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Diseases of Neuro 1
Terms in this set (7)
Muscular Dystrophy generally have the three clinical features...
What are two main factors that distinguish them from other myopathies?
-Proximal muscle weakness
-No UMN or LMN findings
-Steadily progressive muscle weakness and wasting
Describe Duchenne Muscular Dystrophy:
-Most rapidly progressive form of muscular dystrophy
-exclusively affects young boys
-death from respiratory failure in mid-teens or twenties
-altered gene is on band Xp21 (short arm of X-chromosome)
-dystrophin is the missing protein. connects F-actin to the Sarcolemma.
-boys walk late (after 18 months)
-weakness of forward head flexion
-walking is broad-based/waddling with exaggerated lumbar lordosis
-ankle plantar flexor (calves) & ankle inversion (posterior tibialis) remain strong throughout the illness
-Calves undergo hypertrophy
-Gowers' sign is always found in DMD. It is the way they stand up.
-By age 10 most require leg braces.
-By age 12 they are wheelchair dependent
-Death is commonly by respiratory failure around age 20.
Complications of Duschenne Muscular Dystrophy
Respiratory: hypoventilation because of muscle weakness and kyphoscoliosis
Cardiac: Tachycardia w/o heart failure....most patients are free of cardiovascular symptoms until late in the course
CNS: 1 SD below the mean
Creatine Kinase >20x the norm
Muscle biopsy shows absent staining for dystrophin
Give two examples of a woman who would be considered an obligate carrier for DMD.
A female with an affected son + one other affected male relative
A female with 2 affected sons
A lack of dystrophin causes instability of the ........... resulting in ...
sarcolemma; excessive influx of calcium (which causes muscle fiber necrosis)
What is Becker Muscular Dystrophy? What would one consider the daughter of a BMD male?
Pretty much a slowed-down version of DMD.
There is a detectable amount of dystrophin but it is qualitatively altered.
An obligate carrier.
Describe Myotonic Dystrophy.
-has a varied range of disease severity even within a family
-symptomatic onset is generally in the 2nd or 3rd decade
-Myotonia is the inability to quickly relax a muscle once it is contracted.
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