Guillain Barre Syndrome

By Author: Ibrahim Machiwala
Total Articles: 463
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What is GBS?
‘GBS is an acute inflammatory demyelinating polyneuropathy characterized by progressive symmetric ascending muscle weakness, paralysis, and hyporeflexia with or without sensory or autonomic symptoms'

Background:
1859- Landry published a report on 10 patients with ascending paralysis
1916- Guillain, Barre and Strohl described 2 French soldiers with motor weakness, areflexia, and albuniocytological dissociation in the cerebrospinal fluid. They recognized the peripheral nature of the illness

Epidemiology:
1-3 per 100,000 (US)
M:F - 1.5:1
Ages: bimodal distribution with 2 peaks (15-35 yrs) & (50-75 yrs)

Etiology:
Post-infectious AI disease
Cellular and humoral mechanisms
Association with administration of certain vaccinations, and other systemic illnesses
Auto-immunity In GBS
Humoral immunity: antibodies formed against capsular antigens cross-react with myelin
Target: gangliosides and glycolipids, such as GM1 and GD1b, distributed throughout the myelin in the peripheral nervous system
Lmphocytic ...
... infiltration of spinal roots and peripheral nerves, followed by macrophage-mediated multifocal stripping of myelin
Sub-group: primary immune attack directly against nerve axons

Variants:
Miller-Fisher syndrome: ataxia, ophthalmoplegia, and areflexia. Anti-GQ1b antibodies (ophthalmoplegia)
Acute motor axonal neuropathy (AMAN): pure motor axonopathy. Pediatric age groups
Acute motor-sensory axonal neuropathy (AMSAN): axonal degeneration of motor and sensory nerve
Pure sensory variant of GBS
Acute pandysautonomia: postural hypotension, bowel and bladder retention, anhidrosis

Common Infectious Agents:
Bacteria: C jejuni (60% in north China study), Haemophilus influenzae, Mycoplasma pneumoniae, and Borrelia burgdorferi
Viruses: cytomegalovirus (13% in Dutch Study), Ebstein-Barr virus and HIV

Other Associations:
Vaccines: group A streptococci vaccines, the rabies vaccine, and the swine flu vaccine
Systemic illnesses: systemic lupus erythematosus, sarcoidosis, lymphoma, surgery, renal transplantation (ANECDOTAL)

Presentation:
History - Antecedent illness
- Weakness (ascending and symmetrical)
- Sensory changes (ascending paraesthesias)
- CN involvement ( Facial droop, Diplopias, Dysarthria, Dysphagia)
- Pain (Back & leg)
- Autonomic changes
- Respiratory involvement

Preceding illness
2/3 of patients
URTI or GI symptoms
1-3 weeks prior to onset
C jejuni- can cause both URTI or GI symptoms

Weakness
Classic clinical picture is ascending and symmetrical
Develops over days to weeks
Can very from mild to tetraplegia
Peaks 4 weeks after onset
Recovery 2-4 weeks after peak

Sensory change
Frequently ascending as well
Parasthesia, numbness.
Usually mild

Cranial nerve involvement
45-75% of patients
Facial drop
Diplopia
Dysarthria
Dysphagia

Pain
89% of one study experienced pain
50% of these severe and distressing
Back and leg pain

Autonomic symptoms
Tachycardia, bradycardia
Urinary retention
Sweating

Respiratory involvement
40% of patients
Exertional dyspnea
SOB
Slurred speech
Ventilatory arrest
Physical
Tachycardia/bradycardia, tachypnea
BP lability
Lower extremities first affected
If marked asymmetry then GBS
Weakness
Hyporeflexia or absent reflexes
Normal objective sensory exam
If marked then GBS
CN: facial weakness, also VI,III,XII,IX,X

Investigations:
CSF studies- CSF protein (>0.55 g/L) without an elevation of white blood cells (