GBS

Guillain-Barre SyndromePresentor: Izaskun C. GanaoModerator: Dr. Mary Katherine E. Millan-Morales Reactor: Dr. Teresita N. Rabanal ObjectivesTo discuss the approach to a patient with Guillain-Barre Syndrome

To discuss the clinical presentation and management of Guillain-Barre Syndrome

IDENTIFYING DATAJSS4 year oldMaleFilipinoFrom MandaluyongAdmitted for the 1st time in our institution last September 30 2013 Chief ComplaintVomitingAndDoubling of Vision History of Present Illness1 day PTA Patient complained of doubling of vision noted to have inward deviation of the eyes, L>R (-) vomiting (-) history of trauma (-) headache (-) dizziness (-) body weakness (-) seizureSpontaneous resolution of symptomsMorning PTA History of Present Illnessdoubling of vision associated with inward deviation of the right eye(+) 4 episodes of vomiting(-) body weakness(-) headache(-) numbness

Consult at TMC-ER

Advised admissionReview of SystemsSkin(-) jaundice, (-) active dermatoses, (-) pruritus, photosensitivityEyes(-) lacrimation, (-) preferential gaze, (-) strabismus, (-) blurring of vision, lacrimation, rednessEars: (-) discharge, (-) tenderness, (-) ear pain, deafness, tinnitusNose:(-) epistaxis, (-) dischargeMouth: (-) sores, fissures, bleeding gumsNeck:(-) stiffness, limitation of motion on the neckPulmonary:(-) hemoptysis, (-) difficulty of breathingCardiac:(-) chest pain, orthopnea, syncopeGastrointestinal: (-) constipation (-) hematochezia , (-) melenaGenitourinary: (-) urgency, hesitancy, dysuria, hematuria, frequencyMuscular: (-) swelling, (-) trauma, (-) wastingHematopoietic: (-) bleeding, (-) easy bruisingEndocrine: (-) polydipsia, polyuria, polyphagia, nocturia, (-) heat intolerancetremorsNeuro: (-) headache, seizure, head traumaPast Medical History(+) Skin Asthma(+) Measles 2011 (2 years old)No previous head traumaNo known allergiesNo previous surgeriesNewborn screen normal

Family History(+) Asthma(+) Diabetes

Birth HistoryFull termNormal spontaneous delivery31 year old G1P1 (1001) Birth weight: 2.8 kgNo feto-maternal complications

Nutritional HistoryNo particular food preferencesNo known food allergiesImmunization HistoryBCG - 1 doseDPT/OPV - 3 doses + 2 boostersHiB - 3 dosesHepatitis-B - 3 dosesMMR - 2 dosesVaricella - 1 dosePCV - 3 dosesHepatitis - 2 dosesTyphoid - 1 doseInfluenza Last dose: 2012

Physical Examination at the ERGeneral survey Awake, active, not in cardio-respiratory distress

Vital signs:BP: 90/60 CR: 82 bpm RR: 26 cpm T: 36.1C Weight: 16 kg Height: 105 BMI: 14.5

Nonsunken eyeballs, moist lips, no oral soresSymmetrical chest expansion, no retractions, clear breath soundsAdynamic precordium, normal rate, regular cardiac rhythm, no murmursFlat abdomen, normoactive bowel sounds, soft abdomen, nontenderFull pulses, CRT less than 2 seconds, warm extremities

Physical Examination at the ERNeurologic ExaminationGCS 15, awake, follows commands, coherent, able to speak and comprehend spoken languagePhysical Examination at the ERCranial nerves:CN I intactCN II, III pupils equally briskly reactive to lightCN III no ptosis, pupils equally briskly reactive to lightCN IV no diplopia on looking down or inCN VI diplopia on lateral gaze to the rightCN V intact corneal reflexCN VII no facial asymmetryCN VIII intact gross hearingCN IX, X - can swallow, intact gagCN XI good shoulder shrugCN XII tongue midline

Neurologic ExaminationCN V intact corneal reflexCN VII no facial asymmetryCN VIII intact gross hearingCN IX, X - can swallow, intact gagCN XI good shoulder shrugCN XII tongue midline

Physical Examination at the ERNeurologic ExaminationMotor:

Sensory:

Deep tendon reflexes:

Physical Examination at the ER5/55/55/55/5100%100%100%100%++++++++(-) Babinski(-) ClonusSupple neck, non meningeal signsAdmitting ImpressionVomiting, unspecified;t/c abduction deficit, OD more than OS, probably secondary to cranial nerve VI compression ;R/O Intracranial mass

Laboratory ExaminationsCBCHgbHctWbcNeuLymMonoEosPlt9/301213812.82766602409/30Creatinine0.55Ionized calcium4.72Sodium139Potassium4.1Therapeutics:Eye patch application for 4 hours each eye alternatelyVitamin B complex once a day

1st hospital daySubjectiveObjectiveAssessmentPlanAfebrile since admissionDiplopia1 episode of vomitingDecreased appetiteNo abdominal painNo headache

BP: 100/60 CR: 88RR: 24 T: 36.4Inability to abduct right eyeNo hoarseness of the voiceClear breath soundsRegular cardiac rhythmSoft abdomenFull pulsesNo limitation of movement, no decrease in sensationVomiting, unspecified;t/c abduction deficit, OD more than OS, probably secondary to cranial nerve VI compression ;R/O Intracranial mass

HGTFor MRI of the orbitsFor chest x-rayFor ECGEye patch application for 4 hours each eye alternatelyVitamin B complex once a dayHGT: 102 mg/dLECG: sinus bradycardia, normal sinus rhythmChest x-ray: no significant chest findingsMRI of the orbits: polysinusitis, enlarged adenoids, unremarkable MRI of the orbits Laboratory ExaminationsSecond Hospital DaySubjectiveObjectiveAssessmentPlanAfebrile since admissionDiplopia1 episode of vomiting(+) abdominal painNo bowel movement since admissionDecreased appetiteNo headache

BP: 100/60 CR: 80RR: 22 T: 37Inability to abduct right eyeNo hoarseness of the voiceClear breath soundsRegular cardiac rhythmSoft abdomen

Polysinusitis

Eye patch application for 4 hours each eye alternatelyVitamin B complex once a dayCo-AmoxiclavThird Hospital DaySubjectiveObjectiveAssessmentPlanAfebrile since admissionDiplopiaNo headacheNo difficulty of breathingWeakness of the lower extremitiesNo loss of sensation

BP: 90/60 CR: 70RR: 21 T: 36.8Inability to abduct right eyeNo hoarseness of the voiceClear breath soundsRegular cardiac rhythmSoft abdomenGCS 15No sensory deficit, (+) areflexiaMMTs:

R/O Guillain-Barre Syndrome, Miller Fisher variant;Polysinusitis

For Electromyography/Nerve conduction Study of the lower extremitiesEye patch application for 4 hours each eye alternatelyVitamin B complex once a dayCo-AmoxiclavFor transfer to PICU for close monitoring5/55/50/50/5EMG-NCV of the lower extremities: normal findings for age except for the bilateral absence of the H-reflex. Consider beginning or early polyneuropathy. Laboratory ExaminationsThird Hospital Day, PICUSubjectiveObjectiveAssessmentPlanAfebrile since admissionDiplopiaNo headacheNo difficulty of breathingWeakness of the lower extremitiesNo loss of sensation

Abdominal pain, periumbilical area, 4/10No bowel movement since admissionNo vomitingBP: 90/60 CR: 58-72RR: 24 T: 37.1Inability to abduct both eyesNo hoarseness of the voiceIrregular cardiac rhythmNormoactive bowel sounds, soft abdomen, nontenderGCS 15No sensory deficit, (+) areflexiaMMTs:R/O Guillain-Barre Syndrome, Miller Fisher variant;PolysinusitisFor Electromyography/Nerve conduction Study of the lower extremitiesECGEye patch application for 4 hours each eye alternatelyVitamin B complex once a dayCo-AmoxiclavGlycerine suppositoryIVIg, 2g/kg/dose5/55/50/50/5Electromyography/Nerve conduction Study of the lower extremitiesnormal findings for age except for the bilateral absence of the H-reflex Electrical equivalent of the tendon jerkECGsinus arrhythmia Laboratory ExaminationsFourth Hospital Day, PICUSubjectiveObjectiveAssessmentPlanAfebrile since admissionNo diplopia(+) headache, parieto-temporal, 5/10Difficulty swallowing liquidsNo difficulty swallowing solid foodNo difficulty of breathingWeakness of the lower extremitiesNo loss of sensation

