Nontraumatic Neuromuscular Disease Pathologies And Mangement (exam 2) Flashcards

1
Q

What is Transverse Myelitis (TM)?

A

Acute inflammation of the gray and white matter in one or more adjacent spinal cord segments
Demyelination and axonal injury often present
(Inflammation of the spinal cord)

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2
Q

TM etiology

A

Varied; hours to days

Idiopathic TM: abnormal immune response against spinal cord
May present as a feature of MS (10–33% of persons with acute TM develop MS; high percentage if lesions on MRI with TM)
Viral/bacterial infections (especially those with rashes)

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3
Q

TM clinical presentation

A

Rapid progressive weakness/paralysis (LEs first, then possibly UEs)
Diminished light touch, pain, temperature, vibration, proprioception below level of lesion (DCML and ALS)
Often have a “band” sensation around the trunk that is sensitive to touch
Autonomic dysfunction (sexual, B/B)
Upper motor neuron presentation

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4
Q

TM diagnostics

A

Gadolinium enhancement noted on MRI or LP showing elevated CSF WBCs or IgG index
Clearly defined sensory level
Bilateral signs and/or symptoms

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5
Q

What is Post-polio Syndrome (PPS)?

A

New, late-onset symptoms of fatigue, weakness and pain in 20–40% of persons who survived acute paralytic polio

  • Individuals without history of polio diagnosis are presenting with PPS symptoms as well (non-paralytic polio)

Range of PPS onset from initial infection to new symptoms: 8–71 years!

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6
Q

How does PPS present?

A

In addition to fatigue, muscle/joint pain, weakness …
Residual asymmetrical muscle atrophy
Areflexia (in at least one limb)
Cold intolerance
Sleep disorders
Muscle cramps, fasciculations
Breathing/swallowing difficulty
Hypoventilation
Scoliosis

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7
Q

What is Guillain-Barré Syndrome (GBS)?

A

Less commonly known as “acute demyelinating inflammatory polyradiculoneuropathy”

Most common, most severe acute paralytic
neuropathy

Affects nerve roots and peripheral nerves, causing motor neuropathy and flaccid paralysis

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8
Q

Are women or men more likely to be affected by GBS?

A

Men are 2x more likely

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9
Q

What is the etiology of GBS?

A

Two-thirds of patients have an infectious disease about 2–4 weeks before onset of symptoms

There is no definitive causative factor

Macrophages and T-lymphocytes infiltrate spinal roots and peripheral nerves
- Macrophages remove myelin
- Axons may degenerate or may remain intact & nerves remyelinate

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10
Q

GBS clinical presentation

A

Rapidly ascending bilateral weakness
- Distal to proximal: typically starts in distal LEs; could start more proximally in UEs/LEs
- May progress to full tetraplegia (respiratory affected)

Areflexia (absence of DTR) in paretic limbs

Other:
Relative symmetry
Autonomic dysfunction
Pain is common
Cranial nerve involvement
Sensory symptoms (not in AMAN variant)
Absence of fever at onset
Abnormal nerve conduction studies

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11
Q

What is Myasthenia Gravis (MG)?

A

Autoimmune disorder
- Postsynaptic defect of neuromuscular transmission (neuromuscular junction)
- Autoantibodies directed against the postsynaptic nicotinic acetylcholine receptor (AChR)

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12
Q

What is the clinical presentation of MG?

A

Skeletal muscle weakness
- Increases with fatigue and throughout day
- Localized or generalized
- Greater proximally than distally

Fatigue, breathing issues, ptosis, diplopia, hypomimia, difficulty chewing/swallowing, dysarthria, mobility deficits

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13
Q

What is a Myasthenic Crisis?

A

Days to weeks of worsening symptoms
Drastic worsening of respiratory muscle weakness
Respiratory failure (intubation/vent)

Myasthenic Exacerbation: worsened double vision, slurred speech, increased arm weakness, falling, unsteady walking, difficulty swallowing

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14
Q

TM prognosis

A

1/3rd recover completely or with minimal deficits
1/3rd have moderate degree of permanent disability
1/3rd have severe disability

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15
Q

TM approach for interventions

A

Same approach as traumatic ACI with consideration of compensation vs recovery continuum

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16
Q

General considerations for neuromuscular diseases (NMD)

A

Improve and maintain function
Exercise is safe and effective in all dorms of adult NMD

Improvements with 6MWT, STS reps and times, SF-36 and fatigue severity

17
Q

PPS prognosis

A

Not very clear
Variability in functional prognosis

18
Q

PPS strengthening guidelines

A

Exercises should NOT:
cause muscle soreness or pain
lead to fatigue that prevents activities

Only attempt strengthening with muscles that can move through their full ROM
Up to moderate intensity
Progress slowly

19
Q

PPS strengthening guidelines

A

Recommenced unless complaints of overwhelming fatigue
NO muscle soreness or pain
3-4x a week, up to 30 min
Up to moderate intensity

20
Q

Other PPS intervention recommendations

A

Stretching for ROM and pain management
Education! Posture, EC, activity modifications, pacing
Assess for orthotic and/or equipment needs
HEP

21
Q

GBS prognosis

A

About 60% fully recover strength by one year
80% ambulate w/in 6 months and 84% at 1 year
5-10% have delayed or incomplete recovery
3% unable to ambulate long term

22
Q

GBS prognosis factors

A

Older than 60
Severe disease
Rapid progression period to admission (<7 days)
Preceding diarrheal illness
Mean distal monster response reduction to <20%
Required mechanical ventilation

23
Q

Acute GBS interventions

A

Functional retraining (Monitor orthostatics!)
Frequent repositioning
P-AAROM
Splinting and positioning
Airway clearance
Non-fatiguing activity
Pain management

24
Q

GBS subacute intervention

A

Continue functional training
Progress to AROM as able
Self-repositioning for pressure relief and ROM
Assess cushion/mattress
Aerobic exercises training (walking, biking, UBE)
Strength training (functional, bands, weights) - AVOID eccentric exercises

25
Q

GBS chronic interventions

A

Gait, dynamic balance, community mobility and recreation
Self-stretching
Assess orthoses need
Aerobic (mod-high intensity)
Strengthening (60-80% RM)
Neuropathic Pain management (TENS)
Home/work adaptations for safety and minimize fatigue

26
Q

MG Prognosis

A

Most can significantly improve
Remission can be temporary or permanent

27
Q

MG interventions

A

Strength
Aerobic
Posture and balance
Functional mobility
Respiratory training
Patient education

28
Q

Respiratory training with MG

A

16 weeks of mild to moderate showed improved pattern breathing at rest
Home based training can be utilized

29
Q

Patient education for MG

A

Self-management strategies
Brief napping throughout the day may help decreased muscular fatigue