Guillain-Barré Syndrome(Ghee-yan Bah-ray)

Dr Hermanto Swatan, Sp.S, FINS

Quick Overview

Guillain-Barré is a non-contagious autoimmune disorder that affects approximately 1 in every 100,000 individuals in the United States.

It is not clear yet if a specific disease-causing agent is involved so Guillain-Barré is called a syndrome rather than a disease

Guillain-Barre Syndrome (GBS)

Acute, frequently severe fulminant polyradiculoneuropathy

Autoimmune Occurs year round 3500 cases per year in US and Canada Males are 1.5x than females Adults > Children

Pathogenesis

Acute autoimmune disorder

There is involvement of T lymphocytes and B lymphocytes

Brain is unable to send messages

Legs and arms are commonly affected

Etiology

There are varying degrees of severity

Onset usually occurs 1-3 weeks after exposure to a viral infection

Destruction most often occurs in segments between the Nodes of Ranvier

Etiology

Clinical Manifestations

Signs and Symptoms Include…

Paresthesia Muscle Weakness Possible Paralysis

Clinical Manifestations

Rapidly evolving (hours to days) Areflexic motor paralysis: “Rubbery legs” Sensory loss Proprioception Usually upward progressing Lower cranial nerves: OP dysphagia Pain: “Deep aching pain” Transient bladder dysfunction

Other Clinical Manifestations

Autonomic Dysfunction: Wide fluctuations in BP, Orthostatic Hypotension, and Cardiac Arrhythmias

SIADH

Antecedent Events

70% of cases occur 1-3 weeks after infectious process

20-30% of all cases are associated with Campylobacter jejuni (summer outbreaks in China among kids and young adults)

Also HHV, CMV or EBV Mycoplasma pneumoniae Recent Immunizations Lymphoma (Hodgkin’s) HIV SLE

Molecular Mimicry

Immune responses to non-self antigens Misdirect to host nerve tissue through resemblance Neural targets are gangliosides, particularly at the

Nodes of Ranvier

Molecular Mimicry

Diagnosis

Rapid development of muscle paralysis, areflexia, absence of fever

CSF: Elevated protein level without pleocytosis

EMG and Nerve conduction show slowing CSF and EMG are used to verify symptoms

but may not be abnormal until a week after onset

Required Diagnostic Criteria

Progressive, relatively symmetrical weakness of two or more limbs

Areflexia Course < 4 weeks Exclusion of other causes

Supportive Diagnostic Criteria

Symmetric Weakness accompanied by numbness and/or tingling

Mild Sensory Involvement Facial Nerve or other cranial nerve

involvement Absence of fever Typical CSF findings Evidence of demyelination from EMG

Differential Diagnosis Acute Myelopathies Botulism Diptheria Lyme disease with polyradiculitis Porphyria Vasculitis Neuropathy Poliomyelitis CMV polyradiculitis Critical Illness Neuropathy Myasthenia Gravis Poisonings with Organophosphates or arsenic Paresis from West Nile Virus Spinal Astrocytoma Motor Neuron Disease

Treatment

There is no cure for Guillain-Barré Syndrome, but there are treatments available…

Plasmapharesis Immunoglobulins

Treatment

Supportive Management: Telemetry, Blood pressure Rx, DVT prophylaxis, Vital Capacity/NIF

High dose IVIg or PLEX: Two weeks after the first motor symptom immunotherapy is no longer effective

Meta-anaylsis of randomized clinical trials indicate Rx reduces the need for mechanical ventilation by 50% and increases the likelihood of full recovery at one year from 55% to 68%

Steroids have not shown to be effective

Prognosis

Most of the time recovery starts after the 4th week from onset of disease

80% complete recovery within months to a year; Areflexia usually persists

5-10% have relapse CIDP Mortality rate is <5% in optimal settings Death results usually from pulmonary

complications

Thanks for your attention

Good Luck and Never Give Up