Transcript Guillain-Barré Syndrome

Definition
 Group of neuropathic conditions characterized by
progressive weakness & dimished or absent reflexes.
 Inflammatory neuropathy due to cross reactivity
between neural antigens & antibodies induced by
specific infections
 Campylobacter (most common) but also reported
following: Mycoplasma Pneumoniae, Hemophilus
Influenza, CMV, EBV
Epidemiology
 Estimated annual incidence in the United States is 1.65
to 1.79 per 100,000 persons
 Incidence increases from 0.62 per 100,000 in those <9
years old to 2.66 per 100,000 in those 80-89 years old
 Male-to-female ratio is 3:2
Preceding illness
 Most common symptoms reported before onset of
Guillain-Barré Syndrome are: fever, cough, sore throat
and other upper respiratory symptoms
 Infection with Epstein-Barr virus has been linked to
milder forms of Guillain-Barré Syndrome
 GI symptoms may be more likely to precede GuillainBarré subtypes that are related to slower recovery and
higher risk of residual disability
Guillain-Barré Syndrome
Subtypes
 Acute inflammatory demyelinating polyradiculopathy
 Multifocal peripheral demyelination, slow remyelination, both
humoral and cellular immune mechanisms
 Most common subtype (90% of Guillain-Barré Syndrome in
the US)
 Acute motor axonal neuropathy
 Antibodies against gangliosides GM1, GD1a, GalNAc-GD1a
and GD1b in peripheral motor nerve axons
 5-10% of GBS cases
 Only motor symptoms
 Acute motor-sensory axonal neuropathy
 Similar to acute motor axonal neuropathy but with sensory
axonal degeneration, predominantly sensory involvement
Guillain-Barré Syndrome
Subtypes
 Miller Fisher syndrome
 Demyelination, immunoglobulin G antibodies against
gangliosides GQ1b, GD3 and GT1a
 Rare, 3% of GBS cases in US
 Bilateral opthalmoplegia, ataxia, areflexia, facial & bulbar
weakness in 50% of cases
 Trunk, extremity weakness occurs in 50% of cases
 Acute autonomic neuropathy
 Mechanism is unclear
 Autonomic symptoms, sensory loss
 Recovery is slow and may be incomplete
Presentation
 Presentation:
 Symmetrical weakness (ascending)
 Decreased or absent reflexes
 Also commonly seen:
 Weakness, numbness, tingling & pain in the limbs
 25% of patients will have advancing weakness that
compromise the respiratory muscles- will require
mechanical ventilation
Presentation
 Respiratory failure is more common in patients with rapid
progression of symptoms, upper limb weakness, autonomic
dysfunction or bulbar palsy
 Facial, oropharyngeal & oculomotor muscles may be affected
because of cranial neuropathy
 Autonomic symptoms include arrythmias, orthostasis, BP
instability, urinary retention, decreased GI motility
 Pain is reported in 50-89% of Guillain-Barré Syndrome patients
 Pain is severe, deep, aching or cramping in muscles or back
 Difficult to control because pain is nociceptive and/or
neuropathic
Disease progression
 Symptoms typically peak within 4 weeks, then plateau
before resolving
Diagnosis
 Clinical criteria for diagnosis include:
 Symmetric motor weakness (bilateral symptoms)
 Absent or decreased reflexes
 Lumbar puncture:
 Elevated protein in CSF, normal WBC count
 Protein level may be normal in the 1st week of symptoms, protein
will be elevated in 90% of cases by then end of the 2nd week
 Nerve conduction studies:
 Slowed conduction (<60% normal velocity) or blockage of nerve
conduction will be seen
 Must test at least 3 motor nerves & 3 sensory nerves, must avoid
sural nerve (often normal in GBS)
 Can be used to track progression of the illnes
Differential Diagnosis
 Brainstem: Infection, stroke
 Spinal cord: compression, myelopathy, poliomyelitis,
transverse myelitis
 Rhabdomyolysis
 Myasthenia gravis
 Toxicity: industrial chemicals and other toxins
 Infectious, inflammatory or toxic myopathy
 Lyme disease
Complications
 Neuropathic pain
 Autonomic dysfunction
 Hypotension, hypertension, arrythmias, bladder and
bowel dysfuntion
 Increased risk of VTE
 Bulbar dysfunction & swallow difficulty, risk of
aspiration
Complications
 Respiratory failure
 Close respiratory monitoring with frequent
measurement of negative inspiratory force (NIF) should
be instituted initially on all all Guillain-Barré Syndrome
patients
 NIF is a noninvasive method to measure respiratory
muscle strength
