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Guillain-Barré Syndrome

Pathophysiology
by

S Habib

on 25 February 2014

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Transcript of Guillain-Barré Syndrome

Pathophysiology II
Guillain-Barré Syndrome
The atypical autoimmune disease
E & S
Pathophysiology II
Why Atypical?
Men are 1.5 time more likely to develop GBS
The disease is monophasic
Does not respond to immunosuppresive medications
Exogenous in etiology
Absence of typical immunogenic background
Etiology
Infection
respiratory or GI
Examples:
Epstein-Barr
Mycoplasma pneumonia
Campylobacter jejuni
Cytomegalovirus
HIV/AIDS
Pathophysiology
C. jejuni's LOS
structure
= ganglioside GM1's structure
IgG antibodies recognize GM1 as foreign
IgG targets GM1 for immune response
Damage to neurons and presentation of symptoms
References
Signs and Symptoms
Types
Acute Inflammatory Demyelinating Polyneuropathy (AIDP)
a.k.a. Classic Guillain-Barré (97% incidence)
Acute Motor Axonal Neuropathy (AMAN)
Acute Motor Sensory Axonal Neuropathy (AMSAN)
Miller Fisher Syndrome
Pathogenesis
Study related to AMAN and C. jejuni to prove molecular mimic theory
Physiology of nerve cells
Schwann
Myelin Sheath
Ganglioside
Auto-antibodies
IgG
Physiology of C. jejuni
Lipo-oligosaccarides (LOSs)
Outline
Interesting Facts
History
Types
Etiology
Pathogenesis
Pathophysiology
Signs and Symptoms
Diagnostics
Treatment
Prognosis
What's New?
References
¿Your turn?
What's new?
Complement inhibiting factors
Complement activation followed by membrane attack complex (MAC) formation is an improtant mechanism for the glial and neuronal injury
Nafamostat mesilate
Eculizumab (C5 inhibitor)
CSF filtration
Antibodies may be present in CSF fluid
Needs more research
Pathophysiology continued
Disease course is predictable:
Acute phase
Lasts about 4 weeks
Plateau phase
Days - weeks
Recovery phase
Weeks, months, sometimes years
Diagnosis
Challenging, often resulting in extensive differential diagnosis
Medical assessments and history
Stool culture/serology for infection
Lytes, Urea, Creatinin, Lumbar puncture
Blood sugar
Drugs, toxins

Neuromusclular biopsy
Nerve conduction test
MRI: Brain and Spinal cord
Electromyography (EMG)
Cerebrospinal fluid
Acetylecholine receptor
Autoantigens
AIDP
Myelin sheath and Schwann cells
AMAN
Spaces between cells to directly target axons
Dysfunction:
Sensory
Numbness, tingling, pain, paraesthesia
Motor
Ascending weakness/paralysis
Autonomic
Blood pressure and heart regulation
Cranial nerve
Facial weakness/ spasms
Respiratory
IV immunoglobulin G (IVIgG)
Administering IgG
Equally as effective as PE
Fewer adverse effects
Fewer patients drop out of therapy than in PE
Treatment
Plasmapheresis (PE)
Filtering plasma
Outcome associated with:
lower disability rates at 1 year
Fewer deaths
Corticosteroi
ds
Oral suppl
ements
ineffective
Concurrent
use with IgG

therapy
Pain
Support care
Interdisciplinar
y
team
Psychological coun
s
ellin
g
Coping with disability
Prognosis
4-15% patients die each year
20% are disabled 1 year after occurrence

Rule of 3's:
30% have residual weakness after 3 years
33% need to make changes to daily lives
3% remain wheelchair bound >2 years after diagnosis
~3% relapse with muscle weakness and paraesthesia many years after initial event

Preceding infection of C. jejuni
More likely to require ventilation
Worse outcome

Worse outcomes with increased age
Recovery is measured by the Hughes scale or F-score
0 = healthy
1 = minor SS, ablility to run
2 = ability to walk 5km independently, inability to run
3 = ablility to walk 5km with assistance
4 = bed or chair bound
5 = requiring assisted ventilation for at least part of the day
6 = dead
Castro, M.C. (2010). Unraveling Guillain-Barre syndrome. Nursing Management, p. 37-39 DOI-10.1097/01.NUMA.0000383998.78718.3a
Dua, K., & Banerjee, A. (2010). Guillain-Barré syndrome: a review. British Journal Of Hospital Medicine (17508460), 71(9), 495-498. http://web.ebscohost.com/ehost/detail?sid=7af189dc-1930-48e0-8ac6-eedeac6da5ab%40sessionmgr12&vid=3&hid=19&bdata=JnNpdGU9ZWhvc3QtbGl2ZSZzY29wZT1zaXRl#db=c8h&AN=2010831302
Lugg, J. (2010). Recognising and managing Guillain-Barré syndrome. Emergency Nurse, 18(3), 27-30. Retrieved from http://web.ebscohost.com/ehost/detail?sid=948f8960-7af8-48c4-b6c6-8db1cc216a2b%40sessionmgr13&vid=2&hid=19&bdata=JnNpdGU9ZWhvc3QtbGl2ZSZzY29wZT1zaXRl#db=c8h&AN=2010686772
Randall, D.P. (2010). Guillain-Barre Syndrome. Disease-a-month 56(5), pp. 256-261. Doi : http://dx.doi.org/10.1016/j.disamonth.2010.02.004
Raphael, J-C., Chevret, S., Auriant, I., Sharshar, T., Bouget, J. and Bolgert, F. (1999). Treatment of adult Guillain-Barre syndrome: Indications for plasma exchange. Transfusion Science 20, pp. 53-61. Doi: http://dx.doi.org/10.1016/S0955-3886(98(00092-7
Shahrizaila, N., & Yuki, N. (2011). Guillain-Barré Syndrome Animal Model: The First Proof of Molecular Mimicry in Human Autoimmune Disorder. Journal Of Biomedicine & Biotechnology, 1-5. doi:10.1155/2011/829129
Steiner, I. I., Rosenberg, G. G., & Wirguin, I. I. (2010). Transient immunosuppression: a bridge between infection and the atypical autoimmunity of Guillain–Barré syndrome?. Clinical & Experimental Immunology, 162(1), 32-40. doi:10.1111/j.1365-2249.2010.04223.x
Vucic, S., Kiernan, M.C. and Cornblath, D.R. (2009). Guillain-Barre syndrome: An update. Journal of Clinical Neuroscience 16, pp. 733-741.
Your Turn
History
1916 - Guillain, Barré, Strohl: weakness, loss of tendon reflexes, and increased CSF protein without cells (distinguished from polio)
1859 - Landry: acute flaccid paralysis due to neuropathy. Autopsy: no spinal cord involvement
1969 - Asbury, Arnason, Adams: GBS pathology,
- autopsy showed
demyelination
with variable axonal
degeneration and
lymphocytic infiltration.
2 experiments:
Experimental Allergic encephelomyelitis (EAE): immunization with CNS myelin or MBP >T-cell mediated inflammatory demyelination CNS model
Experimental Allergic neuritis (EAN): immunization with PNS myelin or P2 > T-cell mediated inflammatory PNS model
MS, TM, GBS might reflect a cell
mediated immune attack on myelin
proteins.
Varying proportions throughout the world
Vaccinations have
Swine flue vaccine
been shown to predispose
weakness/ failure
Full transcript