical muscle weakness or paralysis, usually beginning in the extremities and spreading to the torso, facial, respiratory, and bulbar muscles. MFS is characterized by ophthalmoplegia, ataxia, and absence of the tendon reflexes. Patients with MFS usually demonstrate serum anti-GQ1b IgG antibodies.

Common clinical findings of AIDP:
  • Progressive (typically over 2 weeks), symmetric muscle weakness in more than 1 limb (can range from mild weakness to total paralysis of all 4 limbs, bulbar muscles, and/or trunk)
  • Absent or depressed deep tendon reflexes
  • Accompanying signs and symptoms may include mild paresthesias in the hands and feet accompanying the weakness, pain in the back and extremities (seen in two-thirds of patients), and dysautonomia (tachycardia, urinary retention, orthostatic hypotension, etc). There is often lack of fever at onset.
Common presenting symptoms in children:
  • Neuropathic pain, typically bilateral leg pain and lower back pain
  • Gait unsteadiness and/or refusal to walk
  • Facial weakness
  • Autonomic dysfunction
Recovery may take weeks to years. About two-thirds of individuals make a full or near full recovery. Approximately 10%-15% have residual weakness, numbness, or pain. Mortality rate is generally <5%. Poor prognostic factors include age >60 years, rapid nadir within first 7 days, ventilator dependency, and preceding infection with CMV or C. jejuni.

Codes

ICD10CM:
G61.89 – Other inflammatory polyneuropathies

SNOMEDCT:
26261000119109 – Acute inflammatory demyelinating polyneuropathy

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

Best Tests

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Management Pearls

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Therapy

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Drug Reaction Data

Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

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References

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Last Reviewed: 11/20/2017
Last Updated: 10/09/2018
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Emergency: requires immediate attention
Acute inflammatory demyelinating polyneuropathy
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View all Images (3)
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Acute inflammatory demyelinating polyneuropathy (AIDP) : Areflexia, CSF protein elevated, Paralysis, Urinary retention, Paresthesias
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