Guillain-Barr Syndrome

(Ghee-yan Bah-ray)

Dr Hermanto Swatan, Sp.S, FINS

Quick Overview
Guillain-Barr 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-Barr 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 (Hodgkins)
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-Barr 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