Guillain Barré syndrome Flashcards
What is Guillain-Barre syndrome?
immune-mediated demyelination of peripheral nervous system, often triggered by an infection (classically Campylobacter jejuni)
What is the classical organism that can cause Guillain-Barre syndrome?
Campylobacter jejuni
What do 65% of patients with GBS experience as initial symptoms?
back/leg pain in initial stages
What is the characteristic clinical picture of Guillain Barre syndrome?
progressive, symmetrical weakness of all the limbs
What are 5 clinical neurological features of Guillain-Barré syndrome?
- Progressive, symmetrical weakness of all the limbs
- Weakness is classically ascending i.e. legs affected first
- Proximal muscles (e.g. hips/shoulders) usually affected before distal ones (e.g. feet/hands)
- Reflexes reduced or absent
- Sensory symptoms tend to be mild (e.g. distal paraesthesia) with very few sensory signs
What type of infection might there be a history of in GBS?
may be history of gastroenteritis
What are 3 possible additional features of Guillain-Barré syndrome?
- Respiratory muscle weakness
- Cranial nerve involvement - diplopia, bilateral facial nerve palsy, oropharyngeal weakness
- Autonomic involvement - urinary retention, diarrhoea
What are 3 types of features that may indicate cranial nerve involvement in Guillain-Barré syndrome?
- Diplopia
- Bilateral facial nerve (CNVII) palsy
- Oropharyngeal weakness (common)
What are 4 signs of autonomic involvement in GBS?
- Urinary retention
- Diarrhoea
- Arrhythmia
- Labile blood pressure
What may be present on fundal examination in GBS and why?
papilloedema - thought to be secondary to reduced CSF resorption
What are 5 key investigations to perform in GBS?
- Spirometry to look for reduced FVC
- ABG to look for T2RF
- Anti-ganglioside antibodies
- Lumbar puncture
- Nerve conduction studies
What will lumbar puncture show in some patients with GBS? In what proportion is this present?
Rise in protein with normal white blood cell count (albuminocytologic dissociation) found in 66%
What is thought to be the pathogenesis of Guillain-Barre syndrome?
- 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
What is the name of a key variant of Guillain-Barré syndrome?
Miller Fisher syndrome
What are 3 key signs of Miller Fisher syndrome?
- Ophthalmoplegia
- Areflexia
- Ataxia
Which muscles are typically affected first in Miller-Fisher syndrome?
eye muscles
How does Miller Fisher syndrome usually present?
descending paralysis (rather than ascending)
+ ataxia, ophthalmoloplegia, areflexia
Which antibodies are typically present in Miller Fisher syndrome?
anti-GQ1b antibodies
After what time frame does Guillain-Barré syndrome typically present?
1-3 weeks after infection
What are 3 causative organisms that can cause Guillain-Barré syndrome?
- Campylobacter jejuni
- Mycoplasma
- Epstein-Barr virus
What proportion of cases of GBS are idiopathic?
40%
What may occur in GBS secondary to respiratory muscle weakness?
Type 2 respiratory failure
What are 2 signs of type 2 respiratory failure that may be present in GBS?
- CO2 retention flap
- bounding pulse
What are 4 aspects of the conservative management of GBS?
- Monitoring of ventilation with serial spirometry and ABG
- Ventilation if required
- Measures to reduce risk of VTE (TEDs, LMWH)
- Protection of pressure areas
What is the medical management of GBS? 2 steps
- IV immunoglobulin
- If ineffective: plasmapheresis
What is the prognosis of GBS?
- 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
What are 3 factors associated with poor prognosis in GBS?
- Rapid progression of symptoms
- Advanced age
- Prolonged ventilation
