Guillain–Barré Syndrome
Jill C. Cash and Cheryl A. Glass
Definition
A.Guillain–Barré syndrome (GBS) is an acute immunemediated polyneuropathy of the peripheral nervous system. GBS often follows an infection.
B.GBS is not contagious, and there is no known cure.
C.GBS usually presents with ascending, progressive, multifocal, or symmetric muscle weakness, as well as paresthesia:
1.The first symptom of GBS is weakness or tingling sensations of the legs. Most people reach the stage of greatest weakness within the first 2 weeks after symptoms appear.
2.By the third week of GBS, 90% of patients are at their weakest.
D.GBS was considered monophasic and remits spontaneously but may also reoccur in 3% of patients. Twenty to thirty percent of patients will have persistent disability measured by tools such as the overall disability sum score (ODSS; see Table 23.2). An electronic version of the ODSS is available online at http://farmacologiaclinica.info/scales/overall-disability-sum-score. This application grades the arm (range 0–5) and the leg (range 0–7) to provide an overall range score. A total score of 0 equals no disability and a total score of 12 equals maximum disability. There are several variants of GBS:
1.Miller Fisher syndrome (MFS) often follows an infection, especially Campylobacter jejuni gastroenteritis.
2.Acute inflammatory demyelinating polyradiculoneuropathy (AIDP): Approximately two-thirds occur after an infection, including C. jejuni, cytomegalovirus (CMV), Mycoplasma pneumonia, influenza virus, or Zika virus.
3.Acute motor axonal neuropathy (AMAN).
4.Acute sensorimotor axonal neuropathy (AMSAN).
5.Acute panautonomic neuropathy (rare).
6.Bickerstaff’s brainstem encephalitis (BBE).
Incidence
A.The incidence of GBS is 0.89 to 1.89 cases per 100,000 adults. A 20% increase is seen in every 10-year rise in age after the first year of life.
B.The lifetime individual incidence is 1:100,000.
C.GBS occurs in all age groups. GBS is more common in older adults, with those older than 50 years of age at greatest risk.
D.80% to 90% become nonambulatory during the illness.
E.Relapses are not uncommon in adults who have been treated with intravenous immunoglobulin (IVIg) and plasma exchange.
F.Approximately 30% have a residual weakness after 3 years.
G.GBS severe enough to require mechanical ventilation is associated with both incomplete recovery and up to 20% mortality.
H.5% of patients with GBS die from medical complications such as sepsis, pulmonary emboli, and cardiac arrest related to dysautonomia.
I.GBS is reported throughout the world.
Pathogenesis
A.GBS is believed to be an immune-mediated response linked to an antecedent infection wherein a patchy demyelination of the motor component of multiple peripheral nerves occurs. This causes a failure of neuromuscular transmission and leads to abrupt, distal weakness, and symmetrical onset of paresthesias. The sensory disturbance is quickly followed by a rapid progressive limb weakness and sometimes paralysis. Most patients are able to identify a specific date of onset of sensory and motor symptoms.
Predisposing Factors
A.Up to two-thirds of patients with GBS have experienced a viral upper respiratory infection (URI) or gastroenteritis 10 to 14 days before onset:
1.C. jejuni gastroenteritis (30% of infections).
2.CMV (the second most common reported infection preceding GBS[10%]).
3.Epstein–Barr virus (EBV).
4.Mycoplasma pneumonia.
5.HIV.
6.Haemophilus influenza.
7.Enteroviruses.
8.Hepatitis A and B.
9.Herpes simplex.
10.Varicella-zoster virus.
11.Chlamydophila (formerly Chlamydia) pneumonia.
12.Zeka polyneuropathy.
B.Trauma.
C.Surgery.
D.Parturition.
E.Immunization:
1.In 1976 there was a small increase in GBS following the flu vaccine formulated to protect against swine flu.
2.Rabies vaccine is prepared from infected brain tissue.
F.There is no genetic factor for GBS.
Common Complaints
A.The classic clinical features:
1.Progressive, fairly symmetric muscle weakness (symmetric):
a.Difficulty walking.
b.Nearly complete paralysis, including the following:
i.All extremities, generally starting in the proximal legs.
ii.Facial muscles/oropharyngeal weakness.
iii.Respiratory muscles (dyspnea on exertion and shortness of breath [SOB]).
iv.Bulbar (ocular) muscles.
2.Accompanying absent or depressed deep tendon reflexes (DTRs).
B.Acute weakness in hands, dropping things, trouble picking up small objects or buttoning buttons, inability to feel textures.
