Muscle Dz of adults and children
Terms
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- Characterize Duchennes Muscular Dystrophy.
- Deterioration of muscle secondary to absence of structural dystrophin gene. Characterized by pseuodhypertrophy, and progressive muscle weakness. Without treatment patients die in 20's from cardiac or respiratory problems.
- Desicribe the typical DMD patient.
- Starts at birth. Dx made by 5-6 years of age.
- What is Beckers dysrophy?
- A milder form of DMD in which patients do not experience as rapid a rate of decline. Abnormal Biopsy.
- What laboratory abnormalities are observed if any at all in Duchennes Muscular Dystrophy?
- Elevations of Creatine Kinase (20-30,000)
- How is DMD treated?
- Prednisone. Allows muscle fibers to last longer due to supression of inflammatory macrophage response following myofiber damage.
- Characterize the pathology of myotonic dystrophy.
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Auo-dom dz characterized by inability to relax muscle typically presenting distally (hands and feet)
Patients characteristically show a steppage gait. - How is Myasthenia Gravis treated?
- Acetylchonlinesterase blockers.
- What is the pathology underlying Myasthenia Gravis?
- Immune mediated response to a viral trigger producing antibodies that bind acetylcholine receptor sites. Thus MG is characterized by a progressive decline of ACH Receptors.
- Discuss symptoms typically associated with Myasthenia Gravis.
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fatigable weakness in which patient utilization of muscle facilitates weakness.
Patients may hold their mouths closed or have difficulty closing their eyes. - What is the mode of DMD inheritance?
- X-linked recessive
- Differentiate Polymyositis vs. Dermatomyositis.
- Rash. Both presesent with proximal weakness and elevated CK and are immune mediated. However, dermatomyositis manifests with maccular rash.