Guillain Barre Syndrome - By: Ibrahim

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%

Me score on the Glasgow Outcome Scale at eight weeks (a score of 5, indicating a favorable outcome, vs. a score of 1 to 4, indicating an unfavorable outcome).
• The mortality rate among adults with acute bacterial meningitis and the frequency of neurologic sequelae among those who survive are high, especially among patients with pneumococcal meningitis.

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.