Pathology Flashcards
(251 cards)
What surrounds the entire skeletal muscle?
epimysium
What surrounds the bundles of skeletal muscle fibres?
perimysium
What surrounds the individual muscle fibres?
endomysium
What are the 3 main indications for muscle biopsy?
- evidence of muscle disease (eg weakness, muscle symptoms, elevated CK)
- presence of neuropathy
- presence of vascular disorder (vasculitis)
what is fasciculation?
uncontrolled twitching of muscle
When CK level is higher than normal, in the ‘low’ classification, what type of disorder does this indicate?
neurogenic disorder
when CK level is higher than normal, in the ‘intermediate’ classification, what type of disorder does this indicate?
inflammatory myopathy
when CK level is higher than normal, in the ‘high’ classification, what type of disorder does this mean?
muscular dystrophy
what are the 5 main groups of myopathies?
- muscular dystrophies
- inflammatory myopathies
- congenital myopathies
- secondary myopathies
- metabolic myopathies
Why are ring fibres sometimes formed during muscle dystrophy?
condensation of the cytoplasm
Why can there be enlarged nuclei and increased number of nuclei during muscle dystrophy?
due to attempted regeneration co-existing with the atrophy
What type of inheritance is Duchenne’s Muscular Dystrophy (DMD)?
x-linked
What muscles does Duchenne’s Muscular Dystrophy (DMD) mainly affect?
muscles of the pelvic girdle
What type of inheritance is Becker’s Muscular Dystrophy (BMD)?
x-linked
What muscles does Becker’s Muscular Dystrophy (BMD) mainly affect?
muscles of the pelvic girdle
When is the onset of Duchenne’s Muscular Dystrophy (DMD)?
2 - 4 years old
What is the life expectancy of someone with Duchenne’s Muscular Dystrophy (DMD)?
20
What happens to the calves of patients with Duchenne’s Muscular Dystrophy (DMD)?
pseudohypertrophy
What mutation causes Duchenne’s Muscular Dystrophy (DMD)?
Mutations in dystrophin gene on long arm of chromosome X
What causes the different severities of Duchenne’s Muscular Dystrophy (DMD)?
mutations resulting in frame shift- severe
mutations resulting in altered binding- moderate to severe
mutations in middle rod- mild (Beckers)
What does the mutation in the dystrophin gene in Duchenne’s Muscular Dystrophy cause? (in terms of basement membrane and actin and Ca++ entry)
changes anchorage of actin cytoskeleton to basement membrane
uncontrolled Ca++ entry into cells
What does the change in anchorage of actin cytoskeleton to basement membrane in Duchenne’s Muscular Dystrophy do to the muscle fibres?
makes them more liable to tear
What gene is mutated in Becker’s Muscular Dystrophy (BMD)?
dystrophin
variant of DMD
compare disease progress of Beckers to Duchennes?
Beckers progression is much slower