BP: 100/60 CR: 72RR: 24 T: 36.4PtosisInability to abduct both eyesNo hoarseness of the voiceIrregular cardiac rhythmClear breath soundsGCS 15No sensory deficit, (+) areflexiaMMTs:Guillain-Barre Syndrome, Miller Fisher variant; s/p IVIg;PolysinusitisDiscontinue eye patch applicationVitamin B complex once a dayCo-AmoxiclavParacetamol5/55/50/50/5Fifth Hospital Day, PICUSubjectiveObjectiveAssessmentPlanAfebrile since admissionNo diplopiaNo headacheWeak coughNo difficulty swallowingNo difficulty of breathingWeakness of the lower extremities, beginning weakness of the upper extremitiesNo loss of sensation

BP: 100/60 CR: 70RR: 23 T: 36.9Inability to abduct both eyesPtosisHoarseness of the voiceRegular cardiac rhythmClear breath soundsGCS 15No sensory deficit, (+) areflexia of LEMMTs:Guillain-Barre Syndrome, Miller Fisher variant; s/p IVIg;PolysinusitisVitamin B complex once a dayCo-Amoxiclav4/54/50/50/5Sixth Hospital Day, PICUSubjectiveObjectiveAssessmentPlanAfebrile since admissionNo diplopiaWeak coughNo difficulty swallowingNo difficulty of breathingWeakness of the lower extremities, beginning weakness of the upper extremitiesNo loss of sensationNo bowel movement for 3 daysNo abdominal pain

BP: 100/60 CR: 62RR: 20 T: 36.8Inability to abduct both eyesPtosisHoarseness of the voiceRegular cardiac rhythmClear breath soundsSoft nontender abdomenGCS 15No sensory deficit, (+) areflexia of LEMMTs:Guillain-Barre Syndrome, Miller Fisher variant; s/p IVIg;PolysinusitisVitamin B complex once a dayCo-AmoxiclavLactulose4/54/50/50/5Seventh Hospital Day, PICUSubjectiveObjectiveAssessmentPlanAfebrile since admissionNo diplopiaCough resolvedNo difficulty swallowingNo difficulty of breathingWeakness of the lower extremitiesNo loss of sensationBetter appetiteNo bowel movement for 3 daysNo abdominal pain

BP: 100/60 CR: 75RR: 23 T: 36.6Limitation of movement of all EOMsNo ptosisNo hoarseness of the voiceRegular cardiac rhythmSoft nontender abdomenGCS 15No sensory deficit, (+) areflexia of LEMMTs:Guillain-Barre Syndrome, Miller Fisher variant; s/p IVIg;PolysinusitisVitamin B complex once a dayLactuloseInosiplexIVF at 80% of maintenance4/54/50/50/5Eighth Hospital Day, PICUSubjectiveObjectiveAssessmentPlanAfebrile since admissionNo diplopiaCough resolvedNo difficulty swallowingNo difficulty of breathingWeakness of the lower extremitiesNo loss of sensationGood oral intake

BP: 100/60 CR: 80RR: 24 T: 36.4Limitation of movement of all EOMsNo ptosisNo hoarseness of the voiceRegular cardiac rhythmSoft nontender abdomenGCS 15No sensory deficit, (+) areflexia of LEMMTs:Guillain-Barre Syndrome, Miller Fisher variant; s/p IVIg;PolysinusitisFor serum potassium, sodium, ionized calciumVitamin B complex once a dayInosiplexPassive range of motion exercisesDiscontinue IVF4/54/50/50/59/3010/08Creatinine0.55Ionized calcium4.724.72Sodium139136Potassium4.13.6Tenth Hospital DaySubjectiveObjectiveAssessmentPlanAfebrile since admissionNo diplopiaCough resolvedNo difficulty swallowingNo difficulty of breathingDecreased weakness of the right lower extremityFurther weakness of the right upper extremityNo loss of sensationGood oral intake

