Guillain-Barre Syndrome

Question 1

what is GBS?

Answer 1

1. acute immune-mediated, inflammatory, demyelinating disorder with potential for chronic implications
   
   • immune system attacks Schwann cells in peripheral nervous system

Question 2

what is the most common form of GBS?

Answer 2

acute demyelinating inflammatory polyneuropathy (AIDP)

Question 3

how is GBS characterized?

Answer 3

rapidly progressing ascending motor weakness and diminished reflexes

• sensory, autonomic and brainstem abnormalities may occur

Question 4

T/F: GBS is the most common type of acute paralytic neuropathy?

Answer 4

TRUE

Question 5

describe the incidence of GBS

Answer 5

• 1-2 cases/100,000
• peaks in frequency in young adults and 5th through 8th decades
  
  • incidence rates increase with age, highest >60
  • increases by 20% for every 10-yr increase in age
• seasonal relationships associated with infections
• males > females

Question 6

describe the etiology of GBS

Answer 6

1. triggering cause can be idiopathic, but 50% of cases occur shortly after a microbial (viral or bacteria) infection
2. autoimmune disease
3. allergic response
4. other connections
   
   • HIV, herpes
   • vaccinations (very rare)
   • surgery

Question 7

describe the difference between bacterial and viral etiologies for GBS

Answer 7

1. Bacterial
   
   • ex → campylobacteriosis
   • found in undercooked food, especially poultry
2. Viral
   
   • 50-75% of pts have history of URI or GI infection within last month
   • usually precedes GBS dx by 1-4 weeks
   • Zika, Epstein-Barr, COVID-19

Question 8

typical progression of symptoms in GBS

Answer 8

1. progression of symptoms from 12hrs to 28 days before plateau is reached
   
   • plateau phase (nadir) for 2-4 weeks, then recovery proximal to distal

Question 9

typical clinical presentation for GBS

Answer 9

1. Motor weakness
2. Hyporeflexia or areflexia
3. Sensory impairments onset much later than motor
4. CN involvement common
   
   • CN 7 most often
   • CN 3, 4, 6, 9 and 10
5. ++Pain (neuropathic and MSK up to 90%)
6. Autonomic dysfunction
7. Respiratory difficulties

Question 10

describe motor weakness in GBS

Answer 10

1. rapidly progressive
2. symmetrical
3. distal → proximal
4. leg weakness before arm in 90% of cases

Question 11

describe CN involvement in GBS

Answer 11

1. CN 7 most frequently invovled
   
   • smiling
   • frowning
   • whistling
   • drinking through a straw
2. CN 3, 4, 6
   
   • double vision
3. CN 9 and 10
   
   • dysphagia
   • larygneal paralysis

Question 12

describe autonomic dysfunciton in GBS

Answer 12

occurs up to 70% of cases and in 20% can be severe

1. tachycardia
2. arrhythmias
3. OH
4. wide fluctations in BP

Question 13

how is GBS diagnosed?

Answer 13

1. Clinical Eval
2. CSF exam
3. Nerve conduction studies
4. MRI

Question 14

CSF exam findings in GBS

Answer 14

1. increased protein levels w/o pleocytosis
2. protein elevation noted in 90% of cases by 2nd week

Question 15

Nerve conduction studies findings in GBS

Answer 15

1. reduced amplitude or absent motor AP
2. decreased conduction velocity
3. increased temporal dispersion
4. latency prolongation of F wave
5. nerve conduction block (axonal GBS)

Question 16

MRI findings in GBS

Answer 16

1. enhancement and swelling/thickening of spinal nerve roots

Question 17

NINDS criteria for GBS (required features)

Answer 17

1. 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 4 limbs, trunk, bulbar and facial muscles, and external opthalmoplegia
2. Areflexia or decreased reflexes in weak limbs

Question 18

NINDS criteria for GBS (supportive features)

Answer 18

1. progressive of symptoms <4 weeks
   
   • ​80% reach nadir in 2 weeks
2. relative symmetry
3. mild sensory S/S
4. CN involvement, especially bilteral facial nerve weakness
5. recovery starting 2-4 weeks after progression halts
6. autonomic dysfunction
7. Pain
8. no fever at onset
9. elevated protein in CSF
10. electrodiagnosis abnormalities consistent with GBS

Question 19

The following features make a dx of GBS doubtful according to the NINDS

Answer 19

1. sensory loss >> motor loss
2. marked, persistent asymmetry of weakness
3. bowel and bladder dysfunction at onset
   
   • or severe and persistent bowel and bladder dysfunction
4. severe pulmonary dysfunction with little or no limb weakness at onset
5. fever at onset

Question 20

how is GBS medically managed?

Answer 20

1. NO CURE
2. management goals
   
   • control inflammatory response
3. Meds:
   
   • IVIg
   • Plasma exhange (plasmapheresis)

Question 21

what is IVIg treatment and what are it’s benefits?

