Transcript Guillain Barre syndrome

Molebatsi Theletsane
 Guillain Barre Syndrome (GBS) is a serious disorder
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that occurs when the body’s defense (immune) system
mistakenly attacks part of the nervous system. This
leads to nerve inflammation that causes muscle
weakness.
There are 6 different subtypes of GBS and they
are:
Acute Inflammatory demyelinating polyneuropathy
Miller Fisher syndrome
Acute motor axonal neuropathy
Acute motor sensory axonal neuropathy
Acute panautonomic neuropathy
Bickerstaff’s brainstem encephalitis
Pathophysiology
 Guillain Barre Syndrome (GBS) is a rare and severe
disease. It occurs after an acute infectious
procedure. GBS initially affects the peripheral
nervous system. Normally it is acute form of
paralysis in lower body area that moves towards
upper limb and face. Gradually patient loses all
his/her reflexes and goes through a complete body
paralysis. GBS is a life threatening disorder and
needs timely treatment and supportive care with
intravenous immunoglobulins or plasmapheresis.
Pathophysiology cont…
 GBS is considered to be an autoimmune disease triggered by a
preceding bacterial or viral infection. Campylobacter jejuni,
cytomegalovirus, Epstein-Barr virus and Mycoplasma
pneumoniae are commonly identified antecedent pathogens.
They have specific antigens in their capsule that they share with
nerves. The immune system usually then response to these
components in the capsule by producing antibodies that crossreact wit the myelin in the peripheral nervous system, causing
demyelination and then damage to the peripheral nervous
system. In GBS there is infiltration of the spinal roots and
peripheral nerves via the lymphatic system, causing stripping of
the myelin. This will lead to a defect in the transmission of
electrical nerve impulses which will then lead to flaccid
paralysis.
Causes
 Nobody knows precisely what the exact cause is. We know it is due to an immune
response to foreign antigens, such as infectious agents (bacteria, etc.) that the
body's immune system mistargets - attacks good tissue by mistake. Experts
believe our immune system mistakenly attacks gangliosides - compounds which
are naturally present in nerve tissues.
 The most common infection which precedes the development of Guillain-Barre
syndrome is Campylobacter jejuni - one of the most widespread causes of human
gastroenteritis. Even so, in over half of all cases no previous infection was
present; in other words, there was nothing to which the doctor could link the
syndrome.
 It is believed the influenza virus may also trigger an autoimmune response which
causes the syndrome.
 Experts believe the foreign agent (bacterium/virus) causes the body's immune
system to attack the myelin sheath of the peripheral nerves. The myelin sheath is
a cover that protects the nerve, a bit like the plastic that covers electrical wires.
The sheath becomes damaged, causing nerve damage, resulting in faulty sending
of signals between nerves and muscles. This faulty wiring causes muscle
weakness, numbness and tingling, and eventually paralysis.
Risk factors
 Age (15-35) and (60-75)
 In men more likely than woman
 Recent gastrointerstinal or respiratory infection by
viruses or bacteria
 Recent vaccination (especially influenza and
meningococcal)
 Recent surgery
 History of lymphoma, Systemic lupus erythromatosus,
or HIV and AIDS
Signs and Symptoms
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Muscle weakness
Numbness
Loss of reflexes in arms and legs
Low blood pressure
Uncoordinated movement
Facial weakness
Clumsiness and falling
Severe pain in the lower back
Sensation changes
Tenderness or muscle pain
Blurred vision
Respiratory problems
Medical management
 Patients with Guillain-Barre syndrome will be
hospitalized initially (medical emergency). It is
important to monitor the individual's respiration
carefully. If breathing problems are severe he/she may
be placed in an ICU (intensive care unit) and put on a
ventilator.
 According to the National Health Service, the two
main initial treatment options for Guillain-Barre
syndrome are intravenous immunoglobulin, which is
safer and easier to give, or plasmapheres (plasma
exchange).
Medical management
 Intravenous immunoglobulin - concentrated
antibodies is injected straight into a vein. The
antibodies are extracted from healthy donors.
 Plasmapheresis - the aim here is to deplete the body
of blood plasma without depleting it of its blood cells.
Plasma is the liquid part of blood. In the case of
Guillain-Barre syndrome, the aim is to remove the cells
that are attacking the nerves.
 After the acute phase of the syndrome, the patient may
need rehabilitation to regain functions that were lost.
Treatment concentrates on improving activities for
daily living, such as brushing teeth, washing, getting
dressed and performing some other everyday tasks.
Physiotherapeutic problems
Acute
 Respiratory failure due to paralysis of diaphram and
secretions
 Decreased A/E due to paralysis of diaphram and secretions
 Decreased chest expansion due to decreased A/E
 Increased secretions due to pneumonia, poor cough
attempt and weakness of respiratory muscles
Post acute
 Decreased Fx w.r.t. ADL due to muscle weakness
 Poor proximal and distal control due to weakness
 Decreased Fx due to immobility
Physiotherapeutic problems cont…
Associated problems
 Muscle atrophy due to weakness and inactivity
 Poor sensation due to nerve damage
 Contractures due to immobility
 Decreased exercise tolerance due to inactivity
 Decreased circulation due to inactivity
 Secondary lung infection due to secretions
 Pressure sores and DVT due to immobility
 Weakness due to inactivity
Short term Aims
 Improve/maintain respiratory function
 Prevention of pressure sores and DVT
 Prevention of muscle atrophy
 Improve/maintain muscle strength
 Improve/maintain function
Long term Aims
 Maintain exercise tolerance
 Maintain function
 Wheelchair handling
 Support groups
 Retrain proximal control
 Maintain muscle strength
 Retraining of gait
Evidence-based article
Recognising and managing guillain barre syndrome
 Jason Lugg (emergency care practitioner) explores
the difficulties of recognising the viral-like symptoms
of this rare condition and offers diagnostic advice to
emergency practitioners.
References
 Hughes RA, Wijdicks EF, Baohn R, et al. Practice
parameter: immunotherapy for Guillain-Barre syndrome:
Report of the Quality Standards Subcommittee of the
American Academy of Neurology. Neurology . 2003;61(6):
736-740. [PubMed: 14504313].
 Jason L. Recognising and managing Guillain-Barre
Syndrome. Emergency nursing: June 2010; 18(3): 27-30
 http://www.thirdage.com/hc/c/guillain-barr-syndromerisk-factors
 Paediatric PowerPoint's presentations (2012), Mrs R. Smith