 Acceptable NIF range in Guillain-Barré Syndrome is 20 to -40 at least
 Normal NIF is more negative than -60
Complications
Complications
Predicts the need for mechanical ventilation
 Bulbar symptoms
 Inability to raise the head or flex the arms
 Inadequate cough
 Maximum expiratory pressure: < 40 cm H2O
 Maximum inspiratory pressure: < 30 cm H2O
 Vital capacity: < 60 percent of predicted or < 20 mL per kg
 Vital capacity, maximum inspiratory pressure, or maximum
expiratory pressure reduced by at least 30 percent
Treatment
 Disease modifying therapy:
 IVIg- 400mg/kg/day x 5days OR 2g/kg/day x 2days
 Plasma exchange (Plasmapheresis) – optimal response if
performed within 7 days onset
 Both IVIg and plasma exchange are equally effective
according to the literature but combining them is not
beneficial
 Mild Guillain-Barré syndrome cases benefit from 2 sessions
of plasma exchange
 Severe disease often requires 4 sessions
 Corticosteroids are not recommended and may in fact delay
long term recovery
Treatment
 Initial response to IVIg does not predict the outcome
because patients may stabilize or continue to decline
after therapy
Treatment
 Supportive care
 SQ anticoagulation & SCD’s to reduce risk of VTE
 Swallow eval in patients with facial or oropharyngeal
weakness- risk of aspiration
 ICU monitoring- ANS dysfunction and risk of
respiratory failure, patient can become very unstable
very quickly
Treatment
 Bladder & bowel care
 Foley catheter, enemas & laxatives, erythromycin for treatment
of ileus
 ANS dysfunction
 Paroxysmal HTN (24%), Orthostatic hypotension (19%),
sustained HTN (3%)
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Severe HTN- use PRN Labetalol, Esmolol, Nitroprusside
Hypotension- IV fluid boluses 1st, then low dose phenylephrine
 Arrythmias
 Sustained sinus tachycardia (37%)- requires no treatment
 Bradycardia & asystole (4%)
Treatment
 Patients with limited mobility should be closely
monitored for skin breakdown and treated
appropriately
 Pain control- neuropathic pain in 40-50% of patients
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Gabapentin (Neurontin)
Carbamazepine (Tegretol)
NSAIDs
TCA’s
Tramadol
Epidural morphine
Treatment
 Physical therapy and rehabilitation is recommended to
decrease residual deficits and increase speed of
recovery
 A supervised exercise program is also recommended to
improve fatigue as well as functional abilities
Prognosis
 Even with appropriate treatment, 3% of patients with
Guillain-Barré Syndrome will die
 25% of patients will require mechanical ventilation
which increases mortality risk
 Prognosis is worse in patients with rapid onset of
symptoms, severe symptoms and in elderly patients
 Neurological deficits persist in 20% of patients, half of
these remain severely disabled
 Up to 80% of patients experience persistent, severe
fatigue after resolution of other symptoms
Prognosis
Predicts long-term disability
 Absence of motor response
 Diarrheal illness
 Axonal involvement
 Campylobacter jejuni or cytomegalovirus infection
 Inability to walk at 14 days
 Older age
 Rapid progression of symptoms
 Severity of symptoms at their peak
CME Questions
 Q: A patient presents with leg pain. Which one of the
following accompanying findings most consistently
suggests Guillain-Barré syndrome? (check one)
 A. Prominent bowel or bladder symptoms.
 B. Cerebrospinal fluid leukocytosis.
 C. Relatively symmetrical weakness of the limbs.
 D. Normal results on nerve conduction studies.
CME Questions
 Answer:
 C. Relatively symmetrical weakness of the limbs
CME Questions
 Q: Which one of the following statements about diseasemodifying therapy for Guillain-Barre syndrome is correct? (check
one)
 A. Plasma exchange should be witheld for the first 30 days after
symptom onset.
 B. Intravenous immune globulin therapy should be started within
two weeks of symptom onset, and should be considered for
patients who are nonambulatory.
 C. Most patients require six sessions of plasma exchange.
 D. Corticosteroids are first-line treatment.
CME Questions
 Answer:
 B. Intravenous immune globulin therapy should be
started within two weeks of symptom onset, and should
be considered for patients who are nonambulatory.
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