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3. History. Referred from Brits hospitalAdmitted for 3 weeksSudden onset inability to walkWeakness started with the legsProgressed to the armsFace involvement shown byDifficulty talkingDifficulty in swallowingDrooling secretionsUnable to close his eyesDiagnosed RVD positive on this present

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1. 1

2. 2 OUTLINE Case presentation Variants GBS Presentation GBS Treatment Prognostic features

3. 3 History Referred from Brits hospital Admitted for 3 weeks Sudden onset inability to walk Weakness started with the legs Progressed to the arms Face involvement shown by Difficulty talking Difficulty in swallowing Drooling secretions Unable to close his eyes Diagnosed RVD positive on this presentation CD4 count was 478

4. 4 Further enquiry No burning sensation and pins and needles in the feet and hands No sphincter involvement no gastro or respiratory illness prior to admission No vaccination No h/o food poisoning

5. 5 Past medical history Not yet put on ARVs No previous hospital admission No previous surgery

6. 6 Social history Single (did not ask about partners) Non smoker Does not take alcohol Unemployed No firms or farms close by

7. 7 On examination Alert and orientated BP 100/60 supine position Pulse 84 Temperature 37.2 Urine dipstick NAD

8. 8 On examination Bilateral lower motor neuron facial 7 palsy Bilateral absent gag reflexes UPPER LIMBS power flexors and extensors of elbow 5/5 Shoulder adduction and abduction bilaterally 5/5 Wrist flexion and extension 3/5 Finger abduction n adduction 3/5 Finger flexion and extension 3/5

9. 9 On examination Hip flexion and extension 3/5 Hip abduction and adduction 3/5 Knee flexion and extension 3/5 Ankle flexion and extension 2/5 Tone normal Global areflexia Sensation glove n stocking impairment both upper and lower limbs Co ordination difficult

10. 10 On examination Chest trachea central Good air entry Clear Cardiovascular-apex beat 5th ICS Normal S1 and S2 No murmurs Abdomen soft non tender

11. 11 summary 25 yrs old RVD reactive CD4 478 presenting acute quadriparesis with involvement of the face Legs more involved than the arm Proximal and distal weakness leg Distal weakness in the arm Glove n stocking sensory impairment

12. 12 Differentials Guillain barre syndrome Electrolyte imbalance (hypokalemia) Botulism Organophosphate poisoning Myasthenia gravis

13. 13 Investigations Full blood count (WCC 5.2o,HB 12.0, PLAT401) UE(NA135, K4.2, UREA7.1, CREAT 64) CSF GLUCOSE 3.1 (SERUM5.2) PROTEIN 0.28 CHLORIDE 126 ADA <1.0 _NO CELLS

14. 14 RX Started on immunoglobulins for 5 days Rehabilitation Physiotherapy Occupational therapy Speech therapy Counselling

15. 15 Guillain Barre Syndrome 1859-Laundry 10 % patients ascending paralysis 1916-3 French physicians First World War 2french soldiers motor weakness, areflexia, albuminocytological dissociation Guillain, Barre and Strohl tendon reflexes of the patients and recognised the peripheral nature of the illness

16. 16 Guillain Barre Syndrome Immune mediated Motor, sensory and autonomic dysfunction Acute inflammatory demyelinating polyneuropathy Progressive symmetric ascending motor weakness Hyporeflexia with or without sensory symptoms or autonomic symptoms

17. 17 Epidemiology 35 population based surveys past 40 years Olmsted county-incidence doubled from 1.2 to 2.4 per 100 000 1970-1980 Italy incidence 1.09 1980-1983 to 2.73 1991-1993 Occurs in all ages

18. 18 Pathophsiology Autoimmune humoral or cell mediated responses to recent infection Antibodies formed against ganglioside like epitopes in the lipopolysaccharide layer Cross react with ganglioside surface molecules of the schwann cells that myelinate peripheral nerves Myelin sparing axonal damage resulting from a direct cellular attack on axon itself

19. 19 Antecedent infections GBS postinfectious illness Two third acute infectious illness (respiratory, gastrointestinal) Interval between prodromal infection and onset varies 1-3 weeks

20. 20 Campylobacter jejuni Major cause of gastroenteritis worldwide Most frequent antecedent pathogen Association 14 large series of GBS Serological or culture evidence 26-46% Strong associated with AMAN Most frequent trigger of miller fischer Serum higher titres of antibody GM1 ang GQ1b

21. 21 Cytomegalovirus Upper respiratory tract infection, pneumonia or non specific flu Most viral triggers 10 -22% Young females Prominent involvement of sensory and cranial nerves High serum titres of antibodies crossreacting with GM1 gangliosides

22. 22 HIV Well recognised Around seroconversion Clinical presentation similar to AIDP Lymphocytic pleocytosis

