Guillain-Barré Syndrome (GBS)

 

Introduction

Guillain-Barré Syndrome (GBS) is an acute demyelinating disorder of the peripheral nervous system that results from an aberrant immune response directed at peripheral nerves.

 

Epidemiology

• All age groups are affected, but primarily seen in adults and rare in children under 2 years old
• Incidence in children: 0.4-1.3 cases per 100,000 per year
• Most common cause of acute flaccid paralysis

 

Pathogenesis

GBS occurs in relatively healthy persons that experience an antecedent infection/event anywhere from days to weeks prior to onset of GBS symptoms.

The most common infections reported in GBS cases are bacterial gastroenteritis (20%) and upper respiratory infection that was present approximately one to three weeks before the onset of any neurological symptoms.

Autonomic abnormalities in GBS are usually transient and reversible.

 

Clinical Presentation

Timing is important, as the clinical presentation is tri-phasic.  This includes an acute phase, a plateau of variable length, and a recovery phase lasting weeks to months.

Acute stage:     Onset and progression of symptoms. Hours to weeks.
                          Acute symptoms peak 2 weeks after onset.

Initial symptoms present between 1-4 weeks after respiratory or diarrheal illness and consist of:

• Weakness that manifests as disturbance of gait (refusal to walk, walking on a wide base, difficulty running or climbing stairs).
  • Often bilateral, not always
  • Usually begins distally and ascends (sometimes rapidly) to arms, face, and muscles of respiration.
  • *Note* Gait disturbance is due to weakness not cerebellar dysfunction
  • Click below for videos of walking with GBS. 

Video 1                 Video 2

• Vague parathesias
• Significant, poorly localized pain.  Pain (90% of patients) described like a “Charlie horse.” Severe with slight movement that should not normally cause pain
• Diminished or absent reflexes early in course.
• Cranial nerve abnormalities frequent (up to 50% of cases) with facial most commonly affected
• Pupillary abnormalities are rare and should lead to other diagnosis (such as botulism).
• Autonomic instability (cardiac dysrhythmias, orthostatic hypotension, hypertension, paralytic ileus, transient bladder dysfunction) common.

 

Classic Presentation

Persons with GBS experience a gradual symmetrical onset of parenthesis and numbness that begins at the feet and ascends at a variable rate. Progression may also include the hands and feet ascending distally to proximally, eventually leading to motor weakness then paralysis.

Sensory or autonomic dysfunction is present in about two thirds of cases, but is not necessarily present in all cases. Autonomic disruption can manifest in many variations such as hyper/hypotension, cardiac arrhythmias with wide variations, and other various symptoms.

 

Diagnosis

Requires that the following criteria are met: progressive weakness of more than two limbs, areflexia, and progression of symptoms in four weeks or less.

 

Variants

Once thought of as a single disease. There are now 5 different recognized variants of GBS. While named and delineated, the variants are not currently correlated with different and standardized treatment modalities or outcomes:

• Acute inflammatory demyelinating polyneuropathy, the prototype of GBS
• Acute motor axonal neuropathy
• Acute motor-sensory axonal neuropathy
• Miller Fisher syndrome
• Polyneuritis cranialis

 

Differential Diagnosis

• CNS:                                    Hysterical Paralysis
• Cerebellum:                        Acute cerebellar ataxia
• Spinal Cord:                        Transverse myelitis, spinal cord compression
• Anterior Horn Cell:             Poliomyelitis
• Peripheral Nerves:            Tick paralysis, toxic neuropathy, diphtheria,
                                              porphyria
• Neuromuscular Junction:  Botulism, Myasthenia gravis, neuromuscular
                                             blocking agents
• Muscle:                              Viral myositis, inflammatory myopathies,
                                             metabolic myopathies

 

Factors Unlikely to be Seen in GBS

• Marked persistent asymmetry of motor function
• Persistent bowel or bladder dysfunction
• Discrete sensory level
• Cerebrospinal fluid pleocytosis > 50 cells/mm3
• Ptosis
• Pupillary abnormalities
• Prominent bulbar signs
• Descending weakness

 

Laboratory Tests

• CSF:   Elevated protein without an elevated cell count (protein usually
             normal in first few days, rises by end of first week and peaks in 4-6
             weeks).
• EMG:   Later in disease, electromyography (EMG) shows signs of
             demyelination: prolonged distal latencies, conduction velocity
              slowing, and evidence of conduction block.
• Nerve conduction velocity
• Pulmonary function tests

 

Treatment

There is no cure for Guillain-Barre syndrome. However, many treatments are available to help reduce symptoms, treat complications, and speed up recovery.

