Guillain-Barré Syndrome

“Ascending Paralysis “
AI-Destruction-Nodes of Ranvier
 Guillain-Barré Syndrome

Acute inflammatory demyelinating

polyneuropathy (AIDP) caused by an autoimmune
disorder affecting the peripheral nervous system,
usually triggered by an acute infectious process
characterized by ascending paralysis.
 Demyelination of Nerve Fibers

Negative conduction abnormalities

slowed axonal conduction, variable conduction blocks occur

in the presence of high- but not -low frequency volleys of
impulse.

Positive conduction abnormalities

generations of ectopic impulses, spontaneous and abnormal

“crosstalk” between demyelinated axons
IMMUNE RESPONSE
5
Immunopathogenesis
Acute autoimmune disorder
There is involvement of T
and B lymphocytes –
↑cytokines and cytokine receptors
in serum (IL 2, soluble IL 2
receptor) and CSF (IL 6, TNF α,
interferon)
Brain is unable to send
messages
Legs and arms are
commonly affected
 Etiology
 75% of cases are preceded by an
acute infectious process usually
GI or Respiratory in origin
 20-35% of cases are preceded by
a Campylobacter jejuni, HV, EBV
infection.
 Recent: swine influenza vaccine
 Destruction most often occurs in
segments between the Nodes of
Ranvier

Immune response to foreign antigens that are

mistargeted at host nerve tissues instead.

Source: Harrison Internal medicine pp 2509

 Why Nodes of Ranvier are the
target of attack?
Neural targets are likely to be
gangliosides

Gangliosides are complex

glycosphingolipids that contain one or
more sialic acid residue

Gangliosides are present in large

quantities in human nervous tissues and
in key sites: NODES OF RANVIER
Pathophysiology of GBS
Etiology Antigens enter
 Autoimmue into the body by
 Campylobacter multifenestrated cells
jejuni
 Virus
 EBV Innate immune response
 HV results in the uptake of the
 SIV pathogens by immature APC

Production of antibodies and

Phagocytosis of the bacteria

B cells are activated by newly

activated Th2 cells. This
produces a cell-mediated and
humoral response against the
pathogen.
 Migration to lymph nodes , a
mature, differentiated APC
activate CD4 T cells that
recognize antigen from the
infectious pathogen

Molecular mimicry

Immune responses directed

against the capsular components,
produce antibodies that
cross-react with myelin.

Lymphocytes and macrophages

circulate in the blood and eventually
find myelin.
 Lymphocytic infiltration of spinal
roots and peripheral nerves,
followed by macrophage-mediated,
multifocal stripping of myelin causing
axonal damage

Defects in the propagation of

electrical nerve impulses with
eventual conduction blocks

Guillain–Barré syndrome M. Fishers

Syndrome

Sensory Acute Dull aching Cranial nerve

changes: progressive pains of the involvement:
Paresthesia ascending lower back, facial droop(VII),
or numbness in weakness flank or lower dysphagia (V),
the hands/feet legs
Cranial Nerves and Their Functions Test

Number Name General Function Specific Function

I Olfactory Sensory Smell
II Optic Sensory Vision
III Oculomotor Motor, Parasympathetic Motor to four of six eye muscles and upper
eyelid; parasympathetic: constricts pupil;
thickens lens
IV Trochlear Motor Motor to one eye muscle
V Trigeminal Sensory, Motor Sensory to cornea face and teeth; motor to
muscles of mastication
VI Abducens Motor Motor to one eye muscle
VII Facial Sensory,Motor, Parasympathetic Sensory: taste; motor to muscles of facial
expression; parasympathetic to salivary and
tear glands
VIII Vestibulo-cochlear Sensory Hearing and balance

