Download - A Case of Guillain-Barre (GBS) Syndrome 1
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Guillain-Barre Syndrome
Florentina Eller8/26/2014
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Case
• CC: Lower extremities numbness and paralysis• HPI: MW is 34 yo female who is admitted to the hospital on 8/5 due to
numbness and paralysis in her right leg, weakness in her left leg, and inability to walk. She also complains of daily, non-bloody diarrhea for the last 2 months and some urinary incontinence. Patient lost 40 lbs in the last month. The burning she feels in both legs started 2 days ago. MW has thoracic, lumbar and sacroiliac pain as well. She denies any trauma, MVA or recent labor. She has not had any recent URI.
• PMH: HTN, anxiety, s/p cholecystectomy, T2DM, RA, fibromyalgia, asthma, hypothyroidism, hx of DVT.
• FH: Significant for CAD and MI• SH: Chronic smoker (1 pack/day for 18 years); denies alcohol or illicit
drug use
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Case (cont.)• Home Medications: Synthroid 25 mcg PO QD; Metformin 1000 mg PO
QD; Prilosec PRN; Arava 20 mg PO QD; Hydroxychloroquine 200 mg PO BID; Endocet 10/325 PO Q 6H PRN pain; Valium 0.5 mg PO BID; Benicar 20 mg QD
• Allergies: codeine; penicillin• PE:
– Vital signs: T 97.6F; P 57bpm; BP 127/79 mmHg; RR 16 bpm; O2 sat 95%; – HEENT: PERRL; EOMI; no JVD; no lymphadenopathy; no meningism;– Cardiovascular: RRR; no murmurs, gallops, rubs; no LE edema; no calf
tenderness– Respiratory: CTA– GI: soft, nontender, nondistended– Psychiatric: Cooperative, but tearful– Integumentary: lower extremity cyst consistent with RA– Neurologic: Cranial nerves 2-12 intact; intact upper motor; lacking tendon
reflexes in LE
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Problems
1. Bilateral LE paraplegia with paresthesias (neurology consult): Suspect AIDP; spine imaging and lumbar puncture is needed to rule out other causes; discuss IVIg tx with patient and if OK start Privigen 10% 35 g/350 ml IVPB every 24 H for 5 days (0.4 g/Kg max 2g/kg for 5 days); rule out infections or structural causes; cardiac telemetry to evaluate for arrhythmia; pain and anxiety rx.
2. Diarrhea: 3. Hypothyroidism, RA, HTN- continue home medications,
T2DM4. DVT prophylaxis
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Guillain-Barre Syndrome (GBS) Introduction
• Definition: heterogeneous group of immune-mediated peripheral neuropathies– Common to all variants: rapidly evolving polyradiculoneuropathy
preceded by a triggering event– Cytomegalovirus or Campylobacter jejuni infection
• Prevalence: – GBS affects between 1 and 4 per 100,000 of the world’s population
annually.
• Prognosis: – 67% - persistent fatigue– 25% - respiratory failure requiring ventilation – 20% - persistent disability – 4% to 15% - death
Hughes RAC, Wijdicks EFM, Barohn R, et al. Practice parameter: immunotherapy for Guillain-Barré syndrome—report of the quality standards subcommittee of the American Academy of Neurology. Neurology 2003; 61: 736–40.
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Date of download: 8/24/2014 Copyright © 2014 McGraw-Hill Education. All rights reserved.
From: Section I. Cellular and Molecular Basis for Medical PhysiologyGanong's Review of Medical Physiology, 24e, 2012
From: Section I. Cellular and Molecular Basis for Medical PhysiologyGanong's Review of Medical Physiology, 24e, 2012
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Subtypes of GBS
Harrison's Principles of Internal Medicine, 18ed. Chapter 385. Guillain-Barré Syndrome and Other Immune-Mediated Neuropathies. Access Pharmacy. Accessed 8/24/2014
.
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GBS Pathophysiology AIDP
Ang CW, Jacobs BC, Laman JD. The Guillain-Barre Syndrome: a true case of molecular mimicry. Trends in Immunology. 2004. 25(2):61-66.
