Motor Disorders Flashcards

Describe the pathophysiology, causes, and manifestations of muscular dystrophy and myasthenia gravis. Compare and contrast the causes and manifestations of peripheral nerve disorders of carpal tunnel, Guillain-Barre syndrome, back pain, herniated intervertebral disk. Differentiate the causes and manifestations of selected UMN and LMN disorders including Amyotrophic Lateral Sclerosis and Multiple Sclerosis. Discuss the causes and manifestations of vertebral and spinal cord injuries.

1
Q

What is the normal function of dystrophin?

A

Dystrophin is a protein that helps stabilize muscle cell membranes by linking the cytoskeleton to the extracellular matrix, protecting muscle fibers from damage during contraction.

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2
Q

What is muscular dystrophy?

A

A group of genetic disorders characterized by progressive muscle weakness and degeneration due to mixed muscle cell hypertrophy, atrophy, and necrosis

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3
Q

What protein is defective in Duchenne Muscular Dystrophy?

A

Dystrophin.

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4
Q

What is the normal function of dystrophin?

A

Dystrophin is a protein that helps stabilize muscle cell membranes by linking the cytoskeleton to the extracellular matrix, protecting muscle fibers from damage during contraction.

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5
Q

what is the pathophysiology of muscular dystrophy? (5 parts)

A

dystrophin integrity is disrupted = increased fragility of dystrophic muscle + excess Ca INFLUX + release of enzymes (CK) into blood = necrosis of muscle = fat and CT replace muscle (psuedohypertrophy) = increase in muscle size and muscle weakness

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6
Q

What are the early signs of Duchenne Muscular Dystrophy?

A

Delayed motor milestones
difficulty running or jumping frequent falls
Gower’s sign (using hands to push up from the floor).

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7
Q

What are some complications of muscular dystrophy?

A

Respiratory failure, cardiomyopathy, scoliosis, and contractures

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8
Q

manifestations of muscular dystrophy (10)

A

breathing problems
weak ineffective cough
(risk of infection)
heart problems (cardiomyopathy)
muscle weakness
trouble walking
muscle stiffness
stiff joints
trouble swallowing
incontinence

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9
Q

what is myasthenia gravis?

A

disorder of neuromuscular junction transmission due to antibody-mediated attacks (autoimmune)

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10
Q

What is the etiology of myasthenia gravis?

A

unknown (body own overrxn)

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11
Q

what is the pathophysiology of myasthenia gravis?

A

antibodies attack AcH receptors on postsynaptic cell + decrease in SA + widened synaptic space = impaired/delayed signal transmission = decreased muscle contraction

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12
Q

what is a myasthenia crisis?

A

sudden, acute onset of all symptoms

severe resp distress

caused by stress (emotional/physical)

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13
Q

s/s of myasthenia gravis (5)

A

weakness and fatigue (esp with exercise)

ptosis (drooping eyelids)

chewing/swallowing issues

weak limb mvmt

lower face m weakness

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14
Q

what are examples of mononeuropathy?

A

carpal tunnel syndrome
bell’s palsy

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15
Q

what is the etiology of carpal tunnel syndrome

A

Compression of median nerve with tendons under/through canal made by carpal bones and transverse carpal ligament

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16
Q

what is mononeuropathy?

A

peripheral nerve disorder that affects a single nerve, leading to weakness, numbness, or pain in the area it supplies

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17
Q

what is the patho of carpal tunnel syndrome? (2)

A

reduction in tunnel capacity

increase in volume of contents through tunnel (d/t inflammation and compressed nerve)

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18
Q

what are manifestations of carpal tunnel syndrome? (4)

A

pain in wrist/hand worse at night

parethesia

numbness fo thumb and first 2.5 digits of hand

weak grip (fine motor diff)

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19
Q

What are risk factors for developing Carpal Tunnel Syndrome?

A

Risk factors include repetitive hand use (e.g., typing, assembly line work), wrist trauma, obesity, pregnancy, hypothyroidism, and rheumatoid arthritis.

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20
Q

what are polyneuropathies?

A

affect multiple peripheral nerves throughout the body

diabetic neuropathy
gullian-barre syndrome
chronic alcholism

21
Q

What is Guillain-Barré Syndrome (GBS)?

