Guillain-Barre Syndrome Flashcards
Definition of Guillain Barre Syndrome
a collection of symptoms and signs, which is usually an immune attack of on part of the peripheral motor or sensory nervous system (myelin sheath, eye nerves, motor and sensory axons)
Causes of Guillain Barrre Syndrome
Most commonly occur after
- Food poisoning (campylobacter)
- Flu
- Viral infections
- Child birth
- Surgery
- Vaccinations
Time from onser of illness to peak disability can vary from hours to weeks, but generally peaks at ______ weeks, and progression always stops within ____ weeks
2 -3 weeks
4 weeks
Risk factors of Guillain Barre Syndrome
- Can occur at any age (slightly more common > 65)
- Female (ratio of 1 : 5 = Male : Female)
- Not hereditary
- Not infectious (間接)感染性のor contagious (接触)伝染性の.
Incidence and prevalence of Guillain Barre Syndrome
Rare disorder-
affects 1 - 2 / 100,000 NZ (40 - 80 a year)
Pathophysiology of Guillain Barre Syndrome
Patchy multifocal mononuclear cell infiltration throughout the nervous system, with the inflammatory distribution correlating to the clinical deficits. Either by:
-Activated helper T-cells react against specific antigens on the surface of Schwann cells or the myelin sheath thereby directing activated macropphages to this region, or
-Antibodies bind to epitopes (抗体と結合する抗原の一部分)
抗原決定基 on the outer surface of Schwann cells inducing activation and subsequent myeline destruction prior to macrophage invasion
Schwann cells supply the myelin for _______, whereas oligodendrocytes myelinate the axons of the _________.
peripheral neurons (in the PNS) central nervous system (CNS)
Lesion location of Guillain Barre Syndrome
lesion occurs throughout the PNS from the spinal nerve roots to the distal termination of both motor and sensory fibres
Although GBS is a monophasic (having a single phase) illness, about ___ to ___% of patients suffer recurrent episodes of worsening after an initial improvement
6 - 17%
Clinical presentation of Guillain Barre Syndrome
- Progressive, ascending weakness - proximal and distal muscles
- Altered sensation - tingling (P & N) and /or numbness (usually starting in feet and rising to hands and sometimes face - 50%)
- Cranial nerves and respiratory muscles are commonly involved
- Hyporeflexia or areflexia (as it is PNS)
- Neurological pain (as a result of inflammatino around the nerves)
- Usually bilateral symptoms
- About 60% are unable to walk at the peak of disability
- Respiratory function will be impaired in 50% of cases with 20 - 30% requiring non-invasive to invasive ventilation and intensive care
- Dysautonomia in 15% - cardiac arrhythmia, hypertension or hypotension (5-61%), urinary retention
- Fatigue
Diagnosis of Guillain Barre Syndrome
- Blood test (raised ESR [erythrocyte sedimentation rate] …the distance red blood cells travel in one hour in a sample of blood as they settle to the bottom of a test tube)
- Lumbar puncture (elevated CSF protein > 40mg/dl)
- Electromyography (EMG)… evaluate the activation signal of muscles
- Nerve conduction tests (reduced motor conduction velocity)
- Altered Sensory Evoked Potentials (SEPs) … non-invasive. assess somatosensory functioning
- CT / MRI
- Stool culture and serology
Medical managent of Guillain Barre Syndrome
-Time - remyelination of nerves
-Plasmapheresis (gold standard) … removal of the attacking antibodies from plasma (clean blood which causes problem) => Reduces time taken to walk from 44 to 33 days; improvement in ADL function
-PEEP (positive end expiratory pressure) ventilation - reduces stasis [うっ血, blood is slowed or stopped] and pooling in lung
-Analgesia
Fluid resuscitation
-No significant effect of steroids
Prognosis of Guillain Barre Syndrome
- Recovery can be between 3 weeks to 9 months - average 3-6 months
- 2/3 make full recovery; remainder left with some degree of disability and need to make substantial changes in their work or social activities
- Mortality of -5% … respiratory or bulbar (眼球の) dysfunction
Differential Diagnosis of GBS
- Disorders of CNS may present with acute generalised flaccid paralysis (weakness or loss of muscle tone resulting from injury or disease of the nerves innervating the muscles)
- Peripheral neuropathy
- Disorders of the neuromuscular junction …. Muasthenia gravis
- Disorders of muscle … infections
- Disorders of the CNS … Acute myelopathy (high cervical), Acute anterior poliomyelitis (polio)
Functional assessment of GBS
- 10MWT (temporal spatial measurement)
- 6MWT (exercise tolerance)
- Balance (Functional reach, Lateral reach, Berg, BESTest, Dynamic Gait Index, Community Balance and Mobility Scale, HiMAT - High Level Mobility Assessment Tool)