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Signs and Symptoms
• Weakness• Tingling dysesthesias in the extremities
– legs more often affected than arms• Paresthesias
– seldom extends past the wrists and ankles• Loss of deep tendon reflexes
– within the first few days of symptom onset• Progressive phase lasts few days to 4 weeks• Plateau phase: persistent, unchanging symptoms• Improvement begins within days of the plateau
– The time to resolution of symptoms varies
Newswanger DL, Warren CR. Guillain-Barre Syndrome. Am Fam Physician. 2004 May 15;69(10):2405-10.
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Diagnosis
Newswanger DL, Warren CR. Guillain-Barre Syndrome. Am Fam Physician. 2004 May 15;69(10):2405-10
• Required Factors:– Progressive weakness in both arms and legs– Areflexia (e.g. loss of knee jerk reflex)
• Strongly Supporting Diagnosis:– Progression of symptoms over days, up to 4 weeks– Relative symmetry of symptoms– Mild sensory symptoms or signs– Cranial nerve involvement ( bilateral weakness of facial muscles)– Recovery beginning 2-4 weeks after progression ceases– Absence of fever at onset– High concentration of protein in CSF
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2003 American Academy of Neurology (AAN) Treatment Guidelines
Hughes RA, Wijdicks EF, Barohn R, et al. Practice parameter: immunotherapy for Guillain-Barré syndrome: report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology. 2003 Sep 23;61(6):736-40.
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AAN Guidelines
• Treatment with PE or IVIg hastens recovery from GBS.• PE and IVIg are equally effective in patients with advance GBS symptoms.• PE may carry a greater risk of side effects and is more difficult to
administer.• Combining the two treatments is not recommended.• Steroid treatment is not beneficial. • High-dose IVIg (400 mg per kg daily for 5 days) or plasmapheresis (5
exchanges over 5-8 days) can be initiated. • Supportive care and monitoring for autonomic dysfunction
• Hughes RA, Wijdicks EF, Barohn R, et al. Practice parameter: immunotherapy for Guillain-Barré syndrome: report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology. 2003 Sep 23;61(6):736-40
• Newswanger DL, Warren CR. Guillain-Barre Syndrome. Am Fam Physician. 2004 May 15;69(10):2405-10
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IVIg, PE or Combination RCT
• International, multicenter, randomized trial of 383 adult patients with Guillain-Barré syndrome.
• Objectives: – Whether IVIg is equivalent/ superior to PE– PE followed by IVIg is superior to single treatment
• Methods:– Random assignment to:
• PE (five 50 mL/kg exchanges over 8–13 days)• IVIg (Sandoglobulin, 0·4 g/kg daily for 5 days)• PE course immediately followed by the IVIg course. • Retreatment with original treatment was permitted if relapse
Plasma Exchange/Sandoglobulin Guillain-Barré Syndrome Trial Group. Randomized trial of plasma exchange, intravenous immunoglobulin, and combined treatments in Guillain-Barré syndrome. Lancet 1997 Jan 25;349(9047):225-30.
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Outcomes Measurements: Disability Assessment Grades
Grades Disability Assessment Arm Assessment
0 Healthy; no signs or symptoms of GBS
Normal
1 Minor symptoms; able to run Minor symptoms; able to put hand on top of head and able to oppose thumb to each finger
2 Able to walk 5 m without assistance
Able to do one or the other @ 1 not both
3 Able to walk 5 m with assistance Some movement ; unable to perform either tasks above
4 Chair - bound; unable to walk No movement
5 Require assisted ventilation ( part of the day)
Dead
6 Dead N/A
Plasma Exchange Group. Lancet 1997.
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IVIg, PE or Combination RCT (cont.)
