Nontraumatic Neuromuscular Disease Pathologies And Mangement (exam 2) Flashcards
What is Transverse Myelitis (TM)?
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)
TM etiology
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)
TM clinical presentation
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
TM diagnostics
Gadolinium enhancement noted on MRI or LP showing elevated CSF WBCs or IgG index
Clearly defined sensory level
Bilateral signs and/or symptoms
What is Post-polio Syndrome (PPS)?
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!
How does PPS present?
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
What is Guillain-Barré Syndrome (GBS)?
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
Are women or men more likely to be affected by GBS?
Men are 2x more likely
What is the etiology of GBS?
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
GBS clinical presentation
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
What is Myasthenia Gravis (MG)?
Autoimmune disorder
- Postsynaptic defect of neuromuscular transmission (neuromuscular junction)
- Autoantibodies directed against the postsynaptic nicotinic acetylcholine receptor (AChR)
What is the clinical presentation of MG?
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
What is a Myasthenic Crisis?
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
TM prognosis
1/3rd recover completely or with minimal deficits
1/3rd have moderate degree of permanent disability
1/3rd have severe disability
TM approach for interventions
Same approach as traumatic ACI with consideration of compensation vs recovery continuum
General considerations for neuromuscular diseases (NMD)
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
PPS prognosis
Not very clear
Variability in functional prognosis
PPS strengthening guidelines
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
PPS strengthening guidelines
Recommenced unless complaints of overwhelming fatigue
NO muscle soreness or pain
3-4x a week, up to 30 min
Up to moderate intensity
Other PPS intervention recommendations
Stretching for ROM and pain management
Education! Posture, EC, activity modifications, pacing
Assess for orthotic and/or equipment needs
HEP
GBS prognosis
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
GBS prognosis factors
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
Acute GBS interventions
Functional retraining (Monitor orthostatics!)
Frequent repositioning
P-AAROM
Splinting and positioning
Airway clearance
Non-fatiguing activity
Pain management
GBS subacute intervention
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
GBS chronic interventions
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
MG Prognosis
Most can significantly improve
Remission can be temporary or permanent
MG interventions
Strength
Aerobic
Posture and balance
Functional mobility
Respiratory training
Patient education
Respiratory training with MG
16 weeks of mild to moderate showed improved pattern breathing at rest
Home based training can be utilized
Patient education for MG
Self-management strategies
Brief napping throughout the day may help decreased muscular fatigue
