GBS

Question 1

What are the dominant forms of GBS in Europe and North America?

Answer 1

Demyelinating forms with a respiratory prodrome.

Question 2

Which GBS subtypes are more common in Asia?

Answer 2

Axonal subtypes with a preceding diarrheal illness.

Question 3

Which gender is more commonly affected by Guillain-Barré syndrome?

Answer 3

Men are more commonly affected than women.

Question 4

In which age group is the incidence of Guillain-Barré syndrome higher?

Answer 4

The incidence is higher in older age groups.

Question 5

What noninfectious factors are associated with preceding/triggering Guillain-Barré Syndrome?

Answer 5

Trauma, surgery, medications (eg ICI).

What about vaccinations?

Question 6

Which pathogens are most consistently associated with GBS?

Answer 6

Campylobacter jejuni, hepatitis E virus, cytomegalovirus, Epstein-Barr virus, Mycoplasma pneumoniae, zika virus.

Question 7

What is the most common infectious trigger for GBS in adults?

Answer 7

C. jejuni, present in 30% of all cases. In kids, M. Pneumonia was the most common pathogen.

Question 8

What was the severity of GBS presentations for patients with C. jejuni infection?

Answer 8

They had the most severe GBS presentations in all geographic areas.

Question 9

What is the maximum duration of the progressive phase in GBS?

Answer 9

Should not progress beyond 4 weeks.

Question 10

What are treatment-related fluctuations in GBS?

Answer 10

Up to two relapses (worsening GBS disability scale or MRC score) within 8 weeks.

Question 11

What percentage of GBS cases experience treatment-related fluctuations?

Answer 11

Up to 10% of cases.

Question 12

What condition should be considered in patients with three or more relapses or progression of GBS symptoms beyond 8 weeks?

Answer 12

Acute-onset chronic inflammatory demyelinating polyradiculoneuropathy (CIDP).

Question 13

What is the most common subtype of Guillain-Barré Syndrome in the US and Europe?

Answer 13

Acute inflammatory demyelinating polyradiculoneuropathy (AIDP).

Question 14

What are the earliest symptoms of Guillain-Barré Syndrome?

Answer 14

Distal paresthesia (acroparesthesia) and low back pain.

Question 15

Which cranial nerve-innervated muscles are affected in GBS?

Answer 15

Facial (50%), oropharyngeal (40%), and extraocular (5%-15%) muscles.

Question 16

What percentage of GBS patients require ventilatory support due to respiratory muscle weakness?

Answer 16

10% to 30% of patients.

Question 17

What proportion of patients with GBS experience autonomic abnormalities?

Answer 17

About two-thirds of patients have autonomic abnormalities.

Question 18

What autonomic subtype is more common in the acute phase of GBS?

Answer 18

Sympathetic activity typically predominates in the acute phase (sinus tachycardia is the most common).

Question 19

What autonomic subtype is more prominent in the recovery phase of GBS?

Answer 19

Parasympathetic failure is more prominent in the recovery phase.

Question 20

What are some uncommon clinical features in GBS?

Answer 20

Papilledema, facial myokymia, hearing loss, meningeal signs, vocal cord paralysis.

Question 21

In what region is AMAN more prevalent?

Answer 21

AMAN is more prevalent in Asia.

Question 22

What infectious illness (diarrheal or respiratory) typically precedes AMAN?

Answer 22

AMAN is typically preceded by diarrhea.

Question 23

What type of infectious illness typically precedes AMSAN?

Answer 23

Respiratory illness.

Question 24

What is more severe, AMAN or AMSAN?

Answer 24

AMSAN is often more severe.

Question 25

Does AMAN or AMSAN typically have more CN and autonomic involvement?

Answer 25

AMSAN.

Question 26

What are reflexes like in AMAN?

Answer 26

Muscle stretch reflexes might be normal or exaggerated in AMAN.

Question 27

What percentage of patients with Miller Fisher syndrome have GQ1b antibodies?

Answer 27

Approximately 85% to 90%.

Question 28

What are the three key clinical features of Miller Fisher syndrome?

Answer 28

Ophthalmoplegia, ataxia, and areflexia.

Question 29

What causes ataxia in Miller Fisher syndrome patients?

Answer 29

Cerebellar pathology (central) or Ia afferent neuron involvement (peripheral).