IX Glossopha-ryngeal Sensory,Motor, Parasympathetic Sensory: taste and touch to back of tongue;

motor to pharyngeal muscles;
parasympathetic to salivary glands
X Vagus Sensory,Motor, Parasympathetic Sensory to pharynx, larynx, and viscera;
motor to palate, pharynx, and larynx;
parasympathetic to viscera of thorax and
abdomen
XI Accessory Motor Motor to 2 neck and upper back muscles

XII Hypoglossal motor Motor to tongue muscles

Fatigue Scale Assessment

Muscle Strength Assessment

 Complications
 Breathing difficulties
 Residual numbness or other sensations

Long term complications:

 Serious, permanent problems with sensation and
coordination, including some cases of severe disability
 A relapse of Guillain-Barre syndrome

 Rarely, death from complications such as respiratory

distress syndrome
 Nursing Diagnosis
1. Acute Pain r/t stimulation of free nerve endings 2ndary
to nonsynaptic transmission of nerve axons.
2. Self care deficit r/t decrease strength and endurance.
3. Low Self–Esteem r/t disruption in how client perceive
one’s own body.
4. Ineffective airway clearance r/t neuromuscular
dysfunction
 Cont..Nursing Diagnosis
5. Bathing/hygiene, feeding, toileting self-care deficit
related to decrease energy production.
6. Fear related to sudden onset of illness.
7. Impaired spontaneous ventilation r/t denervation of
intercostal muscles.
 Diagnosis
 Diagnosis is made by recognizing the
pattern of rapidly evolving paralysis with
areflexia.
 Absence of fever or other systemic
symptoms and characteristics of antecedent
events.
Diagnostics
 Lumbar Puncture
In lumbar puncture “LP” CSF is withdrawn through a needle
inserted into the subarachnoid space of the spinal canal
between the L3-L4 or L4-L5 lumbar vertebrae.
 Measure CSF pressure

 determine viral or bacterial origin

 Increase in WBC count

 presence of cytokines (IL 6, TNF α, interferon)

 Cx: inc. ICP → rapid decrease in pressure within CSF

around spinal cord→ brain herniation

 Electromyography
- Needle electrodes inserted into the muscle .
- Pattern of electrical activity in the muscle both at rest and
during activity may be recorded.
- Relaxed muscles are normally electrically silent except in
motor end plates.
- Abnormal spontaneous activity with denervation or
inflammatory changes in the affected muscle.
- Fibrillation potentials and positive sharp waves – reflect
muscle irritability.
 Serum Antibody titer

 IgM and IgG are highest in the early course

of the disease.
 Diagnostic Tests
 Nerve conduction studies
 (Sensory) determining the conduction
velocity and amplitude of APs where
these fibers are stimulated at one
point.
 Helpful in determining whether sensory
symptoms arising from pathology are
proximal or distal to the root of ganglia
Normal conduction:
- Arms: 50-70 m/s
- Legs: 40-60 m/s
 Treatment
There is no cure for Guillain-Barré Syndrome, but there
are treatments available…

 Plasmapharesis
 Immunoglobulins
Question?
 references
 (1) Guillain-Barre Syndrome. Davids, Dr. Heather. University of Colorado School
of Medicine. 2006. Viewed at: http://www.emedicine.com/pmr/topic48.htm
 "Guillain-Barré Syndrome Fact Sheet." NINDS. NIH Publication No. 05-2902. Viewed
at http://www.ninds.nih.gov/disorders/gbs/detail_gbs.htm
 Van Doorn, P. A., (March 2003). Gullain-Barré syndrome. Retrieved September 2,
2008 from http://www.orpha.net/data/patho/GB/uk-Guillain.pdf
 Thomas. C. L. (18). (1997). Taber’s Encyclopedic Medical Dictionary . Philadelphia:
F.A. Davis Company.
 Http://www.healthscout.com/ency/68/653/main.html
 http://www.ninds.nih.gov/disorders/gbs/gbs.htm
 http://www.neurologychannel.com/guillain/treatment.shtml
 http://www.emedicine.com/pkm/topic48.htm#section~treatment