Guillain-Barré Syndrome

1. Description: also referred to as “acute inflammatory demyelinating polyradiculoneuropathy” (AIDP), which reflects its localization,
pathology, and pathogenesis
2. Epidemiology: incidence is 1/100,000 population
3. Pathogenesis
a. Immune response with myelin damage
b. Two-thirds of patients have antecedent respiratory tract infection (Epstein-Barr virus, cytomegalovirus, Mycoplasma) or
gastrointestinal tract infection (especially Campylobacter jejuni, cytomegalovirus)
c. Other possible precipitating events: vaccinations, trauma, surgery, cancer
4. Clinical presentation
a. Presentation: approximately 2 to 4 weeks after viral illness
b. “Ascending paralysis”: patients may develop distal paresthesias in extremities, followed within hours to days by lower
extremity weakness that may ascend to involve upper extremities and
cranial nerves (facial weakness, dysphagia, ptosis) c. Aspiration pneumonia may occur if dysphagia is severe
d. Radicular pain or cramps may occur
e. Respiratory failure may not be presenting symptom, but can occur early after other symptoms requiring frequent monitoring
(see below)
5. Physical examination findings
a. Symmetric weakness
b. Hyporeflexia or areflexia
c. Minimal loss of sensation despite paresthesias
d. Autonomic symptoms: cardiac conduction arrhythmias, orthostatic hypotension, hypertension, paralytic ileus, bladder
dysfunction, abnormal sweating
6. Variant forms
a. Miller Fisher variant
1) Patient presents with ophthalmoplegia, ptosis, ataxia, and hyporeflexia
2) Associated with the GQ1b ganglioside
b. Pharyngeal-cervical-brachial variant
7. Differential diagnosis (see Chapter 21)
8. Diagnostic evaluation
a. Head or spine imaging: may be important to rule out alternative lesion
b. Electromyography
1) Demyelinating polyradiculopathy, but can also have features of axonal degeneration
2) Demyelinating: slowed motor conduction velocities and distal latencies, motor conduction block, temporal
dispersion, slowed or absent F waves
3) H-reflex may be helpful
c. CSF
1) Protein is usually more than 50 mg/dL (usually within first week)
2) Few cells: if high leukocyte count, consider alternative diagnosis such as Lyme disease, human immunodeficiency
virus infection, or other meningeal processes
d. GM1b ganglioside: found in some cases of
Campylobacter-associated AIDP
e. GQ1b ganglioside: found in some cases of Miller Fisher variant
9. Specific therapy
a. Intravenous immunoglobulin (IVIG) or plasma exchange can be considered depending on cost and availability
b. Treatments can improve strength outcome earlier than placebo and decrease time of ventilator
c. Generally not considered for mild cases when patients are ambulatory
d. If clinical symptoms progress after reaching a plateau with treatment, additional therapy or alternate therapy can be
considered
e. IVIG (immune globulin)
1) Utility: can improve strength earlier than natural history
2) Regimen: 0.4 g/kg daily for 5 days
3) Side effects: aseptic meningitis, acute renal failure, anaphylaxis, pseudohyponatremia
4) Contraindications
a) Hypersensitivity to IVIG, human albumin, or thimerosal
b) Isolated immunoglobulin A deficiency with antibodies to IgA
 f. Plasma exchange
1) Utility: improve strength earlier than natural history
2) Regimen: five exchanges on alternate days with 5% albumin
3) Side effects: hypovolemia, allergy, catheter-related problems (infection, access), hypocalcemia, thrombocytopenia,
hypokalemia
4) Contraindications: septic shock, recent myocardial infarction, marked dysautonomia, active bleeding
10. Supportive therapy
a. Intensive care unit warranted for following:
1) Patients with rapid progression of symptoms
2) Bulbar and/or facial dysfunction
3) Severe autonomic dysfunction (arrhthymia, hypotension)
4) Respiratory compromise
a) Occurs in about one-third of patients
b) Prediction of respiratory compromise in Guillain- Barré syndrome (the 20/30/40 rule): if vital capacity is
less than 20 mL/kg, maximum inspiratory pressure less than –30 cm H2O, maximum expiratory pressure
less than 40 cm H2O
b. Other supportive measures
1) Monitor for respiratory changes
2) Deep venous thrombosis prevention
3) Decubitus ulcer prevention
4) Gastritis prevention
5) Pain control
6) Enteral feeding if necessary
7) Stool softener
8) Recognition and treatment of ileus
9) Monitoring for arrhythmia
10) Consider tracheostomy if prolonged course
11) Recognition and treatment of bladder paralysis
12) Recognition and treatment of depression
11. Clinical course and outcome
a. Clinical course: most patients reach plateau at 2 weeks and slowly improve over months
b. Serial respiratory monitoring is required because patient’s condition may deteriorate rapidly, requiring mechanical ventilation
(occurs in about one-third of patients)
c. Majority of patients have good long-term outcome

Medication used to manage status epilepticus

Medication Loading dose, mg/kg, Maintenance dose Therapeutic level, µg/mL
intravenous
Lorazepam 0.1 ---
Diazepam 0.15 ---
Phenytoin 18-20 300mg/d (adults) 10-20
4-6 mg/kg daily (children)
Fosphenytoin 18-20 mg/kg phenytoin Same as phenytoin Same as phenytoin
equivalents
Valproate (depacon) 15-20 50-100
Phenobarbital 20 200-320 mg/d (adult) 10-40
3-6 mg/kg daily (children)
Propofol 1-3 6-10 mg/kg per hour ---
Midazolam 0.2 1-10 µg/kg per minute ---
Pentobarbital 5 1-5 mg/kg per hour ---

Reference:
Mowzoon, N. Flemming, K.D., (2007). Principles of critical care neurology. In Neurology board review an illustrated study guide (pp.423-
424). Mayo Foundation for medical education and research