Guillain-Barre Syndrome Flashcards
What is GBS?
Most common cause of rapidly evolving motor paresis and paralysis and sensory deficits
What is the incidence? M vs F? Mortality?
- Incidence is 1-2 cases per 100,000 people
- Affects all ages (esp. young adults and individuals between 50-80 yrs)
- M>F
- Mortality > 5%
GBS is a syndrome. What 4 conditions does it include?
- Flaccid Paralysis
- Areflexia
- Respiratory Compromise
- Autonomic Dysfunction
What are the characteristics of flaccid paralysis?
- Maximal weakness ~2-3 weeks after onset
- Facial weakness >50% of cases
- Pharyngeal weakness
- Laryngeal weakness
Pharyngeal and laryngeal weakness can lead to ______.
ASPIRATION
Mechanical ventilation is needed in ____% of cases
25-30%
Autonomic dysreflexia occurs in _____% of cases. Characteristics?
70%
- Fluctuations in heart rate, rhythm, and BP
- Excessive or loss of sweating
- Flushing of face
What is the etiology of GBS?
Autoimmune disorder –> antibody mediated demyelination
Myelin sheath on peripheral nerves is attacked causing demyelination (Classic type of GBS)
______ cells are spared allowing for future recovery and re-myelination.
SCHWANN CELLS
60-90% of patients with GBS report experiencing ____ 30 days before onset of GBS.
INFECTION (minor illness)
How long does it take for the body to initiate its healing processes following the onset of GBS?
~ 2 to 4 weeks
What is the onset of GBS like? When does it peak?
- Onset and progression are fast
- 50% of cases peak in 2 weeks
- 90% of cases peak in 4 weeks
- 10% recur
What are the clinical signs and symptoms of GBS?
- “Stocking Glove” presentation at first
- Begins with paraesthesias in toes / fingers and distal leg weakness / hand weakness
- Weakness and paraesthesias progress proximally including trunk
Face and palate in ~50% of cases - Respiratory paralysis requiring mechanical ventilation in ~ 25-30% of cases within ~ 18 days
- May cause total paralysis/dysphagia
- Bowel and bladder dysfunction are rare
- Fever is not typical
What are possible differential diagnoses for GBS?
Hysteria Tick paralysis Toxic neuropathy Myasthenia gravis Neuromuscular blocking agents Transverse myelitis Anterior spinal artery syndrome Poliomyelitis Stroke Metabolic disorders
How is GBS diagnosed?
Basic lab studies ordered to r/o other pathologies
Albuminocytologic Dissociation is present
(Increased protein in the cerebrospinal fluid without increase in white blood cell count = widespread inflammation of nerve roots)
- Nerve conduction studies to detect demyelination
What diagnostic criteria must be met to establish a diagnosis of GBS?
- Progressive, symmetric weakness in more than one extremity
- Sensory deficits
- Loss of deep tendon reflexes
- Possible tachycardia, cardiac arrhythmias, labile blood pressures
- Absence of fever
- Electrophysiological tests reveal signs of demyelination
- Cerebral spinal fluid analysis via lumbar puncture 1 week post-onset of symptoms reveals
increased protein (albumin) and <10 WBC’s and lymphocytes
Plasmapheresis is used to manage GBS. What is it? What are 2 benefits associated with plasmapheresis?
- The process of removing plasma from the circulatory system and filtering it in order to remove antibodies
- Plasma is then returned to the circulatory system
- Performed 4-6 x over period of 1 week
- Shown to reduce time on respirator
- Shown to reduce time to independent ambulation
***Intravenous Immunoglobulins (IV Ig) also used to manage GBS
_______ do not have any beneficial effect in classic GBS
Corticosteroids
The period between peak impairment and recovery is called the ______ phase.
Plateau or static phase
How does recovery from GBS typically progress ?
Proximal to distal (opposite to the onset)
How long does it take to recover from GBS?
- May take weeks to years for recovery
- One year post onset, 67% of people have full recovery
- Up to 20% have lasting neurological impairments
- After 2 years, up to 8% have not recovered
What are 5 poor prognostic indicators of GBS?
- Advanced age at onset
- Protracted time between peak impairment and onset of recovery
- Need for mechanical ventilation
- Evidence of axonal degeneration (reduced evoked motor potential)
- Cranial nerve involvement
What are 5 good prognostic indicators of GBS?
- Younger age at onset
- Less time between peak impairment and onset of recovery
- Less total impairment
- No need for mechanical ventilation
- Intact axonal integrity
What are 6 variants of GBS?
- Acute Inflammatory Demyelinating Polyradiculoneuropathy (AIDP) –> classic form
- Acute Motor Axonal Neuropathy (AMAN)
- Acute Sensory Ascending Neuropathy (ASAN)
- Acute Motor and Sensory Axonal Neuropathy (AMSAN)
- Miller Fisher Syndrome
- Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)
