Clinical Aspect of Guillain Barre Syndrome
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Transcript of Clinical Aspect of Guillain Barre Syndrome
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CLINICAL ASPECT OF GUILLAIN BARRE SYNDROMEAlwi ShahabBagian Neurologi FK Unsri RSMH Palembang
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Landry
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History of GBSLandry (1859) first described a case of a febrile illness followed by sensory and motor symptoms with subsequent respiratory failure and death.Autopsy unrevealing. Peripheral nerves probably not examinedThe disease was named Landrys Ascending Paralysis
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History of GBSGuillain, Barre and Strohl (1916) further described the disease when two soldiers in Amiens developed paralysis and loss of deep tendon reflexesA new diagnostic feature: albuminocytologic dissociation in the CSF
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Group of immune mediated disorder targeting the peripheral nervesUnknown etiologyAn acute autoimmune polyneuropathy encompassing heterogenous group of pathologic and clinical entitiesVarious antecedent infectionsPost infective acute polyradiculoneuropathyINTRODUCTION
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Cont.Incidence : 1-3/100,000 populationBoth sexes especially maleAny age group especially young adult and the elderlyProgressive symmetrical weakness and areflexiaMortality rate : 4-15%Persistent disability : 20%
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SGB SUBTYPEAIDP: Acute Inflammatory Demyelinating PolyneuropathyAMAN: Acute Motor Axonal NeuropathyAMSAN: Acute Motor Sensory Axonal NeuropathyMiller Fisher Syndrome
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CLINICAL FEATURES OF AIDP2/3 have identifiable preceding event Progressive for days to weeksMostly begin with paresthesia and followed by weakness on legs ascending to armsPartial opthalmoplegia happens in 5% of cases; total opthalmoplegia for 5% of cases as wellAutonomic dysfunction accounts for 65% of cases
- CLINICAL FEATURES OF AMANProgressive in similar manner to AIDPFlaccid paralysis No sensory dysfunctionOften preceeded by diarrhoeaSimilar prognosis to AIDPMortality rate of
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CLINICAL FEATURES OF AMSANCommonly preceeded by diarrhoeaAbrupt onset of weakness quadriplegia respiratory insufficiencyLonger period of recoveryMore residual symptomsMortality rate of 10-15%
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CLINICAL FEATURES OF MF SYNDROME
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LABORATORY FINDINGSCSF :- Protein : normal during early phase elevated after 2 weeks- Cell count < 10
Antibodies :- AIDP : Non spesific- AMAN: IgG anti GM1- AMSAN: IgG anti GD1a- MFS: IgG anti GD1b
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ELECTROPHYSIOLOGY FINDING AIDP:- prolonged F & distal motor latencies- conduction block
AMAN:- reduced CMAP- normal F & distal motor latency - normal sensory studies
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Cont.AMSAN:- no response in some motor nerve- decreased amplitude of CMAP- fibrillation- absence of SNAP
MFS: - reduced sensory & motor NAP- absence of H reflex- normal motor & sensory conduction velocity
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DIAGNOSTIC Clinically:- rapidly progressive ascending symmetric paralysis- areflexia- antecedent infection history (mostly)
Laboratory :CSF : cyto-albuminique dissociation especially after 2nd week from onset
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Cont.EMGAIDP: - evidence of demyelination on CNS- absence of F waveAMAN:- reduced motor amplitude - normal sensory studyAMSAN:- reduced motor and sensory amplitudeMFS:- decreased of sensory NAP
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DIFFERENTIAL DIAGNOSISAcute spinal cord diseasesAcute neuromuscular junction disorderAcute metabolic myopathyNeuropathy
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TREATMENTPlasma exchange - 50ml/kgBW 3-5times over 1-2 weeks- limited, invasive, serious side effectsIVIg- 0,4gr/kgBW/day over 5 days- less invasive, easy administration, less serious side effectsRespiratory carePhysical therapy
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Cont.Supportive therapy- DVT prophylaxis- Psychiatric consult for reactive depressionNutritional supportPain management
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Case illustration A 39 year old male was admitted with chief complaint of progressive quadriplegia starting 2 days ago. Initially the patient felt painful and tingling sensation on both lower limbs which later ascended to the upper limbs. Patient was still able to walk. A day after all the symptoms worsen and the patient was not able to walk anymore. 2 weeks before, the patient suffered from varicella. No history of spinal trauma or chronic cough.
