Week 7- Guillain-Barre Syndrome Flashcards
PART 1: INTRO AND CLINICAL PRESENTATION
PART 1: INTRO AND CLINICAL PRESENTATION
What is Guillain-Barre Syndrome?
-Acute immuno-mediated, inflammatory, demyelinating disorder with potential for chronic implications.
Immune system attacks _______ cells in PNS.
-Schwann
Guillain-Barre has multiple variant forms, what is the most common in US/EU?
-Acute Inflammatory Demyelinating Polyneuropathy (AIDP)
What is Guillain-Barre characterized by? (2)
- Rapidly progressive motor weakness
- Diminished reflexes
Guillain-Barre is the most common type of Acute Paralytic _________.
-Acute Paralytic Neuropathy
Incidence:
- Peaks in frequency in young adults and ___-____ decades.
- Seasonal relationship associated with _________.
- Does it affect males or females more?
- 5th-8th
- infections
- Males > Females
- What is the cause of Guillain-Barre?
- What is the most common?
- Triggering can be idiopathic, but 50% of cases occur shortly after a microbial (viral or bacterial) infection.
- Viral
GBS Clinical Presentation:
- Progression of symptoms from _____ to ______ before plateau is reached. (Plateau phase (“nadir”) for 2-4 weeks, then recovery from ________ to _________).
- What are the 3 ways motor weakness will present itself in patients with Guillain-Barre?
- _____reflexia
- _____ symptoms onset much later than motor impairments.
- 12 hours to 28 days (recovery from proximal to distal)
- RAPIDLY progressive and symmetrical, Distal → Proximal, leg weakness before arm weakness in 90% of cases
- Hyporeflexia/areflexia
- sensory symptoms (paresthesia and hyperesthesia)
GBS Clinical Presentation:
- _______ ______ involvement is common (45-75%).
- Which is the most frequently involved? What are some others?
- ++____ ( neuropathic and MSK up to 90% reported)
- _______ dysfunction (up to 70% of cases, 20% can be severe)
- _________ difficulties (15-30%)
- Cranial Nerve involvement (45-75%)
- CN VII most frequently involved (smiling, frowning, whistling, or drinking through straw). CN III, IV, and VI (double vision) and CN IX and X (dysphagia and laryngeal paralysis)
- ++Pain (90%)
- Autonomic dysfunction
- Respiratory difficulties
With regards to clinical presentation, what is the first complaint that most patients will report?
-Foot drag
- How does CN VII involvement present?
- How does CN III, IV, and VI present?
- How does CN IX and X present?
- CN VII = smiling, frowning, whistling, drinking through straw
- CN III, IV, and VI = double vision
- CN IX and X = dysphagia and laryngeal paralysis
Diagnosis of Guillain-Barre involves clinical evaluation plus what (3) things?
- CSF examination
- Nerve Conduction studies
- MRI
What does a CSF examination look for?
-Increased protein levels (noted in 90% of cases by 2nd week).
What is a Nerve Conduction study looking for?
- Reduced amplitude or absent distal motor AP
- Decreased conduction velocity
- Nerve conduction block
What is an MRI looking for?
-Enhancement and swelling/thickening of spinal nerve roots.
National Institute of Neurological Disorders and Strokes (NINDS) Required Features for GBS? (2)
- Progressive, symmetrical weakness of the legs and arms (sometimes only initially in the legs)
- Areflexia or decreased reflexes in weak limbs
NINDS Supportive Features for GBS? (6)
- <4 weeks
- mild sensory S/Sx
- CN
- 2-4 weeks
- Autonomic dysfunction
- Elevated protein in CSF
NINDS Features That Make Diagnosis Doubtful? (5)
- sensory loss > motor loss
- persistent asymmetry of weakness
- B&B dysfunction at onset
- severe autonomic dysfunction with little/no limb weakness
- fever at onset
What are some other clinical variants of GBS? (5)
- Miller-Fisher Syndrome
- AMAN
- AMSAN
- Bickerstaff encephalitis
- Pharyngeal-cervical-brachial
With AIDP, do we see axonal damage?
No, we mostly knock out myelin.
PART 2: MEDICAL MANAGEMENT, CLINICAL COURSE, PROGNOSIS
PART 2: MEDICAL MANAGEMENT, CLINICAL COURSE, PROGNOSIS
Is there a cure for GBS?
-No
- What are the management goals of treating GBS?
- What do we use for this?
- Control inflammatory response.
- IVIg and Plasmapheresis
IVIg:
- Plasma product made of _________.
- Hypothesized to do what?
- antibodies
- block macrophage and antibody binding
IVIg Benefits:
- Has been shown to make significant improvements for up to 50-75% of patients with GBS. Thought to prevent further _______ loss and ______ loss.
- Can aid in sustained _________.
- prevent further myelin and axonal loss
- sustained remission
Plasmapheresis:
- 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 the goal of removing __________.
- Typical treatment is __ exchanges over a __-week period.
- Recommended when patients not able to walk ____m w/o assistance.
- antibodies
- 5 exchanges over a 2-week period
- not able to walk 10m w/o assistance
Plasmapheresis Benefits:
-Associated with reduced _____ damage and faster clinical improvement.
