Pathology of Skeletal Muscle, Pt. 2 Flashcards
What is arthrogryposis? What is it typically associated with?
congenital condition in aborted fetuses and stillborns characterized by rigid joints and limbs in abnormal, grotesque positions
developmental abnormalities of the nervous system (spina bifida, syringomyelia) that causes a lack of innervation, which leads to muscle hypoplasia
Other than denervation, what is another probable cause of arthrogryposis?
ingestion of toxins
in-utero viral infections
(Akabane disease, Cache Valley fever, blue tongue)
What is muscular dystrophy? Dogs and cats are models for what type of muscular dystrophy?
genetical determined, progressive, degenerative disease of the muscle where regenerative attempts are inadequate or absent
Duchenne’s muscular dystrophy
What causes Duchenne’s muscular dystrophy?
animal or person affect lack dystrophin, a cytoskeletal protein that strengthens muscle and protects it from injury
In what kinds of dogs is the X-linked dystrophin gene defect most identified? What organs tend to be involved?
young male Golden Retrievers (Irish Terriers, Samoyeds, Labs, Rottweilers, Dalmations)
heart —> cardiomyopathy
esophagus —> megaesophagus
weakened diaphragm
What are the characteristic symptoms of the X-linked dystrophin gene defect leading to muscular dystrophy? What typically causes death in patients with this?
muscle wasting
outward rotation of forelimbs (splaying)
aspiration pneumonia
cardio-respiratory failure
What is Equine Polysaccharide Storage Myopathy (EPSSM)? How can this be diagnosed microscopically?
inherited disease reported mainly in QH, Arabians, Morgans, Ponies, and draft-related breeds, where an underlying carbohydrate metabolic disorder causes gate abnormalities (hind limb lameness) and reduced muscle mass
accumulation of PAS positive material (polysaccharides) in muscle associated with Type II fibers
What is Porcine Stress Syndrome? What pigs are more susceptible? Why have instances dropped?
(porcine malignant hyperthermia)
an inherited hypermetabolic syndrome characterized by dyspnea, muscle rigidity and convulsions, hyperthermia, lactic acidosis, hyperkalemia, cardiovascular collapse, and death
heavy-muscle pigs
genetic testing
What causes Porcine Stress Syndrome?
single point mutation in the skeletal muscle ryanodine receptor gene (ryr1), which is a calcium release channel
the mutation in this gene causes uncontrolled calcium release, which leads to the excessive muscle contraction and heat production
What characteristics point toward Porcine stress syndrome during a necropsy? What muscles are most affected?
pale, swollen, and wet muscles (PSE = pale, soft, exudative)
longissimus dorsi and psoas
What secondary lesions are common in pigs that died due to Porcine Stress Disorder?
acute heart failure:
- pulmonary edema and congestion
- hydropericardium
- hydrothorax
- hepatic congestion
What are common microscopic characteristics of Porcine Stress Syndrome?
acute myofiber degeneration - interstitial edema, myofiber swelling, fragmentation, segmental contraction with “retraction cap”
What is White Muscle Disease? Why is it most common in young animals and those that are the best nourished and rapidly growing?
nutritional myopathy as a result of a vitamin E and selenium deficiency
these animals have the highest metabolism, leading to the production of oxygen radicals,; lacking in vitamin E and selenium (radical scavenger co-enzymes to glutathione peroxidase/reductase) leads to the lipoperoxidation of cell membranes
What are 2 other vitamin E/selenium syndromes that co-exist with nutritional myopathy in pigs?
hepatosis dietetica
mulberry heart disease
What leads to the “white muscles” of nutritional myopathy?
calcification (dystrophic mineralization)
What are 2 common clinical signs of White Muscle Disease? What muscles are most affected?
- stiffness
- dyspnea
those that are used the most and create the most free radicals
- intercostals in animals where there is little involvement of other muscles
- thigh in shoulders in cases of high skeletal muscle involvement
- tongue and neck muscles in nursing animals
Neck muscles, White Muscle Disease:
pale, granular mineralization appearance (gritty)
Thigh muscles, White Muscle Disease:
pale, hemorrhage
Nutritional Myopathy, histology:
sarcoplasm fragmentation
Nutritional Myopathy, heart:
white brush strokes - mineralization, chalky, gritty
How do lambs and calves differ in their presentation of White Muscle Disease in their heart?
LAMB - common in right ventricle
CALF - common in left ventricle
How can mineralization in White Muscle Disease be visualized histologically?
Von Kossa stain highlights calcium in a dark brown color
What are exertional myopathies?
group of diseases characterized by muscle damage initiated by muscle activity (can be intensive/exhaustive, but minimal exercise can cause damage in susceptible individuals)
What is hyperkalemic periodic paralysis (HYPP)? What is the underlying defect?
inherited condition that affects descendants of the famous QH, Impressive
characterized by well-defined muscle groups rewarded in halter competitions
alteration in muscle sodium channel, leading to a release of potassium in the bloodstream and extracellular space —> transient muscle spasms (myotonia), flaccid paralysis, death