Question 30

What symptoms are associated with Bickerstaff brainstem encephalitis?

Answer 30

Impaired consciousness (reticular formation), paradoxical hyperreflexia (pyramidal tracts), ataxia, and ophthalmoparesis. MFS except increased reflexes and impaired consciousness

Question 31

What are the GBS variants?

Answer 31

- 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

Question 32

What does the pharyngeal-cervical-brachial variant mimic?

Answer 32

Botulism.

Question 33

Which GBS variant mimics an acute spinal cord lesion?

Answer 33

Paraparetic variant.

Question 34

What antibody is common in axonal variants of GBS?

Answer 34

GM1 antibodies.

Question 35

Which infection often precedes the axonal variants of GBS with GM1 antibodies?

Answer 35

C. jejuni infection.

Question 36

GQ1b is strongly expressed in which areas?

Answer 36

Extraocular muscles, muscle spindles, and reticular formation.

Question 37

What explains dysphagia in the context of GT1a expression?

Answer 37

GT1a expression in the glossopharyngeal and vagal nerves explains dysphagia.

Question 38

What nodal antibodies are involved in AMAN?

Answer 38

Presence of IgG anti-GM1 or anti-GD1a autoantibodies.

Question 39

What % of GBS pts have abnormal elevated CSF protein at 1 week? At 2 weeks?

Answer 39

At 1 week CSF protein can be normal in up to 50%. At 2 weeks it is increased in > 90%.

Question 40

By when do more than 90% of GBS patients show increased CSF protein levels?

Answer 40

By the end of the second week.

Question 41

In GBS, mild CSF pleocytosis (5-10) can be seen in what percentage of patients?

Answer 41

5%.

Question 42

What CNS imaging findings are associated with Miller Fisher syndrome?

Answer 42

Enhancement of cranial nerves or posterior columns.

Question 43

What does the '20/30/40 rule' assess in GBS patients?

Answer 43

Risk for respiratory failure based on vital capacity, inspiratory, and expiratory pressures.

Question 44

What vital capacity measurement indicates risk for respiratory failure in GBS?

Answer 44

Vital capacity <20 ml/kg.

Question 45

What maximum inspiratory pressure indicates risk for respiratory failure in GBS?

Answer 45

Maximum inspiratory pressure <30 cm H2O.

Question 46

What maximum expiratory pressure indicates a risk for respiratory failure in GBS?

Answer 46

<40 cm H2O.

Question 47

What factors prompt ICU admission consideration in GBS patients?

Answer 47

Dysautonomia, bulbar dysfunction, severe weakness, evolving respiratory distress.

Question 48

What does an Erasmus GBS Respiratory Insufficiency Score (EGRIS) score of more than 4 indicate?

Answer 48

A high (≥65%) risk of respiratory failure.

Question 49

What percentage of GBS patients require mechanical ventilation?

Answer 49

Mechanical ventilation is required for 10% to 30% of all patients with GBS.

Question 50

Do IVIg or plasma exchange stop GBS progression?

Answer 50

No, they do not stop disease progression or change nerve damage degree.

Question 51

What is less likely in patients with acute-onset CIDP than GBS patients?

Answer 51

Autonomic nervous system involvement, facial weakness, preceding infectious illness, or need for ventilation.

Question 52

What does the modified Erasmus GBS Outcome Score predict?

Answer 52

Probability of walking independently at 1, 3, and 6 months.

Question 53

When is the modified Erasmus GBS Outcome Score assessed?

Answer 53

At hospital admission or 1 week after admission.

Question 54

What factors are used in the modified Erasmus GBS Outcome Score?

Answer 54

Age, presence of preceding diarrhea, and the MRC sum score.

Question 55

What percentage of GBS patients can walk independently after 1 yr?

Answer 55

Approximately 80% of GBS patients can walk independently and 50% recover fully after 1 yr.

Question 56

How many GBS patients fully recover after 1 year?

Answer 56

More than half of GBS patients fully recover after 1 year.

Question 57

What is the overall mortality rate for GBS?

Answer 57

3% to 7%.

Question 58

What is the mortality rate for ventilator-dependent GBS patients?

Answer 58

Around 20%.

Question 59

What are indicators of a higher chance of death in GBS patients?

Answer 59

Advanced age, severe disease, 'associated comorbidities', pulmonary/cardiac complications, mechanical ventilation, systemic infection.