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.
What is the normal function of dystrophin?
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.
What is muscular dystrophy?
A group of genetic disorders characterized by progressive muscle weakness and degeneration due to mixed muscle cell hypertrophy, atrophy, and necrosis
What protein is defective in Duchenne Muscular Dystrophy?
Dystrophin.
What is the normal function of dystrophin?
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.
what is the pathophysiology of muscular dystrophy? (5 parts)
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
What are the early signs of Duchenne Muscular Dystrophy?
Delayed motor milestones
difficulty running or jumping frequent falls
Gower’s sign (using hands to push up from the floor).
What are some complications of muscular dystrophy?
Respiratory failure, cardiomyopathy, scoliosis, and contractures
manifestations of muscular dystrophy (10)
breathing problems
weak ineffective cough
(risk of infection)
heart problems (cardiomyopathy)
muscle weakness
trouble walking
muscle stiffness
stiff joints
trouble swallowing
incontinence
what is myasthenia gravis?
disorder of neuromuscular junction transmission due to antibody-mediated attacks (autoimmune)
What is the etiology of myasthenia gravis?
unknown (body own overrxn)
what is the pathophysiology of myasthenia gravis?
antibodies attack AcH receptors on postsynaptic cell + decrease in SA + widened synaptic space = impaired/delayed signal transmission = decreased muscle contraction
what is a myasthenia crisis?
sudden, acute onset of all symptoms
severe resp distress
caused by stress (emotional/physical)
s/s of myasthenia gravis (5)
weakness and fatigue (esp with exercise)
ptosis (drooping eyelids)
chewing/swallowing issues
weak limb mvmt
lower face m weakness
what are examples of mononeuropathy?
carpal tunnel syndrome
bell’s palsy
what is the etiology of carpal tunnel syndrome
Compression of median nerve with tendons under/through canal made by carpal bones and transverse carpal ligament
what is mononeuropathy?
peripheral nerve disorder that affects a single nerve, leading to weakness, numbness, or pain in the area it supplies
what is the patho of carpal tunnel syndrome? (2)
reduction in tunnel capacity
increase in volume of contents through tunnel (d/t inflammation and compressed nerve)
what are manifestations of carpal tunnel syndrome? (4)
pain in wrist/hand worse at night
parethesia
numbness fo thumb and first 2.5 digits of hand
weak grip (fine motor diff)
What are risk factors for developing Carpal Tunnel Syndrome?
Risk factors include repetitive hand use (e.g., typing, assembly line work), wrist trauma, obesity, pregnancy, hypothyroidism, and rheumatoid arthritis.
what are polyneuropathies?
affect multiple peripheral nerves throughout the body
diabetic neuropathy
gullian-barre syndrome
chronic alcholism
What is Guillain-Barré Syndrome (GBS)?
an acute autoimmune polyneuropathy where the immune system attacks the myelin sheath of peripheral nerves, leading to progressive muscle weakness and paralysis
What disease?
motor axonal degeneration and axonal degeneration of motor and sensory nerves
guillain-barre syndrome
what causes gullian-barre syndrome? (3)
Infiltration of mononuclear cells around capillaries of peripheral neurons
edema of endoneurial compartment
demyelination of ventral spinal roots
manifestations of gullian-barre syndrome? (11)
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