Disorders of Peripheral Nervous System Flashcards

1
Q

What is the most common cause of acute evolving motor & sensory deficits?

A

Guillain-Barré syndrome (GBS)

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2
Q

What age groups are affected by GBS?

A
  • Young adults
  • 50-80 y/o
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3
Q

T/F: females are more likely to get GBS

A

False- slightly more males develop GBS

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4
Q

What is the etiology of GBS and what often precedes GBS?

A
  • Immune-mediated disorder
  • Acute infection often precedes
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5
Q

What are some common triggers for GBS?

A
  • Viral (Haemophilus influenza, Epstein-Barr, Cytomegalovirus)
  • Bacterial (Campylobacter jejuni)
  • Surgery
  • Vaccines
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6
Q

What is the pathogenesis of GBS in regards to demyelination of PNS?

A
  • Antibodies bind to myelin of Schwann cells
  • Macrophages respond to inflammatory signals & strip myelin from nerves
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7
Q

In regards to pathogenesis of GBS how does remyelination occur?

A

Schwann cells divide & remyelinate nerve axons

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8
Q

Why may there be axonal damage in an individual with GBS?

A

Inflammation

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9
Q

What axons are affected by GBS?

A
  • Motor
  • Motor and sensory
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10
Q

What is the clinical presentation of Acute Inflammatory demyelinating polyneuropathy (AIDP)?
A variant of GBS

A

progressive paralysis & areflexia due to demyelination

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11
Q

What is the clinical presentation of acute motor axonal neuropathy (AMAN)?
A variant of GBS

A

Axon involved, more severe, more respiratory involvement, more significant residual impairments

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12
Q

What is the clinical presentation of acute motor & sensory axonal neuropathy (AMSAN)?
A variant of GBS

A

Same as AMAN but with sensory involvement

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13
Q

What is the clinical presentation of Acute sensory ascending neuropathy (ASAN)?
A variant of GBS

A

Sensory > Motor

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14
Q

What is the clinical presentation of Miller Fisher syndrome?
A variant of GBS

A

Opthalmoplegia, ataxia, areflexia with sparing of strength

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15
Q

What is the clinical presentation of chronic inflammatory demyelinating polyneuropathy?
A variant of GBS

A

Slower onset, relapses & remissions or slow progression

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16
Q

What are the clinical manifestations of GBS?

A
  • Variation b/w subtypes
  • Ascending symmetrical weakness & distal sensory impairments
  • Flaccid paralysis
  • Absence of DTRs
  • Time from onset to peak impairment < 4 weeks
  • 30% require mechanical ventilation
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17
Q

What are the GBS stages and what occurs in each?

A
  • Progressive impairment (distal to proximal)
  • Static phase (2-4 wks)
  • Recovery (proximal to distal, may take months or years)
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18
Q

What symptoms are required for diagnosis of GBS?

A
  • Progressive weakness in > 1 extremity
  • Loss of DTRs
19
Q

What symptoms are supportive of a diagnosis of GBS?

A
  • Weakness developing rapidly ceasing to progress by week 4
  • Symmetric weakness
  • Mild sensory signs & symptoms
  • Facial weakness
  • Recovery starts 2-4 wks after progression ceases
  • Tachycardia, cardiac arrhythmias, & labile BP possbile
  • Absence of fever
20
Q

In order to diagnose GBS what features must the CSF have?

A
  • CSF protein levels increase after 1 week (continue to increase with serial exams)
  • CSF contains < 10 mononuclear leukocytes/mm3
21
Q

In order to diagnosis GBS what must the electrodiagnostic test show?

A

Nerve conduction velocity slowed

22
Q

What are some interventions for GBS?

A
  • Control of autoimmune responses
  • Mechanical ventilation
  • Prevention of effects of immobility
23
Q

What is the prognosis of GBS in regards to:
- Mortality rate?
- Recurrent form?
- 1 year?

A
  • Mortality rate: 5%
  • Recurrent form: 10%
  • 1 year: 67% complete recovery, 20% significant disability)
24
Q

What are the indications of a poor prognosis of GBS?

A
  • Older
  • Increase time before recovery begins
  • Need for artificial respiration
  • Reduced evoked potential (sign of axonal damage)
25
What is polio?
Virus invades cell bodies of lower motor neurons in ventral horn of spinal cord resulting in asymmetric flaccid paresis or paralysis
26
What is post- polio syndrome?
New neuromuscular symptoms occurring decades (average 25 years) after recovery from the acute paralytic episode
27
How many survivors are there of acute poliomyelitis in US? And how many will develop post-polio?
- 1.63 million survivors - 1/4 to 1/2 will develop post-polio
28
What is the etiology of post-polio syndrome?
- Denervated muscles were reinnervated by collateral sprouting from surviving nerves - Axons innervating more muscle fibers than originally intended - Nervous system can no longer support giant motor units - Prune back axonal sprouts
29
What are the clinical manifestations of post-polio syndrome?
- Declining muscle strength in previously affected & unaffected muscles - Myalgia - Joint pain - Muscle atrophy - excessive fatigue
30
How is post-polio diagnosed?
- Clinical - EMG - Muscle biopsy
31
What are the intervention strategies for post-polio syndrome?
- Treat symptoms - Modify lifestyle - Surgery (for deformities) - Avoid working to fatigue
32
What is the prognosis of post-polio syndrome?
- Slowly progressive - Stable period (3-10 years) - Decreased function & quality of life
33
What is Myasthenia Gravis?
- Motor end plate disorder - Most common neuromuscular transmission disorder
34
What is Myasthenia Gravis characterized by?
Fluctuating weakness & fatiguability of skeletal muscles
35
What is the age of onset of Myasthenia Gravis?
- Males: 50 to 60 years - Females: 20 to 30 years
36
Is Myasthenia Gravis more common in females or males?
Females > males (3:2)
37
What is the etiology of Myasthenia Gravis?
Autoimmune disorder affecting neuromuscular junction & motor end plate
38
What is the pathogenesis of Myasthenia Gravis?
- Anti-Ach receptor antibodies (block receptor site & cause endocytosis of receptor) - Acetylcholine receptors decreased & flattened which decreases efficiency of neuromuscular transmission - so failure of nerve impulses to cross neuromuscular junction to stimulate muscle contraction - Thymus may trigger the autoimmune response
39
What are the clinical manifestations of Myasthenia Gravis?
- Skeletal muscle fatigue & weakness (Activity causes fatigue & rest restores activity) - Weak neck & facial muscles
40
Patients with Myasthenia Gravis have weak neck & fascial muscles. How does this manifest?
- Diplopia - Ptosis - Weak neck muscles - Nasal speech - Dysphagia - Nasal regurgitation - Aspiration of food
41
What are the three diagnostic methods of Myasthenia Gravis?
1. Immunologic - Detect anti-Ach receptor antibodies 2. Pharmacologic - acetylcholinesterase inhibitor (blocks Ach reuptake) Improves strength & endurance 3. Electrophysiological - repeated stimulation shows a rapid decrease in motor action potential amplitude
42
What are some interventions for Myasthenia Gravis?
- AChE inhibitor meds - Surgery (removal of thymus) - Corticosteroids - Plasmapheresis
43
What is the prognosis of Myasthenia Gravis?
- Slowly progressive disorder (periods of exacerbation & remission) - Myasthenia crisis
44
What is Myasthenia crisis?
- medial emergency - Respiratory muscle weakness