1. Guillain-Barré Syndrome: What you need to know?
France Ellyson Kuwait, 2014

2. GUILLAIN BARRÉ SYNDROME (GBS)
Post infectious disorder in which the body’s immune system attacks the myelin sheath that surrounds the axons of many peripheral nerves

3. PATHOPHYSIOLOGY
An immune-mediated response triggers destruction of myelin sheath
Demyelination process is accompanied by edema and inflammation peripheral nerves
Demyelination of axons result in loss of conduction

4. PATHOPHYSIOLOGY
The inflammation process may lead to various degrees axonal injury
This signals poorer recovery

5. PATHOLOGY
MMN Multifocal Motor Neuropathy

6. EPIDEMIOLOGY Rare disorder: Average yearly incidence of
1-3 cases / population
Occurs world wide / Affects all races
Affects ♂ and ♀ equally
Persons of all ages are at risk (peak ages 50-74)
Unknown etiology
Upper respiratory tract infection, viral infection, Gi infection, ? vaccination

7. CLINICAL PRESENTATION
Characterized by motor weakness and areflexia
Usually begins in legs and progresses to trunk and arms
Resp. failure is due to mechanical failure and fatigue of intercostals and diaphragm
85% have cranial nerve involvement.
Facial nerve (VII) most frequently affected along with CN IX, X, XI and XII.
With sensory changes – paresthesia , tingling, pins and needles, heightened sensitivity to touch or numbness
Facial cranial nerve: inability to smile, frown, whistle, drink with straw
Cranial Nerve !X and X: Dysphagia and laryngeal paralysis and Vagus nerve deficit – autonomic dysfunction

8. CLINICAL PRESENTATION
About 25 % patients experience pain
Pain is often worse at night and interferes with sleep
Many patients experience autonomic dysfunction
Decreased or absent deep tendon reflexes
Vibration and proprioception are typically affected
GBS does not affect consciousness, pupils or cognitive function

9. CLINICAL PRESENTATION
3 phases:
Acute (Progressive) stage lasting 3 –4 weeks
Plateau stage : symptoms remain unchanged for 2 – 4 weeks
Recovery stage

10. DIAGNOSTIC CRITERIA Patient history CSF analysis
There are approximately 50 differential diagnosis
CSF analysis
Increase protein (Albumin-cytological dissociation)
Normal cell count
Electromyography (EMG)
Shows segmental demyelination
Conduction slowing or block (more apparent in the motor nerves than in sensory nerves)

11. MANAGEMENT OF GBS No known preventive treatments Therapies aim:
Lessen the severity of the illness
Accelerate the recovery
There are two components to care
Supportive care
Specific therapies

12. SUPPORTIVE CARE Respiratory failure Dysautonomia
Venous Thromboembolism
Pain
Dysautonomia (or autonomic dysfunction) is any disease or malfunction of the autonomic nervous system (ANS). The autonomic nervous system controls a number of functions in the body, such as heart rate, blood pressure, digestive tract peristalsis, and sweating, amongst others. Dysfunction of the ANS can involve any of these functions.

13. RESPIRATORY FAILURE Most serious and common complication
Occurs in approximately 40% of cases
Secondary to weakness of respiratory muscles and diaphragm
Vital capacity needs to be measured regularly
Patients will be intubated electively when vital capacity falls below 20 ml/kg (ICU admission)
Anxiety and insomnia are classic symptoms

14. DYSAUTONOMIA Occurs to some extent in most patients with GBS
More acute in those patients with respiratory problems and severe motor deficits
Dysfunction in the sympathetic /parasympathetic systems:
Hypertension
Orthostatic hypotension
Cardiac arrhythmia

15. VENOUS THROMBOEMBOLISM
5% PE in patients immobilized ≥ 2 weeks
Accounts for 28% of the deaths due to GBS
Preventative treatments:
Anticoagulation, SCDs
TED stockings
Passive ROM /early mobilization

16. PAIN Commonly presents as lumbar pain.
Discomfort is secondary to acute pain in nerve roots
Treat with analgesia
Due to immobilization
Passive range of motion
Pins and needles or burning pain
Anticonvulsant drugs / elavil / neurontin / Lyrica/ Cymbalta

17. SPECIFIC THERAPIES Immunoglobulin (IVIgG)
Plasma exchange (Plasmapheresis or PLEX)

18. IMMUNOGLOBULIN (IVIG)
IVIG is usually given on 5 sequential days for overall dose of 2 gms/kg of body weight
Some patients need second course of treatment
IVIG is derived from fractionated, purified human plasma collected from a large pool of multiple donors
Mechanism of action is unclear

19. IVIG SIDE EFFECTS Most side effects are mild and transient
Most frequent: rashes, chills, fever, mild hypotension, nausea, malaise, headache, mild arthralgias and short term aseptic meningitis
More serious and rare: fatal anaphylactic shock, stroke, renal impairment, hemolytic anemia and transmission of blood born pathogens

20. PLASMA EXCHANGE
Removal of immunoglobulins and antibodies from the serum by removing the blood from the body, separating cells from the plasma, and replacing the cells in fresh frozen plasma, albumin, or saline
Most common regime 5 – 10 days of treatments

21. PLASMA EXCHANGE SIDE EFFECTS
Hypotension /dizziness
Fluctuation in circulatory volumes
Anemia / Hemorrhage
Infection
Hypocalcemia

22. REFERENCES
Barker, E. (2002). Neuroscience Nursing. A Spectrum of Care (2nd ed.). St. Louis: Mosby.
Hickey, J. V. (2003). The Clinical Practice of Neurological and Neurosurgical Nursing (5th ed.). Philadelphia: Lippincott Williams & Wilkins, Chapter 32
Info Neuro: Neuro-Patient Resource Centre:
Kirmse,MSN,RN, CNS., J., (2009). The Nurse’s Role in the Administration of Intravenous Immunoglobulin Therapy. Home Healthcare Nurse. 27(2): February 2009.
Ruts, L., van Koningsveld, R. & van Doorn, P.A. (2005). Distinguishing acute onset CIDP from Guillain-Barré syndrome with treatment related fluctuations. Neurology 2005 ;6:
 Van Doorm,P.A. Ruts, L. &Jacobs, B. (2008). Clinical features, pathogenesis, and treatment of Guillian- Barré syndrome. Lancet Neurol 2008;7: