Objectives

• Describe the symptoms and clinical signs of Guillain-

Barré syndrome

• Cite the lumbar puncture and electrodiagnostic data

• List the main infectious agents involved

• List the main differential diagnosis

• State the principles of treatment.

 Introduction

• GBS is a rapidly progressive polyneuropathy that can result in complete

paralysis with respiratory failure within 24–48 h

• Incidence of GBS is 1.2–2.3 per 100 000 per year

• Men are about 1.5 times more frequently affected than women

• GBS is a post-infectious demyelinating polyradiculoneuropathy (diarrhea

due to Campylobacter jejuni or upper respiratory tract infection)

• Auto-antibodies produced against the myelin antigens

 Introduction

• The illness has been recognized as GBS since 1916, when Guillain, Barré,
and Strohl described two French soldiers who contracted the illness
during World War I.

• They described the clinical features including elevation of CSF protein.

• For unclear reasons, Strohl’s name was dropped from the term GBS
beginning in the early 20th century.
 Introduction

• Interest :
- Epidemiology : Since the eradication of polio, GBS has become the
most frequent cause of acute or subacute flaccid weakness

- Diagnostic and therapeutic emergency

- Prognostic : respiratory failure +++

 Pathophysiology

• Autoimmune disease: demyelination lesions are secondary to passage

through the endo-neural spaces of antibodies (Abs) directed against
myelin antigens.

• Mechanism of immunological cross-reaction: due to antigenic

similarities between the virus responsible and constituents of peripheral
nerves myelin sheet.
- Fixation of Abs on myelin sheet, activation of complement,

- Destruction of myelin and influx of T-cell and macrophages.

 Pathophysiology

• Mechanism of nerve injury

- Primary segmental demyelination
Slow nerve conduction velocities

- Secondary axonal degeneration

Muscle denervation with neurogenic pattern on EMG
 Pathophysiology

• About 70% of patients can identify a preceding illness:

- Viral infections: Cytomegalovirus, EpsteinBarr virus, influenza A,

- Bacterial infections: Mycoplasma pneumoniae, Haemophilus

influenzae, Enterovirus, and Campylobacter jejuni (40% of
cases)

- Other precipitants : surgery, pregnancy, cancer, and vaccinations