C.Acute onset of persistent tingling or pins and needles, crawling-skin
sensation in feet, possibly in hands, and inability to feel pain.
D.Pain most severe in lower back pain, buttocks, thighs, and shoulder girdle.
Other Signs and Symptoms
A.Sinus tachycardia or other arrhythmias.
B.Bilateral, generally symmetric muscle weakness not improved by rest.
C.Urinary retention.
D.Ileus-gastric motility disorders.
E.Severe residual fatigue that may persist for years.
F.Loss of sweating.
G.Facial or pharyngeal weakness.
Subjective Data
A.Ask the patient about any dyspnea. If there is SOB, assess the need to go immediately by ambulance to a hospital.
B.Elicit information regarding the onset and duration of symptoms.
C.Question the patient regarding change or progression of symptoms.
D.Ascertain if the patient has had recent URI, flu, gastroenteritis, other infections, recent trauma, or surgery: Determine if there was an associated fever.
E.Look for paresthesias preceding weakness by approximately 24 to 48 hours.
F.Look for recent exposure to environmental hazards: Lead, pesticides, volatile solvents, or ticks.
Physical Examination
A.Check temperature (if indicated), pulse, respirations, and blood pressure (BP; persistent hypertension [HTN] alternating with hypotension, or orthostatic hypotension). HTN is seen in about one-third of patients with GBS and can be labile or followed by hypotension.
B.Inspect: Observe overall appearance; look for gait disturbance and respiratory distress.
C.Auscultate:
1.Note heart rate and rhythm: Tachycardia is common; bradycardia and other arrhythmias may be noted.
2.Auscultate the lung fields.
3.Abdomen assessment for bowel sounds/dysfunction. Gastrointestinal motility disorders occur in 15% of severely affected GBS patients.
D.Palpate:
1.Palpate the extremities.
2.Note muscle tone, normal muscle bulk.
3.Assess DTRs, muscle strength, areflexia (lack of reflexes), or hyporeflexia (diminished), although onethird of patients may have hyperreflexia.
4.Look for symmetric weakness. Incidence of weakness is greater in the ankle and knee than in biceps and triceps.
5.Palpate the abdomen: Assess urinary retention.
E.Neurologic exam:
1.Perform complete neurologic exam: Generally no sensory deficits to touch or pinprick are noted. Decreased proprioception or vibration may be seen. In approximately 50% of clients, GBS may progress rapidly, sometimes within hours, to severe respiratory muscle weakness and respiratory failure.
Diagnostic Tests
Prompt treatment mandates that the clinician make the diagnosis of GBS solely on history and examination. The presence of distal paresthesia increases the likelihood that the diagnosis is GBS. Testing done in the inpatient setting includes the following:
A.Lumbar puncture (LP):
1.The LP primarily rules out other infectious disease.
2.Most spinal fluid results for patients with GBS have elevated cerebrospinal fluid (CSF) protein levels with normal CSF cell counts.
B.Needle electromyogram.
C.Nerve conduction studies used to confirm the presence, pattern, and severity of neuropathy.
D.Antibody testing.
E.Serum testing of electrolytes, liver function tests, creatine phosphokinase (CPK), and erythrocyte sedimentation rate (ESR).
Differential Diagnoses
A.GBS.
B.Myasthenia gravis (MG).
C.Poliomyelitis.
D.Acute intermittent porphyria.
E.West Nile encephalomyelitis.
F.Tick paralysis: Lyme neuroborreliosis.
G.Diphtheria.
H.HIV.
I.Cervical myelopathy.
J.Sarcoidosis.
K.Poisoning:
1.Heavy metal poisoning (arsenic, lead, thallium).
2.Hexacarbon abuse (glue sniffing neuropathy).
3.Organophosphate poisoning.
4.Botulism.
Plan
A.General interventions: Early diagnosis is crucial to appropriate management. A possible diagnosis of GBS requires immediate hospitalization at a facility with ICU capabilities and consultation with a neurologist who has experience managing GBS.
B.Hospital medical management:
1.Plasmapheresis (plasma exchange).
2.IVIg administration.
3.Steroids have not been shown to be helpful and may be detrimental.
4.Because of the associated autonomic instability, HTN should be treated with short-acting intravenous (IV) agents.
5.The presence of at least four of the six predictors indicates the need for support/mechanical ventilation:
a.Onset of symptoms less than 7 days.
b.Inability to cough.
c.Inability to stand.
d.Inability to lift the elbows.