Guillan Barre Syndrome Flashcards

1
Q

Guiilanne Barre Syndrome Pathology

A

Immune-Mediated Polyneurpathy

Resulting from a preceding infection that reacts with the ANS and produces inflammation in the peripheral nerve

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2
Q

Where is Guillain Barre Syndrome directed?

A

Directed toward the Nerve Root

Leading to a Motor and Sensory Neuropathy
Most common and severe Paralytic Neuropathy

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3
Q

3 Types of Gullian-Barre

A

Acute Motor Axonal Neuropathy (AMAN)

Acute-Motor Sensory Axonal Neuropathy (AMSAM)

Miller-Fischer Syndrome

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4
Q

Acute Motor Axonal Neuropathy (AMAN)

A

Axons (Nerve Fibers) rather than Myelin Sheath
Arm and Leg involvement

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5
Q

Acute Motor-Sensory Axonal Neuropathy (AMSAN)

A

Combination of Myelin and Axons damage

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6
Q

Miller-Fischer Syndrome

A

Rare Variant
Weakness or paralysis of the Eye Muscles

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7
Q

Epidemiology of Guillain Barre

A

Incidence: 1-2/100,000 per year)

Increases by ~20% with every decade of life beyond the first 10 years of age

Greater in MALES than females
Children 0-18 years

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8
Q

Guillain Barre: Autoimmune Disease

A

ACUTE demyelinating process of the peripheral nerves

Cell mediated PN disruption (Immune Cell Mediated)
Secondary Hypersensitivity Reaction

Elevated Cytokines in CSF, PN, Serum

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9
Q

Guillain Barre: Pathogenesis

A

2/3rds associated with bacterial or viral origin (2 wks prior)

Moves through the GI or Respiratory System
(Mucosal or Epithelium)

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10
Q

Guillain Syndrome Subtypes

A

(Demyelinating)
Acute Inflammatory Demyelinating Polyneuropathy

(Axonal)
Acute Motor Axonal Neuropathy

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11
Q

Guillain Syndrome Subtypes:
(Demyelinating)

A

Anti body injures myelin membranes

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12
Q

Guillain Syndrome Subtypes:
(Axonal)

A

Antibody injures Axonal Membranes

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13
Q

Pathogenesis of Guillain Barre (EXAM)

A

Infiltration of macrophages and T lymphocytes into the Spinal Roots and PN

Macrophages attack and strip the myelin
(Mild Cases = Axons intact)
(Severe Cases = Axons Destroyed)

Saltatory Conduction is reduced or disrupted
Varies from partial to complete

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14
Q

Guillain Barre Pathogens

A

Respiratory or diarrhea prior to illness (2-3 weeks)

Campylobacter Jejuni infection is most common
(Bacterial Gastroenteritis)

Cytomegalovirus, Epstein-Barr virus, and Human Immunodeficiency Virus (HIV) infection

A small % of patients develop GBS after another triggering event such as immunization, surgery, trauma, and bone-marrow transplantation

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15
Q

Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)

A

Similar clinical presentation to GBS

Slow progression up to 8 weeks
Persists for years (Chronic)

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16
Q

Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)

A

Does not involve the respiratory system

Rarely precluded by infection
Relapses are much more frequent
Longer recovery time
Corticosteroids effective

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17
Q

Clinical Presentation GBS:
Evolution

A

Acute (hours) or slowly over 3-4 weeks

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18
Q

Clinical Presentation of GBS:
Symptoms

A

Symmetrical ascending weakness from distal LEs to UE / Respiratory Systems

Varies from mild distal to total paralysis / intubation
Accompanied by severe fatigue

20-38% are intubated from intercostal and diaphragmatic weakness

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19
Q

Guillain Barre:
Clinical Presentation 1st Symptoms

A

Symmetrical
Numbness and Tingling
Ascending Muscular Weakness Distal to Proximal

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20
Q

Guillain Barre:
Clinical Presentation Areflexia

A

Ascending Loss and hypotonia

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21
Q

Guillain Barre:
Clinical Presentation Autonomic Symptoms

A

50% of persons
Tachycardia
Low Cardiac Output: Hypotension (Postural)
Cardiac Arrhythmias (20%)
Hypertension: BP Dysregulation
Peripheral pooling of blood
Urinary retention

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22
Q

Guillain Barre:
Clinical Presentation

A

Sensory Loss and Pain

23
Q

Guillain Barre: Clinical Presentation of Sensory Loss

A

Vibration or Position Sense
Paresthesias
Stocking and Glove Pattern

24
Q

Guillain Barre:
Clinical Presentation of Pain

A

Prominent: Muscular Aching associated with activity (stretching)

Severe burning or hypersensitivity
(Demyelination)

