Guillain-Barre syndrome is a life-threatening, immune-related disorder of acute onset often precipitated by a preceding infection. Symptoms include ascending paralysis (leg weakness that "ascends" into the arms), paresthesias, and loss of reflexes.
CASES/YEAR
6,300 (US); 126,000 (Global)
OTHER NAMES
GBS; Acute inflammatory demyelinating polyneuropathy (AIDP); Acute infective polyneuritis; Postinfectious polyneuritis;
INCUBATION
7 days to 6 weeks
INITIAL SYMPTOMS
Ascending paralysis (leg weakness that "ascends" into the arms); paresthesias; loss of reflexes; 3 main features are: 1.) Acute onset; 2.) Elevated CSF protein; 3.) At least partial recovery; [Cecil, p. 2492-3]
PRECAUTIONS
Standard; "Not an infectious condition." [CDC 2007 Guideline for Isolation Precautions]
COMMENTS
FINDINGS:
Guillain-Barre syndrome (GBS) is the most common cause of acute paralytic disease in young adults. The illness begins with symmetrical weakness of the legs that ascends proximally over subsequent days and weeks to cause respiratory paralysis in severe cases. The arms and both sides of the face are frequently affected. Sensory symptoms are mild, but some patients complain of back and radicular pains. Patients often have autonomic nerve involvement with hypotension, hypertension, arrhythmias, and sweating abnormalities. In addition to typical GBS (85% of cases), other types are chronic inflammatory demyelinating polyneuropathy (13%), polyneuritis cranialis (1%), and Miller-Fisher syndrome (1%). Polyneuritis cranialis involves the cranial nerves only, and Miller-Fisher syndrome is an acute illness with ophthalmoplegia, areflexia, ataxia and some weakness. [ID, p. 1342] Limb weakness reaches its lowest point within 4 weeks. About 20-30% of patients will require mechanical ventilation. [5MCC-2020] About 5-10% of patients will require mechanical ventilation. [Merck Manual, p. 1983]
PRECIPITATING CAUSES:
It is often associated with a preceding infection: CMV, EBV, HIV, hepatitis C, Mycoplasma pneumoniae, Chlamydia psittaci, Lyme disease, and (most commonly) Campylobacter jejuni. Other precipitating factors are vaccination, surgery, malignancy, and other stressful events. [ID, p. 1342] GBS is a complication of C. jejuni infection (1 case per 2000 infections); it occurs 2-3 weeks after C. jejuni gastroenteritis. About 20-50% of GBS cases are caused by C. jejuni. [PPID, p. 2655]
DIFFERENTIAL DIAGNOSIS:
GBS is to be distinguished from Lyme disease, transverse myelitis, myasthenia gravis, botulism, diphtheria, poliomyelitis, advanced HIV infection, West Nile encephalitis, hypokalemia, toxic neuropathy, tick paralysis, porphyria, and toxin-induced syndromes from ingestion of fish or shellfish. [ID, p. 1342] The onset of ascending paralysis is rapid (24-48 hours) in tick paralysis, compared to slower onset (days to weeks) in GBS. [PPID, p. 3522t] "If the CSF cell count is greater than 50 WBCs/ml, another diagnosis, such as HIV infection or Lyme disease should be considered." [Cecil, 24th Ed, p. 2401]
DIAGNOSTIC
Clinical; Nerve conduction (slowing and segmental demyelination in 2/3 of patients); CSF: elevated protein but normal WBC count (CSF may be normal in first week and remains normal in 10% of patients); [Merck Manual, p. 1983]
SIGNS & SYMPTOMS
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E dysphagia
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N muscle weakness
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N paresthesia
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*cranial neuropathy
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*paralysis
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*peripheral neuropathy
ENTRY
Ingestion, Needle (Includes Drug Abuse), Scalpel or Transfusion, Skin or Mucous Membranes (Includes Conjunctiva), Sexual Contact
SOURCE
Person-to-Person, Human Fecal-Oral, Animal Excreta, Eating Contaminated Food, Eating Contaminated or Infected Meat, Eating Unpasteurized Milk or Cheese, Waterborne (Ingesting, Inhaling, or Swimming)
RESERVOIR
Birds and Poultry, Cattle, Goats and Sheep, Cats, Dogs, Horses, Rodents, Swine, Human, Wild Animals
TREATMENT
Measure forced vital capacity (FVC) to monitor for respiratory compromise (FVC <15 mL/kg). [Merck Manual, p. 1983]
REFERENCES FOR CASES/YEAR
1. (US) 1.2-3 cases per 100,000; [Gorbach, p. 286] Calculate: 2.1 x 3000 = 6300;
2. (Global) 20 X US cases/yr;