guillain-barré syndrome (gbs)

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Guillain-Barré Guillain-Barré syndrome syndrome (GBS) (GBS) By Usman Bari By Usman Bari #1054 #1054

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Guillain-Barré syndrome (GBS). By Usman Bari #1054. IntroDuction. Fisrt described by Jean Landry in the mid 1800 ’ s and discovered in 1916 by Jean Alexandre Barre and Georges Gillain. (tests on soldiers) Rare – 1- 2 cases per 100,000 (most common non-trauma related paralysis) - PowerPoint PPT Presentation

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Page 1: Guillain-Barré syndrome (GBS)

Guillain-Barré syndromeGuillain-Barré syndrome(GBS)(GBS)By Usman BariBy Usman Bari

#1054#1054

Page 2: Guillain-Barré syndrome (GBS)

IntroDuctionIntroDuction

• Fisrt described by Jean Landry in the mid Fisrt described by Jean Landry in the mid 1800’s and discovered in 1916 by Jean 1800’s and discovered in 1916 by Jean Alexandre Barre and Georges Gillain. (tests on Alexandre Barre and Georges Gillain. (tests on soldiers)soldiers)

• Rare – 1- 2 cases per 100,000 (most common Rare – 1- 2 cases per 100,000 (most common non-trauma related paralysis)non-trauma related paralysis)

• Guillain-Barré syndrome can affect anybody: Guillain-Barré syndrome can affect anybody: age, sex, race.age, sex, race.

• inflammatory demyelinating polyneuropathy of inflammatory demyelinating polyneuropathy of the peripheral nervesthe peripheral nerves

Page 3: Guillain-Barré syndrome (GBS)

Signs and SymptomsSigns and Symptoms

• Ascending Paralysis Ascending Paralysis - -

• Varying degrees of symmetric weakness with tingling Varying degrees of symmetric weakness with tingling sensations. These symptoms can increase in intensity sensations. These symptoms can increase in intensity until certain muscles cannot be used at all and, when until certain muscles cannot be used at all and, when severe, the person is almost totally paralyzed. severe, the person is almost totally paralyzed.

• Dysfunction of the autonomic nervous system. Wide Dysfunction of the autonomic nervous system. Wide fluctuations in blood pressure, orthostatic hypotension, fluctuations in blood pressure, orthostatic hypotension, and sinus tachycardia among other cardiac and sinus tachycardia among other cardiac arrhythmias. (65% of pts)arrhythmias. (65% of pts)

• Loss of deep tendon reflexes, areflexiaLoss of deep tendon reflexes, areflexia

• Severe cases with loss of respiratory muscle Severe cases with loss of respiratory muscle weakness. . Such an individual is often put on a weakness. . Such an individual is often put on a ventilator (30% of patients) to assist with breathing and ventilator (30% of patients) to assist with breathing and is watched closely. is watched closely.

Page 4: Guillain-Barré syndrome (GBS)

PathophysiologyPathophysiology

• Not exactly known however is believed to have a Not exactly known however is believed to have a multifactorial cause that is an auto-immune disease multifactorial cause that is an auto-immune disease which occurs 3-4 weeks after an onset of gastrointestinal which occurs 3-4 weeks after an onset of gastrointestinal or respiratory viral infection (campylobacter jejuni, or respiratory viral infection (campylobacter jejuni, influenza virus, CMV). Sometimes also seen in after influenza virus, CMV). Sometimes also seen in after vaccination (1977 swine flu pandemic).vaccination (1977 swine flu pandemic).

• GBS onset is rapid, it can be from a few days to 4 weeks.GBS onset is rapid, it can be from a few days to 4 weeks.

• Through molecular mimicry the immune system attacks Through molecular mimicry the immune system attacks gangliosides.(GM1) Ganglioside-like epitopes exist in the gangliosides.(GM1) Ganglioside-like epitopes exist in the bacterial wall of C. jejuni.bacterial wall of C. jejuni.

• This leads to inflammation and demyelination of the This leads to inflammation and demyelination of the peripheral nerves there isn’t any damage to the brain or peripheral nerves there isn’t any damage to the brain or spinal cord. (80% is myelin loss, 20% axon loss).spinal cord. (80% is myelin loss, 20% axon loss).

Page 5: Guillain-Barré syndrome (GBS)

PathoGenesisPathoGenesis

Page 6: Guillain-Barré syndrome (GBS)

diagnosisdiagnosis

Aside from the classic symptoms of GBS along with Aside from the classic symptoms of GBS along with cranial nerve involvment bilateral weakness of facial cranial nerve involvment bilateral weakness of facial muscles, CSF analysis and electrodianostic tests of muscles, CSF analysis and electrodianostic tests of nerves are needed.nerves are needed.

