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Path Skeletal Muscle
KYCOM Block 12
44
Pathology
Graduate
12/12/2013

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Cards

Term
what are the three sheaths of skeletal m and what do they cover
Definition
epimysium: entire muscle
perimesium: surrounds bundles of fibers
endomesium: surrounds fibers
Term
what three clinicl changes occur in muscle atrophy
Definition
decreased muscle mass, strength, bone density
Term
causes of m atrophy
Definition
disuse of muscle occurs after immobility and aging
Term
how is m atrophy treated
Definition
usually with exercise
Term
how does m atrophy show histologically
Definition
few fibrils
increased fat
Term
what are three conditions that cause m atrophy
Definition
cachexia
congestive heart failure
liver disease
Term
define cachexia, what are three causes
Definition
body wasting syndrome, severe generalized atrophy

cancer, chemo, AIDS
Term
what occurs clinically in m hypertrophy
Definition
increase in mass of a muscle due to increased stimuli
Term
what are some histological signs of hypertrophy
Definition
large fibrils
more numerous fibrils
Term
what are two conditions that cause m hypertrophy
Definition
exercise hypertrophy
acromeagly
Term
what is affected in acromeagly
Definition
type 1 skeletal m fibers
Term
what are the inflammatory myopathies
Definition
infectious myositis
non-infectious myositis (autoimmune)
Term
what are the non-infectious myopathies
Definition
polymositis
dermatositis
inclusion body myositis
Term
define polymositis
Definition
chronic inflammation of the muscles
Term
who gets polymositis
Definition
adults
Term
where is polymositis located
Definition
hip, thigh, upper arm, upper back, shoulder, beck
Term
clinical signs of polymositis
Definition
pain, weaness in bilateral proximal muscles (shoulder and palvis)

hip extensors make it difficult to stand from chair or ascend stairs

dysphagia

low fever

peripherial lymphadenopathy
Term
histological signs of polymositis
Definition
endomysial (around fibers) lymphocitic inflammation between muscle fibers

inflammatory cells invade fibers which become more round

skeletal muscle fiber degeneration and regeneration
Term
how is polymositis diagnosed
Definition
CK elevation (marer of m inflammation and damge)
EMG alteration
positive m biopsy
Term
define dermatatositis
Definition
CT disease related to polymositis
additionally affects joints, esophagus, lungs, sometimes heart
Term
clinical signs of dermatotositis
Definition
bilateral proximal m weakness and pain
helitrope/iliac rash
peri-orbital edema
telangestica
gottron lesions
small Ca deposits under skin
25% of adult pt prone to cancer
Term
what and where is a helitrope / iliac rash
Definition
red purple rash on upper eyelid with itching and swelling, esp over upper eyelids in dermatositis
Term
what and where is a guttron lesion
Definition
scaly red erruptions on knuckles, elbows, knees in dermatositis
Term
histological signs of dermatositis
Definition
inflammatory cells around blood vessels and perimysial (around bundles)

skeletal m fiber degeneration and regeneration

x-ray dystrophic carcifications in m (specks of white)
Term
how is dermatositis diagnosed
Definition
increased CK
EMG abnormalities
liver enzymes (creatining phosphokinase) assess progression
confirmed by m biopsy
Term
who gets dermatositis
Definition
children and adults
Term
who gets inclusion body myositis
Definition
adults >50yo
Term
define inclusion body myositis
Definition
slow progressive m weakness asymmatrical
Term
clinical signs of inclusion body myositis
Definition
slow progressive weakness of mucles
asymmetric distal m weakness
life expectancy isnt significantly effected
may require cane or wheel chair for long distances
Term
histological signs of inclusion body myositis
Definition
cytoplasmic vacolues with basophillic granules and amyloid

inflammatory cells invate m tissue
Term
diagnostic digns of inclusion body myositis
Definition
elevated CK
EMG abnormalities
muscle biopsy
Term
general cause of muscular dystrophies
Definition
heterogrnous inherited disorders
Term
who gets muscular dystrophies
Definition
begin in childhood
Term
in general what is the histology of muscular dystrophy
Definition
replacement of muscle tissue by fibrofatty tissue (distinguishes dystrophies from myopathies
Term
what are the types of muscular dystrophy
Definition
myotonic dystrophy
duchennes
beckers
Term
what is the most common and most severe muscular dystrophy
Definition
duchenne
Term
what is the most common less severe muscular dystrophy
Definition
becker
Term
what is the cause of duchenne and backer
Definition
XP21 gene that codes for dystrophin which helps with contraction of actin and myosin (have no dystrophin in duchenne some in becker)

1/3 have de novo mutations
2/3 have obligate female carriers (x-linked) that are usually asymptomatic but may have elevated CK or minor histological changes
Term
what is the timeline of symptoms of duchennes muscular dystrophy
Definition
babies normal at birth
normal eary motor milestones
walking delayed
wheel chair by 10-12yo
can die in 20s from respiratory insufficiency, pneumonia, cardiac decomposition
Term
clinical signs of duchenne MD
Definition
weakness begining in pelvic girdle, ascends to shoulder girdle

pseudohypertrophy of calf muscles

CT making them larger

gower's sign

heart failure, arrhythmia

cognative impairment (maybe MR)
Term
histology of ducnenns MD
Definition
fat in CT in calf muscles
elevated CK
variation in muscle fiber sizes
increased endomysial CT
regenerating fibers
Term
timeline of beckers muscular dystrophy
Definition
patients live into 30s
less severe
Term
what causes metabolic myopathy
Definition
glycogen storage diseases
mitochondrial myopathies
Term
what are diseases of NMJ
Definition
myasthenia gravis
lambert eaton syndrome
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