Sosnowski Myopathies and Muscle Disorders

Question 1

this stain brings out slow and fast twitch muscle (dark and light stains) checkerboard pattern

Answer 1

NADH

Question 2

inflammation of what tissue is pathologic (disease)

Answer 2

endomysium

Question 3

surrounds single axon

Answer 3

endoneurium

Question 4

inflammation of what tissue (nervous) is pathologic (disease)

Answer 4

endoneurium

Question 5

surrounds groups of axons and blood vessels

Answer 5

perineurium

Question 6

surrounds entire nerve

Answer 6

epineurium

Question 7

myocytes without neuronal input become small until innervated by another type of neuron indicative of what

Answer 7

nerve damage

Question 8

problem with muscle itself

Answer 8

myopathy

Question 9

axonal degeneration that leads to angular atrophic (pointed) muscle fibers

Answer 9

neurogenic myopathy

Question 10

happens when nerve damage continues

Answer 10

fiber type grouping atrophy

Question 11

a lot of CT b/t muscle fibers and makes them small and round

Answer 11

myopathic

Question 12

a demyelinating disorder thats deals with a problem with Schwann cells

Answer 12

chronic inflammatory demyelinating polyneuropathy

Question 13

vision loss, muscle weakness, stiffness, spasms; demyelination and remyelination of peripheral nerves “onion bulb formation”

Answer 13

chronic inflammatory demyelinating polyneuropathy (CIDP)

Question 14

most common damaged nerves in legs and feet; pain and numbness, weakness and loss of reflexes

Answer 14

diabetic neuropathy

Question 15

histopathology shows amyloid deposition in endoneurium

Answer 15

diabetic neuropathy

Question 16

any protein in an organ that malfunctions

Answer 16

amyloid

Question 17

Answer 17

inflammatory neuropathy

Question 18

inflammatory disorder of skin (rash) followed by myositis; perifascicular atrophy (rash around eyes followed by muscle weakness)

Answer 18

dermatomyositis

Question 19

this has high association with a new cancer in adults; paraneoplastic syndrome

Answer 19

dermatomyositis

Question 20

caused by lymphocytes eating up muscles and inflammation in CT around each muscle fiber

Answer 20

polymyositis (chronic myositis)

Question 21

progressive muscle weakness and wasting; begin in childhood

Answer 21

muscular dystrophies

Question 22

muscle fibers slowly replaced by fibrosis and adipose tissue

Answer 22

muscular dystrophies

Question 23

caused by mutation in dystrophin gene (which forms an interface b/t sarcomere and extracellular CT matrix)

Answer 23

X-linked Duchenne and Becker muscle dystrophy

Question 24

a finding of this is pseudohypertrophy of calf muscles due to fat infiltration of muscle

Answer 24

duchenne and becker muscular dystrophy

Question 25

deals with breathing problems and infections and gowers sign

Answer 25

duchenne muscular dystrophy

Question 26

people with this muscular dystrophy live longer and will develop heart problems later

Answer 26

becker muscular dystrophy

Question 27

this would have 50% normal fibers stained compared to just one normal stained

Answer 27

beckers

Question 28

weakness and hypotonia at or shortly after birth; due to rod shaped intracytoplasmic inclusions

Answer 28

nemaline myopathy

Question 29

maternal inheritance, ragged red fibers, epilepsy

Answer 29

MERRF

Question 30

diseases in which motor neurons undergo degeneration and die; starts at brain and ends at muscles

Answer 30

motor neuron disease

Question 31

deals with damage to both upper and lower motor neurons

Answer 31

ALS

Question 32

die from respiratory failure; sx's are overlooked including cramps, weakness, muscle stiffness and slurred speech

Answer 32

ALS

Question 33

UMN damage dealing with difficulty swallowing

Answer 33

dysphagia

Question 34

UMN damage dealing with difficulty speaking

Answer 34

dysarthria

Question 35

muscle spasticity and hyperreflexia are what motor neuron damage

Answer 35

UMN

Question 36

weakness and atrophy, cramping and fasciculations are what motor neuron damage

Answer 36

LMN

Question 37

see pale spots in brain CT

Answer 37

ALS

Question 38

affects lower motor neurons only and muscle weakness; trapped in body that won't move; mutation in SMN gene

Answer 38

spinal muscular atrophy

Question 39

weakness and rapid fatigue of skeletal muscles; Ach receptor is attacked by autoantibodies and Ach can't bind

Answer 39

Myasthenia Gravis

Question 40

smooth muscle tumor, benign

Answer 40

leiomyoma

Question 41

smooth muscle tumor, malignant

Answer 41

leiomyosarcoma

Question 42

skeletal muscle tumor, benign

Answer 42

rhabdomyoma

Question 43

skeletal muscle tumor, malignant

Answer 43

rhabdomyosarcoma

Question 44

uterine leiomyoma

Answer 44

Question 45

firm and stains + for desmin

Answer 45

Question 46

rhabdomyosarcoma

Answer 46

Question 47

liposarcoma

Answer 47