Guillain-Barre Syndrome

Question 1

define guillain- Barre syndrome?

Answer 1

Acute inflammatory demyelinating polyneuropathy

Question 2

summarise Guillain Barre syndrome?

Answer 2

URTI/Campylobacter jej/ CMV/HIV

LMN sign

progresses acutely, ascending paraesthesia and pain + symmetrical limb weakness

Question 3

outline the aetiology of Guillain Barre syndrome?

Answer 3

inflammatory process where antibodies after a recent infection react with self- antigen on myelin or neurons

Triggers-> post-infection (Campylobacter jejuni, CMV, mycoplasma, zoster, HIV, EBV, vaccinations) and malignancy cause antibodies.

For 40% of cases, there is no aetiological trigger identified (idiopathic)

progressive phase of 4 weeks followed by recovery. It may advance quickly affecting all limbs at once causing paralysis.

Question 4

summarise the epidemiology of Guillain Barre syndrome?

Answer 4

UK incidence: 1-2/100,000/yr

Affects all age groups

Question 5

summarise the features of the Miller- Fish syndrome variant of Guillain Barre syndrome?

Answer 5

opthalmoplegia, areflexia and ataxia but NO MUSCLE WEAKNESS

Question 6

what are the presenting symptoms of Guillain Barre syndrome?

Answer 6

PROGRESSIVE symptoms

BILATERAL, near symmetry of symptoms

< 1 month duration of:

• ASCENDING symmetrical limb weakness (lower > upper)
• ASCENDING paraesthesia

Cranial nerve involvement (leading to, dysphagia, dysarthria, facial weakness)

Respiratory muscles may be affected in SEVERE cases

Pain common

Autonomic dysfunction: sweating, raised pulse, BP changes, arrhythmias

Miller-Fisher Variant (RARE) = ophthalmoplegia, ataxia, arreflexia

Question 7

what are the signs of Guillain Barre syndrome on physcial examination?

Answer 7

General MOTOR Examination

• Hypotonia
• Flaccid paralysis
• hyporeflexia (ascending upwards from feet to head)

General SENSORY Examination

Impairment of sensation in multiple modalities (ascending from feet to head)

Cranial Nerve Palsies

• Facial nerve weakness
• Abnormality of external ocular movements
• If pupil constriction is affected, consider botulism (DDx)

Type II Respiratory Failure

• Due to paralysis of respiratory muscles

Autonomic Function

• Assess postural blood pressure change and arrhythmias

NOTE: required for diagnosis: progressive weakness of all 4 limbs + areflexia

Question 8

what are the appopriate investigations for Guillain Barre syndrome?

Answer 8

Lumbar Puncture (CSF)

• HIGH protein
• NORMAL cell count and glucose

Nerve Conduction Study - confirmatory​-> reduced nerve conduction velocity

LFTs – more severe disease causing elevated AST and ALT (bilirubin may be transiently high but not enough to cause jaundice)

Anti-ganglioside antibodies in Miller-Fisher variant + 25% of Guillain-Barre cases

Spirometry

• Reduced fixed vital capacity - suggests ventilatory weakness
• Should be performed every 6 hrs initally