Emergency: requires immediate attention
Acute inflammatory demyelinating polyneuropathy
Alerts and Notices
Synopsis
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, HIV, and Japanese encephalitis. 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. There have been rare reports of GBS occurring about 2 weeks after having the Johnson & Johnson (Janssen) COVID-19 vaccine.
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 immunoglobulin G (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.
- Neuropathic pain, typically bilateral leg pain and lower back pain
- Gait unsteadiness and/or refusal to walk
- Facial weakness
- Autonomic dysfunction
Codes
ICD10CM:G61.89 – Other inflammatory polyneuropathies
SNOMEDCT:
26261000119109 – Acute inflammatory demyelinating polyneuropathy
Look For
Subscription Required
Diagnostic Pearls
Subscription Required
Differential Diagnosis & Pitfalls
- Nutritional neuropathy (eg, deficiencies of thiamine, niacin, vitamin B12, vitamin B6, vitamin E; celiac disease)
- Vasculitis of the peripheral nervous system
- Monoclonal gammopathies (multiple myeloma, osteosclerotic myeloma, Waldenstrom macroglobulinemia)
- Acute myelopathy
- Myasthenia gravis
- Poliomyelitis
- Botulism
- Diphtheria (see cutaneous, respiratory)
- Periodic paralysis (eg, familial hypokalemic periodic paralysis)
- Acute intermittent porphyria
- West Nile virus
- Acute uremic polyneuropathy
- Tick paralysis
- Alcoholic neuropathy (see Wernicke-Korsakoff syndrome)
- HIV, Lyme disease, sarcoidosis, central nervous system lymphoma when facial diplegia is prominent
Best Tests
Subscription Required
Management Pearls
Subscription Required
Therapy
Subscription Required
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.Subscription Required
References
Subscription Required
Last Reviewed:11/19/2017
Last Updated:04/03/2023
Last Updated:04/03/2023
Emergency: requires immediate attention
Patient Information for Acute inflammatory demyelinating polyneuropathy
Overview
Guillain-Barré syndrome (GBS) is a group of nerve disorders. In the United States, acute inflammatory demyelinating polyneuropathy (AIDP) is the most common type, causing progressive muscle weakness, numbness, and poor reflexes in the tendons, usually starting in the feet, legs, and arms. The muscle weakness is symmetrical, occurring on both sides of the body, such as both legs. Described as ascending (moving upward), it comes on rather quickly, usually over a 2-week period, and can progress to other parts of the body (lungs, face, back), leading to paralysis in severe cases.Miller Fisher syndrome (MFS) is another variation of the disorder. MFS has several presentations, which may include eye movement paralysis, walking problems, and reflex problems in various combinations. Other types of GBS include acute motor axonal neuropathy and acute motor-sensory axonal neuropathy.
GBS usually occurs following a respiratory or gastrointestinal illness. It is a type of autoimmune disorder, which means the body produces antibodies that attack the body's own healthy nerve cells.
Who’s At Risk
GBS has many variations and is not associated with any one cause. Children and adults who have recently had a viral or bacterial infection, certain systemic illnesses and malignancies, bone marrow transplant, surgery with blood transfusion, and other conditions have been found to develop GBS. It is also associated with certain vaccinations and medications. It may run in families due to a genetic link. A small percentage of patients with possible Zika virus have been reported to have GBS.Signs & Symptoms
Following a respiratory or gastrointestinal illness, AIDP may begin with muscle tingling, numbness, or weakness on both sides of the body, usually starting with the feet, legs, and arms and progressing upward to the body, face, lungs, and back. Muscle function can become impaired to the point of paralysis. Other symptoms can include weak tendon reflexes, rapid heart rate, difficulty urinating, back pain, shortness of breath, difficulty breathing or swallowing, facial weakness, hearing loss, and a sudden drop in blood pressure upon standing (orthostatic hypotension).A child may commonly present with pain and a wobbly gait within 2-4 weeks of an upper respiratory or gastrointestinal illness.
Self-Care Guidelines
Monitor your symptoms and report them to your health care provider. Ask your doctor for instructions on when to go to the emergency room by ambulance for extreme weakness, paralysis, or breathing impairment.When to Seek Medical Care
If you develop numbness, muscle weakness, and poor reflexes that progress rapidly, contact your health care provider.Treatments
Your doctor will examine you or your child and run tests to determine the cause of muscle weakness. Although there is no cure for GBS, your doctor may place you in intensive care, insert breathing tubes to improve lung function if needed, and monitor blood pressure. Standard treatments are intravenous immune globulin (IVIG) and plasma exchange. Physical, speech, and occupational therapy can help you to regain muscle function.Emergency: requires immediate attention
Acute inflammatory demyelinating polyneuropathy