Guillain Barre Syndrome - By: Dr. D.S. Merchant

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 (<10 lymphocytes/mm3)
• EMG / NCV - demyelination: nerve conduction slowing
- Axonal variant: absent or markedly reduced distal compound muscle action potentials (CMAP)
• Pulmonary Function tests- Max Insp. Pressure, VC

Management:
• Constant vigilant monitering
• Rehab
• Physical
• Speech
• Mental
• Respiratory support
• Immune therapy

Monitoring
• Monitor
• RR
• Vitals
• ABG
• PFT
• Pressure sores, DVT prophylaxis
• Enteric/Parenteral feedings
• Requires SCU/ICU admission

Immunotherapy:
Plasmapheresis

• IVIG- blocks macrophage receptors, inhibits antibody production, complement binding, and neutralizes pathologic antibodies

Prognosis:
• Most patients (up to 85%) with GBS achieve a full and functional recovery within 6-12 months
• 7-15% of patients have permanent neurologic sequelae
• Mortality rate less than 5%

About the Author

Dr. D.S. Merchant is a Gold Medalist in (Anatomy & Histology), Resident AKUH, Pakistan. For more information on Gastroenterology or visit http://www.ehealthguide.info is a popular website that offers information on Phd Public Health, Health Insurance and Masters in Public Health.

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