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Neuromuscular practice 2 > Neuromuscular conditions > Flashcards

Neuromuscular conditions Flashcards

1
Q

disorder of the motor neuron-anterior (ventral) horn cell

A

Motor neuron disease (LMN)

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2
Q

disorder of the peripheral nerve (ventral and dorsal)

A

Radiculopathy [one nerve affected from spine downwards]

Neuropathy [usually many nerves affected but more towards hands and feet]

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3
Q

disorder of the neuromuscular junction/motor end plate

A

where nerves meet muscles

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4
Q

disorder of the muscle

A

myopathy

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5
Q

Neuropathy vs. Myopathy

A 
Neuropathy:
-soma/axons involved
-distal involvement (symm. > asymm)
-stocking-glove pattern
-longest nerves affected 1st (LE before UE)
Myopathy
-MEP/mm involvement
-proximal involvement (symm)
-difficulty arising
-overhead activities
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6
Q

LMN vs. UMN

A 
LMN:
-flaccid or reduced stiffness
-decreased tone
-decreased muscle stretch reflexes
-profound muscle atrophy
-fasciculations seen or twitches
- +/- sensory disturbances
UMN:
-spasticity or very stiff
-increased tone
-increased muscles stretch reflexes
-minimal muscle atrophy
-fasciculations absent
- +/- sensory disturbances
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7
Q

Motor neuron conditions (LMN-Anterior horn cell)

A

Spinal muscular atrophy (progressive)
post-polio syndrome (slowly progressive after recovery from polio)
amyotrophic lateral sclerosis

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8
Q

Peripheral nerve conditions

A

Gullian-Barre (initial worsening with recovery)

Charcot Marie Tooth (initial worsening with limited recovery)

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9
Q

Motor end plate conditions

A

Myasthenia Gravis (exacerbations result in wekaness in the face, neck and hands)

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10
Q

muscle conditions

A

Muscular dystrophy (progressive)

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11
Q

Components of medical diagnosis

A 
medical and family hx
physical exam
electrodiagnostic testing: EMG, NCV
Blood labs
genetic testing
muscle biopsy
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12
Q

Components of movement system diagnosis

A 
PIP
movement observation
hypothesize impairments
-weakness (symm/asymm)
-hypo/hypertonia
-muscle flexibility
-bulbar function
-pain
-fatigue
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13
Q

Keys to prognosis to inform intervention strategies

A

Long-term and short-term goals
Expected natural progression of condition
-onset period/age
-progression rate
-recovery potential
contextual factors that would contribute to the achievement of the goal
contextual factors that may prevent or delay the acquisition of the goal
likely time frame for achieving the goal [PROGNOSIS]

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14
Q

interventions for optimizing function

A 
education
encourage activities and participation
strengthening (moderate intensity)
aerobic training (moderate intensity)
flexibility
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15
Q

interventions for adaption to limitation

A

prevent overuse
prevent respiratory dysfunction (breathing techniques/coughing)
prescribe assistive technology (orthotics, AD, w/c) to support participation

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16
Q

interventions for maximizing quality of life

A

addressing respiratory dysfunction (postural drainage and assistive coughing)
positioning
supporting and training the caregiver

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17
Q

Spinal muscular atrophy

A
  • genetic disorder: 5q gene deletion
  • premature death of alpha motor neurons in spinal cord and brainstem (CN V, VII, IX, XII)
  • premature death of alpha-motor neurons –> weakness & wasting of voluntary muscles (more sever in legs vs arms)
  • classified according to type I-IV
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18
Q

Type I spinal muscular atrophy

A
  • acute infantile (Werdnig-Hoffman)
  • onset birth-6 mos
  • progression : rapid
  • movement system: never sits, poor head control/suck/swallow/cry
  • prognosis: usually fatal within 2 years
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19
Q

Type II spinal muscular atrophy

A
  • chronic infantile (Dubowitz Disease)
  • onset: 6 mo - 18 mo
  • progression: slower
  • movement system: delayed motor milestones; able to sit but not stand or walk
  • prognosis: variable
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20
Q