-reduced nerve damage
IVIg and Plasmapheresis should be given in what timeframe?
-2-4 weeks after
What are the (3) phases of GBS?
- Acute Phase
- Plateau Phase
- Recovery Phase
Acute Phase:
- ______ progression of symptoms which peak after __-__ weeks.
- ___% reach max impairment with 1 week, 70% by 2 weeks, 80% by 3 weeks, and 98% by 4 weeks.
- Rapid progression peaking after 2-4 weeks
- 50%
Plateau Phase:
- “______”
- Characterized by _______ of symptoms.
- May last only days but can last weeks or months.
- “Nadir”
- stability
Recovery Phase:
- Gradual improvement in symptoms.
- Most patients show gradual recovery of muscle strength __-__ weeks after plateau.
- Sensory disturbance and fatigue can persist for ________.
- 2-4 weeks after plateau
- years
Plateau = ________
-Nadir
How is the recovery phase able to occur?
-Myelin can be reproduced in PNS.
List some potential complications of GBS. (6)
- Respiratory Impairment/Failure
- Autonomic Instability
- Pain
- Pneumonia
- Prolonged Hospitalizations and Immobility (DVT, skin breakdown, contracture)
- Relapse if treatment inadequate
What is the “good” thing to note about relapses?
- More likely that you don’t relapse, and if it does happen they aren’t as bad.
- Occur in only 10% of patients.
GBS Prognosis:
- ___% recover ambulation within 6 months (20% may experience persistent disability, 50% may continue to experience motor neurological deficits).
- Lingering symptoms may persist (persistent fatigue in __% of patients).
- Long term morbidity and mortality is generally _____.
- Total recovery time can take _______.
- 80% recover ambulation within 6 months
- Persistent fatigue in 67% of patients
- long-term morbidity generally low
- up to 2 years
What are the negative prognostic indicators for GBS? (5)
- Older age onset (>60)
- Need for vent support
- Rapid onset (<7 days) prior to admission
- An average distal motor response amplitude reduction <20% of normal
- History of GI illness (presence of diarrhea)
List some GBS Outcome Measures. (3)
- International GBS Outcome Scale (IGOS)
- GBS Disability Scale
- Overall Disability Sum Score (ODSS)
What are the 2 subcomponents to the IGOS?
- EGRIS
- EGOS
EGRIS:
- Risk of developing ________ failure in first week of admission.
- What 3 things does it look at, that are going to lead to increase risk?
-Respiratory failure
- ) Days between onset of weakness and hospital admission.
- ) Facial or bulbar weakness at time of admission.
- ) UE/LE strength at time of admission.
EGOS:
- Prognostic scoring system can be used at 1 and 2 weeks after admission to estimate ability to _____ at 6 months.
- 1 week = _________________
- 2 weeks = _________________
- Estimate ability to walk at 6 months
- 1 week = Modified Erasmus GBS Outcome Score (mEGOS)
- 2 week = Erasmus GBS Outcome Score (EGOS)
What are the 3 things mEGOS looks at?
- ) Age of onset
- ) Preceding diarrhea in last 4 weeks
- ) UE/LE strength at day 7 of admission
What are the 3 things EGOS looks at?
- ) Age of onset
- ) Preceding diarrhea in last 4 weeks
- ) GBS disability score at 2 weeks after hospital admission
__/__ patients with GBS will require ventilatory support.
-1/4
GBS Disability Scale is a 7 point scale rating level of ________ disability.
-global disability
\List the GBS Disability Scale (0-6).
- ) A healthy state.
- ) Minor symptoms and capable of running.
- ) Able to walk 10m or more without assistance but unable to run.
- ) Able to walk 10m across an open space with help
- ) Bedridden or chair bound
- ) Requiring assisted ventilation for at least part of the day.
- ) Dead
Overall Disability Sum Score (ODSS) includes ___ and ____ functional tasks scored from 0 (no signs of disability) to 12 (severe disability) and can be scored through interview or by individual.
-UE and LE functional tasks
What is a good thing about the ODSS?
-You get to see what the patient’s perceived disability is. (get into their head)
What is CIDP (Chronic Inflammatory Demyelinating Polyneuropathy)?
-Much more chronic presentation of GBS.
What is the difference between GBS and CIDP?
GBS presents much more acutely, and reaches its most severe state in less than 4 weeks. CIDP presents more slowly and reaches its more severe state typically in over 8 weeks. Because of this, GBS is considered a classic acute autoimmune neuropathy while CIDP is a classic chronic autoimmune neuropathy.
CIDP:
- ________ onset (>__ weeks from onset before any signs of plateau)
- Motor or sensory involvement?
- Symmetric or asymmetric?
- Do we see recovery with this? Do they have relapses?
- Biopsy reveals __________ changes.
- Responds to ____________.
- gradual onset (>8 weeks from onset before any signs of plateau)
- motor and sensory involvement
- symmetric or asymmetric
- Not as likely to see recovery, and if we do it is not to the extent of AIDP. Will have relapses.
- onion bulb changes
- Responds to glucocorticoids.