PROM: proximal Muscle groups
Sensory Nerve regrowth from WB & Ex

25
Guillain Barre Syndrome
Symmetrical weakness begins in the legs and ascends Severe respiratory muscle weakness (Ventilatory support) Treatment through plasma exchange and immune globulin Most cases preceded by an infection
26
Guillain Barre: Pathological Involvement
Can lead to: Loss of mobility Assisted transfers Inability to stand Inability to sit upright Also limited by fatigue and orthostatic/autonomic issues
27
Guillain Barre: Clinical Progression
Progression of disease process over 2-4 weeks 90% reach nadir by 4 weeks Progressive weakness ascends May involve respiratory musculature & diaphragm Acute ICU on Ventilation -> Poorer prognostic outcome
28
CIDP vs. Guillain Barre
CIDP -Slow onset & progress for a longer period, may return -Needs sustained treatment (even with remission) Guillain Barre Syndrome -Rapid onset that progresses quickly & stops in 2-4 wks -After stabilizing, rarely any further deterioration
29
Guillain Barre: Medical Diagnosis with Presentation
Initial based upon clinical presentation -Progressive weakness of one or more limbs -Absent or depressed DTRs -Absence of fever -SYMMETRICAL -CN involvement (V, VII, IX, X, XI, XII)
30
Guillain Barre: Medical Diagnosis with CSF
Confirmed with CSF (Lumbar Puncture) -Decreased Leukocytes -Elevated CSF fluid protein
31
Guillain Barre: Medical Diagnosis Gold Standard
Nerve Conduction Velocity will be abnormal
32
Lumbar Spinal Tap
Needle placed between the L3 and L4 spinous processes
33
3 Phase Course of GBS
Initial phase with definitive symptoms: 1-3 weeks Plateau Phase: Days to 2 weeks Recovery Phase: 4 to 6 months, up to 2 years
34
Guillain Barre: Treatment with Plasmapheresis
First Treatment option through Plasma Exchange -Removing plasma from the blood -Centrifugal separation to remove antibodies -Re-inject plasma into patient with albumin -Variable outcome -Initiated within two weeks
35
Guillain Barre: Medical Management with Intravenous
Intravenous Administration of Immunoglobulins (IVIg) Inhibit autoantibodies Reduce or block secondary immune attack by reducing phagocytic damage (-400 mg over six days) As effective as plasmapharesis Must be implemented immediately
36
Guillain Barre: Immunoglobulin
Immunoglobulin replaces missing antibodies in patients with immune deficiencies and helps them fight diseases
37
Medical Prognosis of GB
50% reach nadir by one week 70% by two weeks 80% by 3 weeks 3-5% expire due to respiratory or organ failure Recovery emerging in 2 to 4 weeks Fatigue or endurance long term consequence
38
Negative Prognostic Indicators
Older age: >40 Rapid rate of progression from onset (<7 days) Length of time till Nadir Severe muscle weakness CN involvement Ventilatory support Average distal motor response amplitude reduction to <20% of normal Preceding diarrheal illness or cytomegalovirus
39
Long Term Outcomes of GB
Independent ambulation at 6 months: >80% Full strength recover at 1 year: 60% 5-10% have prolonged course with several moths of Ventilatory dependency Death: 5% of those with GBS, 20% who become ventilator dependent Relapse: up to 10% Develop Chronic Inflammatory Demyelinating Polyneuropathy: 2%
40
Physical Therapy Management Acute Care
Medical Hx (precluding infection) Comorbidities Medications Social History Environment Language/Learning Style Cognition Integumentary
41
Special Considerations for GB
Skin Integrity DVT Autonomic Dysfunction: Hypertensive events (Sweating, BP changes, Color change) Aspiration: Watch for Dysphagia
42
PT Assessment of GB
Neuromuscular Assessment Muscular Assessment Cardiopulmonary Assessment CV: BP HR Supine, BP HR Sitting (every 2-3 minutes) Precautions: Autonomic changes (sweat, BP/HR) DVT: Homan’s Sign Aspiration: Elevate Bed
43
Additional Assessments of GB
CN Assessment Pain Assessment at rest MMT: UE/LE Sensation: Proprioception & Touch Respiratory: Diaphragm, Cough, Mobility Sitting: Tolerance, BP/HR/RR/O2 sats Transfers and Gait
44
ICU Care Management of GB
Patient in ICU on Ventilator & reached Nadir -Nursing and family training -ROM/positioning Supported upright sitting with Cardiac Respiratory monitoring: Respiratory Therapy -Initial 10-20 minutes -Supervised by PT (BP/HR checks 3min) Prevent contractures and skin breakdown
45
Overall Goals: ICU and Acute Care for GB EXAM
Prevent Contractures: -ROM exercises and splints -Positioning for longer stretch -Ankle foot orthosis Promote Mobility: -Management of Orthostatic Hypotension -Abdominal binder -Stockings or Leg Wraps -W/C Tilt with LE elevated
46
Items used in GB Treatment
Cardiac Chair Compression STockings
47
Things to avoid with GB
Avoid prolonged hip and knee flexion Chang position every 2 hours Positioning: Side lying, Supine Every 2 hours (float heels)
48
Transfers for GB
W/C Transfers -Transfer Board -Standing Pivot -Dependent
49
Overall Goals of Acute Care GB
Upright sitting in chair 2-3 hours per day Initiating transfer training and bed mobility Promote graded activity (AROM & PROM ex) Avoid prolonged positioning Initiate WB Activities (Orthotics) -Parallel Bars -Tilt Table -Walker
50
Inpatient Rehabilitation for GB
3 hours per day Consider Dysphagia (Language) Strengthening Activities Functional Rehabilitation -Gait -Transfers -Physical Tolerance -Postural Control
51
What about exercise in the Acute Phase of GB?
Avoid fatigue or over-exertion in Acute Phase -Inflammatory Process -Up to 86% have fatigue -Up to level of movement without pain -Halt at the first signs of fatigue Upright Tolerance is exercise!
52
Exercise for GB
Passive to Active - Assisted when below grade 3/5 Low repetitions and Low resistance Powder board or Hydrotherapy Painful muscles greater than 12 hours
53
Gait Activities for GB
High risk for falls due to Fatigue and Weakness Lack of Equilibrium and protective responses Orthotics (AFO or Ground Reaction Orthotics) 1. Initiate in Parallel Bars 2. Advance to Walker 3. Short Distances These individuals fall straight down! Collapse secondary to muscular fatigue
54
Summary of Guillain Barre
Autoimmune Mediated Process -Demyelinating and Axonal Loss -IVIg or Plasmapheresis Ascending Symptoms: Rapid -Ventilatory Assistance Recovery in 6 months with continue recovery -Up to 2 years