Type III spinal muscular atrophy

A
  • chronic juvenile (Kugelberg-Welander)
  • onset: after 18 mo
  • progression: slower
  • movement system: most can stand and walk, but have trouble going up/down stairs; may lose ability to walk later; in w/c- develop scoliosis; bulbar dysfunction later in life
  • prognosis: typical life expectancy
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21
Q

Type IV spinal muscular atrophy

A
  • adult
  • onset in 30s
  • lose ability to walk
  • typical life expectancy
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22
Q

Tools for medical diagnosis of spinal muscular atrophy

A 
genetic testing (5q)
EMG (reduced amplitude, fibrillations)
muscle biopsy (muscle fiber atrophy)
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23
Q

common impairments associated with spinal muscular atrophy

A

several proximal muscles weakness
flaccidity or hypotonicity
bulbar dysfunction (difficulty swallowing and breathing) in more severe cases, as in type I or in later disease progression of type II or III

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24
Q

Common PT diagnoses for spinal muscular atrophy

A
  • difficulty rolling secondary to weakness in SCM, pec major, AO, and RF
  • difficulty sitting secondary to weakness of paraspinals (including cervical), and abdominals
  • difficulty reaching secondary to weakness in deltoid and triceps
  • difficulty lying prone secondary to poor flexibility in iliopsoas and hamstrings
  • difficulty transitioning to standing secondary to poor strength in glute max and quadriceps
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25
Q

intervention guidelines for spinal muscular atrophy

A
  • strength training of proximal muscle groups: shoulder flexors/extensors, elbow flexors/extensors, hip flexors, extensors, and knee extensors
  • 2 sets of 15 reps for each muscle group with 5 minute rest between sets
  • initial intensity should emphasize biomechanics using body weight as resistance and progressed by adding light resistance
  • monitor with RPE; goal under “somewhat hard” to “hard”
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26
Q

Post-polio syndrome

A
  • onset is adulthood (35 yrs)
  • late manifestation of acute poliomyelitis
  • degeneration of large motor neuron pools
  • slowly progressive new muscle weakness in previously affected areas
  • asymmetrical distribution
  • distal or proximal or patchy
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27
Q

Keys to medical diagnosis of post-polio syndrome

A

prior paralytic poliomyelitis
EMG (polyphasic motor unit potentials, fibrillations)
muscle biopsy (muscle atrophy)

28
Q

movement system diagnosis for post-polio syndrome

A

difficulty walking and standing secondary to…
fatigue
pain
weakness

29
Q

Intervention guidelines for post-polio syndrome

A
  • lifestyle modifications
  • energy conservation techniques
  • avoid overuse of muscle groups
  • non-fatiguing muscle strengthening program can result in strength gains
  • be aware of poor body mechanics and malalignment and incorporate postural exercises
  • monitor fatigue and pain
30
Q

Amyotrophic Lateral Sclerosis

A

acquired disease
degeneration of cortical spinal tract: UMN and LMN
primarily anterior horn cells and motor cranial nuclei
progressive asymmetrical weakness: distal then proximal
sensory function intact
onset: adulthood (avg. 50 yr.)
progression: rapid (fatal in 3.5 yrs)

31
Q

Macro/microscopic exam findings in ALS

A
  • anterior spinal nerve roots are shrunken/atrophied
  • loss of cell bodies
  • loss of corticospinal tracts
32
Q

Classification of ALS

A

Suspected ALS: LMN signs only in 1 or more regions
Suspected ALS: UMN signs only in 1 or more regions
Probable ALS: LMN and UMN signs in 2 regions
Definite ALS: LMN and UMN signs in 3 regions

33
Q

clinical presentation of ALS

A

LMN sxs
-bulbar–dysarthria, dysphagia, tongue atrophy & fasciculations
-Peripheral– weakness, MM atrophy, fasciculations, hypotonia, dyspnea
UMN sxs
-bulbar– dysphagia, dysarthria
peripheral– spasticity/clonus/hyperreflexia, +Babinski, spastic gait, poor balance

