GBS Flashcards
What are the dominant forms of GBS in Europe and North America?
Demyelinating forms with a respiratory prodrome.
Which GBS subtypes are more common in Asia?
Axonal subtypes with a preceding diarrheal illness.
Which gender is more commonly affected by Guillain-Barré syndrome?
Men are more commonly affected than women.
In which age group is the incidence of Guillain-Barré syndrome higher?
The incidence is higher in older age groups.
What noninfectious factors are associated with preceding/triggering Guillain-Barré Syndrome?
Trauma, surgery, medications (eg ICI).
What about vaccinations?
Which pathogens are most consistently associated with GBS?
Campylobacter jejuni, hepatitis E virus, cytomegalovirus, Epstein-Barr virus, Mycoplasma pneumoniae, zika virus.
What is the most common infectious trigger for GBS in adults?
C. jejuni, present in 30% of all cases. In kids, M. Pneumonia was the most common pathogen.
What was the severity of GBS presentations for patients with C. jejuni infection?
They had the most severe GBS presentations in all geographic areas.
What is the maximum duration of the progressive phase in GBS?
Should not progress beyond 4 weeks.
What are treatment-related fluctuations in GBS?
Up to two relapses (worsening GBS disability scale or MRC score) within 8 weeks.
What percentage of GBS cases experience treatment-related fluctuations?
Up to 10% of cases.
What condition should be considered in patients with three or more relapses or progression of GBS symptoms beyond 8 weeks?
Acute-onset chronic inflammatory demyelinating polyradiculoneuropathy (CIDP).
What is the most common subtype of Guillain-Barré Syndrome in the US and Europe?
Acute inflammatory demyelinating polyradiculoneuropathy (AIDP).
What are the earliest symptoms of Guillain-Barré Syndrome?
Distal paresthesia (acroparesthesia) and low back pain.
Which cranial nerve-innervated muscles are affected in GBS?
Facial (50%), oropharyngeal (40%), and extraocular (5%-15%) muscles.
What percentage of GBS patients require ventilatory support due to respiratory muscle weakness?
10% to 30% of patients.
What proportion of patients with GBS experience autonomic abnormalities?
About two-thirds of patients have autonomic abnormalities.
What autonomic subtype is more common in the acute phase of GBS?
Sympathetic activity typically predominates in the acute phase (sinus tachycardia is the most common).
What autonomic subtype is more prominent in the recovery phase of GBS?
Parasympathetic failure is more prominent in the recovery phase.
What are some uncommon clinical features in GBS?
Papilledema, facial myokymia, hearing loss, meningeal signs, vocal cord paralysis.
In what region is AMAN more prevalent?
AMAN is more prevalent in Asia.
What infectious illness (diarrheal or respiratory) typically precedes AMAN?
AMAN is typically preceded by diarrhea.
What type of infectious illness typically precedes AMSAN?
Respiratory illness.
What is more severe, AMAN or AMSAN?
AMSAN is often more severe.
Does AMAN or AMSAN typically have more CN and autonomic involvement?
AMSAN.
What are reflexes like in AMAN?
Muscle stretch reflexes might be normal or exaggerated in AMAN.
What percentage of patients with Miller Fisher syndrome have GQ1b antibodies?
Approximately 85% to 90%.
What are the three key clinical features of Miller Fisher syndrome?
Ophthalmoplegia, ataxia, and areflexia.
What causes ataxia in Miller Fisher syndrome patients?
Cerebellar pathology (central) or Ia afferent neuron involvement (peripheral).
What symptoms are associated with Bickerstaff brainstem encephalitis?
Impaired consciousness (reticular formation), paradoxical hyperreflexia (pyramidal tracts), ataxia, and ophthalmoparesis.
MFS except increased reflexes and impaired consciousness
What are the GBS variants?
- Acute bulbar palsy
- Bilateral Facial Palsy with Paresthesias
- Pharyngeal-Cervical-Brachial (can look like botulism d/t oropharynx/neck/shoulder involvement)
- Paraparetic (BLE)
- Pure sensory Ataxia
- Acute pandysautonomia
What does the pharyngeal-cervical-brachial variant mimic?
Botulism.
Which GBS variant mimics an acute spinal cord lesion?
Paraparetic variant.
What antibody is common in axonal variants of GBS?
GM1 antibodies.
Which infection often precedes the axonal variants of GBS with GM1 antibodies?
C. jejuni infection.
GQ1b is strongly expressed in which areas?
Extraocular muscles, muscle spindles, and reticular formation.
What explains dysphagia in the context of GT1a expression?
GT1a expression in the glossopharyngeal and vagal nerves explains dysphagia.
What nodal antibodies are involved in AMAN?
Presence of IgG anti-GM1 or anti-GD1a autoantibodies.
What % of GBS pts have abnormal elevated CSF protein at 1 week? At 2 weeks?
At 1 week CSF protein can be normal in up to 50%. At 2 weeks it is increased in > 90%.
By when do more than 90% of GBS patients show increased CSF protein levels?
By the end of the second week.
In GBS, mild CSF pleocytosis (5-10) can be seen in what percentage of patients?
5%.
What CNS imaging findings are associated with Miller Fisher syndrome?
Enhancement of cranial nerves or posterior columns.
What does the ‘20/30/40 rule’ assess in GBS patients?
Risk for respiratory failure based on vital capacity, inspiratory, and expiratory pressures.
What vital capacity measurement indicates risk for respiratory failure in GBS?
Vital capacity <20 ml/kg.
What maximum inspiratory pressure indicates risk for respiratory failure in GBS?
Maximum inspiratory pressure <30 cm H2O.
What maximum expiratory pressure indicates a risk for respiratory failure in GBS?
<40 cm H2O.
What factors prompt ICU admission consideration in GBS patients?
Dysautonomia, bulbar dysfunction, severe weakness, evolving respiratory distress.
What does an Erasmus GBS Respiratory Insufficiency Score (EGRIS) score of more than 4 indicate?
A high (≥65%) risk of respiratory failure.
What percentage of GBS patients require mechanical ventilation?
Mechanical ventilation is required for 10% to 30% of all patients with GBS.
Do IVIg or plasma exchange stop GBS progression?
No, they do not stop disease progression or change nerve damage degree.
What is less likely in patients with acute-onset CIDP than GBS patients?
Autonomic nervous system involvement, facial weakness, preceding infectious illness, or need for ventilation.
What does the modified Erasmus GBS Outcome Score predict?
Probability of walking independently at 1, 3, and 6 months.
When is the modified Erasmus GBS Outcome Score assessed?
At hospital admission or 1 week after admission.
What factors are used in the modified Erasmus GBS Outcome Score?
Age, presence of preceding diarrhea, and the MRC sum score.
What percentage of GBS patients can walk independently after 1 yr?
Approximately 80% of GBS patients can walk independently and 50% recover fully after 1 yr.
How many GBS patients fully recover after 1 year?
More than half of GBS patients fully recover after 1 year.
What is the overall mortality rate for GBS?
3% to 7%.
What is the mortality rate for ventilator-dependent GBS patients?
Around 20%.
What are indicators of a higher chance of death in GBS patients?
Advanced age, severe disease, ‘associated comorbidities’, pulmonary/cardiac complications, mechanical ventilation, systemic infection.
