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11 Cards in this Set

  • Front
  • Back
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.
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.
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.