34
Q

Components of medical diagnosis for ALS

A

History– progressive weakness/atrophy of mm: bulbar, cervical, thoracic & lumbosacral neurons
Genetics–most cases sporadic
clinical course– unrelenting deterioration months > years
neuro exam–UMN & LMN sxs; EMG/NCV; imaging, biopsy

35
Q

prognosis for ALS

A

Devastating, rapidly progressive neurodegeneration of AHC, LMN, and UMN with highly predictable clinical course
patient survival: 2.5-4 yrs post-diagnosis; 50% survival 1yr after bulbar onset.
Medications: Riluzole (prolongs survival by 2-3 mo)

36
Q

Intervention guidelines for ALS

A
  • Old controversial idea: epidemiological data suggests vigorous activity is a risk factor for motor neuron loss.
  • animal studies suggest lifetime of vigorous activity unrelated to onset time or progression of ALS
  • positive effect of moderate exercise in individuals with ALS
  • monitor post-exercise fatigue to monitor intensity
37
Q

Non-pharmacological management of ALS

A 
PT, OT, Speech T
respiratory
dietitians
social workers
nursing
psychologists
spiritual care
38
Q

Pharmacological management for ALS

A 
Riluzole
Symptom management:
-anxiety-- lorazepam
-spasms--Quinine sulphate
-spasticity-- Baclofen
-drooling-- Atropine
39
Q

Symptomatic management for ALS

A

dysphagia– diet modification, PEG, speech-language
Dysarthria– communication device
Thick mucous– humidifier, chest PT
dyspnea– chest PT, morphine
respiratory failure–non-invasive positive pressure ventilation, tracheotomy, ventilation

40
Q

Guillain-Barre Syndrome

A

Acute inflammatory demyelinating polyneuropathy

Onset is adulthood and initially rapidly progressive (within 24 hours - 4 weeks) but recovery occurs

41
Q

GBS variant: AIDP

A
  • demyelinating polyneuropathy
    - myelin more than axons
  • Sensorimotor GBS
    • progressive a-reflexic weakness, mild sensory changes, autonomic dysfunction, some CN deficits
42
Q

GBS variant: MFS

A
  • Normal NCV
  • Sensory changes
  • opthalmoplegia, ataxia, areflexia
43
Q

GBS variant: AMAN

A
  • pure motor GBS
  • Axonal polyneuropathy (motor axon involvement–not myelin)
  • CN rarely affected
44
Q

GBS variant: AMSAN

A
  • Severe variant of AMAN but with sensory deficits
  • axonal polyneuropathy
    • sensory and motor axons (not myelin)
  • primarily in Asia, Central & South America
45
Q

Pathology of GBS

A
  • Autoimmune response happens within 2 weeks of infection
  • antibodies made to eliminate virus
  • antigens of virus look like those on myelin nodes = molecular mimicry
  • antibodies attack Schwann cells
  • breaks down myelin
  • some attack node-axonal degeneration (bystander effect)
  • affects nerve roots and peripheral nerves
  • recovery: proximal to distal (remyelination is faster than regeneration)
46
Q

Phases of GBS

A

Acute: progressive, ascending, symmetrical weakness and sensory loss (distal to proximal)
Plateau phase
Recovery phase

47
Q

“Classic” features of GBS

A 
LE weakness
UE weakness
Areflexia
Paresthesia
Sensory loss
Dysautonomial (HR, BP, etc)
Oropharyngeal weakness
pain
respiratory failure
sphincter involvement
48
Q

key components for medical diagnosis of GBS

A 
lumbar puncture (presence of elevated albumin)
NCV (delays in latency suggesting demyelination)
EMG (suggests axonal degeneration)
49
Q

differential diagnosis for GBS

A 
Botulism
Lyme Disease
Tick Paralysis
Poliomyelitis
other Neuropathies (diphtheria, toxins)
50
Q

Medical management of GBS

A

admit to ICU
respiratory changes: when FVC is ~1.5 L –> tracheostomy & vent
psychoemotional support
immunomodulation (shortens disease duration)
-IVIG
-plasmapheresis/plasma exchange
- oral corticosteroids significantly slow recovery

51
Q

Movement system diagnosis for GBS

A

difficulty rolling, transitioning to sitting & standing, standing, walking…
secondary to weakness, sensory deficit, autonomic symptom

