CLINICAL ASPECT OF GUILLAIN BARRE SYNDROMEAlwi ShahabBagian Neurologi FK Unsri RSMH Palembang

Landry

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

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

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

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%

SGB SUBTYPEAIDP: Acute Inflammatory Demyelinating PolyneuropathyAMAN: Acute Motor Axonal NeuropathyAMSAN: Acute Motor Sensory Axonal NeuropathyMiller Fisher Syndrome

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

CLINICAL FEATURES OF AMSANCommonly preceeded by diarrhoeaAbrupt onset of weakness quadriplegia respiratory insufficiencyLonger period of recoveryMore residual symptomsMortality rate of 10-15%

CLINICAL FEATURES OF MF SYNDROME

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

ELECTROPHYSIOLOGY FINDING AIDP:- prolonged F & distal motor latencies- conduction block

AMAN:- reduced CMAP- normal F & distal motor latency - normal sensory studies

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

DIAGNOSTIC Clinically:- rapidly progressive ascending symmetric paralysis- areflexia- antecedent infection history (mostly)

Laboratory :CSF : cyto-albuminique dissociation especially after 2nd week from onset

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

DIFFERENTIAL DIAGNOSISAcute spinal cord diseasesAcute neuromuscular junction disorderAcute metabolic myopathyNeuropathy

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

Cont.Supportive therapy- DVT prophylaxis- Psychiatric consult for reactive depressionNutritional supportPain management

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.

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----

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

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 : (+)

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

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.

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

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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