Tests:Tests:

•CSF test through lumbar puncture shows increased CSF test through lumbar puncture shows increased protein levels with an absence of pleocytosis. (albiumino-protein levels with an absence of pleocytosis. (albiumino-cytological dissociation). Normal protein levels ( 15-60 cytological dissociation). Normal protein levels ( 15-60 mg/dL) in the first week but they significantly increase by mg/dL) in the first week but they significantly increase by 90% by the end of second week (100-1000mg/dL).90% by the end of second week (100-1000mg/dL).

•Nerve conduction tests: prolonged distal latencies, Nerve conduction tests: prolonged distal latencies, conduction slowing, conduction blockconduction slowing, conduction block

•ElectromyographyElectromyography

Page 7: Guillain-Barré syndrome (GBS)

Differential diagnosisDifferential diagnosis

• PoliomyelitisPoliomyelitis

• BotulismBotulism

• Myasthenia GravisMyasthenia Gravis

• Porphyria polyneuropathyPorphyria polyneuropathy

Page 8: Guillain-Barré syndrome (GBS)

Variations of GBSVariations of GBS

• Acute inflammatory demyelinating Acute inflammatory demyelinating polyneuropathy (AIDP) – against schwann cell polyneuropathy (AIDP) – against schwann cell membranesmembranes

• Acute motor axonal neuropathy (AMAN) – Acute motor axonal neuropathy (AMAN) – against nodes of ranvieragainst nodes of ranvier

• Acute motor sensory axonal neuropathy Acute motor sensory axonal neuropathy (AMSAN) – similar to AMAN but with severe (AMSAN) – similar to AMAN but with severe axonal damageaxonal damage

• Miller Fisher syndrome (MFS)Miller Fisher syndrome (MFS)

Page 9: Guillain-Barré syndrome (GBS)

Management/ Management/ treatmenttreatment

• Regular monitoring to prevent potentially fatal Regular monitoring to prevent potentially fatal complications: complications:

• Monitoring respiratory function (vital capacity, Monitoring respiratory function (vital capacity, respiation frequency) may be need to put on respiation frequency) may be need to put on ventilatory support.ventilatory support.

• Regular check for autonomic dysfuntion (ECG, Regular check for autonomic dysfuntion (ECG, pulse and blood pressure) pulse and blood pressure)

• Physical therapy (mobilization for DVT)Physical therapy (mobilization for DVT)

• PlasmaphpheresisPlasmaphpheresis

• Intravenous Immunoglobulin (IVIg) – high dose Intravenous Immunoglobulin (IVIg) – high dose every 3 to 4 weeks every 3 to 4 weeks

Page 10: Guillain-Barré syndrome (GBS)
Page 11: Guillain-Barré syndrome (GBS)

Prognosis/ Prognosis/ complicationscomplications

• Prognosis is usually good if managed well. Prognosis is usually good if managed well. 80% fully recover after the onset of the 480% fully recover after the onset of the 4thth week of GBS. The recovery period can take week of GBS. The recovery period can take anywhere from a few months to a year.anywhere from a few months to a year.

• Minor fiindings such as areflexia may persist. Minor fiindings such as areflexia may persist. 5 - 10% develop one or more recurrent 5 - 10% develop one or more recurrent episodes, which is classified as chronic episodes, which is classified as chronic inflammatory demyelinating polyneuropathy.inflammatory demyelinating polyneuropathy.

Page 12: Guillain-Barré syndrome (GBS)

Case StudyCase Study

• A 23-year-old man with a recent history of a viral-type illness presents to the emergency department because of complaints of weakness in his legs. He is admitted to the hospital and the weakness progresses into his thighs and hips. What is his prognosis?

• A. Fair, with treatment the progression of the muscle weakness will stop, but the weakness will remain for the remainder of his life

• B. Good, with treatment the muscle weakness will definitely stop and not progress further

• C. Good, with treatment the condition will run its course, and his life can be saved and he should have no residual sequelae

• D. Dismal, the condition will continue to progress, involving his respiratory muscles, causing his death even if treated

Page 13: Guillain-Barré syndrome (GBS)

referencesreferences

• http://www.med.unc.edu/pedclerk/schedules/clerkship-at-moses-cone/readings-and-resources/clinical-problem-solving-conferences-readings/14.%20Guillain-Barre%20syndrome.pdf

• http://www.youtube.com/watch?v=c-http://www.youtube.com/watch?v=c-8ia81XPMw8ia81XPMw

• http://www.webmd.com/brain/tc/guillain-http://www.webmd.com/brain/tc/guillain-barre-syndrome-topic-overviewbarre-syndrome-topic-overview

• http://www.uptodate.com/contents/http://www.uptodate.com/contents/pathogenesis-of-guillain-barre-syndrome-in-pathogenesis-of-guillain-barre-syndrome-in-adultsadults