R2 LOCO1 Flashcards
Describe happens when muscle fibres become deinnervated [1]
All muscle fibres innervated by a damaged motor unit undergo atrophy
Damaged: arrow
Describe what a target fibre is [1]
Why does a target fibre arise? [1]
Describe the apperance [1]
When a damaged muscle is re-innervated; nuclei of muscle moves centrally and causes the production of new actin and myosin fibres
Occurs due to satellite cells differentiating and proliferating
Which are the slow & fast fibres in this stain? [2]
Slow fibres (Type 1): more mitochondria - darker stain
Fast fibres (Type 2): less mitochondria - light stain
Describe the pathophysiology of sarcopenia
- 0.5 - 1% of muscle mass is lost each year after age of 50; 3-5% if inactive
- Muscle replaced with fat
- No difference in men and women
- Causes an increase likelihood of fractures
- Impaired balance
Which muscle fibres degenerate more in sarcopenia? [1]
Generally, a significant decline of type II, but not type I muscle fibers are observed in sarcopenic patients
How does an EMG present with patients who have myopathy?
Compared to healthy and neuropathy patients [2]
In myopathic motor units, the number of functional muscle fibers is reduced. Therefore motor unit action potentials (MUAPs) are smaller in amplitude and duration. Because of the asynchronous firing of affected muscle fibers, the morphology of MUAPs become polyphasic.
How does a patient with dermatoymositis present? [4]
Rash around eyes
Peri-orbital oedema
V sign of neck due to increased photosynsetivity
Gottron’s sign
How does a biospy from a patient with polymyositis / dermatomyositis present? [1]
- (some have subclinical muscle involvement)
- Muscle generally not involved; CD8 T cells cells infiltrate the periphery of the muscle fasicle
- Central nuclei
- Variation in fibre size
Describe the effect of CD8 infiltration in dermatomyositis [1]
CD8 T cells bind to MHC 1 to create: CD8-MHC1 complex
Describe the change that occurs in subcutaneous calcifications [3]
Dermis-epidermal junction: vacuoles develop, mucin deposite and calcifications
Describe treatment regime of dermatomyositis [4]
Prednisilone (type of corticosteroid):
* 1 mg / kg per day until creatine kinase normal
Azthioprine
Methotrexate
Rituximab
Which muscular dystrophies are the most common? [2]
Describe their inheritance [1]
Duchenne & Becke Muscular Dystrophies;
X-linked - recessive
Describe pathophysiology of Duchenne MD [2]
Which serum marker is raised in DMD? [1]
Healthy muscle: dystrophin is a ring around muscle fibre that connects the ECM to contractile apparatus so that endomesium moves with muscle
Duchenne: no muscle fibres with dystrophin; causes elevated creatine kinase
Which muscle groups are most at risk in Duchenne MD? [2]
What does this mean clinically? [2]
Respiratory muscles & diaphragm; die from resp. failure
Dilated cardiomyopathy occurs; heart failure
How does early DMD appear histologically? [3]
Fibre size variation
endomysial & perimysium fibrosis
Degenerating muscle fibres undergoing myophagocytosis
How does late DMD appear histologically? [2]
Loss of muscle; atrophy of fibres and death - replaced with fibrotic material and fat
Treatment for DMD? [2]
Prednisilone
Gene alterations
How do statins affect 10% muscle? [2]
Rhabdomyolysis; get vacuoles in type 2 muscle fibres
Name and describe how two therapeutic drugs cause myopathy [2]
Corticosteroids; cause dose dependent type 2 atrophy
Hydroxychloroquine: not dose dependent atrophy; lysosomes damaged
Hydroxychloroquine causing lysosome damage
Which tissue is affected in fibromyalgia? [1]
Which muscles are painful? [1]
Which antibodies are present in 50% of patients? [1]
Connective tissue damaged; causes widespread pain in both sides and above & below waist axial skeleton pain
Antipolymer antibodies
How do you diagnose fibromyalgia? [1]
Apply 2kg of pressure on 18 specific tender points in the body; 11/18 is a positive diagnosis
Treatment options for fibromyalgia? [4]
Amitriptyline (TCA)
Fluxetine (SSRI)
Exercise
Complementary therapy
Give three pieces of evidence that RA is autoimmune disease [3]
1- Patients produce auto-antibodies (in blood and joints).
2- Inflamed joints are filled with activated immune cells, cytokines
3- Joint conditions are strongly associated with MHC genes, required for T cell activation.
What is the role of MHC class molecules? [3]
- MHC determines which peptides are presented to T-lymphocytes.
- MHC molecules determines T-lymphocyte tolerance in the thymus (i.e. what mature T cells enter the periphery / survive) and also determines if and how mature T lymphocytes respond to antigens upon encounter.
Therefore: any strongly recognised self-T cells are killed off