Pathology of Skeletal Muscle - Part 2 Flashcards
Arthrogryposis is a _______
condition in ______ fetuses
(______ & _____) and _______
characterized by rigid ______ and ____ in abnormal grotesque
positions. Unclear etiology but usually
associated with _________
abnormalities of the _____ system
(e.g.: spina bifida, syringomyelia etc.,).
What is the most common cause? What are some less common causes?
congenital, aborted, cattle, sheep, stillborns, joints, limbs, developmental, nervous
Lack of innervation (denervation), leading to muscle hypoplasia, is the most common cause.
May be the result of ingestion of toxins or in-utero viral infections (e.g. Akabane disease, Cache Valley fever and blue tongue cause clusters of arthrogryposis in cattle and sheep)
Illanes notes: Easy to diagnose b/c animals usually have ? hair. Associated with developmental anomalies in CNS –> lack of innervation of muscles in limbs. Can also be associated with toxins. If you have several animals born with this issue, may have to think that this may not be an isolated incident. A viral infection may cause this, or rule out a viral toxin from a plant.
This animal is suffering from?
Arthrogryposis
Muscle Dystrophy is _______ determined, ________ degenerative disease of muscle. Regenerative attempts are _______ or _____.
Described in multiple species but most commonly occurs in ______ ______. The ______, _____, and ____ are animal models for Duchenne’s muscular dystrophy –> affected animals
and people lack _______, a _______ protein that ______ muscle and protects it from _______.
genetically, progressive, inadequate, absent, golden retrievers, mouse, dog, cat, dystrophin, cytoskeletal, strengthens, injury
Explain why muscular dystrophy is most commonly found in golden retrievers compared to other dog breeds?
An X-linked dystrophin gene defect has been identified in Golden Retrievers.
Similar disorders have been diagnosed in other breeds including Irish Terriers,
Samoyeds, Labrador Retrievers, Rottweilers, Dalmatians etc.) Muscle
degeneration/ necrosis and fibrosis lead to progressive clinical disability in young
male dogs. Dogs are considered the best animal model for human muscular
dystrophy. The heart and esophagus are also involved (progressive
cardiomyopathy and megaesophagus).
Note muscle wasting and a characteristic outward rotation (splaying) of fore limbs
(image). Death may be the result of aspiration pneumonia or cardio-respiratory
failure.
Illanes notes: Very well documented in golden retrievers. Outward rotation is very characteristic. Also reported in other breeds, but GR very common.
Present in skeletal muscle and heart, leading to significant cardio myopathy and can die of cardio respiratory failure. Diaphragm is involved. Esophagus of dogs there is skeletal muscle as well so you can also have megaesophagus. Possible result of megaesophagus –> Aspiration pneumonia. Animal usually has to be euthanized.
This animal is suffering from?
Golden Retriever suffering from muscular dystrophy
Equine Polysaccharide Storage Myopathy (EPSSM) is an ________ disease reported mainly in what horse breeds?
Some animals are ________, others exhibit ____ abnormalities (_____
limb lameness) and ______ muscle mass. Microscopically there is an accumulation of ____ ______ material
(_______) in ______ muscle associated with
degeneration/ necrosis of type ___ fibers.
• An underlying ______ _____ disorder is also suspected.
inherited, QH, Arabian horses,
Morgans, Ponies and draft-related breeds.
asymptomatic, gate, hind, reduced, PAS positive, polysaccharides, skeletal, II, carbohydrate metabolic
Illanes notes: mainly effects quarter horses.
Only thing you can see if an animal is effected or suspected of this condition, take muscle bipsy and see accumulation of poly saccharides. PAS stain will show accumulation of polysaccharides in skeletal muscle and the condition will result in degeneration and necrosis of type 2 fibers b/c undergo glycolytic metabolism. May not present clinical signs, some may have lameness, muscle fibrilation? sometimes. Important condition in horses. Etiology is not completely elucidated.
- Porcine Stress Syndrome (porcine malignant hyperthermia
– pale soft exudative [PSE]pork ) is what type of disorder? What animals does it affect? What mutation does this syndrome cause? - What is this syndrome characterized by?
- What specifically about the animals effected makes them most susceptible to disease?
- Describe the pathogenesis of PSS.
- Inherited disorder of domestic pigs (Pietrain, Yorkshire,
Duroc, Landrace etc.,) – single point mutation in the skeletal
muscle ryanodine receptor gene (ryr1). - Hypermetabolic syndrome characterized by dyspnea, muscle
rigidity and convulsions, hyperthermia, lactic acidosis,
hyperkalemia, cardiovascular collapse and death. - Heavy-muscle pigs are more susceptible to clinical disease,
- Pathogenesis related to the uptake, storage and release of Ca
ions –> the ryanodine receptor is a Ca release channel –> uncontrolled Ca release leads to excessive muscle contraction
and heat production (hyperthermia).
Illanes notes: Animals with this Ryr1 gene defect there is an uncontrolled Ca release which leads to muscle contraction especially in situations of stress and these animals can develop hyperthermia and die. Can also suffer from seizures. Reported sometimes in slaughter house, because pigs are very smart. Once people knew that this was a genetic defect, they were able to test for it and remove it from the breeding line.