BP: 100/60 CR: 72RR: 21 T: 36.8Limitation of movement of all EOMsNo ptosisNo hoarseness of the voiceRegular cardiac rhythmSoft nontender abdomenGCS 15No sensory deficit, (+) areflexia of LEMMTs:Guillain-Barre Syndrome, Miller Fisher variant; s/p IVIg;PolysinusitisVitamin B complex once a dayInosiplexPassive range of motion exercisesTransfer to a regular room3/54/52/50/5Eleventh Hospital DaySubjectiveObjectiveAssessmentPlanAfebrile since admissionNo diplopiaCough resolvedNo difficulty swallowingNo difficulty of breathingDecreased weakness of both lower extremitiesWeakness of the right upper extremitiesNo loss of sensationGood oral intake

BP: 100/60 CR: 80RR: 23 T: 37.2Limitation of movement of all EOMsNo ptosisNo hoarseness of the voiceRegular cardiac rhythmSoft nontender abdomenGCS 15No sensory deficit, (+) areflexia of LEMMTs:Guillain-Barre Syndrome, Miller Fisher variant; s/p IVIg;PolysinusitisVitamin B complex once a dayInosiplexPassive range of motion exercises4/54/52/52/5Thirteenth Hospital DaySubjectiveObjectiveAssessmentPlanAfebrile since admissionNo diplopiaCough resolvedNo difficulty swallowingNo difficulty of breathingDecreased weakness of both lower extremitiesWeakness of the right upper extremitiesNo loss of sensationGood oral intake

BP: 100/60 CR: 86RR: 24 T: 36.4Limitation of movement of all EOMs, less pronounced adduction deficitNo ptosisNo hoarseness of the voiceRegular cardiac rhythmSoft nontender abdomenGCS 15No sensory deficit, (+) areflexia of LEMMTs:Guillain-Barre Syndrome, Miller Fisher variant; s/p IVIg;PolysinusitisVitamin B complex once a dayInosiplexPassive range of motion exercises4/54/52/52/5Sixteenth Hospital DaySubjectiveObjectiveAssessmentPlanAfebrile since admissionNo diplopiaCough resolvedNo difficulty swallowingNo difficulty of breathingGradual decrease in weakness of both lower extremitiesWeakness of the right upper extremities resolvedNo loss of sensationGood oral intake

BP: 90/60 CR: 70RR: 24 T: 36.4Limitation of movement of all EOMs, less pronounced adduction deficitNo ptosisNo hoarseness of the voiceRegular cardiac rhythmSoft nontender abdomenGCS 15No sensory deficit, (+) areflexia of LEMMTs:Guillain-Barre Syndrome, Miller Fisher variant; s/p IVIg;PolysinusitisPassive range of motion exercisesFor dischargeHome medication: Vitamin B complex5/55/54/54/5Final DiagnosisGuillain-Barre Syndrome,Miller-Fisher Variant;s/p IVIg;PolysinusitisdiscussionSalient Features4 year old maleAfebrileDoubling of visionVomitingAbdominal painConstipationAscending weakness of the extremitiesAreflexia of the lower extremitiesLimitation of movement of extraocular musclesArrhtyhmia and bradycardiaPtosisHeadacheDifficulty swallowing liquidsHoarseness of the voice

Differential DiagnosEsIntracranial massheadache,altered mental status ataxianausea, vomitingweakness, and gait disturbancefocal seizuresfixed visual changesspeech deficitsfocal sensory abnormalitiesBotulismNauseaVomitingExtremely dry mouth unrelieved by drinking fluidsSore throatCranial nerve paralysis manifesting as:blurred visiondiplopiaPtosisextraocular muscle weakness or paresisfixed/dilated pupilsdysarthriadysphagia or suppressed gag reflexParalytic ileus advancing to severe constipationGastric dilatationBladder distention advancing to urinary retentionOrthostatic hypotensionReduced salivationReduced lacrimationBotulismChanges in deep tendon reflexes, which may be either intact or diminishedIncoordination due to muscle weaknessAbsence of pathologic reflexes and normal findings on sensory and gait examinationsNormal results on mental status examinationBotulismSymmetrical descending paralysis or weakness of motor and autonomic nervesRespiratory muscle weakness subtle or progressive, advancing rapidly to respiratory failureBotulismPoliomyelitisFeverHeadacheNausea/VomitingAbdominal painOropharyngeal hyperemiaNuchal rigidityBack and lower extremity painMeningitis with lymphocytic pleocytosis (usually)Paralytic poliomyelitis occurs in fewer than 5% of affected patients and is characterized by the following:Compromise of the motor neurons may be localized or widespread.asymmetric loss of muscleMuscle atrophy is generally observed several weeks after the beginning of symptoms.Myasthenia Gravisspecific muscle weakness rather than generalized muscle weaknessThe severity of the weakness typically fluctuates over hours being least severe in the morning and worse as the day progressesincreased by exertion and alleviated by restThe degree of weakness also varies over the course of weeks or months, with exacerbations and remissionsExtraocular muscle weakness or ptosis is present initially in 50%Patients progress from mild to more severe disease over weeks to months. Weakness tends to spread from the ocular to facial to bulbar muscles and then to truncal and limb muscles

