Guillain-Barré Syndrome

Question 1

GBS is characterized by

Answer 1

rapidly progressive ascending motor weakness and diminished reflexes

Question 2

what is GBS

Answer 2

Acute immune-mediated, inflammatory, demyelinating disorder with potential for chronic implications

Immune system attacks Schwann cells in peripheral nervous system

Question 3

GBS is the most common type of

Answer 3

acute paralytic neuropathy

Question 4

causes of GBS

Answer 4

1. Bacterial etiology
2. Viral etiology

Other causes
-Autoimmune disease
-Allergic response
-Other connections
-HIV, herpes
-Vaccinations (very rare)
-Surgery or trauma
-Post-partum

Question 5

when does GBS peak in frequency

Answer 5

Peaks in frequency in young adults and 5th through 8th decades

more common in males

Question 6

GBS has multiple variant forms

Answer 6

Acute demyelinating inflammatory polyneuropathy (AIDP) is most common in

Question 7

timeline of GBS

Answer 7

Initially, rapid progression of symptoms distal to proximal from 12 hours to 28 days before plateau is reached

Plateau phase (“nadir”) for 2-4 weeks

Gradual recovery proximal to distal

Question 8

most cases of GBS are LMN dominant
presentation:

Answer 8

Motor weakness
- Rapidly progressive, symmetrical
- Distal –> proximal
- Leg weakness before arm in 90% of cases
Hyporeflexia or areflexia
Hypotonia

Question 9

CN involvement

Answer 9

CN VII is most frequently involved– Smiling, frowning, whistling, or drinking through a straw
CN III, IV, VI– Double vision
CN IX and X– Dysphagia and laryngeal paralysis

Question 10

second most common clinical presentation of GBS

Answer 10

PAIN

Question 11

most common clinical exam dx findings

Answer 11

elevated proteins

or lumbar puncture

Question 12

required features of GBS

Answer 12

Progressive, symmetrical weakness of the legs and arms (sometimes initially only in the legs)
–> Ranging from minimal weakness of the legs to total paralysis of all four limbs, the trunk, bulbar and facial muscles, and external ophthalmoplegia

Areflexia/hyporeflexia in involved limbs

Question 13

supportive features of GBS

Answer 13

Progression of symptoms <4 weeks (80% reach nadir in 2 wks)
Relative symmetry
Mild sensory symptoms or signs
Cranial nerve involvement, especially bilateral facial nerve weakness
Recovery starting 2-4 wks after progression halts
Autonomic dysfunction
Pain
No fever at the onset
Elevated protein in CSF
Electrodiagnostic abnormalities consistent with GBS

Question 14

symptoms you should not see with GBS

Answer 14

Sensory loss > motor loss
Marked, persistent asymmetry of weakness
Bowel and bladder dysfunction at onset
Or, severe and persistent bowel and bladder dysfunction
Severe pulmonary dysfunction with little or no limb weakness at onset
Fever at onset

Question 15

clinical variants

Answer 15

AIDP
miller-fisher syndrome
AMAN
AMSAN

Question 16

AIDP

Answer 16

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

Question 17

miller fisher syndrome

Answer 17

Ophthalmoplegia, ataxia, areflexia
25% develop extremity weakness

Question 18

AMAN

Answer 18

Axonal involvement
Muscle weakness with occasional preservation of DTRs. Sensory spared.

Question 19

AMSAN

Answer 19

Axonal involvement
Motor + sensory nerves impacted

Question 20

Intravenous immunoglobulin (IVIg)

Answer 20

Plasma products made of antibodies extracted from blood
Hypothesized the block macrophage and antibody binding.
Blood products administered to boost antigen production

Question 21

Intravenous immunoglobulin (IVIg)
benefits

Answer 21

Has been shown to make significant improvements for up to 50-75% of patients with GBS
Thought to prevent further myelin loss and prevent axonal loss
Can aid in sustained remission

Question 22

Plasma Exchange (Plasmapheresis)

Answer 22

A method of removing blood plasma from the body by withdrawing blood, separating it into plasma and cells, and transfusing the cells back into the bloodstream.
Typical treatment is 5 exchanges over a 2-week period.
Recommended when patients are not able to walk 10 meters w/o assistance

Question 23

Plasma Exchange (Plasmapheresis)
Benefits

Answer 23

Associated with reduced nerve damage and faster clinical improvement.

Question 24

GBS medical management

Answer 24

NO CURE
control inflammatory response

Question 25

acute phase of GBS

Answer 25

Rapid progression of symptoms Symptoms peak (nadir) after 2-4 weeks 50% reach max impairment within 1 week, 70% by 2 weeks, 80% by 3 weeks, and 98% by four weeks

Question 26

plateau phase of GBS

Answer 26

“Nadir” Characterized by stability of symptoms May last only days, up to about a month

Question 27

recovery phase of GBS

Answer 27

Gradual improvement in symptoms. Individual time frame Most patients show gradual recovery of muscle strength 2-4 weeks after plateau Sensory disturbance and fatigue can persist for years

Question 28

potential complications

Answer 28

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

Question 29

prognosis

Answer 29

80% recover ambulation within 6 months 20% experience persistent disability 50% may continue to experience minor neurological deficits lingering symptoms may persist long term morbidity and mortality is generally low

Question 30

total recovery time for GBS can take up to

Answer 30

2 years

Question 31

negative prognostic indicators

Answer 31

older age at onset >60 need for ventilatory support rapid onset prior to admission (less than 7 days) An average distal motor response amplitude reduction on electrodiagnostic testing to <20% of normal History of GI illness prior to onset (presence of diarrhea)

Question 32

Erasmus GBS Respiratory Insufficiency Score (EGRIS): what is it and what 3 things does it look at

Answer 32

Risk of developing respiratory failure in first week of admission Days between onset of weakness and hospital admission Facial or bulbar weakness at time of admission UE/LE strength at time of admission

Question 33

Erasmus GBS Outcome Score (EGOS): what is it and what is the difference between administration at 1 week and 2 weeks

Answer 33

Prognostic scoring system can be used at 1 and 2 weeks after admission to estimate ability to walk at 6 months 1 week: Modified Erasmus GBS Outcome Score (mEGOS) Age at onset - Preceding diarrhea in last 4 weeks - UE/LE strength at day 7 of admission 2 weeks: Erasmus GBS Outcome Score (EGOS) - Age at onset - Preceding diarrhea in last 4 weeks - GBS disability score at 2 weeks after hospital admission (replaces UE/LE strength)

Question 34

GBS disability scale

Answer 34

7 point scale rating level of global disability ranks 0-6 (alive to dead)

Question 35

ODSS-- overall disability sum score

Answer 35

includes UE and LE functional tasks scored on range of 0 to 12 can be scored through interview or by individual reliable, valid and responsive to change across spectrum of care significant association with patients own perception of clinical condition

Question 36

Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)

Answer 36

Unknown etiology, less likely to be preceded by viral or infectious event Chronic course Gradual Onset -- >8 weeks from onset before any signs of plateau -- Motor & sensory involvement -- Symmetric or asymmetric Often does not improve after plateau occurs, despite medical management -- Relapses are common Biopsy reveals onion bulb changes Responds to glucocorticoids