Guillain-Barre Syndrome Flashcards
what is GBS?
- acute immune-mediated, inflammatory, demyelinating disorder with potential for chronic implications
- immune system attacks Schwann cells in peripheral nervous system
what is the most common form of GBS?
acute demyelinating inflammatory polyneuropathy (AIDP)
how is GBS characterized?
ie. do you see rapid or slow progression?
motor weakness or sensory deficits?
What other key areas may be hit?
rapidly progressing ascending motor weakness and diminished reflexes
- sensory, autonomic and brainstem abnormalities may occur
T/F: GBS is the most common type of acute paralytic neuropathy?
TRUE
describe the incidence of GBS
is it more common in older or younger?
Male or female?
- 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
describe the etiology of GBS
- triggering cause can be idiopathic, but 50% of cases occur shortly after a microbial (viral or bacteria) infection
- autoimmune disease
- allergic response
- other connections
- HIV, herpes
- vaccinations (very rare)
- surgery
describe the difference between bacterial and viral etiologies for GBS
- Bacterial
- ex → campylobacteriosis
- found in undercooked food, especially poultry
- Viral
- 50-75% of pts have history of UTI or GI infection within last month
- usually precedes GBS dx by 1-4 weeks
- Zika, Epstein-Barr, COVID-19
typical progression of symptoms in GBS
how long do symptoms normally progress?
What happens after progression stops?
- progression of symptoms from 12hrs to 28 days before plateau is reached
- plateau phase (nadir) for 2-4 weeks, then recovery proximal to distal
typical clinical presentation for GBS (7)
- Motor weakness
- Hyporeflexia or areflexia
- Sensory impairments onset much later than motor
- CN involvement common
- CN 7 most often
- CN 3, 4, 6, 9 and 10
- ++Pain (neuropathic and MSK up to 90%)
- Autonomic dysfunction
- Respiratory difficulties
describe motor weakness in GBS
- rapidly progressive
- symmetrical
- distal → proximal
- leg weakness before arm in 90% of cases
describe CN involvement in GBS
- CN 7 most frequently invovled
- smiling
- frowning
- whistling
- drinking through a straw
- CN 3, 4, 6
- double vision
- CN 9 and 10
- dysphagia
- larygneal paralysis
describe autonomic dysfunciton in GBS (4)
occurs up to 70% of cases and in 20% can be severe
- tachycardia
- arrhythmias
- OH
- wide fluctations in BP
how is GBS diagnosed? (4)
- Clinical Eval
- CSF exam
- Nerve conduction studies
- MRI
CSF exam findings in GBS
- increased protein levels w/o pleocytosis
- protein elevation noted in 90% of cases by 2nd week
Nerve conduction studies findings inf GBS (5)
- reduced amplitude or absent motor AP
- decreased conduction velocity
- increased temporal dispersion
- latency prolongation of F wave
- nerve conduction block (axonal GBS)
MRI findings in GBS
- enhancement and swelling/thickening of spinal nerve roots
NINDS criteria for GBS (required features) (2)
- 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
- Areflexia or decreased reflexes in weak limbs
NINDS criteria for GBS (supportive features) (10)
- progressive of symptoms <4 weeks
- 80% reach nadir in 2 weeks
- relative symmetry
- mild sensory S/S
- CN involvement, especially bilteral facial nerve weakness
- recovery starting 2-4 weeks after progression halts
- autonomic dysfunction
- Pain
- no fever at onset
- elevated protein in CSF
- electrodiagnosis abnormalities consistent with GBS
The following features make a dx of GBS doubtful according to the NINDS (5)
- 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
how is GBS medically managed?
- NO CURE
- management goals
- control inflammatory response
- Meds:
- IVIg
- Plasma exhange (plasmapheresis)
what is IVIg treatment and what are it’s benefits?
- IVIg
- plasma product made of antibodies extracted from blood
- hypothesized to block macrophage and antibody binding
- blood product admin to boost antigen production
- 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
- been shown to make sig improvements for up to 50-75% of pts with GBS
what is Plasmapheresis and what are it’s benefits?
When is it reccomended? What is the usual dosage?
- 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
- Benefits
- associated with r_educed nerve damage and faster_ clinical improvement
Acute Phase of GBS
- 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
- 98% by 4 weeks
Plateau Phase of GBS
- Nadir
- characterized by stability of symptoms
- may last only days, but can last weeks or months
Recovery phase of GBS
- gradual improvement in symptoms
- individual time frame
- most pts show gradual recovery of muscle strength 2-4 weeks after plateau
- sensory disturbances and fatigue can persist for years
List some potential complications with GBS (6)
- respiratory impairment and failure
- autonomic instability
- pain
- pneumonia
- prolonged hospitalizations and immobility
- DVT, skin breakdown, contracture
- relapse if treatment inadequate
GBS Prognosis (5)
- 80% recover ambulation within 6 months
- 20% experience persistent disability
- 50% may continue to experience minor neuro deficits
- lingering symptoms may persist
- long term morbidity and mortality is generally low
- total recovery time can take up to 2 years
- **Relapses, though not common, CAN occur**
what types of lingering symptoms may persist in GBS? (4)
- paresthesias
- distal muscle weakness (foot drop)
- moderate to severe pain
- extreme fatigue
- persistent fatigue in 67% of patients
negative prognostic indicators for GBS (5)
- older age at onset (>60)
- need for ventilatory support
- rapid onset (less than 7 days) prior to admission
- an average distal motor response amplitude reduction to <20% of normal
- history of GI illness (presence of diarrhea)
IGOS subscales
International GBS Outcome Scale
two subscales are EGRIS and EGOS
what does the EGRIS help with?
what are the 3 things it looks at?
helps determine the risk of developing respiratory failure in first week of admission
- days between onset of weakness and hospital admisssion
- facial or bulbar weakness at time of admission
- UE/LE strength at time of admission
what does the EGOS help with?
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)
factors in the mEGOS (3)
- age at onset
- preceding diarrhea in last 4 weeks
- UE/LE strength at day 7 of admission
factors in the EGOS (3)
- age at onset
- preceding diarrhea in last 4 weeks
- GBS disability score at 2 weeks after hospital admission (replaces UE/LE strength)
GBS Specific Outcome Measures
- GBS disability scale
- Overall Disability Sum Score (ODSS)
what is the GBS disability scale?
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)
what is the ODSS (4)
- Includes UE and LE functional tasks scored on range of 0 (no signs of disability) to 12 (severe disability)
- can be scored through interview or by individual
- reliable, valid and responsive to change across spectrum of care
- sig association with pt’s own perception of clinical condition
what is CIDP?
Chronic Inflammatory Demyelinating Polyneuropathy
briefly describe CIDP
- 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 and sensory involvement
- symmetric or asymmetric
- Often does not improve after plateau occurs, despite med management
- relapses are common
- biospy reveals onion bulb changes
- responds to glucocorticoids
compare CIDP to GBS
- CIDP
- Onset → slow onset and progress for a longer period; may return in the future
- Treament → often needs sustained trx (even with remission)
- GBS
- Onset → rapid onset that progresses quickly and stops progressing within 2-4 weeks
- Treatment → once symptoms stabilize there is rarely any further deterioration