Transverse MyelitisSymptoms include weakness and numbness of the limbs as well as motor, sensory, and sphincter deficitsSevere back pain may occur in some patients at the onset of the diseaseThe signs and symptoms depend upon the level of the spinal cord involvedParalysis and sensory loss below the level of the lesion

weakness is ascending and symmetrical in nature. The lower limbs are usually involved before the upper limbs.Cranial nerve involvementFacial droopDiplopiasDysarthriaDysphagiaOphthalmoplegiaPupillary disturbances

Guillain-Barre SyndromeVisceral painTachycardiaBradycardiaFacial flushingParoxysmal hypertensionOrthostatic hypotensionAnhidrosis and/or diaphoresisDyspnea on exertionShortness of breathDifficulty swallowingSlurred speech

Guillain-Barre SyndromeGuillain-Barre SyndromeGuillain-Barre SyndromePostinfectious polyneuropathyMainly Motor Nerves, occ. Sensory and Autonomic nervous system.Most common cause of acute generalized paralysis in all age groupsEstimated incidence: 0.4 -1.7 cases/100,000 populationVery rare in children < 1 year of ageM:F-1.5:1, M predominance more significant in adults rather than children.

CAUSESUsually follows a nonspecific viral infection by about 10 days - 4 weeksUp to two thirds of patients report an antecedent bacterial or viral illness prior to the onset of neurologic symptomsAGENTS: Campylobacter jejuni,H.pylori, EBV, CMV Mycoplasma pneumoniaeWest Nile virus also may cause GB like syndromeAlso reported following administration of vaccines against rabies, influenza,OPV, and possibly the conjugated meningococcal vaccine. PATHOPHYSIOLOGYGBS is an immune-mediated Disease: Precise mechanism of the immunologic injury is unclearUsually postinfectiousImmune-mediated: infectious agents thought to induce Ab production against specific gangliosides/glycolipidsLymphocytic infiltration of spinal roots/peripheral nerves & then macrophage-mediated, multifocal stripping of myelinResult: defects in the propagation of electrical nerve impulses, with eventual conduction block and flaccid paralysis CLINICAL FEATURESMajor clinical features are progressive muscle weakness and diminished DTRs with symmetric distributionWeakness begins usually in the lower extremities, progressively involves the trunk, the upper extremities, and finally the bulbar muscles: Landry ascending paralysis Sensory disturbances also are quite common40% of patients complain of pain or paresthesias in the extremities, around the mouth, or in the back as the presenting symptom. Position and vibratory sensations also diminishedThe weakness, sensory loss, and pain, independently or together, often present as a gait disturbance with an ataxic component out of proportion to the muscle weakness. CLINICAL FEATURESBulbar involvementoccurs in about of casesCN involvement with facial weakness, difficulty in swallowing, and occasionally, ophthalmoplegia~15%- 20% of pts. progress to respiratory failure requiring mechanical ventilationAutonomic disturbances, are infrequent but potentially life-threatening: Tachy-/bradyarrhythmias, hyper-/hypotension, orthostatic hypotension, occ. asystoleBowel/bladder dysfunction is rare: Transient urinary incontinence/ retention is a complication in ~ 20% of cases Fever is not a typical component of the syndrome, and when present, suggests a potential secondary infection.Meningismus and papilledema are troubling signs, and when present, other diagnoses must be considered.The Miller-Fisher variant of GBS is unique in that this subtype begins with cranial nerve deficits

CLINICAL FEATURESSubtypes ofGuillain-Barr syndrome

Several variants of GBS are recognized. These disorders share similar patterns of evolution, symptom overlap, and probable immune-mediated pathogenesis.