A

an acute autoimmune polyneuropathy where the immune system attacks the myelin sheath of peripheral nerves, leading to progressive muscle weakness and paralysis

22
Q

What disease?
motor axonal degeneration and axonal degeneration of motor and sensory nerves

A

guillain-barre syndrome

23
Q

what causes gullian-barre syndrome? (3)

A

Infiltration of mononuclear cells around capillaries of peripheral neurons

edema of endoneurial compartment

demyelination of ventral spinal roots

24
Q

manifestations of gullian-barre syndrome? (11)

A

porgressive ASCENDING muscle weakness of limbs

flaccid paralysis (symmetric)

parethesia

numbness

loss of motor function

ANS: postural hypotension, arrhythmia, facial flushing, abnormalities, sweat, and urine retention

25
Risk factors of back pain: (4)
heavy lifting smoking obesity depressive symptoms
26
etiology/cause of back pain (3)
interrelated issues: spinal strucutres and spinal nerve roots musculoligamentous injuries age-related degenerative changes in intervertebral discs and facet joints
27
determining diagnosis of back pain
history/physical neuro exam
28
what is a herniated disc?
when the nucleus pulposus (soft inner gel) of an intervertebral disc pushes through a tear in the annulus fibrosus (outer ring). This can compress nearby spinal nerves
29
What is the etiology/cause of a herniated intervertebral disk?
trauma age (center loses elasticity) more common in cervical/lumbar
30
How does general back pain differ from a herniated intervertebral disc?
General back pain is often due to muscle strain, poor posture, or degenerative changes and usually improves with rest and conservative treatment. Herniated disc pain is more severe, can radiate down the arms or legs (radiculopathy), and may cause numbness, tingling, or weakness due to nerve compression. Symptoms worsen with movement, coughing, or sneezing.
31
What is Amyotrophic Lateral Sclerosis (ALS)?
progressive neurodegenerative disease that affects: upper (cerebral cortex) motor neurons lower motor neurons motor nuclei of brain stem
32
ALS is more extensive in _______ parts of affected tracts
distal
33
ALS is a _________ disease so the pt has no __________ issues
functional cognitive ex) three dolls Crim Minds ep
34
What is the pathophysiology of ALS?
degeneration of motor neurons in UMN and LMN = impaired nerve signaling, resulting in muscle atrophy, weakness, and loss of voluntary movement
35
what is the etiology of ALS?
uncertain
36
manifestations of ALS UMN: (3) UMN or LMN: (2) LMN: (4)
UMN: weakness, spasticity, impaired fine motor control both: dysphagia or dysarthria LMN: fasciculations, weakness, muscle atrophy, hyporeflexia
37
What is Multiple Sclerosis (MS)?
chronic autoimmune disease that attacks and destroys the myelin sheath of neurons in the central nervous system (CNS) CNS spared
38
What is the pathophysiology of MS? (3)
1. Demyelination – Myelin is damaged, slowing nerve impulses. 2. Inflammation – Immune cells (T-cells, B-cells) attack myelin and oligodendrocytes. 3. Axonal degeneration – Neurons lose their ability to transmit signals effectively, leading to permanent disability over time.
39
What are the 4 different types of MS?
Relapsing-Remitting MS (RRMS) – Most common (85% of cases), involves attacks (relapses) followed by partial recovery (remission). Secondary Progressive MS (SPMS) – Begins as RRMS but worsens progressively over time. Primary Progressive MS (PPMS) – Gradual worsening from onset, without relapses/remissions. Progressive-Relapsing MS (PRMS) – Rare, steadily worsening with occasional acute relapses.
40
what are the manifestations of MS?
optic nerve: visual field corticobulbar tracts: speech/swallowing corticospinal tracts: muscle strength cerebellar tracts: gait, coordination spinocerebellar tracts: balance medial longitudinal fasciculus: conjugate gaze function posterior cell columns of spinal cord: position, vibratory sensation
41
what is a vertebral/spinal cord injury?
damage to neural elements of the spinal cord fractures, dislocations, subluxations
42
what are the etiology/cause of vertebral/spinal cord?
MVC falls violence (GSW sports compression, flexion, extension
43
SCI's commonly involve ______ and _______ function
sensory motor
44
with SCI's , what is spinal shock?
when there is injury to spinal cord = inflammation/swelling = further comrpession = more/severe symptoms wait a bit to see the actual damage
45
C1 SCI
little to no sensation or control of head and neck no diaphrga, control = continuous ventilation very dependent (dressing/eating/toilet) wheelchair
46
C5 SCI
full head and neck control shoulder strength elbow flexion assistance needed (dressing/eating/toilet) electronic wheelchair
47
T1-T5 SCI
full hand and finger control use of intercostal and thoracic muscles independent (dressing/eating/toilet) manual wheelchair
48
SCI T6 or above can lead to: ______ NS disruption (3) disruption of: _______, _____ and sex disruption of other (3)
autonomic = vasovagal response, autonomic dysreflexia, postural hypotension bladder and bowel temperature regulation (no receptors) DVT and edema (dec acitivty) skin integrity (immbolie = ulcers)