Guillan Barre Syndrome Flashcards
Guiilanne Barre Syndrome Pathology
Immune-Mediated Polyneurpathy
Resulting from a preceding infection that reacts with the ANS and produces inflammation in the peripheral nerve
Where is Guillain Barre Syndrome directed?
Directed toward the Nerve Root
Leading to a Motor and Sensory Neuropathy
Most common and severe Paralytic Neuropathy
3 Types of Gullian-Barre
Acute Motor Axonal Neuropathy (AMAN)
Acute-Motor Sensory Axonal Neuropathy (AMSAM)
Miller-Fischer Syndrome
Acute Motor Axonal Neuropathy (AMAN)
Axons (Nerve Fibers) rather than Myelin Sheath
Arm and Leg involvement
Acute Motor-Sensory Axonal Neuropathy (AMSAN)
Combination of Myelin and Axons damage
Miller-Fischer Syndrome
Rare Variant
Weakness or paralysis of the Eye Muscles
Epidemiology of Guillain Barre
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
Guillain Barre: Autoimmune Disease
ACUTE demyelinating process of the peripheral nerves
Cell mediated PN disruption (Immune Cell Mediated)
Secondary Hypersensitivity Reaction
Elevated Cytokines in CSF, PN, Serum
Guillain Barre: Pathogenesis
2/3rds associated with bacterial or viral origin (2 wks prior)
Moves through the GI or Respiratory System
(Mucosal or Epithelium)
Guillain Syndrome Subtypes
(Demyelinating)
Acute Inflammatory Demyelinating Polyneuropathy
(Axonal)
Acute Motor Axonal Neuropathy
Guillain Syndrome Subtypes:
(Demyelinating)
Anti body injures myelin membranes
Guillain Syndrome Subtypes:
(Axonal)
Antibody injures Axonal Membranes
Pathogenesis of Guillain Barre (EXAM)
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
Guillain Barre Pathogens
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
Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)
Similar clinical presentation to GBS
Slow progression up to 8 weeks
Persists for years (Chronic)
Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)
Does not involve the respiratory system
Rarely precluded by infection
Relapses are much more frequent
Longer recovery time
Corticosteroids effective
Clinical Presentation GBS:
Evolution
Acute (hours) or slowly over 3-4 weeks
Clinical Presentation of GBS:
Symptoms
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
Guillain Barre:
Clinical Presentation 1st Symptoms
Symmetrical
Numbness and Tingling
Ascending Muscular Weakness Distal to Proximal
Guillain Barre:
Clinical Presentation Areflexia
Ascending Loss and hypotonia
Guillain Barre:
Clinical Presentation Autonomic Symptoms
50% of persons
Tachycardia
Low Cardiac Output: Hypotension (Postural)
Cardiac Arrhythmias (20%)
Hypertension: BP Dysregulation
Peripheral pooling of blood
Urinary retention
Guillain Barre:
Clinical Presentation
Sensory Loss and Pain
Guillain Barre: Clinical Presentation of Sensory Loss
Vibration or Position Sense
Paresthesias
Stocking and Glove Pattern
Guillain Barre:
Clinical Presentation of Pain
Prominent: Muscular Aching associated with activity (stretching)
Severe burning or hypersensitivity
(Demyelination)
PROM: proximal Muscle groups
Sensory Nerve regrowth from WB & Ex
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
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
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
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
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)
Guillain Barre:
Medical Diagnosis with CSF
Confirmed with CSF (Lumbar Puncture)
-Decreased Leukocytes
-Elevated CSF fluid protein
Guillain Barre:
Medical Diagnosis Gold Standard
Nerve Conduction Velocity will be abnormal
Lumbar Spinal Tap
Needle placed between the L3 and L4 spinous processes
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
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
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
Guillain Barre:
Immunoglobulin
Immunoglobulin replaces missing antibodies in patients with immune deficiencies and helps them fight diseases
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
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
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%
Physical Therapy Management Acute Care
Medical Hx (precluding infection)
Comorbidities
Medications
Social History
Environment
Language/Learning Style
Cognition
Integumentary
Special Considerations for GB
Skin Integrity
DVT
Autonomic Dysfunction:
Hypertensive events (Sweating, BP changes, Color change)
Aspiration:
Watch for Dysphagia
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
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
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
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
Items used in GB Treatment
Cardiac Chair
Compression STockings
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)
Transfers for GB
W/C Transfers
-Transfer Board
-Standing Pivot
-Dependent
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
Inpatient Rehabilitation for GB
3 hours per day
Consider Dysphagia (Language)
Strengthening Activities
Functional Rehabilitation
-Gait
-Transfers
-Physical Tolerance
-Postural Control
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!
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
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
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