23. 23 Vaccines Temporal association No causal relation Rabies vaccine prepared from infected brain tissues carried increased risk Cotroversy 1976-1977, swine influenza vaccine Small excess risk of developing GBS existed up to 6 weeks Subsequent mass influenza vaccination no increased incidence of GBS

24. 24 Acute inflammatory demyelinating polyneuropathy Most commonly identified Preceded by bacterial or viral infection 40% seropositive for campylobacter jejuni Lymphocytic infiltration and macrophage mediated demyelination of peripheral nerves Electrophysiologically-slowing of n conduction velocities _prolongation of distal and f wave latencies -conduction block

25. 25 Acute motor axonal neuropathy Prevalent among paediatric age groups 70-75% seropositive for campylobacter One third hyperreflexia Associated with presence of anti GM1 antibodies Rapidly progressive weakness ensuing respiratory failure and good recovery Electrphysiologically-reduction or absence of distally evoked motor action potential -early signs of denervation on needle electromyography -normal conduction velocities and normal action potential in sensory nerves

26. 26 Acute motor sensory axonal neuropathy Acute severe illness Affects sensory nerves and roots Adults with both motor and sensory dysfunction Marked muscle wasting Feasby & colleagues-unusual finding in 7 GBS -fulminant onset of paralysis after diarrhoea or flulike _severe generalised paralysis _6 needed ventilation within 2-4 weeks from onset of neurological symptoms Electrophysiologically-within 2-7 days reduced or absent evoked responses on distal stimulation of motor or sensory nerves -total loss of electrical excitability -severe generalised muscle atrophy with delayed and very poor recovery

27. 27 Miller Fischer syndrome Rare variant Ataxia, areflexia and opthalmoplegia Ataxia out of proportion to the degree of sensory loss Mild limb weakness, ptosis, facial palsy or bulbar palsy Anti GQ1b prominent Absent or reduced sensory nerve action potentials

28. 28 Acute panautonomic neuropathy Rarest Sympathetic and parasympathetic involvement Cardiac involvement is common Dysrhythmias significant course of mortality Experience sensory symptoms Recovery gradual and often incomplete

29. 29 History 2-4 weeks after respiratory or gastrointestinal illness Dysesthesias of fingers and lower extremity proximal muscle weakness Weakness progress over hours to days to involve arms, truncal muscles, cranial nerves Illness progresses from days to weeks, reaching a nadir by 4 weeks Plateau phase ensues followed days later by gradual symptom improvement One third of patients require mechanical ventilation

30. 30 Motor dysfunction Symmetric limb weakness Inability to stand or walk despite reasonable strength especially when opthalmoparesis or impaired propioception is present Respiratory muscle weakness with shortness of breath Cranial nerve palsies 3-7, 9-11 with facial weakness mimicking Bells palsy, dysphagia, dysarthria, opthalmoplegia and pupillary disturbances Absent deep tendon reflexes

31. 31 Sensory dysfunction Paresthesia begins in the fingers and toes progresses upward, not beyond wrists or ankles Pain sever shoulder girdle Loss of vibration, propioception, touch and pain distally

32. 32 Autonomic dysfunction Cardiovascular-tachycardia, bradycardia, dysrhythmias, wide fluctuations in BP and postural hypotension Urinary retention Constipation Facial flushing and venous pooling secondary to abnormal vasomotor tone Hypersalivation Anhydrosis Tonic pupils

33. 33 Diagnosis Clinical ground Lab studies rule out other diagnoses, assess functional status and prognosis Lumbar puncture- elevated proteins, no cells Normal CSF does not rule out GBS, remain normal 10% of patients CSF pleocytosis in HIV associated GBS Stool for culture Forced vital capacity Nerve conduction studies-delay F wave, nerve motor action potentials decreased, conduction block and prolonged distal latencies

34. 34 Indications for intubation Hypoxia Declining respiratory function Poor or weak cough Suspected aspiration FVC < 15ml/kg Forced expiratory < 40ml Forced inspiratory <30ml

35. 35 Admission Prevention of thromboembolic complications Cardiac monitoring Serial assessment of ventilatory reserve Oropharngeal weakness Airway protection Appropriate bowel & pain management Adequate nutrition Psychological support

36. 36 Medication Plasma exchange and immunoglobulin proven effective Shorten recovery time by 50% IVIG easier to administer, fewer complications than plasma exchange Cost and efficacy comparable Combination no improvement I outcomes Corticosteroids ineffective Immune adsorption early stages

37. 37 Prognostic features Age >60 Rapid onset <7 days Isolation campylobacter jejuni Ventilatory assistance Mean distal motor amplitudes of less than 20%nof the lower limit

38. 38 THANK YOU

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