Sosnowski Myopathies and Muscle Disorders Flashcards
this stain brings out slow and fast twitch muscle (dark and light stains) checkerboard pattern
NADH
inflammation of what tissue is pathologic (disease)
endomysium
surrounds single axon
endoneurium
inflammation of what tissue (nervous) is pathologic (disease)
endoneurium
surrounds groups of axons and blood vessels
perineurium
surrounds entire nerve
epineurium
myocytes without neuronal input become small until innervated by another type of neuron indicative of what
nerve damage
problem with muscle itself
myopathy
axonal degeneration that leads to angular atrophic (pointed) muscle fibers
neurogenic myopathy
happens when nerve damage continues
fiber type grouping atrophy
a lot of CT b/t muscle fibers and makes them small and round
myopathic
a demyelinating disorder thats deals with a problem with Schwann cells
chronic inflammatory demyelinating polyneuropathy
vision loss, muscle weakness, stiffness, spasms; demyelination and remyelination of peripheral nerves “onion bulb formation”
chronic inflammatory demyelinating polyneuropathy (CIDP)
most common damaged nerves in legs and feet; pain and numbness, weakness and loss of reflexes
diabetic neuropathy
histopathology shows amyloid deposition in endoneurium
diabetic neuropathy
any protein in an organ that malfunctions
amyloid
inflammatory neuropathy
inflammatory disorder of skin (rash) followed by myositis; perifascicular atrophy (rash around eyes followed by muscle weakness)
dermatomyositis
this has high association with a new cancer in adults; paraneoplastic syndrome
dermatomyositis
caused by lymphocytes eating up muscles and inflammation in CT around each muscle fiber
polymyositis (chronic myositis)
progressive muscle weakness and wasting; begin in childhood
muscular dystrophies
muscle fibers slowly replaced by fibrosis and adipose tissue
muscular dystrophies
caused by mutation in dystrophin gene (which forms an interface b/t sarcomere and extracellular CT matrix)
X-linked Duchenne and Becker muscle dystrophy
a finding of this is pseudohypertrophy of calf muscles due to fat infiltration of muscle
duchenne and becker muscular dystrophy
deals with breathing problems and infections and gowers sign
duchenne muscular dystrophy
people with this muscular dystrophy live longer and will develop heart problems later
becker muscular dystrophy
this would have 50% normal fibers stained compared to just one normal stained
beckers
weakness and hypotonia at or shortly after birth; due to rod shaped intracytoplasmic inclusions
nemaline myopathy
maternal inheritance, ragged red fibers, epilepsy
MERRF
diseases in which motor neurons undergo degeneration and die; starts at brain and ends at muscles
motor neuron disease
deals with damage to both upper and lower motor neurons
ALS
die from respiratory failure; sx’s are overlooked including cramps, weakness, muscle stiffness and slurred speech
ALS
UMN damage dealing with difficulty swallowing
dysphagia
UMN damage dealing with difficulty speaking
dysarthria
muscle spasticity and hyperreflexia are what motor neuron damage
UMN
weakness and atrophy, cramping and fasciculations are what motor neuron damage
LMN
see pale spots in brain CT
ALS
affects lower motor neurons only and muscle weakness; trapped in body that won’t move; mutation in SMN gene
spinal muscular atrophy
weakness and rapid fatigue of skeletal muscles; Ach receptor is attacked by autoantibodies and Ach can’t bind
Myasthenia Gravis
smooth muscle tumor, benign
leiomyoma
smooth muscle tumor, malignant
leiomyosarcoma
skeletal muscle tumor, benign
rhabdomyoma
skeletal muscle tumor, malignant
rhabdomyosarcoma
uterine leiomyoma
firm and stains + for desmin
rhabdomyosarcoma
liposarcoma
