Guillain-Barré Syndrome Flashcards
General characteristics, background, clinical presentation, diagnosis, clinical variants, clinical course and potential complications, identify prognostic considerations, outcome measures, CIDP
What is the pathophysiology behind GBS?
Immune system attacks Schwann cells in PNS
What populations does this diagnosis
demonstrate incidence trends towards?
- Young adults, 50-80s (*Highest in those >60, 20% increase for every 10-year increase in age)
- MALES > females
Describe the etiology of GBS.
- Idiopathic vs viral/bacteria
- Autoimmune
- Allergic response
- HIV/herpes
- Vaccinations
- Surgery/trauma
- Post-partum
Bacterial etiology of GBS is often associated with:
- Epstein Barr
- Upper respiratory infections
- GI infections
- Undercooked food
Undercooked food
T/F viral etiologies of GBS co-incide (simultaneous occurance) with GBS symptoms
False, virual infection preceeds GBS by 1-4 weeks
Explain the typical clinical presentation of GBS.
Initially rapid progression of symptoms distal to proximal, 12-28 hours > plateau (“nadir”) for 2-4 weeks > gradual recovery proximal to distal
What are common signs and symptoms associated with this diagnosis?
- Weakness (rapid, progressive, symmetrical, distal to proximal, LE before UE 90% of time)
- Hyporeflexia/areflexia
- Hypotonia
- Facciculations
- CN involvement: CN VII, CN III, IV, VI, CN IX, X
- PAIN
- Autonomic dysfunction
- Respiratory difficulties
- Sensory symptoms (late)
How is GBS diagnosed?
- CSF Examination: increased protein levels
- NCV:
- reduced amplitude/absent distal motor AP
- ↓ conduction velocity
- ↑ temporal dispersion
- Latency prolongation of F-wave
- Nerve conduction block (axonal GBS)
- reduced amplitude/absent distal motor AP
- MRI (RO): enhancement/swelling of spinal n. root
Required Featuers of GBS:
NINDS Criteria
- Progressive, symmetrical weakness of LE + UE (minimal to total paralysis)
- Areflexia/hyporeflexia
Supportive Features of GBS
NINDS Criteria
- Progression of symptoms <4 weeks (80% reach nadir in 2 wks)
- Relative symmetry
- Mild sensory S&S
- Cranial nerve involvement (esp bilateral facial n. weakness)
- Recovery starts 2-4 weeks after progression halts
- Autonomic dysfunction
- Pain
- No fever at onset
- Elevated protein in CSF
- Electrodiagnostic abnormalities consistent with GBS
Doubtful Features of GBS Dx
NINDS Criteria
- Sensory > motor loss
- Marked, persistent asymmetry of weakness
- Bowel and bladder dysfunction at onset (or persistent)
- Severe pulmonary dysfunction with little to no limb weakness at onset
- Fever at onset
Which GBS clinical variant am I?
- Progressive, symmetrical muscle weakness, absent or depressed DTRs
- Often with preceding illness
Acute Inflammatory Demyelinating Polyradiculoneuropathy (AIDP)
Which GBS clinical variant am I?
- Ophthalmoplegia
- Ataxia
- Areflexia
(Especially of face) - 25% develop extremity weakness
Miller-Fisher Syndrome
Which GBS clinical variant am I?
- Axonal involvement
- Muscle weakness
- Occasional preservation of DTRs
- Sensory spared.
Acute Motor Axonal Neuropathy
Which GBS clinical variant am I?
- Axonal involvement
- Motor + sensory impacted
Acute Motor-Sensory Axonal Neuropathy
What is the goal of medical management of GBS?
Control the inflammatory response
What are the major interventions for GBS in the acute setting?
IV Immunoglobulin (IVIg)
Plasma Exchange
How does Intravenous Immunoglobulin (IVIg) work?
Plasma products made of antibodies extracted from blood given to patient. Thought to block macrophage/antibody binding.
How does Plasma exchange (plasmapheresis) work?
Blood removed and separated into plasma and cells - cells transferred back into bloodstream.
Typical treatment 5 exchanges/2-week period
When is plasma exchange reccommended?
If a pt is unable to walk 10 meters w/o assistance
What are the benefits of Intravenous Immunoglobulin (IVIg)?
- Thought to prevent myelin loss and axonal damage = significant improvements
- Aid in sustained remission
What are the benefits of Plasma exchange (plasmapheresis)?
- Reduced n. damage
- faster clinical improvement
Describe the Acute Phase of the clinical course of GBS
- Rapid progression of symptoms that peak after 2-4 weeks
- 50% reach max impairment w/in 1 week.
- 70% reach max impairment w/in 2 weeks.
- 80% reach max impairment w/in 3 weeks.
- 98% reach max impairment w/in 4 weeks.
Describe the Plateau Phase of the clinical course of GBS
- Stability of symptoms
- Days - 1 Month
Describe the Recovery Phase of the clinical course of GBS
- Gradual improvement in symptoms (indv. time frame)
- Most 2-4 weeks after plateau
- Sensory disturbance and fatigue can persist for years
What are some potential complications in the acute phases of GBS?
- Respiratory impairment and failure
- Autonomic instability
- Pain
- Pneumonia
- Prolonged hospitalization and immobility (DVT, skin breakdown, contracture)
- Relapse if treatment inadequate
Which of the following is true regarding total recovery time from GBS:
a. Increased risk of relapses with subsequent infections.
b. Moderate to severe sensory impairments are common
c. 20% of patients experience persistent disability
d. 80% recover previous level of ambulation status by 6 month
c. 20% of patients experience persistent disability
What are common lingering symptoms with GBS?
- Paresthesias, distal muscle weakness (foot drop)
- Mod - severe pain
- Extreme fatigue (67% of patients)
- Minor neurological deficits
- 2% progress to CIDP
What factors increase the risk of long term morbidity and mortality?
- Prolonged course with months of ventilatory support/incomplete recovery
- Pulmonary/cardiac complications
- Organ failure
What does the EGRIS seek to determine?
Risk of developing respiratory failure in 1st week of admission.
Negative Prognostic Indicators for GBS
- Older age at onset (>60)
- Need for ventilatory support
- Rapid onset (<7 days) prior to admission
- Avg. distal motor response amplitude reduction on electrodiagnostic testing to <20% normal
- Hx of GI illness prior to onset (presence of diarrhea)
What patient characteristics does the EGRIS include in it’s assessment?
- Days between onset of weakness and hospital admission
- Facial or bulbar weakness at time of admission
- UE/LE strength at time of admission
1 in 3 pt’s requires ventilatory support!!
What does the EGOS seek to determine?
ability to walk at 6 months
What patient characteristics does the EGOS include in it’s assessment?
- Age at onset
- Preceding diarrhea in last 4 weeks
- Modified (1 week): UE/LE strength at day 7 of admission
- Original (2 week): GBS disability score at 2 weeks after hospital admission
What does the GBS Distability Scale Measure?
Global Disability (health to death, mobility status, assisted vent)
What does the Overall Disability Sum Score assess?
- UE/LE functional tasks (no to severe disability score)
- Significant association with patient’s own perception of clinical condition
How does Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) differ from GBS?
- Unknown etiology
- Chronic
- Gradual onset
- > 8 weeks from onset before plateau signs
- Motor AND sensory involvement
- Symmetric or asymmetric
- No improvement after plateau occurs
- Onion bulb changes
- Responds to glucocorticoids (minimal)
