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21 Cards in this Set
- Front
- Back
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What is a myopathy?
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Is a motor dysfxn due to a disease of the muscle.
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What can cause myopathies?
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Muscle membrane abnormalities, cytoskeletal protein abnormalities, metabolism errors, inflammatory destruction, toxins
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What are things to look out for clinically in regards to myopathy?
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muscle bulk, palpation, and percussion. Also reflexes and weakness.
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What is important about weakness, muscle bulk, palpation, percussion, and reflexes?
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The distribution (proximal and symmetric).
Preserved or enlarged. Palpation induarated or tender (unreliable). Diminished muscle contraction. Parallel degree of muscle strength. |
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What clinical fx's may suggest that weakness is not due to myopathy?
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If it is distal, fasciculations occur, tremors are present, sensory signs, fatigue, and abscence of reflexes occur.
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What do the following things indicate:
1) distal weakness, 2)fasciculations, 3) tremor, 4) sensory signs, & 5) pathologic fatigue |
1) neuropathy
2) ant. horn cell syndrome 3) per. neuropathy, ant. horn cell disease, or central process 4) per. neuropathy or central process 5) NMJ disorder |
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What dose MERRF stand for and what are its symptoms?
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Myoclonic epilepsy and ragged red fibers
Myoclonus, gen. seizures, ataxia, dementia, hearing loss, optic atrophy, & muscle weakness |
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What does MELAS stand for and what are its symptoms?
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Mitochondrial lactic acidosis and strokes
Encephalopathy, high lactic acid levels in serum/CSF, and stroke-like episodes |
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What are fx's of KEARNS-SAYRES syndrome?
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Ophthalmoplegia, retinitis pigmentosa, abnormal cardiac conduction, & myopathy
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What is a dynamic glycogen metabolism disorder?
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It relates to exercise intolerance (cramps, pain, myoglobinuria)
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What is the one fx of static glycogen metabolism disorders?
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Fixed weakness
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Mitochondrial myopathies are classified by defects in what things?
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tranport, substrate utilization, Kreb's cycle, oxidation/phosphorylation, or coupling
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Most mitochondrial proteins are encoded by what?
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Nuclear DNA
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What fx of cell division causes some mitochondria DNA to have no mutants, some mutants, or be predominantly mutants?
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Segregation
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Why is the nervous system more susceptible to mitochondrial disorders?
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It requires a constant supply of energy
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Why do most mitochondrial myopathies have a maternal pattern of transmission and affect males & females equally?
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Mitochondria are inherited from the mother
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What does a classic muscle biopsy demonstrate in reference to mitochondrial disorders?
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ragged red fibers, and abnormalities in oxidative enzymes stains (NADH, succinyl dehydrogenase, & cytochrome oxidase)
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What clinical fx's suggest weakness is not due to myopathy?
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distal weakness, fasciculations, tremor, sensory signs, pathologic fatigue & early absence of reflexes
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What do distal weakness and fasciculations suggest?
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Neuropathy and ant. horn cell syndrome.
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What does a tremor suggest?
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Peripheral neuropathy, ant. horn cell disease, or central process
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What do sensory signs (symptoms) and pathologic fatigue suggest?
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Peripheral neuropathy, and a NMJ disorder
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