Describe the image below
Porcine Stress Syndrome (PSS) – Pale swollen and “wet” muscles (longissimus dorsis)
Extramuscular lesions in pigs that died of PSS are those of acute heart failure: pulmonary edema and congestion, hydropericardium, hydrothorax and hepatic congestion.
Illanes notes: Usually died acutely of acute cardio respt failure, will see pulmonary edema, accumualtion of fluid in pericardial sac, pericardium, and thorax = hydrothorax.
main lesion in muscles.
In cases of PSS, muscles commonly affected are
the ?
muscles the back (longissimus dorsis & psoas),
thigh, and shoulders.
Microscopically, PSS is characterized by?
Acute myofibre degeneration characterized by interstitial edema, myofiber swelling and fragmentation – PSS, AV as well as segmental hypercontraction with “retraction cap.”
Illanes notes:
Animals do not survive very long. If you look at muscle grossly affected, you will see acute muscle degeneration. Swelling of muscle fibers, loss of straitions, see fragmentation of cytoplasm of cells, presence of interstitial edema. See image to right. Retraction caps due to hyperthermia from muscle contraction (see image above).
Nutritional Myopathy (White Muscle Disease)
1. Is the result of?
2. Primarily a disease that affects?
3. What parts of the body are mainly effected?
4. Most common in what species?
5. White Muscle Disease can coexist with what other disease in pigs?
6. Describe the pathogenesis of this disease.
- The result of Vit E/ Se deficiency
- Primarily a disease of young animals; Usually affects the rapidly growing and best nourished animals in the herd/ flock
- In addition to skeletal muscle the heart may also be affected
- Most common in sheep, cattle and pigs
- In pigs can co-exist with other Vit E/ Selenium syndromes (hepatosis dietetica, mulberry heart disease).
- Pathogenesis: Oxydative damage (lipoperoxydation of cell membranes)
due to deficiency in oxygen-radical scavengers: Vit E and selenium- containing enzymes like glutathione peroxidase/ reductase.
Illanes Notes: Not confined to ruminants and pigs, but seen in these animals more commonly.
Lesions produced in heart (marble heart disease), liver (hepatosis diatectica), in pigs.
Normally affect young animals growing rapidly. high metabolic rate. normal metabolism results in normal oxidative damagebut we have vitamin e and seleniumthat protect us from further damage and myofiber degeneration of skeletal muscle and heart. Animals affected may be lame, reluctant to move. In ruminants, may see respiratory distress associated with involved of diaphgram.
Calcification is a frequent outcome of _____ ______ in cases of
Vit E/ Se deficiency –> ____ muscles –> “_____ ______ disease”
muscle degeneration, pale, white muscle
Nutritional Myopathy: White Muscle Disease
1. In cattle, WMD primarily affects?
2. Common clinical signs?
3. What muscles are commonly affected?
4. In cases in which skeletal muscle lesions predominate they are most severe in?
5. What muscles are most affected in nursing calves?
- In cattle primarily affects calves 1-6 months of age.
- Stiffness and dyspnea are common clinical signs.
- Intercostal muscles and diaphragm (type I fibers) are often affected even when there is little involvement of other skeletal muscles.
- Muscles of the thigh and shoulders.
- In nursing calves, the tongue and neck muscles are often affected (more active muscles –> Increase production of free radicals).
Describe the image below
White Muscle Disease
In young calves or lambs, muscles more affected ar eneck muscles and tongue muscles because they are used for suckling. The more active muscles are the ones that are more suscpetible to oxidative damage and you will find lesions here.
Muscle is more pale, granular appearance (mineralization - gritty sensation due to calcification).
Describe this image below
Foal nutritional myopathy
Describe this image below
Nutritional myopathy
manifestation of injury in muscles are the same:
myofiber swelling, loss of straitions, fagmentation of sarcoplasm, areas of regenerative ?, etc.
Describe this image below
Nutritional myopathy
Vitamin e and selenium deficiency can produce lesions in heart. If you see a lamb wiht this nutritional myopathy and it has lesiosn in the heart, for some reason are confined to the right side as seen here. Pale muscle in the right side, slightly raised whitish region of mineralization.
Describe the image below
Calf, “white muscle disease”
In calves, region affected is on the left
Describe the image belowmuscle damage
Calf, 2 days-old, heart, nutritional myopathy
may be born from vitamin e/selenium deficient mothers, born with condition, and then when move around develop oxidtative muscle damage and die.
selenium deficient soil –> vitamin e to pregnant cows –> incidence of condition decreased.
dystrophic mineralization; von kossa stain can highlight caclium mineralization; looks like dark brown or black. Very easy to recognize when use this stain.
Exertional Myopathies are a group of diseases characterized by ______ ______ initiated by _____ ______ (exercise, stress) in which the underlying cause is ______ or _____ ______. Such activity may be intensive or exhaustive but
in susceptible individuals exertional myopathy
may occur with only ______ _____. Some believe that there is a _______ component
muscle damage, muscle activity, unknown, poorly understood, minimal exercise, genetic