Guillain-Barré syndrome

Question 1

Define Guillain-Barré syndrome

Answer 1

Acute inflammatory demyelinating polyneuropathy characterised by motor difficulty, absence of deep tendon reflexes, paraesthesia’s without objective sensory loss, and increased CSF albumin with absence of cellular reaction (albuminocytological dissociation)

Question 2

Aetiology of Guillain-Barré syndrome

Answer 2

Immune-mediated attack on the myelin sheath or Schwann cells of sensory and motor nerves due to cross-reaction of antibodies with gangliosides in the peripheral nervous system
Frequently triggered by an antecedent infection
Often no aetiological trigger identified (idiopathic in 40%). In other cases:
→ Post-infection (1-3 weeks): bacterial e.g. campylobacter jejuni, HIV, herpes
→ Malignancy (lymphoma, Hodgkin’s disease)
→ Post-vaccination

There are rare axonal variants with no demyelination.

Question 3

Risk factors for Guillain-Barré syndrome

Answer 3

Preceding viral or bacterial illness
Preceding mosquito-borne viral infection
Hep E infection
Immunisation
Cancer and lymphoma
Older age
HIV infection
Male

Question 4

Symptoms of Guillain-Barré syndrome

Answer 4

Progressive symptoms of <1 month duration of ascending symmetrical limb weakness (lower>upper) and ascending paraesthesia
- Muscle weakness
- Respiratory distress (dyspnoea on exertion + SOB)
- Speech problems (due to facial and oropharyngeal weakness)
- Paraesthesia
- Back/leg pain (65% preceding weakness)
- Diplopia
- Dysphagia
- Dysarthria

Question 5

Signs of Guillain-Barré syndrome

Answer 5

General
- Altered consciousness level
- Dysautonomia - sinus tachycardia, hypertension, postural hypotension, urinary retention, ileus.
Limbs
- ASCENDING symmetrical limb weakness and paraesthesia (LMN)
- Arreflexia/hyporeflexia/flaccid paralysis
Impaired sensation - tends to mild with few sensory signs
- Ataxia - slurred speech, stumbling, falling and incoordination due to cerebellum damage
CNs
- Facial weakness
- Bulbar dysfunction causing oropharyngeal weakness
- Facial droop
Eyes
- Anisocoria
- Non-reactive pupil
- Extra-ocular muscle weakness
- Ophthalmoplegia
- Ptosis
Generally:
- Hypotonia/flaccid paralysis/arreflexia
- Facial nerve weakness
- TIIRF - identify via CO2 flap, bounding pulse, drowsiness
- Autonomic dysfunction - postural BP and arrhythmias

Question 6

What is Miller-Fisher variant

Answer 6

Ophthalmoplegia, ataxia and arreflexia

Question 7

Investigations for Guillain-Barré syndrome

Answer 7

ECG: ?arrhythmia

Anti-ganglioside antibodies: positive 25%, positive in Miller-Fisher variant

Lumbar puncture: raised CSF protein/albumin, cell count + glucose normal
Nerve conduction studies: reduced conduction velocity/conduction block (may be normal if early), prolonged distal motor latency, increased F wave latency

Spirometry: ?ventilator weakness (reduced FVC)

Question 8

Management for Guillain-Barré syndrome

Answer 8

1. IVIg / plasma exchange
2. Monitor: BP, pulse
3. Thromboprophylaxis e.g. DOAC, LMWH, unfractionated heparin
4. Respiratory maintenance: calculate the Erasmus GBS respiratory insufficiency score (EGRIS)
   a. Bulbar dysfunction or risk of aspiration → elective intubation

Question 9

Complications of Guillain-Barré syndrome

Answer 9

Respiratory failure
Bladder areflexia
Adynamic ileus
Paralysis
Fatigue
Immobilisation hyeprcalcaemia
DVT

Question 10

Prognosis for Guillain-Barré syndrome

Answer 10

Overall good prognosis, 85% of survivors make good functional recovery and can walk independently
Miller-Fisher has a better prognosis, recovers without treatment in 6 months