Guillain-Barre syndrome

Guillain-Barré Syndrome (GBS),an acquired immune-mediated inflammatory disorder ofperipheral nervous system (e.g. notbrainspinal cord). Italso called acute inflammatory demyelinating polyneuropathy, acute idiopathic polyradiculoneuritis, acute idiopathic polyneuritisLandry's ascending paralysis.

The pathologic hallmark ofdiseasemyelin lossperipheral nerves duean acuteprogressive inflammationunknown cause. Itsuggested that itan autoimmune disease,whichsufferer's immune systemtriggered into damagingnerve covering. Theresome supportthisthat halfall cases occur soon aftermicrobial infection or respiratory or gastrointestinal viral infection. Many cases developedpeople who received1976 swine flu vaccine.

Peripheral nerves originate inspinal cordproceedtheir target tissues (mainly muscle, skinall internal organs). Their most proximal parts emerging fromspinal cordcalled nerve roots andinflammationmost (but not all) typical Guillain-Barré syndrome cases startsthese roots. Therefore, this conditionalso referredas acute polyradiculoneuritis.

GBS israre - affecting about 12 peopleevery 100,000 per year. It does not discriminateregard toage or sexsufferers.

About one halfpatients havehistorypreceding viral infection. Guillain-Barré syndrome may also be associatedimmunizations, recent surgery or trauma, pregnancy, Hodgkin's diseaseconnective tissue diseases. The most frequently associated viral agentscytomegalovirus (CMV), HIV, measlesherpes simplex virus. A bacteria called Campylobacter jejuni has recently been shownbe closely relatedcertain subtypes ofdisease.

Extensive damagemyelin causes disturbancesperipheral nerve functions, which can be classified as motor (affectingmuscle), sensory (affectingskin) or autonomic (affectinginternal organs). Therefore, patients usually show two or more offollowing symptoms: weakness (often symmetrical,ascending fashion, leadingrespiratory failure1/3cases), decreased sensation (numbness, lossposition sense), severe fluctuationsblood pressure, irregularitiesheart rate, constipationincontinence. Additional symptoms may be blurred vision, difficulty moving facial muscles, difficulty swallowing,drooling.

The diagnosisestablished by electromyography examination, nerve conduction studies (NCS),cerebrospinal fluid (CSF) examination. ElectromyographyNCS show slowingconduction velocities, indicating myelin loss; CSF examination reveals high protein contentusually normal or slightly elevated cell count, indicating severe nerve damage. These findingsusually prominent afterfirst week ofdisease, soclinical symptomsfindingsmore valuable inearly stages.

Recent studies ondisease have demonstrated that approximately 80% ofpatients have myelin loss, whereas, inremaining 20%,pathologic hallmark ofdiseaseindeed axon loss. The cases indicatingdemyelinating form (AIDP)called "acute motorsensory axonal neuropathy" (AMSAN);cases showing only motor symptoms (diffuse weakness)called "acute motor axonal neuropathy" (AMAN). Indifferentinfrequent variant called Miller Fisher syndrome, patients develop ataxia, losstendon reflexes,difficulty moving eye muscles but not weakness or sensory loss. All variantsGuillain-Barré syndromenow supposedbe an autoimmune disease caused by antibodies againstvarietygangliosides foundabundant amounts inperipheral nerve tissue.

The symptomsascending weaknessabnormal sensationsthen paralysis oflegs, arms, facepossibly breathing muscles. Itrarely fatal but thereno direct curerecovery may need care inintensive care unitcan take years (although people can recover infew weeks as as well).

Supportive caremonitoringall vital functions iscornerstonesuccessful management. Becauseimmune mechanisms playrolepathogenesis, plasma exchange or intravenous immunoglobulins may improveoutcome. Althoughcorticosteroids may be usedtreatment, theynot usually considereddrugfirst choice becausemay occasionally worsensymptoms.

Approximately 80%patients havecomplete recoveryabout 5-10% recoversevere disability. However this isgrave diseasedespite all improvementstreatmentsupportive care,death rate among patientsthis diseasestill about 2-3% even inbest intensive care units.

The disease was first described byFrench physician Jean Landry1859. In 1916, Georges Guillain, Jean Alexandre BarréAndre Strohl discoveredkey diagnostic abnormalityincreased spinal fluid protein production, but normal cell count.