Guillain-Barré Syndrome

Hare Ram Karn2009928111

•Guillain-Barré syndrome is a postinfectious polyneuropathy involving mainly motor but sometimes also sensory and autonomic nerves.

•Body's immune system begins to attack the body itself, causing autoimmune disease. In this syndrome, the immune system starts to destroy the myelin sheath that surrounds the axons nerves.

Epidemiology

• The incidence <18 years is 0.34 to 1.34 cases per 100,000/year • It can occur at any age, The average age is 4 to 8 years and is rare below the age of 2 years • Males more susceptible• More common in devloping countries.• Germany 7.9% ,Mexico (44%) ,India (28%).• Emerged as the major cause of AFP.• Immunization with the H1N1 vaccine in 2009 resulted in an

increased incidence of 0.8 cases per million doses

Etiology

• Campylobacter jejuni, Helicobacter pylori, Mycoplasma pneumoniae• West Nile virus,Epstein-Barr Virus,Echo Virus,Coxsakie Virus,Influenza

Virus• Mumps,Measles• Vaccines against rabies,influenza, poliomyelitis (oral), conjugated

meningococcal vaccine particularly serogroup C and recently with H1N1.• Some Surgery

Mechanism of Development of GBS

•Exogenous triggers are believed to activate T cells, which act against a variety of specific endogenous antigens(myelin),Resemblance of the triggering pathogens to antigens on peripheral nerves leads to activation of autoimmune response mounted by T lymphocytes in cooperation with B lymphocyte

Clinical Features• Initially Pain in the muscle•Weakness of muscle-The onset is gradual and progresses over days or weeks.-By the 3rd week 90% of the patient are weak.-Usually begins in the lower extremities and progressively involves the

trunk, the upper limbs, and finally the bulbar muscles.-This pattern is known as Landry ascending paralysis.-Relatively symmetrically,but asymmetry is found in 9% of patients-Paresthesias occur in some cases.

•Respiratory insufficiency can result due to Intercostal and diaphragmatic muscle paralysis•Dysphagia and facial weakness are often impending

signs of respiratory failure. They interfere with eating and increase the risk of aspiration•Papilledema is found in some cases, oculomotor and

other cranial neuropathies are severe

•The autonomic nervous system is also involvement causing lability of blood pressure and cardiac rate, postural hypotension, episodes of profound bradycardia, and occasional asystole occur.

Diagnosis

•History•Affected children are irritable•Unable or refuse to walk and later to flaccid tetraplegia• In the early course Tendon reflexes are lost but sometimes preserved until later.Planter reflex is normal.

Diagnosis

1 CSF examination Albumin-Cytologic Dissociation The albuminocytologic dissociation in a patient with an acute or subacute polyneuropathy is diagnostic of Guillain-Barré syndrome. Usually seen after 48 hours of onset2 Motor NCVs• Motor NCVs are greatly reduced, and sensory nerve conduction time is often slow.3 MRI of the spinal cord•Thickening of the cauda equina and intrathecal nerve roots

• Antiganglioside antibodies Antiganglioside antibodies,mainly against GM1 and GD1, are sometimes elevated• Serum creatine kinase (CK) level may be mildly elevated or

normal.• Electromyography (EMG) shows evidence of acute

denervation of muscle.• Serologic testing for Campylobacter and Helicobacter

infections• Sural nerve biopsy tissue shows segmental demyelination,

focal inflammation and wallerian degeneration

Treatment

• There is no known cure for Guillain-Barré syndrome.• In a patients with slow progression closely monitered to

prevent respiratory failure and respiratory arrest• Supportive care, such as respiratory support, and treatment

of secondary bacterial infections• Rapidly progressive ascending paralysis IVG 0.4gm/kg/day

for 5 consecutive day• Plasmapheresis and/or immunosuppressive drugs are

alternatives if IVIG is ineffective.

•Combined administration of immunoglobulin and interferon is effective in some patients.•Respiratory paralysis may necessitate tracheostomy and assisted ventilation.•Patients need tube feeding, care of bowel & bladder & frequent change of posture to prevent bedsores•Physiotherapy is done to prevent handicaps

Complications

•Acute Flaccid Paralysis•Pneumonia•Bed Sores•Psychological trauma. •Urinary incontinence or retention of urine

Prognosis• Usually benign, and spontaneous recovery begins within 2-3

wk.•Most patients recover within 1-6 months without functional

disability, with 80% of patents having complete recovery within 12 months.• The mortality associated with GBS is approximately 3%. •Mortality is 10-20% from respiratory insufficiency.• The most common causes for death are autonomic

disturbances.