INFLAMMATORY DEMYELINATING NEUROPATHIES

Question 1

What is Guillain-Barre syndrome?

Answer 1

Weakness of the limbs as a result of an acute polyneuropathy, a disorder affecting the peripheral nervous system. Usually triggered by an infection, which provokes immune-mediated nerve dysfunction.

Question 2

What types of infections usually precede a diagnosis of Guillain-Barre syndrome?

Answer 2

Respiratory infection

Diarrhoea

Question 3

What bacteria has been particularly associated with Guillain-Barre syndrome?

Answer 3

Campylobacter jejuni

Question 4

How long after the infection do patient’s tend to start experiencing symptoms of Guillain Barre syndrome?

Answer 4

1-3 weeks

Question 5

Which virus has been particularly associated with Guillain Barre syndrome?

Answer 5

Cytomegalovirus

Question 6

What are the classical features of Guillain Barre syndrome?

Answer 6

Distal parasthesia (often with no sensory loss)
Proximal or generalized weakness
Facial weakness (often asymmetrical)
Back pain
Muscle pain
Pain in neck and head

Question 7

What is the time course of disease progression in Guillain Barre syndrome?

Answer 7

Symptoms ascend up lower limbs and body over days to weeks - often reaching a peak within 6 weeks
Plateau phase where disease persists but at a stable level
Recovery over many months

Question 8

What is the pattern of affected muscles in Guillain Barre syndrome?

Answer 8

Symmetrical weakness in arm and legs, and possible neck involvement
50% have some cranial nerve involvement - leading to eye problems, problems using face muscles and dysphagia.

Question 9

How long does the plateau phase of Guillain Barre syndrome tend to last for?

Answer 9

Median time is a week

Question 10

What are the classical signs of Guillain Barre syndrome that would be elicited on examination?

Answer 10

Reduced power

Reduced or absent reflexes

Question 11

What are the most worrying complications of Guillain Barre syndrome?

Answer 11

Weakness of breathing leading to respiratory failure, as well as infection.
Autonomic dysfunction leading to arrythmias
PE from not moving

Question 12

What is the treatment for those with respiratory failure as a result of Guillain Barre syndrome?

Answer 12

Intubation of the windpipe and breathing support through mechanical ventilation, generally on an intensive care unit.

Question 13

How might you be able to anticipate the need for ventilatory support in a patient with Guillain Barre syndrome?

Answer 13

Measurement of two spirometry-based breathing tests: the forced vital capacity (FVC) and the negative inspiratory force (NIF). An FVC of less than 15 ml per kilogram body weight or an NIF of less than 60 cmH2O are considered markers of severe respiratory failure.

Question 14

Other than respiratory failure, what other serious complications do a proportion of Guillain Barre syndrome patients experience?

Answer 14

Autonomic dysfunction

Question 15

What are the autonomic complications associated with Guillain Barre syndrome, affecting up to 60% of patients?

Answer 15

Severe blood pressure fluctuations 
Irregularities in the heart beat, sometimes to the point that the heart beat stops and requiring pacemaker-based treatment.
Abnormalities in perspiration
Changes in the pupillary response
Urinary retention
Constipation

Question 16

What is the proximal variant of Guillain Barre syndrome?

Answer 16

Miller-Fisher syndrome

Question 17

What is affected in Miller-Fisher syndrome?

Answer 17

This is a variant of Guillain Barre, affecting the more proximal muscles such as ocular muscles, muscles associated with areflexia and ataxia.

Question 18

How would you diagnose Guillain Barre syndrome?

Answer 18

Usually a clinical diagnosis
Supported by slowed conduction velocity studies
Raised CSF protein
Normal CSF cell count or mild lymphocytosis

Question 19

What antibodies will patients with Miller-Fisher syndrome have?

Answer 19

Anti-GQ1b ganglioside antibodies

Question 20

What is the differential diagnosis for someone showing the signs and symptoms of Guillain Barre syndrome?

Answer 20

Poliomyelitis
Myasthenia gravis
Botulism
Primary muscle disease

Question 21

How do you treat someone with Guillain Barre syndrome?

Answer 21

Recovery is usually spontaneous as long as the patient is monitored and maintained well enough - by the MDT team
Constant monitoring of heart and lung function is necessary - often in ITU
Pain management
Plasmapheresis
Intravenous immunoglobulin (IVIg)

Question 22

Are steroids used in the management of Guillain Barre syndrome?

Answer 22

Steroids have not been shown to be particularly successful in treatment of Guillain Barre syndrome.

Question 23

What is the mortality rate of Guillain Barre syndrome and what is this associated with?

Answer 23

5% - associated with inadequate ventilatory support, arrythmias, as well as complications of immobility (infection, PE).

Question 24

At what point do we no longer classify an inflammatory demyelinating neuropathy as Guillain Barre syndrome and what do we call it?

Answer 24

8 weeks

Chronic inflammatory demyelinating polyradiculoneuropathy

Question 25

What are the clinical features of chronic inflammatory demyelinating polyradiculoneuropathy?

Answer 25

Symmetrical weakness (both proximal and distal)
Sensory loss
Parasthesia

Question 26

Which muscles are more commonly affected by chronic inflammatory demyelinating polyradiculoneuropathy?

Answer 26

Arms
Legs
Neck
(NOT facial muscles)

Question 27

How could the pattern of sensory loss in inflammatory demyelinating neuropathies be described?

Answer 27

Glove and stocking

Question 28

What will examination of the CSF reveal in someone with chronic inflammatory demyelinating polyradiculoneuropathy?

Answer 28

High protein

Normal cell count

Question 29

Are steroids used in the treatment of chronic inflammatory demyelinating polyradiculoneuropathy?

Answer 29

Yes, unlike in Guillain Barre syndrome.

Question 30

What is the treatment for someone newly diagnosed with chronic inflammatory demyelinating polyradiculoneuropathy?

Answer 30

Management must always involve the MDT
Prednisolone
Plasmapheresis
Intravenous immunoglobulin
Immunosuppressants - eg Rituximab, cyclophosphamide or cyclosporins, azathioprine

Question 31

What is multifocal motor neuropathy?

Answer 31

Lower motor neuron variant of motor neuron disease, characterised by progressive, asymmetrical weakness without sensory involvement. Rare disease.

Question 32

What are the antibodies associated with multifocal motor neuropathy?

Answer 32

Anti-GM1 ganglioside antibodies

Question 33

What is the treatment for multifocal motor neuropathy?

Answer 33

Intravenous immunoglobulins