• Inclusion criteria: – GBS diagnosis by a qualified neurologist
• Clinical and • Cerebrospinal-fluid diagnostic criteria
– Severe disease (requiring aid to walk or worse)– Older than 16 years– Onset of neuropathic symptoms within the previous 14 days
• Statistics– Equivalence definition: If the true mean difference in the improvement of
the disability grade among the two groups <0.5 – Power: 122 patients were expected to give 90% power to detect a true
difference of ≥0.5 in the IVIg versus PE comparison and to exceed 0
Plasma Exchange Group. Lancet 1997.
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Treatment Groups Features
Plasma Exchange Group. Lancet 1997.
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Primary Outcome and Results
Mean disability-grade improvement after 4 weeks: No significant difference between the groups
– Difference of improvement between PE vs IVIg : 0.09 grade (95% CI 0.23 to 0.42)
– Proved equivalence between groups– Difference between the PE+IVIg and IVIg alone groups: 0.29 grade
(95% CI 0.04 to 0.63)– The difference between the PE+IVIg and PE alone groups: 0.20 grade
(0.14 to 0.54). – The combined treatment was not superior to either treatment
alone
Plasma Exchange Group. Lancet 1997.
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Adverse Events
• 8 cases in PE group : hypotension in five, septicemia, pneumonia, malaise, abnormal clotting, and hypocalcaemia
• 6 cases in IVIg group: nausea or vomiting, meningism, exacerbation of chronic renal failure, possible myocardial infarction, and painful erythema at the infusion site
• Conclusion– IVIg may be preferable to PE (in severe GBS that requires aid to walk
and the disorder was diagnosed < 2 weeks of onset) based on :• equal therapeutic benefit• greater convenience
Plasma Exchange Group. Lancet 1997.
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Treatment of the patient• Bilateral LE paraplegia with paresthesias (neurology consult): Suspect AIDP; spine
imaging and lumbar puncture is needed to rule out other causes; discuss IVIg tx with patient and if OK start Privigen 10% 35 g/350 ml IVPB every 24 H for 5 days (0.4 g/Kg max 2g/kg for 5 days); rule out infections or structural causes; cardiac telemetry to evaluate for arrhythmia; pain and anxiety rx.
• Workup– MRI of cervical and thoracic spine: soft tissue density in the epidural space
causing effacement of the right lateral thoracic cord at C7, T1 and T2– LP: 2WBC, 11RBC, protein 48
• Patient was treated with 5-day course of IVIg; – Weakness in leg improved, discharged home after 6 days of hospitalization
• MW came back to hospital less than 24 hours later for almost the same complaint: – Progressive weakness in her LE and UE – Was able to walk but with difficulty
• Patient treated successfully with PE @5
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Assessment of the Treatment• Patient received Provigen 10% 35 g/350 ml IVPB every 24 hours for 5
days • MW weighs 119Kg ( IBM 75.4Kg, AdjBW 92.6 Kg)
– If dosed at 0.4g/Kg* 119Kg= 47.6 g/day– Patient received 35 g/day (dosed based on ~ Adj BW : 37 g/day)
• Patient came back less than 24 hours later– MRI was ordered to asses for possible worsening of abnormal tissue density at
level C7-T12
• PE was ordered, @ 5 exchanges patient improved
Assessment: Starting with a higher dose of IVIg could have been more effective (use ABW instead of Adj BW)
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Future Possible Treatment
• Chinese herbal medicine Tripterygium polyglycoside ( Thunder God Vine) – Significantly better outcome after 8 weeks compared with
high-dose corticosteroids*• Very low quality of evidence RCT, found after• Meta analysis of 1271 references to possible RCT
• Other trials not large enough to find clinical significant benefits – Interferon beta-1a (IFNb-1a) vs placebo– Brain-derived neurotrophic factor (BDNF) vs placebo– CSF filtration vs regular PE
*Hughes RA, Pritchard J, Hadden RD. Pharmacological treatment other than corticosteroids, intravenous immunoglobulin and plasma exchange for GBS. Cochrane Database of Systematic Reviews, Feb 01, 2013, No. 2.
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Tripterygium wilfordii (Thunder God Vine)
http://en.wikipedia.org/wiki/Tripterygium_wilfordii