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GBS : Guillain- Barré syndro me SUHAS PAI 09-07-2012 1

Guillain barre syndrome

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GBS : Guillain-Barré syndrome

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GBS is also known as:

• Acute idiopathic polyradiculoneuritis

• Acute idiopathic polyneuritis

• French polio

• Landry's ascending paralysis • Landry guillain barré syndrome.

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CLASSIFICATIONSix different subtypes of GBS:

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• Acute inflammatory demyelinating 

polyneuropathy (AIDP) is the most common form of GBS, and the term is often used synonymously with GBS.

• It is caused by an auto-immune response directed against Schwann cell membranes.

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• Miller Fisher syndrome (MFS) is a rare variant of GBS.

• Accounting for approximately 5% of GBS cases, it manifests as a descending paralysis, proceeding in the reverse order of the more common form of GBS.

• It usually affects the eye muscles first and presents with the triad of ophthalmoplegia, 

ataxia, and areflexia.

• The ataxia predominantly affects the gait and trunk, with the limbs relatively spared. 

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• Acute motor axonal neuropathy (AMAN),  also known as Chinese paralytic syndrome, attacks motor nodes of Ranvier and is prevalent in China and Mexico.

• It is probably due to an auto-immune response directed against the axoplasm of peripheral nerves.

• The disease may be seasonal and recovery can be rapid.

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• Acute motor sensory axonal neuropathy 

(AMSAN) is similar to AMAN but also affects sensory nerves with severe axonal damage.

• Like AMAN, it is probably due to an auto-immune response directed against the axoplasm of peripheral nerves.

• Recovery is slow and often incomplete.

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• Acute panautonomic neuropathy is the most rare variant of GBS, sometimes accompanied by encephalopathy.

• It is associated with a high mortality rate, owing to cardiovascular involvement, and associated dysrhythmias.

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• Bickerstaff's brainstem encephalitis (BBE) is a further variant of Guillain–Barré syndrome.

• It is characterized by acute onset of ophthalmoplegia, ataxia, disturbance of consciousness, hyperreflexia or Babinski's sign.

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FACTS 

• Guillain-Barré syndrome occurs when the immune system attacks the peripheral nervous system

• leading to weakness or tingling in the legs. Symptoms sometimes affect the arms and upper body.

• Severe cases of Guillain-Barré can lead to paralysis and are life-threatening.

  

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• Guillain-Barré is a very rare condition

• afflicts about one person out of 100,000.

• The condition often manifests after a respiratory or gastrointestinal viral infection.

• Surgery or vaccines may also trigger GBS.

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• The autoimmune reaction in Guillain-Barré is directed against the myelin sheaths that surround the axons of peripheral nerves or the axons (parts of the nerve) themselves.

• The greatest point of weakness or paralysis can occur in days or weeks after the first symptoms occur.  

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• Because the signs and symptoms of GBS vary, it can be difficult to diagnose the condition in the early stages.

• A physical exam as well as an examination of the cerebrospinal fluid (CSF) obtained from a spinal tap may help aid diagnosis.

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• Treatment of Guillain-Barré syndrome may include • plasma exchange (

plasmapheresis) • high-dose immunoglobulin

therapy.

• A respirator may be used if the patient requires assistance to breathe.

• Physical therapy can begin after the patient recovers limb control.

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• The recovery period after about of Guillain-Barré may be as little as a few weeks or as long as a few years.

• About 30% of those with Guillain-Barré may suffer from residual weakness after 3 years.

• Ongoing research seeks to identify the cause of Guillain-Barré and develop new and better treatments.

 

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What is GBS?

• Body's immune system attacks part of the peripheral nervous system.

• Can affect anybody, can strike at any age and both sexes

• Rare : afflicts 1 or 2 in 100,000

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• Usually occurs a few days or weeks after the patient has had symptoms of a respiratory or gastrointestinal viral or bacterial infection.

• First symptoms • varying degrees of weakness

in legs• tingling sensations in the

legs

• Weakness and abnormal sensations spread to the arms and upper body.

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• Severe: Muscle Wasting, Paralysis.

• Life threatening - potentially interfering with breathing and with blood pressure or heart rate.

• Most patients recover from even the most severe cases of GBS, although some continue to have a certain degree of weakness.

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CAUSES

• Not yet exactly known.

• Body's immune system begins to attack the body itself

• Destroy the myelin sheath that surrounds the axons of many peripheral nerves, or even the axons themselves

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• Peripheral nerve’s myelin sheaths are injured or degraded, the nerves cannot transmit signals efficiently

• Motor and sensory response becomes weak.

• Signals to and from the arms and legs must travel the longest distances : most vulnerable to interruption

• So muscle weakness and tingling sensations first appear in the hands and feet and progress upwards

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• GBS is preceded by a viral or bacterial infection

• It is possible that the virus has changed the nature of cells in the nervous system so that the immune system treats them as foreign cells

• Virus makes the immune system itself less discriminating about what cells it recognizes as its own

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• Symptoms can progress over the course of hours, days, or weeks.

• Most people reach the stage of greatest weakness within the first 2 weeks after symptoms appear

• By the third week of the illness, 90 percent of all patients are at their weakest.

• Cause and course of GBS is an active area of neurological investigation

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DIAGNOSIS

• Reflexes such as knee jerks are usually lost.

• As the signals traveling along the nerve are slower, a nerve conduction velocity (NCV) test aids in diagnosis.

• The CSF contains more protein than usual. (spinal tap test, a procedure in which needle is inserted into the patient's lower back to draw csf from the spinal column)

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TREATMENT

• No known cure

• Plasmapheresis (Exchange of Plasma)

• High-dose immunoglobulin therapy.

• Both reduce the severity and duration of the Guillain-Barré episode.

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THANK YOU

By

Suhas PaiIII Prof BAMS