Guillain Barre Syndrome

By Author: Ibrahim Machiwala
Total Articles: 396
Comment this article

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 (