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50 Cards in this Set

  • Front
  • Back
Describe denervation atrophy
Any process that affects anterior horn cells in PNS
manifestation- spinal muscular atrophy
Most common form of denervation atrophy
Werdnig- Hoffman disease
onset- birth to 4 months, death within 3 years due to muscle atrophy
Describe muscular dystrophies
inherited disorders, often beginning in childhood, characterized clinically by progressive muscle weakness and wasting. Histologically, muscle fibers replaced by fibrofatty tissue
Which muscular dystrophy is more severe - DMD or BMD
What is most common type of muscular dystropy
DMD - 1 per 3500 live male births
When is DMD clinically manifested
By age of 5, with weakness leading to wheelchair dependness by age of 10-12,death in early 20's
Do females have DMD
Females are carriers, but they are asymptomatic but often have elevated serum creatin kinase.
-females are at risk of developing dilated cardiomyopathy later in life
What is the role of dystrophin in DMD and BMD
in DMD there is no or minimal dystrophin
in BMD there is abnormal diminished dystrophin
Weakness in DMD begins at
Pelvic girdle, and progresses to shoulder
Enlargement of calf muscles associated with weakness in DMD is called
Pseudohypertrophy - caused by initial increase in muscle fibers and then by replacement with fat and connective tissue
Which plasma protein is elevated in DMD
Serum creatin kinase is elevated during first decade of life, and then returns to normal
Death in DMD results from
Respiratory insufficiency, pulmonary infection and cardiac decompensation
Boys with BMD develop symptoms earlier or later then DMD
Later, with slower progression, and can live normal life span
Ion channel myopathies
Group of familial disorders characterized by myotonia, relapsing episodes of hypotonic paralysis(induced by cold, vigorous exercise or high carbohydrate meal)
Malignant hyperthermia
Rare clinical symdrome charaterized by dramatic hypermetabolic state - tachycardia, tachypnea, muscle spasms, triggered by induction of anesthesia
Caused by mutation of gene coding for voltage gated Ca channel
Name 3 inflammatory myopathies
Dermatomyositis, polymyositis, inclusion body myositis
Inflammation of both skin and muscle
Distinctive skin rash in dermatomyositis
Lilac discoloration of upper eyelids with periorbital edema
Also can be accompanied by Grotton lesions - scaling red eruptions at knuckles, elbows, knees
Muscular involvement in dermatomyositis
Slow onset
Affects proximal muscles first
40% correlation with cancer
Polymyositis differs from DM only in
There is no skin involvement in PM, only proximal muscle involvement
Inclusion body myositis differs from DM and PM in
distal muscle involvement, rather then proximal
Ethanol myopathy
Binge drinking of alcohol produces acute rhabdomyolysis with accompanying myoglobinuria, which may lead to renal failure
Drug induced myopathy
Steroid myopathy, characterized by muscle fiber atrophy, predominantly affecting type II fibers
Chloroquine myopathy
Drug originally used to treat malaria can produce myopathy
Myasthenia Gravis
Muscle disease caused by immune mediated loss of Ach receptors
MG prevalence
1 in 100 000
What else is often found in patients with MG
Thymic hyperplasia in 65% and thymoma in 15 %
How does MG present clinically
Ptosis and diplopia (double vision), generalized weakness
Which drugs are best to treat MG
Ach esterase inhibitors
Lambert Eaton syndrome
Disease of NMJ, paraneoplastic process commonly assoicated with small cell lung CA (60%)
Patients develop proximal muscle weakness along wih autonomic dysfunction
Two processes of repair of tissue damage
Regeneration and healing
Definition of regeneration
Growth of cells and tissue to replace lost structures (liver + kidney)
Definition of healing
Response to

Regeneration requires
intact connective tissue scaffold
Cell cycle
G1 presynthetic
S - DNA synthesis
Labile tissue
Cells proliferate throughout life, replacing those that are destroyed - surface epithelia - skin, vagina, cervix, lining of glands
Quiescent (stable) tissue
low level of replication, but can undergo rapid division under certain stimulus, in G0 but can enter G1 - parenchymal cells of liver, kidney, pancreas, fibroblasts
Nondividing (permanent) tissue
Cells that have left cell cycle and cannot undergo mitosis in postnatal period

Neurons, cardiac and skeletal muscle cells
Difference between embryonic and embryonic stem cells
EMbryonic are pluripotent
Which growth factor causes migration and proliferation of fibroblasts
PDGF - platelet deived GF
Can liver regenerate?
Yes --> even with 70% removed
3 processes of formation of scar
-emigration and proliferation of fibroblasts
-deposition of ECM
-tissue remodeling
Best example of wound healing by first intention
surgical incision
Within 24 hours
neutrophils appear at the margin, formation of thin epithelial layer
By day 3
replacement of neutrophils by macrophages
appearance of granulation tissue
collagen fibers at margins of incision
epithelial proliferation thickens epidermal layer
Day 5
incisional space is filled with granulation tissue
maximal neovascularization
Abundant collagen fibers
Epidermis recovers normal thickness
2nd week
Continued accumulation of collagen and fibroblasts
Increased vasculariy and edema disappear
Scar has formed, covered by normal epidermis with no inflammation
Are dermal appendages recover in wound healing
No, permanently lost
Healing by 2nd intention
-more intense inflammatory reaction
-larger amount of granulation tissue
-wound contraction
-scar formation