Progressive Neuromuscular Disorders Pt 2: GBS Flashcards
Guillain-Barre Syndrome
Another disorder of the PNS; an acute inflammatory demyelinating ____ (simultaneous neuropathy of peripheral nerves)
Often occurs > an infection & leads to a rapidly progressing flaccid paralysis
polyneuropathy
GBS: Epidemiology
- Respiratory or GI infections are common sources
- Campylobacter jejuni is most freq cause
- Also CMV, EBV, Mycoplasma pneumoniae, & Haemophilus influenzae
- About 2/3 of those who develop GBS show clinical manifestations of an infection 3 wks prior to onset
GBS: Pathophysiology
- Mediated by an immune response w/acute lower extremity weakness w/areflexia or diminished reflexes that develop over several days
- Pt’s own immune system begins to destroy the myelin sheath that surrounds the peripheral nerves
- Destruction occurs between the nodes of Ranvier that impairs saltatory (jumping) conduction & results in slowing of impulses or conduction block
GBS: Clinical Manifestations
▻ Symmetrical ascending motor weakness & paralysis
▻ Starts in feet & extends to trunk & arms
▻ Paresthesias & pain (that involves shoulders, back, buttocks, & upper legs)
▻ Diminished or absent DTRs
▻ Cranial nerve sx’s
▻ No atrophy
▻ Autonomic sx’s
- Most common form ▻ acute inflammatory demyelinating polyneuropathy
- The CN ___, facial nerve, being one of the most common involved, so pt develops problems w/facial expression such as smiling or frowning
VII
Other CN’s involved might be IX & XII which may cause dysphagia/problems swallowing
- If CN ___ is affected, the pt may develop autonomic dysfunction w/possible cardiac dysrhythmias, paroxysmal hypotension, & potential SIADH
X
- Up to 40% of pts develop resp impairment; resp failure is c/b weakness of the diaphragm & intercostal muscles
- Pt may require intubation & mechanical ventilation
- LOC & cognitive fxn remain intact throughout the course of the illness
! Muscle atrophy is not a part of the dz process but if someone is bedridden or vent dependent for a long time, normal atrophy of muscle will occur
- The progression of the sx’s is ascending, meaning that it begins @ the feet/legs & progressively moves up the body
▻ When it begins to resolve, it goes backwards, so descending; the last areas to be affected are the first to heal
3 Phases of GBS
▻ Acute
▻ Plateau
▻ Recovery
?
Is when the demyelination stops; lasts from a few days to weeks
There will be no change in pt’s sx’s; no worse, no better
Plateau
?
Is when the remyelination & axonal regeneration begins & there’s a gradual improvement in the pt’s s/s
Recovery
?
Is when GBS starts & the demyelination occurs (destruction of myelin sheath), edema, & inflammation
Can last approx 4 wks
Acute
?
Is when GBS starts & the demyelination occurs (destruction of myelin sheath), edema, & inflammation
Can last approx 4 wks
Acute
GBS: Management
- Focuses on supportive care, reducing severity, potential complications, suffering, & recovery time
- Diagnostic tests
- Rx’s & diet
> enteral feeding if needed - Complications
GBS: Treatments
- Plasmapheresis
- IVIG
- Support resp functions
- Dec complications/immobility
- Recovery - rehabilitation
Management
- Established diagnostic criteria for GBS includes progressive weakness of 2 or more limbs c/b neuropathy, areflexia, & h/o recent viral or bacterial infection
Diagnostic
- Criteria will be progressive weakness of 2 or more limbs c/b a neuropathy, areflexia, & h/o recent viral or bacterial infection
- LP will be done & the CSF will be tested for indicative findings of elevated protein & normal cell count
