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Clinical aspects / Socioeconomical impact of Guillain-Barre Syndrome (GBS) dr. B. Jacobs Campylobacter jejuni & GBS Erasmus MC, Neurologie Molecular mechanisms / Epidemiology of C. jejuni-associated GBS

Q: What is the therapy for GBS?

Specific treatment • Plasma exchange or plasma pheresis • Intravenous immunoglobulins (IVIg) Supportive therapy • Artificial respiration • Prevention and treatment of complications (infections, pain) • Physiotherapy Rehabilitation

Q: What are intravenous immunoglobulins (IVIg)?

• Pooled from plasma from 3.000-10.000 healthy blood donors • Purification by enzymatic treatment, fractionation and chromatography • Contains: • Immunoglobulins: IgG >95% • Cytokines, soluble CD4, CD8 • Many other immune modulating peptides Effects: • Many immunomodulatory effects, but: • Therapeutic effect in GBS unknown

16 important questions on Clinical aspects / Socioeconomical impact of Guillain-Barre Syndrome (GBS) dr. B. Jacobs Campylobacter jejuni & GBS Erasmus MC, Neurologie Molecular mechanisms / Epidemiology of C. jejuni-associated GBS

What is the Most frequent cause of acute neuromuscular paralysis?

Guillain-Barre Syndrome

What is the incidence and life-time risk of Guillain-Barre Syndrome?

Incidence 1 to 2 per 100.000 persons per year; • Life-time risk 1 in 1.000

What are the neurological deficits in GBS?

Muscle paralysis

• Arms and legs

• Eye movements, facial muscles, swallowing, speech

• Respiratory insufficiency

Sensory deficits

• Pain, tingling

• Numbness, absent pain and touch sense

• Absent position sense (ataxia)

Autonomic dysfunction

• Tension fluctuations and cardiac arrhythmia

Areflexia

Name the two immune-mediated diseases of the nervous system and their affected areas:

Multiple sclerosis (MS)

Central nervous system:

- brain

- myelum (spinal cord)

Guillain-Barré syndrome (GBS)

Peripheral nervous system:

- nerve roots

- peripheral nerves

Which three types of GBS are there?

Sensory-motor forms (80%)

• Arms and legs muscle weakness and sensory deficits

• Frequently cranial nerves and autonomic dysfunction

Pure motore GBS (15%)

• Only arms and legs muscle weakness

• No cranial nerve, sensory nerve and autonomic nerve deficits

Miller Fisher syndroom (MFS) (5%)

• Only weakness of muscles involved in eye movements

• Ataxia

What are the diagnostic criteria for GBS?

Clinical features

• Rapidly progressive (<4 weeks)

• Symmetrical paresis of arms and legs

• Reduced or absent reflexes

Additional examinations

• Blood : no abnormalities

• Cerebrospinal fluid (liquor) : no cells, increased protein level

• Electrophysiology : demyelination, axonal degeneration

What is the therapy for GBS?

Specific treatment

• Plasma exchange or plasma pheresis

• Intravenous immunoglobulins (IVIg)

Supportive therapy

• Artificial respiration

• Prevention and treatment of complications (infections, pain)

• Physiotherapy

Rehabilitation

What are intravenous immunoglobulins (IVIg)?

• Pooled from plasma from 3.000-10.000 healthy blood donors

• Purification by enzymatic treatment, fractionation and chromatography

• Contains:

• Immunoglobulins: IgG >95%

• Cytokines, soluble CD4, CD8

• Many other immune modulating peptides

Effects:

• Many immunomodulatory effects, but:

• Therapeutic effect in GBS unknown

What is the prognosis of GBS?

Acute phase

• 20% artificial ventilation

• 5% mortality

Recovery phase

• Hospital admission median 2 months (1 week to >1 year)

• 20% unable to walk independently at 6 months

• Time to recovery to work median 10 - 12 months

• Usually severe residual fatigue

• Frequently ‘post-traumatic stress syndrome

Name 7 markers/mechanisms of disease progression and recovery, that could be researched in prognostic studies in GBS

 Clinical features

 Electrophysiology

 Preceding infections

 Anti-neural antibodies

 Genetic polymorphisms

 Pharmacokinetic

 Other biomarkers

What is the Socio-economic impact of GBS in The Netherlands?

High socio-economic impact because of:

• Expensive treatment of GBS and complications

• Long term admission to Intensive and Medium care

• Long term rehabilitation and physiotherapy

• Frequent psychological care

• Frequent unemployment

Estimation of gastro-enteritis-related GBS:

• 340 Disability Adjusted Life Year (DALY)

GBS is not a typical classic auto-immune disease, why?

 No predominance in females

 No association with other auto-immune diseases

 No relapsing-remitting or chronic disease course

 No association with specific HLA haplotypes

 No improvement after corticosteroids

GBS is a typical post-infectious disease, why?

 Two-third of patients have symptoms of a recent respiratory or gastro-intestinal infection or a vaccination.

 50-60% has serological evidence for a recent infection.

 Association between type of infection and clinical features.

 Association between type of infections and prognosis.

 >95% has a monophasic disease course.

How can mild infections or vaccinations trigger the onset of post-infectious diseases such as GBS?

Molecular mimicry: cross-reactive immune response

What are the Criteria for a molecular mimicry mediated disease?

1. Establishment of an epidemiological association between an infectious agent and the disease.

2. Identification of cross-reactive T cells or antibodies directed against microbial and host target antigens in patients.

3. Identification of microbial mimic of target antigen.

4. Induction of a cross-reactive immune response in an animal model.

5. Reproduction of the disease in an animal model.

What is the microbial mimic of target antigen in GBS?

Campylobacter jejuni lipo-oligosaccharide

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Clinical aspects / Socioeconomical impact of Guillain-Barre Syndrome (GBS) dr. B. Jacobs Campylobacter jejuni & GBS Erasmus MC, Neurologie Molecular mechanisms / Epidemiology of C. jejuni-associated GBS

16 important questions on Clinical aspects / Socioeconomical impact of Guillain-Barre Syndrome (GBS) dr. B. Jacobs Campylobacter jejuni & GBS Erasmus MC, Neurologie Molecular mechanisms / Epidemiology of C. jejuni-associated GBS

What is the Most frequent cause of acute neuromuscular paralysis?

What is the incidence and life-time risk of Guillain-Barre Syndrome?

What are the neurological deficits in GBS?

Name the two immune-mediated diseases of the nervous system and their affected areas:

Which three types of GBS are there?

What are the diagnostic criteria for GBS?

What is the therapy for GBS?

What are intravenous immunoglobulins (IVIg)?

What is the prognosis of GBS?

Name 7 markers/mechanisms of disease progression and recovery, that could be researched in prognostic studies in GBS

What is the Socio-economic impact of GBS in The Netherlands?

GBS is not a typical classic auto-immune disease, why?

GBS is a typical post-infectious disease, why?

How can mild infections or vaccinations trigger the onset of post-infectious diseases such as GBS?

What are the Criteria for a molecular mimicry mediated disease?

What is the microbial mimic of target antigen in GBS?

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