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Guillain Barre Syndrome

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 alb

uniocytological 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%

By: Dr. D.S. Merchant

Article Directory: http://www.articledashboard.com

Dr. D.S. Merchant is a Gold Medalist in (Anatomy & Histology), Resident AKUH, Pakistan. For more information on Gastroenterology or visit 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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