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Physical examinationGeneral status : Compos mentisBP : 120/80mmHg RR : 20xHR : 78x T : 36.8 CNeurological status :Cranial nerve functions : in normal limitMotor function
Right upper limbLeft upper limbRight lower limbLeft lower limbMovementInadequateInadequateInadequateInadequateStrength3434Tone
Clonus --Physiiological reflex
Pathological reflex----
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Sensory function: paresthesia on tip of four limbsHigher cortical function : in normal limitAutonomic function : in normal limitMeningeal reflexes : -Gait and balancing : steppage gaitAbnormal movement : -
Diagnosis : Flaccid quadriplegia e.c suspected Guillain Barre Syndrome
Radiology findings :Chest X-ray : in normal limit
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Laboratory findings :Hb : 14,6 mg/dlLeu: 12,700 mm3Diff. Count : 0/01/79/13/7Glucose : 120 md/dlTotal cholesterol : 228 mg/dlHDL : 39 mg/dlLDL : 139 mg/dlTriglycerides : 258 mg/dl
Dermatology and venerology consult : Varicella in recovery process + hyperpigmentation
- Ureum : 42 mg/dl- Creatinine : 0,9 mg/dl- Uric acid : 1,8 Natrium : 145 mmol/L- Potassium : 4,1 mmol/L- Calsium : 1,96 mmol/L- Widal O titer : 1/80- Widal H titer : 1/80- ASTO : (-)- CRP : (+)
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Day 1 : Compos mentisBP : 120/80 mmHg, HR : 78x/minSpontaneous breathing, reguler, adequate, RR : 20x/minStrength of upper limb 3, lower limb 4Tingling sensation on the tip of hands and feetGiven neurotonic and roborantiaDay 2 : BP : 140/90 mmHg, HR : 84x/min RR : 20x/minStrength of upper limb 3, lower limb 2Day 3 :SomnolenceBP : 170/110 mmHg, HR : 102x/minDyspnoe, RR : 28x/minStrength of upper limb 1, lower limb 1Moved to ICU
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Day 6 : Spontaneous respitarory (-) BP : 143/98 mmHg, HR : 85x/minVentilator is usedAdministration of IVIG 400 mg/kg BW/day for 5 daysDay 9 : BP : 181/113 mmHg, HR : 118 x/mntDay 10 : Compos mentisBP : 98/60 mmHg, HR : 117x/minLast day of IVIGTracheostomy Strength of upper limb 2, lower limb 1Hari 17 : BP: 140/85 mmHg, HR: 112x/min.Spontaneous respiratory (+), RR : 40x/min.
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Day 19 : BP : 147/85 mmHg, N: 103x/minSpontaneous respiratory (+)RR : 26x/min.Strength of upper limb 2, lower limb 2.Day 29 : BP : 130/90 mmHg, RR : 98 x/minSpontaneous respiratory (+), adequate, RR : 22x/minMoved to regular roomStrength of upper limb 3, lower limb 1Day 37 : BP:120/80 mmHg, HR: 86x/minStrength of upper limb 3 lower limb 2
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Day 53 : BP : 120/80 mmHg, HR: 82x/minStrength of upper limb 4, lower limb 3Day 69 : BP : 120/90 mmHg, HR : 78x/minStrength of upper limb 4, lower limb 4Tracheostomy off
Patient was sent home at day 72 with strength of upper limb 4 and lower limb 4.
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