Answer 21

1. IVIg
   
   • plasma product made of antibodies extracted from blood
   • hypothesized to block macrophage and antibody binding
   • blood product admin to boost antigen production
2. Benefits
   
   • been shown to make sig improvements for up to 50-75% of pts with GBS
     
     • thought to prevent further myelin loss and prevent axonal loss
   • can aid in sustained remission

Question 22

what is Plasmapheresis and what are it’s benefits?

Answer 22

1. 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 blood stream. Often performed with goal of removing antibodies in treating autoimmune conditions
   
   • typical treatment is 5 exchanges over a 2 week period
   • recommended when pts not able to walk 10 m w/o assistance
2. Benefits
   
   • associated with reduced nerve damage and faster clinical improvement

Question 23

Acute Phase of GBS

Answer 23

1. rapid progression of symptoms
2. symptoms peak (nadir) after 2-4 weeks
3. 50% reach max impairment within 1 week
   
   • 70% by 2 weeks
   • 80% by 3 weeks
   • 98% by 4 weeks

Question 24

Plateau Phase of GBS

Answer 24

1. Nadir
2. characterized by stability of symptoms
3. may last only days, but can last weeks or months

Question 25

Recovery phase of GBS

Answer 25

1. gradual improvement in symptoms 2. individual time frame 3. most pts show gradual recovery of muscle strength 2-4 weeks after plateau 4. sensory disturbances and fatigue can persist for years

Question 26

List some potential complications with GBS

Answer 26

1. respiratory impairment and failure 2. autonomic instability 3. pain 4. pneumonia 5. prolonged hospitalizations and immobility * DVT, skin breakdown, contracture 6. relapse if treatment inadequate

Question 27

GBS Prognosis

Answer 27

1. 80% recover ambulation within 6 months * 20% experience persistent disability * 50% may continue to experience minor neuro deficits 2. lingering symptoms may persist 3. long term morbidity and mortality is generally low 4. total recovery time can take up to 2 years 5. \*\*Relapses, though not common, CAN occur\*\*

Question 28

what types of lingering symptoms may persist in GBS?

Answer 28

1. paresthesias 2. distal muscle weakness (foot drop) 3. moderate to severe pain 4. extreme fatigue * persistent fatigue in 67% of patients

Question 29

negative prognostic indicators for GBS

Answer 29

1. older age at onset (\>60) 2. need for ventilatory support 3. rapid onset (less than 7 days) prior to admission 4. an average distal motor response amplitude reduction to \<20% of normal 5. history of GI illness (presence of diarrhea)

Question 30

IGOS subscales

Answer 30

International GBS Outcome Scale two subscales are EGRIS and EGOS

Question 31

what does the EGRIS help with?

Answer 31

helps determine the risk of developing respiratory failure in first week of admission 1. days between onset of weakness and hospital admisssion 2. facial or bulbar weakness at time of admission 3. UE/LE strength at time of admission

Question 32

what does the EGOS help with?

Answer 32

prognostic scoring system that 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) * 2 weeks: Erasmus GBS Outcome Score (EGOS)

Question 33

factors in the mEGOS

Answer 33

1. age at onset 2. preceding diarrhea in last 4 weeks 3. UE/LE strength at day 7 of admission

Question 34

factos in the EGOS

Answer 34

1. age at onset 2. preceding diarrhea in last 4 weeks 3. GBS disability score at 2 weeks after hospital admission (replaces UE/LE strength)

Question 35

GBS Specific Outcome Measures

Answer 35

1. GBS disability scale 2. Overall Disability Sum Score (ODSS)

Question 36

what is the GBS disability scale?

Answer 36

7 point scale rating level of global disability between: * 0 (healthy) * 3 (able to walk with a stick, appliance or support 5m across an open space * 6 (death)

Question 37

what is the ODSS

Answer 37

1. Includes UE and LE functional tasks scored on range of 0 (no signs of disability) to 12 (severe disability) 2. can be scored through interview or by individual 3. reliable, valid and responsive to change across spectrum of care 4. sig association with pt's own perception of clinical condition

Question 38

what is CIDP?

Answer 38

Chronic Inflammatory Demyelinating Polyneuropathy

Question 39

briefly describe CIDP

Answer 39

1. unknown etiology, less likely to be preceded by viral or infectious event 2. chronic course 3. gradual onset * \>8 weeks from onset before any signs of plateau * motor and sensory involvement * symmetric or asymmetric 4. Often does not improve after plateau occurs, despite med management * relapses are common 5. biospy reveals onion bulb changes 6. responds to glucocorticoids

Question 40

compare CIDP to GBS

Answer 40

1. CIDP * Onset → slow onset and progress for a longer period; may return in the future * Treament → often needs sustained trx (even with remission) 2. GBS * Onset → rapid onset that progresses quickly and stops progressing within 2-4 weeks * Treatment → once symptoms stabilize there is rarely any further deterioration