GUILLAIN BARRE SYNDROMEBagian Ilmu Penyakit SarafRS BayukartaKarawang

INFLAMMATORY-DEMYELINATING DISORDERSACUTE INFLAMMATORY-DEMYELINATING POLYRADICULONEUROPATHY (AIDP)

CHRONIC INFLAMMATORY-DEMYELINATING POLYRADICULONEUROPATHY (CIDP)CLASSIFIED BY COURSE

GUILLAIN BARRE SYNDROME ACUTE INFLAMMATORY DEMYELINATING POLYRADICULOPATHY (AIDP) 80% of GBS patients get this form

ACUTE MOTOR SENSORY AXONAL NEUROPATHY (AMSAN) a serious axonal form of GBS that attacks motor and sensory nerves

ACUTE MOTOR AXONAL NEUROPATHY (AMAN) a particularly severe form; rural areas of China,children and young adults during summer months

MILLER-FISHER SYNDROME (MFS) : Acute Disseminated Encephalo-myeloradiculopathy

SUBDIVIDED INTO SUBGROUPS OF CASES :Variants with rapid progressive phase

A Very rare form of GBSAffects about 5% of GBS patientsCauses descending paralysisLoss of tendon reflexes/coordinationDifficulty in walking and standingExperience tingling and numbness, dizziness and nauseaBlurred or double vision,facial sagging

Chronic Inflammatory Demyelinating Polyneuropathy(CIDP)Variants with slow progressive phaseKnown as chronic GBS or Chronic Relapsing PolyneuropathyMuch less common than GBSEvolves over months or yearsRelapses occur more frequentlyMMN (Multifocal Motor Neuropathy)MMSD (Multifocal Motor Sensory Demyelinating Neuropathy)

GUILLAIN BARRE SYNDROME0.6 1.9 CASES PER 100,000 PEOPLEOCCCUR AT ANY AGEINCREASES GRADUALLY WITH AGETHE SEXES ARE EQUALLY AFFECTEDPRECIPITATED BY VACCINATIONS, GENERAL SURGERY, PREGNANCY, AND VIRAL INFECTIONINCIDENCE

CHARACTERISTICS OF GBSACUTE ONSET OF PERIPHERAL AND CRANIAL NERVE DYSFUNCTIONVIRAL UPPER RESPIRATORY OR GI INFECTION, IMMUNIZATION, OR SURGERY OFTEN PRECEDES NEUROLOGIC SYMPTOMS BY 5 DAYS TO 3 WEEKSRAPIDLY PROGRESSIVE SYMMETRIC WEAKNESSLOSS OF TENDON REFLEXES, FACIAL DIPLEGIABULBAR AND RESPIRATORY PARESISWORSENS FOR SEVERAL DAYS TO 3 WEEKSGRADUALLY IMPROVES TO NORMAL OR NEARLY NORMAL FUNCTION

GUILLAIN BARRE SYNDROMEPathogenesisA popular theory suggest that the organism (virus or bacteria) responsible for the preceding infection somehow confuses the the immune system, perhaps by mimicking the characteristics of the nerve cells (molecular mimicry), making it less discriminating about what cells it attacksAnn Neurol 1995

GUILLAIN BARRE SYNDROMEPathogenesis Another suggest that the organism perhaps changes the characteristics of the nerve cells, causing the immune system to see them as foreign cells

GUILLAIN BARRE SYNDROMEPathogenesis Special proteins: the antibodies or immunoglobulins are produced by the immune system as a reaction to the presence of antigens; in GBS patients are somehow produced against myelin.These antibodies circulate in the blood, and eventually find myelin, attack and destroy it with the help of white blood cells, producing inflammation in the nerves.

GUILLAIN BARRE SYNDROMEPathogenesis The inflamed cells in turn secrete chemicals that affect the Schwann cells (which produced the fatty materials required to produce myelin)The Schwann cells reduce myelin production, and some of them may even die, further reducing myelin production, while at the same time the existing myelin is destroyed by the antibodies

GUILLAIN BARRE SYNDROMEPathogenesis Tumor necrosis factor-alpha : a cytokine that has well-recognized toxic effects on myelin, may be important in the pathogenesis of peripheral demyelination in GBSAnn Neurol 1993

GUILLAIN BARRE SYNDROMEPathogenesis Recently, Campylobacter Jejuni was claimed to be a predominant precipitating agent that may also trigger a hunoral immune response to glycoconjugates of peripheral myelin in GBS Related with axonal type and poor outcomeAnn Neurol 1993N Engl J Med 1995

GUILLAIN BARRE SYNDROMEPathogenesis A form of GBS occurs after respiratory infection by Haemophilus Influenzae in the Japanese population A particular strain of non-typable Haemophilus influenzae has a ganglioside GM1-like structure Elicits axonal GBSBrain 2000

PATHOLOGIC FEATURES AND PROGNOSIS Segmental demyelination (the predominant structural change) : rapidly reversible once repair is initiated. Axonal interruption which occurs distally : the nerve cell body will survive and slow regeneration with eventual recovery will ensue.