• Symptomatic treatments and preventing complications:
      
  • Blood thinners may be used to prevent blood clots.
  • If the diaphragm is weak, breathing support or even a breathing tube and ventilator may be needed.
  • Pain is treated with anti-inflammatory medicines and narcotics, if needed.
  • Proper body positioning or a feeding tube may be used to prevent choking during feeding if the muscles used for swallowing are weak.
      
• Plasma exchange
  • ​This removes antibodies from the blood. Plasma exchange is a method by which whole blood is removed from the body and processed so that the red and white blood cells are separated from the plasma, or liquid portion of the blood. The blood cells are then returned to the patient without the plasma, which the body quickly replaces.
      
• IVIG
     
  • A second method is to block the antibodies using high-dose immunoglobulin therapy (IVIG). In this case, the immunoglobulins are added to the blood in large quantities, blocking the antibodies that cause inflammation.
  • Both plasma exchange and IVIG are equally effective, but immunoglobulin is easier to administer.
        
• Steroids
      
  • The use of steroid hormones has also been tried as a way to reduce the severity of GBS, but controlled clinical trials have demonstrated that this treatment not only is not effective but may even have a deleterious effect on the disease.

 

Recovery

The severity of GBS in children does not correlate with long-term outcome. Recovery better in children than adults: 85 percent of children can be expected to have an excellent recovery; 50% are ambulatory by six months; 70 percent walk within a year after onset . A better prognosis is associated with a gradual evolution of weakness.

Mortality is approximately 3 to 4 percent, and usually is secondary to respiratory failure or cardiac complications. The deaths due to GBS are attributed to autonomic disturbances with cardiac arrest being the most common cause (20-30% of deaths).

The recovery period may be as little as a few weeks or as long as a few years. About 30 percent of those with GBS still have a residual weakness after 3 years. About 3 percent may suffer a relapse of muscle weakness and tingling sensations many years after the initial attack.

 

Notes about previous infections (common) and immunizations (rare)

• 75% of GBS patients document a preceding infection.
• The most frequent infection preceding GBS is due to the pathogen Campylobacter jejuni (C. jejuni).
• The Center for Disease Control (CDC) estimates that over 2.5 million cases of Campylobacter enteritis occur each year in the United States alone. Food poisoning via C. jejuni is caused by consumption of raw or undercooked meat (mainly poultry), unpasteurized milk, home-canned foods, prepared foods (restaurants and home), or foods incubated in anaerobic environments.
• There was a small association with Swine Flu Vaccine in 1976, but current vaccination of influenza does NOT show increase rates of GBS.
• Medical events occur regardless of vaccination, and background rates are used to assess vaccine safety by comparing the expected rate of disease or death to the actual or observed rate in any given timeframe. The background rate for GBS in the U.S. is about 80 to 160 cases of GBS each week, regardless of vaccination.

http://www.cdc.gov/flu/protect/vaccine/guillainbarre.htm

• The CDC has a link at http://vaers.hhs.gov/index to report any adverse incidents with vaccines.

 

Patient Information and Support

Guillain-Barré Syndrom & Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) Foundation International

www.gbs-cidp.org

 

References

1. Rees J.H. et al. Campylobacter jejuni infection and Guillain-Barre Syndrome. NEJM November 1995
2. Newswanger and Warren Guillain-Barre’ Syndrome.  American Family Physician 2004
3. DiMario F.J. Intravenous Immunoglobulin in the Treatment of Childhood Guillain-Barre Syndrome: A Randomized Trial. Pediatrics 2005
4. McClellan K. Armeau E. Parish T. Recognizing Guillain-Barre Syndrome in the Primary Care Setting. The Internet Journal of Allied Health Sciences and Practice. Jan 2007
5. Rosen B.A. Guillian-Barre Syndrome. Pediatrics in Review. April 2012