Guillain Barré syndrome Flashcards

1
Q

What is Guillain-Barre syndrome?

A

immune-mediated demyelination of peripheral nervous system, often triggered by an infection (classically Campylobacter jejuni)

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2
Q

What is the classical organism that can cause Guillain-Barre syndrome?

A

Campylobacter jejuni

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3
Q

What do 65% of patients with GBS experience as initial symptoms?

A

back/leg pain in initial stages

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4
Q

What is the characteristic clinical picture of Guillain Barre syndrome?

A

progressive, symmetrical weakness of all the limbs

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5
Q

What are 5 clinical neurological features of Guillain-Barré syndrome?

A
  1. Progressive, symmetrical weakness of all the limbs
  2. Weakness is classically ascending i.e. legs affected first
  3. Proximal muscles (e.g. hips/shoulders) usually affected before distal ones (e.g. feet/hands)
  4. Reflexes reduced or absent
  5. Sensory symptoms tend to be mild (e.g. distal paraesthesia) with very few sensory signs
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6
Q

What type of infection might there be a history of in GBS?

A

may be history of gastroenteritis

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7
Q

What are 3 possible additional features of Guillain-Barré syndrome?

A
  1. Respiratory muscle weakness
  2. Cranial nerve involvement - diplopia, bilateral facial nerve palsy, oropharyngeal weakness
  3. Autonomic involvement - urinary retention, diarrhoea
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8
Q

What are 3 types of features that may indicate cranial nerve involvement in Guillain-Barré syndrome?

A
  1. Diplopia
  2. Bilateral facial nerve (CNVII) palsy
  3. Oropharyngeal weakness (common)
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9
Q

What are 4 signs of autonomic involvement in GBS?

A
  1. Urinary retention
  2. Diarrhoea
  3. Arrhythmia
  4. Labile blood pressure
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10
Q

What may be present on fundal examination in GBS and why?

A

papilloedema - thought to be secondary to reduced CSF resorption

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11
Q

What are 5 key investigations to perform in GBS?

A
  1. Spirometry to look for reduced FVC
  2. ABG to look for T2RF
  3. Anti-ganglioside antibodies
  4. Lumbar puncture
  5. Nerve conduction studies
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12
Q

What will lumbar puncture show in some patients with GBS? In what proportion is this present?

A

Rise in protein with normal white blood cell count (albuminocytologic dissociation) found in 66%

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13
Q

What is thought to be the pathogenesis of Guillain-Barre syndrome?

A
  • cross reaction of antibodies with gangliosides in the peripheral nervous system
  • correlation between anti-ganglioside antibody (e.g. anti-GM1) and clinical features has been demonstrated, present in 25% of patients
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14
Q

What is the name of a key variant of Guillain-Barré syndrome?

A

Miller Fisher syndrome

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15
Q

What are 3 key signs of Miller Fisher syndrome?

A
  1. Ophthalmoplegia
  2. Areflexia
  3. Ataxia
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16
Q

Which muscles are typically affected first in Miller-Fisher syndrome?

A

eye muscles

17
Q

How does Miller Fisher syndrome usually present?

A

descending paralysis (rather than ascending)

+ ataxia, ophthalmoloplegia, areflexia

18
Q

Which antibodies are typically present in Miller Fisher syndrome?

A

anti-GQ1b antibodies

19
Q

After what time frame does Guillain-Barré syndrome typically present?

A

1-3 weeks after infection

20
Q

What are 3 causative organisms that can cause Guillain-Barré syndrome?

A
  1. Campylobacter jejuni
  2. Mycoplasma
  3. Epstein-Barr virus
21
Q

What proportion of cases of GBS are idiopathic?

A

40%

22
Q

What may occur in GBS secondary to respiratory muscle weakness?

A

Type 2 respiratory failure

23
Q

What are 2 signs of type 2 respiratory failure that may be present in GBS?

A
  1. CO2 retention flap
  2. bounding pulse
24
Q

What are 4 aspects of the conservative management of GBS?

A
  1. Monitoring of ventilation with serial spirometry and ABG
  2. Ventilation if required
  3. Measures to reduce risk of VTE (TEDs, LMWH)
  4. Protection of pressure areas
25
Q

What is the medical management of GBS? 2 steps

A
  1. IV immunoglobulin
  2. If ineffective: plasmapheresis
26
Q

What is the prognosis of GBS?

A
  • excellent for most patients
  • Neuro problems persist in 20% of patients; half of these severely disabled
  • 20% cannot walk unaided 6 months after onset
  • mortality rate 3-7%, mostly due to respiratory failure, pulmonary emboli or infection
27
Q

What are 3 factors associated with poor prognosis in GBS?

A
  1. Rapid progression of symptoms
  2. Advanced age
  3. Prolonged ventilation