Pathophysiology of muscle disease Flashcards
Types of muscle dz
- Exertional
- Non-exterional
Exertional muscle dz
Acquired:
- ‘tying-up’
Genetic:
- PSSM-1
- PSSM-2
- RER
- HYPP
- Myofibrillar myopathy
Non-exertional muscle dz
Acquired:
- Toxic: AM
- IMM
- Traumatic: atrophy
- Infectious: Clostridium spp
Genetic:
- MH
- Mitochondrial myopathies
- IMM
Other names for sporadic rhabdomyolysis
- tying-up
- monday-morning disease
- rhabdomyolysis
- azoturia
What is the most common muscular disorder in horses?
- Sporadic rhabdomyolysis
Sporadic rhabdomyolysis - CS
- stiff
- stilted gait
- excessive sweating
- increase respiratory rate after exercise
- firm painful muscles (hindquarters, back)
- reluctant to move
forward - occasional dark urine
Causes of sporadic rhabdomyolysis
- overexertion
- dietary imbalances
- exhaustion
Overexertion causing sporadic rhabdomyolysis
- increase in work intensity without a strong musculoskeletal foundation Z- disk instability
- overstretching of myofibers
- sarcolemma stretching
Dietary imbalance causing sporadic rhabdomyolysis
- Electrolytes (Na and Ca) both important in neural transmission and muscle contraction
- Low Vitamin E intake: poor antioxidant state
Exhaustion causing sporadic rhabdomyolysis
TB and endurance horses training in hot, humid weather: electrolytes loss in sweat and dehydration
What is RER
= recurrent exertion rhabdomyolysis
- intermittent form of rhabdomyolysis in horses likely linked to abnormal calcium regulation in myofibres
– SERCA receptor dysfunction?
Prevalence of RER
- 5% TB
- 6.5% SB
RER risk factors
➢ Fit horse with nervous temperament»epinephrin/adrenaline release?
➢ Young mares more at risk, but no associated with oestrus cycle
➢ Lack of turnout
➢ Held back during gallop: epinephrin and cortisol release?
What does the SERCA receptor do?
- allows removal Ca from sarcoplasm leading to relaxation
What does the RYR-1 receptor do?
- allows release of Ca to stimulate contraction
How does adrenaline affect the RYR-1 receptor?
- it increases it’s activity
What is PSSM-1?
= polysaccharide storage myopathy 1
➢Abnormal storage of glycogen in muscle fibres difficult to be metabolized by muscle cells
➢Genetic mutation in GY1: Glycogen synthase enzyme turn on constantly: PSSM-1
➢A subset of cases: amylase sensitive glycogen»>PSSM-2»>Myofibrillar myopathy & PSSM-2
Prevalence of PSSM-1
➢Highest in continental European draft breeds
➢Percheron Belgians
➢Shires and Claydesdales low in UK: Irish draft, Connemaras, Cobs present
➢Quarter horses (28%), Appaloosas (10%)
➢Very rare in light breeds: SB, TB, Arabians
PSSM-1 risk factors
➢breed and exercise of more than 20min in one session
➢Owner perception : high quality grass available
What do both PSSM-1&-2 cause?
- a myofibrillar myopathy (oxidative deficit)
PSSM-1 breeds & clinical presentation
➢Most draft horses are asymptomatic
– unless performance drafts
➢Stiffness after a short period of exercise
–reluctance to move forward, poor performance, sweating or more severe clinical signs: myoglobinuria
➢Mean age at diagnosis 4-8 years-old
What is PSSM-2?
➢Big box of muscle conditions with some accumulation of glycogen
– likely different aetiologies and/or mutations
Prevalence of PSSM-2
➢ 28% QH or American breeds
➢ Other light breeds such as Friesians, Selle francais, Morgans, Appaloosas, Arabs, occasional TB
PSSM-2 CS
- more subtle with poor performance mainly, milder elevation of muscle enzymes post exercise