Peripheral Neuropathy - Guillain-Barre Syndrome

Question 1

What is Guillain-Barré syndrome?

Answer 1

Acute Paralytic Polyneuropathy that is immune-mediated demyelination of the PNS.

Question 2

Triggers of Guillain-Barré syndrome (3).

Answer 2

1. Campylobacter jejune.
2. CMV.
3. EBV.

Question 3

What is Miller Fisher Syndrome?

Answer 3

A variant of GBS associated with ophthalmoplegia, arreflexia and ataxia presenting as descending paralysis (anti-GQ1b Antibodies).

Question 4

Pathophysiology of GBS.

Answer 4

Molecular Mimicry : B-Cells create antibodies against antigens that cause the preceding infection; these antibodies also match proteins on nerve cells and so target proteins on the myelin sheath of the motor nerve cell or nerve axon e.g. anti-Ganglioside (anti-GM1) antibodies.

Question 5

Clinical Presentation of GBS (4).

Answer 5

1. Symptoms start within 4 weeks of preceding infection e.g. Gastroenteritis.
2. Typically begin in feet and progress upwards.
3. Symptoms peak within 2-4 weeks.
4. Recovery period can last months-years.

Question 6

Clinical Features of GBS (4).

Answer 6

1. Symmetrical Ascending Weakness (beginning in feet and moving upwards).
2. Hyporreflexia.
3. Peripheral Loss of Sensation/Neuropathic Pain.
4. Progression to Cranial Nerves e.g. Facial Nerve Weakness.

Question 7

Investigations of GBS.

Answer 7

1. Nerve Conduction Studies (Reduced Signal through Nerves - Demyelination).
2. Lumbar Puncture for CSF (Raised Protein + Normal Cell Count + Glucose) - Albuminocytologic Dissociation.
3. Diagnosis : Clinical using Brighton Criteria.

Question 8

Management of GBS (5).

Answer 8

1. IV Immunoglobulins (1st Line).
2. Plasma Exchange (2nd Line).
3. Supportive Care.
4. VTE Prophylaxis (PE is a cause of death).
5. Intubation, Ventilation and ITU Admission if Respiratory Failure.

Question 9

Prognosis of GBS (3).

Answer 9

1. 80% Fully Recover.
2. 15% Remain with Neurological Disability.
3. 5% Die.