Guillain-Barré Syndrome

Question 1

What is the pathophysiology behind GBS?

Answer 1

Immune system attacks Schwann cells in PNS

Question 2

What populations does this diagnosis
demonstrate incidence trends towards?

Answer 2

• Young adults, 50-80s (*Highest in those >60, 20% increase for every 10-year increase in age)
• MALES > females

Question 3

Describe the etiology of GBS.

Answer 3

• Idiopathic vs viral/bacteria
• Autoimmune
• Allergic response
• HIV/herpes
• Vaccinations
• Surgery/trauma
• Post-partum

Question 4

Bacterial etiology of GBS is often associated with:
- Epstein Barr
- Upper respiratory infections
- GI infections
- Undercooked food

Answer 4

Undercooked food

Question 5

T/F viral etiologies of GBS co-incide (simultaneous occurance) with GBS symptoms

Answer 5

False, virual infection preceeds GBS by 1-4 weeks

Question 6

Explain the typical clinical presentation of GBS.

Answer 6

Initially rapid progression of symptoms distal to proximal, 12-28 hours > plateau (“nadir”) for 2-4 weeks > gradual recovery proximal to distal

Question 7

What are common signs and symptoms associated with this diagnosis?

Answer 7

• 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)

Question 8

How is GBS diagnosed?

Answer 8

• 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)
• MRI (RO): enhancement/swelling of spinal n. root

Question 9

Required Featuers of GBS:

NINDS Criteria

Answer 9

• Progressive, symmetrical weakness of LE + UE (minimal to total paralysis)
• Areflexia/hyporeflexia

Question 10

Supportive Features of GBS

NINDS Criteria

Answer 10

• 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

Question 11

Doubtful Features of GBS Dx

NINDS Criteria

Answer 11

• 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

Question 12

Which GBS clinical variant am I?

• Progressive, symmetrical muscle weakness, absent or depressed DTRs
• Often with preceding illness

Answer 12

Acute Inflammatory Demyelinating Polyradiculoneuropathy (AIDP)

Question 13

Which GBS clinical variant am I?

• Ophthalmoplegia
• Ataxia
• Areflexia
  (Especially of face)
• 25% develop extremity weakness

Answer 13

Miller-Fisher Syndrome

Question 14

Which GBS clinical variant am I?

• Axonal involvement
• Muscle weakness
• Occasional preservation of DTRs
• Sensory spared.

Answer 14

Acute Motor Axonal Neuropathy

Question 15

Which GBS clinical variant am I?

• Axonal involvement
• Motor + sensory impacted

Answer 15

Acute Motor-Sensory Axonal Neuropathy

Question 16

What is the goal of medical management of GBS?

Answer 16

Control the inflammatory response

Question 17

What are the major interventions for GBS in the acute setting?

Answer 17

IV Immunoglobulin (IVIg)
Plasma Exchange

Question 18

How does Intravenous Immunoglobulin (IVIg) work?

Answer 18

Plasma products made of antibodies extracted from blood given to patient. Thought to block macrophage/antibody binding.

Question 19

How does Plasma exchange (plasmapheresis) work?

Answer 19

Blood removed and separated into plasma and cells - cells transferred back into bloodstream.

Typical treatment 5 exchanges/2-week period

Question 20

When is plasma exchange reccommended?

Answer 20

If a pt is unable to walk 10 meters w/o assistance

Question 21

What are the benefits of Intravenous Immunoglobulin (IVIg)?

Answer 21

• Thought to prevent myelin loss and axonal damage = significant improvements
• Aid in sustained remission

Question 22

What are the benefits of Plasma exchange (plasmapheresis)?

Answer 22

• Reduced n. damage
• faster clinical improvement

Question 23

Describe the Acute Phase of the clinical course of GBS

Answer 23

• 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.

Question 24

Describe the Plateau Phase of the clinical course of GBS

Answer 24

• Stability of symptoms
• Days - 1 Month

Question 25

Describe the Recovery Phase of the clinical course of GBS

Answer 25

- Gradual improvement in symptoms (indv. time frame) - Most 2-4 weeks after plateau - Sensory disturbance and fatigue can persist for years

Question 26

What are some potential complications in the acute phases of GBS?

Answer 26

- Respiratory impairment and failure - Autonomic instability - Pain - Pneumonia - Prolonged hospitalization and immobility (DVT, skin breakdown, contracture) - Relapse if treatment inadequate

Question 27

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

Answer 27

c. 20% of patients experience persistent disability

Question 28

What are common lingering symptoms with GBS?

Answer 28

- Paresthesias, distal muscle weakness (foot drop) - Mod - severe pain - Extreme fatigue (67% of patients) - Minor neurological deficits - 2% progress to CIDP

Question 29

What factors increase the risk of long term morbidity and mortality?

Answer 29

- Prolonged course with months of ventilatory support/incomplete recovery - Pulmonary/cardiac complications - Organ failure

Question 30

What does the EGRIS seek to determine?

Answer 30

Risk of developing respiratory failure in 1st week of admission.

Question 31

Negative Prognostic Indicators for GBS

Answer 31

- 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)

Question 32

What patient characteristics does the EGRIS include in it's assessment?

Answer 32

- 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!!**

Question 33

What does the EGOS seek to determine?

Answer 33

ability to walk at 6 months

Question 34

What patient characteristics does the EGOS include in it's assessment?

Answer 34

- 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

Question 35

What does the GBS Distability Scale Measure?

Answer 35

Global Disability (health to death, mobility status, assisted vent)

Question 36

What does the Overall Disability Sum Score assess?

Answer 36

- UE/LE functional tasks (no to severe disability score) - Significant association with patient's own perception of clinical condition

Question 37

How does Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) differ from GBS?

Answer 37

- 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)