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Group A
23
4 - 5 PTA
sensory
2 - 3 PTA
proximal grade 5
distal grade 4
Axonal polyneuropathy
Demyelinating polyneuropathy
Multiple mononeuropathy/ mononeuropathy multiplex
Demyelinating polyneuropathy
Hereditary polyneuropathy
Immune-mediated polyneuropathy
- Guillain-Barre syndrome (GBS)
- Chronic demyelinating polyneuropathy (CIDP)
Guillain-Barr Syndrome
is an acute inflammatory demyelinating polyneuropathy (AIDP),
a disorder affecting the peripheral nervous system.
It is usually triggered by an acute infectious process.
ETIOLOGY
The etiology of Guillain-Barr syndrome is unclear, but
an autoimmune response is strongly suspected.
There is a preceding event or trigger that is often an infection.
Occasionally, vaccinations have been known to trigger
Guillain-Barr syndrome.
Approximately half of the people who develop Guillain- Barr syndrome
have a mild febrile illness 2 to 3 weeks before the onset of symptoms.
The febrile infection is usually respiratory or gastrointestinal.
Approximately 25% of patients with this disease have antibodies
to either cytomegalovirus or Epstein-Barr virus.
PATHOPHYSIOLOGY
In Guillain-Barr syndrome, the myelin sheath surrounding the axon
is lost.
Demyelination is a common response of neural tissue to many agents
and conditions, including physical trauma, hypoxemia, toxic chemicals,
vascular insufficiency, and immunological reactions.
Loss of the myelin sheath in Guillain-Barr syndrome makes nerve impulse
transmission is aborted.
Diagnosis of Typical GBS
Required for Diagnosis
- Progressive weakness in both arms & legs from mild
- paresis to complete paralysis
- Generalized hypo- or areflexia
Strongly Supportive of Diagnosis
Progression of symptoms from days to 4 weeks
Relative symmetry of symptoms
Mild sensory symptoms
Cranial nerves involvements
Autonomic dysfunction
High CSF protein
Typical electro diagnostic features
Features that raise doubt about the
diagnosis
Severe pulmonary dysfunction with limited limb weakness
Severe sensory signs with limited weakness
Bladder or bowel dysfunction at onset
Fever at onset
Sharp sensory level
Marked persistent asymmetry of weakness
Persistent bladder dysfunction
Increased number of mononuclear cells in CSF
Investigation
polyneuropathy
CBC, ESR
FBG, LFT, BUN/Cr
TFT
Vit. B12 level
Serum & urine immunoelectropheresis
ANA, rheumatoid factor
HBsAg, anti HIV
Blood, hair, nail for heavy metal
Genetic testing
Specific investigation
Supportive care
- admitted to hospital
- ABCs, intravenous treatment, oxygen, and assisted ventilation may be indicated.
- Intubation should be performed on patients who develop any degree of respiratory
failure. Clinical indicators for intubation in the ED include the following:
Hypoxia
Rapidly declining respiratory function
Poor or weak cough
Suspected aspiration
monitoring for vital capacity, heart rhythm, blood pressure, nutrition, DVT
prophylaxis, early consideration (after 2 weeks of intubation) for tracheostomy,
and chest physiotherapy.
Specific treatment
IVIG
Dose: 0.4 gm/kg/day for 5 consecutive
C/I : congestive heart failure and renal insufficiency
Side effects : expands the plasma volume, fever, myalgia, headache, nausea
and vomiting, aseptic meningitis, neutropenia and hypertension
**previous anaphylaxis to IVIg is C/I to repeat treatment
Plasmapheresis
40 - 50ml/kg
4-5 times over 2 wks.
complications : sepsis
**If fresh frozen plasma is used as replacement fluid
Risk of acquiring viral infections such as hepatitis and HIV.