52
Q

Prognosis for GBS

A

return to motor/sensory takes months to years
(15% full recovery, 67% residual weakness, 10% severe weakness/sensory loss, 8% die in acute phase)
recovery occurs mainly during first year post-onset, however motor and sensory impairments are still detectable in 50% of patients at 2 years
(31% decrease grip strength, 7% unable to walk 10 m independently, 52% sensation affected)

53
Q

Interventions for GBS

A

avoid fatigue
after plateau phase and recovery has begun:
-short periods of non-fatiguing exercise (PROM/AAROM/AROM)
-increase activity if pt improves or not deteriorating after 1 wk
-exercise to promote functional activities; gait & balance, including endurance
-equipment: ambulatory aids, AFOs
-monitor neuro signs & ANS (tachy/bradychardia, facial flushing, labile BP, abnormal sweating, paresthesia) may suggest overwork

54
Q

Charcot Marie Tooth

A

Hereditary motor and sensory neuropathy
polyneuropathy impacting myelin or axons
distal, symmetrical “stocking glove” weakness and sensory loss
onset is childhood and initially rapidly progressive (within 24 hours- 4 months) but recovery occurs

55
Q

key components for medical diagnosis of CMT

A 
genetics
NCV (delays in latency suggest demyelination)
56
Q

Activity limitations in CMT

A

reduced walking

reduced hand function

57
Q

Common impairments in CMT

A

Paresthesias: cutaneous and proprioceptive
Muscle weakness: symmetrical with distal involvement, weak TA, weak Peroneals
Pes Cavus: elevation of medial longitudinal arch, progressing to clawing of toes (loss of intrinsic muscle innervation)

58
Q

Movement system diagnosis for CMT

A

difficulty running/jumping, climbing stairs, walking, standing, reaching and grasping…
…secondary to weakness, sensory loss, reduced muscle flexibility

59
Q

Intervention guidlines for CMT

A 
ROM
strengthening
balance training
task-oriented
AFOs
Gait: AD
adaptive home equipment for safety in tub/shower
60
Q

Myasthenia Gravis

A
  • decreased number and defective functioning of ACh receptor sites at neuromuscular junction
  • autoimmune condition where antibodies bind the ACh receptor
  • painless disorder of strength occurring during sustained or repetitive muscle contractions: loss of muscle power occurs in association with continuous effort
  • relatively uncommon; women: men = 2:1; 20-30 years for women, but 60-70 years for men
61
Q

Clinical signs of MG

A 
ptosis
diplopia
facial weakness
dysarthria
dysphagia
neck mm weakness
weak grip
62
Q

Lab tests used for diagnosis of MG

A 
IV tensilontest (short acting anti-AChE) --> reverse weakness
ACh receptor antibody titer
63
Q

treatment for MG

A

Mestinon (cholinesterase inhibitor), corticocosteroids, thymectomy

64
Q

Prognosis for MG

A

with treatment, most individuals with MG can significantly improve their muscle weakness and lead normal or nearly normal lives.
Sometimes, severe weakness of MG can cause respiratory failure, which requires immediate medical care.
Some cases of MG may go into remission–either temporarily or permanently–muscle weakness may disappear completely so that medications can be discontinued.
Stable, long-lasting complete remissions are the goal of thymectomy and may occur in about 50% of individuals who undergo the procedure.

65
Q

Interventions for MG

A

Muscle weakness worsens as affected mm are used repeatedly, therefore sx improve with rest. PT typically not involved.
Stable patients with MG may exercise following ‘rules’:
-exercise at your best time of day
-exercise at peak dose of meds
-exercise large, proximal mm groups for short periods of time, building up to only moderate intensity
Do NOT exceed moderate intensity exercise levels
-HR should not elevate > 30 bpm form baseline
-exercise should not cause you to become SOB
-Sx should not become worse during exercise
-should not be tired 2 hours after exercise
-should not have residual soreness day(s) post exercise
Postural exercises
Breathing exercises (abdominal); inspiratory trainer

Neuromuscular practice 2 (8 decks)

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