Miller-Fisher syndromeobserved in about 5% of all GBS casestriadataxiaareflexiaophthalmoplegia (cardinal feature)Ataxia tends to be out of proportion to the degree of sensory loss.Patients may also have mild limb weakness, ptosis, facial palsy, or bulbar palsyPatients have reduced or absent sensory nerve action potentials and absent tibial H reflex.

Subtypes ofGuillain-Barr syndrome

Acute inflammatory demyelinating polyneuropathythe most commonly identified form in the United States.generally preceded by a bacterial or viral infection40% of patients are seropositive for C jejuniLymphocytic infiltration and macrophage-mediated peripheral nerve demyelination is presentSymptoms generally resolve with remyelination.

Subtypes ofGuillain-Barr syndrome

Acute motor axonal neuropathypurely motor disorder that is more prevalent in pediatric age groups.generally characterized by rapidly progressive symmetric weakness and ensuing respiratory failure.Nearly 70-75% of patients are seropositive for Campylobacter.Patients typically have high titers of antibodies to gangliosidesOne third of patients with AMAN may actually be hyperreflexic.

Subtypes ofGuillain-Barr syndrome

Acute motor-sensory axonal neuropathyacute severe illness differing from AMAN in that AMSAN also affects sensory nerves and roots.Patients are typically adultsoften presents as rapid and severe motor and sensory dysfunction.Marked muscle wasting is characteristicassociated with preceding C jejuni diarrheaPathologic findings show severe axonal degeneration of motor and sensory nerve fibers with little demyelination.

Subtypes ofGuillain-Barr syndrome

Acute panautonomic neuropathyrarest GBS variantinvolves both the sympathetic and parasympathetic nervous systemsPatients have severe postural hypotension, bowel and bladder retention, anhidrosis, decreased salivation and lacrimation, and pupillary abnormalitiesCardiovascular involvement is common, and dysrhythmias are a significant source of mortality. Significant motor or sensory involvement is lacking

Subtypes ofGuillain-Barr syndrome

Pure sensory Guillain-Barr syndromeIt is typified by a rapid onset of sensory loss and areflexia in a symmetric and widespread patternLumbar puncture studies show albuminocytologic dissociation in the CSF, and electromyography (EMG) results show characteristic signs of a demyelinating process in the peripheral nerves.

Subtypes ofGuillain-Barr syndrome

LAB FINDINGSCSF studies are essential for diagnosis : elevated protein, N cell count~50% of pts. will demonstrate an initial elevation of CSF protein if measured in the 1st week of the illness; the majority will have an increase in protein if measured after the first several weeks of the illness. ELECTRODIAGNOSISElectrodiagnostic abnormalities are found in approx. 80% of the pts. The most important electrodiagnostic feature in GBS is evidence of multifocal demyelination in motor + sensory nerves.MANAGEMENTThe mainstay of treatment is supportive care. Most common causes for death are autonomic disturbances and when +nt careful monitoring in an ICU is needed.2nd most common cause of death and the most common cause of prolonged hospitalization is respiratory failurePts. should have FVC and NIF measured oftenIVIGRapidly progressive ascending paralysis is treated with intravenous immunoglobulin (IVIG), administered for 2, 3, or 5 days. Commonly recommended protocol is IVIG 0.4 gm/kg/day for 5 consecutive daysPlasmapheresis &/or immunosuppressive drugs are alternatives if IVIG is ineffective. Steroids are not effective. Combined administration of immunoglobulin and interferon is effective in some patients PROGNOSISThe clinical course is usually benign, and spontaneous recovery begins within 23 wk. Most patients regain full muscular strength, although some are left with residual weakness. The tendon reflexes are usually the last function to recover. Improvement usually follows a gradient inverse to the direction of involvement, with recovery of bulbar function first and lower extremity weakness resolving last. 65% can walk independently @ 6 mosOverall, 80% usually recover completely5-10% have prolonged course w/ incomplete recovery, ~3% wheelchair boundApprox 5% die despite ICU care2% will develop chronic relapsing Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP)

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