PATHOLOGIC FEATURES AND PROGNOSIS Axonal interruption which occurs proximally: the nerve cell body may die.

But if a nerve remain non-functional for a sufficiently long time, recovery may no longer possible.

PATHOLOGIC FEATURES AND PROGNOSIS Relative proportions of segmental demyelination and axonal interruption with muscle denervation determine recovery: * complete and rapid * initially rapid but incomplete and followed by gradual further improvement over many months/years * incomplete permanently

GUILLAIN BARRE SYNDROMESYMMETRIC WEAKNESS OF THE LIMBSOFTEN ACCOMPANIED BY PARESTHESIAOCCASIONALLY, FACIAL, EXTRAOCULAR,OR OROPHARYNGEAL MUSCLES MAY BE THE FIRST TO BE AFFECTEDSOME PATIENTS REQUIRE MECHANICAL SUPPORT OF VENTILATIONHYPORELEXIA OR REFLEXIA IS PRESENTDEGREE OF SENSORY IMPAIRMENT IS VARIABLEEVIDENCE OF AUTONOMIC DYSFUNCTIONSYMPTOMS AND SIGNS

GUILLAIN BARRE SYNDROMEProgressive phase: last typically 2-3 weeks, measured from the observation of the first symptom until no further deterioration occurs.Plateau phase: neither worsening nor improvements occurs during unpredictable span of time (a few days-several months).Recovery phase: spontaneous improvement and recovery which is individual, achieved in a few weeks or after several years

Course of disease

GUILLAIN BARRE SYNDROMEELEVATED CSF PROTEINCSF NORMAL CELL COUNT (cyto-albumine dissociation)THE OCCURRENCE OF ANTECEDENT VIRAL DISEASES MAY BE DOCUMENTED BY SEROLOGIC STUDIESLABORATORY DATA

GUILLAIN BARRE SYNDROMEDiagnosisClinical examinations of the symptoms and their distribution: symmetrical symptoms,increasing weakness, signs of preceding infectionHistory: contact with poisons,alcohol consumption,recent infections, diabetes,family history of nerve diseaseCourse of the disease

GUILLAIN BARRE SYNDROMEDiagnosisLABORATORY TESTS: blood and urine tests, stool test, x-rays, scans, lumbal punctureELECTRODIAGNOSTIC STUDIES: nerve conduction velocity test, EMG, ECG FURTHER EXAMINATIONS

GUILLAIN BARRE SYNDROMETreatment and care of patientsSymptomatic : to reduce symptomsImmunotherapy : to shorten the duration of the diseasePhysiotherapy and hydrotherapy : to maintain the bodys muscles and to reduce stiffness and discomfort of the extremitiesPsychotherapyVentilator treatment and tracheostomyTreatment begins as soon as the diagnosis is established

GUILLAIN BARRE SYNDROMETreatment and care of patientsPlasmapheresis or Plasma Exchange (PE) : a mechanical process that involves the exchange of plasma and removal of disease-causing antibodies from the patients bloodIntravenous Immunoglobulin (IVIg) : consists of the slow injection of high doses of donor antibodies (immun-globulins), into the patients blood

GUILLAIN BARRE SYNDROMETreatment and care of patientsPlasmapheresis or Plasma Exchange (PE) : a mechanical process that involves the exchange of plasma and removal of disease-causing antibodies from the patients bloodIntravenous Immunoglobulin (IVIg) : consists of the slow injection of high doses of donor antibodies (immun-globulins), into the patients blood* Immunadsorption (Imad) : resembles PE, but only the immunoglobulins are removed

GUILLAIN BARRE SYNDROMETreatment and care of patientsGiving the high doses of other antibodies causes the patients own destructive antibodies to disappear into the crowd.Some of the donor antibodies inactivate these destructive antibodies, and the disease is slowed down. The activity of the white blood corpuscles that produce the undesirable antibodies is also slowed down.

GUILLAIN BARRE SYNDROMERecoveryMaking a prognosis about recovery is impossibleRecovery begins as suddenly as when gbs symptoms appearThe symptoms disappear gradually, but may take weeks, months or yearsThe course of the disease varies for each patientRecovery takes 3-6 months for most patientsAbout two thirds of them recover completely

GUILLAIN BARRE SYNDROMERecoveryDEATH : UP TO 5% OF THE CASES, DUE TO CARDIOVASCULAR OR RESPIRATORY COMPLICATIONSOF THE REST, 70% MAKE AN EXCELLENT RECOVERY WITH NO PERMANENT DAMAGE, EVEN AFTER A SEVERE ATTACKABOUT 20% ARE DISABLEDABOUT 10% ARE SEVERELY DISABLED

GUILLAIN BARRE SYNDROMEPrognosisThe onset of recoveryAgeThe intensity of infection phaseNeed for a ventilatorMajor loss of coordinationDegree of paralysisPreceding diarrhoeaSigns of axonal damageDuration of the treatment

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