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•CJ " Item: 13 of 44

Q. ld: 3809 [
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5 A 33-year-old Caucasian female comes to the office and complains of occasional diplopia and ptosis. These
6 symptoms become especially prominent w hen she looks above her head for some time. She also complains
7 of fatigue in her hands and leg muscles after exercising, such as sw imming. W hat is the level of the lesion in
8 the disease that is being described?
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10 ~ n A. Neuromuscular junction [92%]
11 B. Nerve conduction [3%]
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C. Muscle contraction [1 %]
14 D. Corticospinal tract [1 %]
15 ~ E . Autonomic nervous system [1 %]
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18 Explanation: User ld:
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20 This patient's clinical presentation is suggestive of m yasthenia gravis (MG). MG is generally seen in females
21 in their second or third decade, or in males older than sixty years. The typical presentation includes fatigability
22 and w eakness of the skeletal muscles. There are no reflex, sensory or coordination abnormalities. A
23 characteristic feature is that the symptoms are typically seen on exertion and resolve w ith rest. W eakness
24 of the eye muscles can lead to diplopia and ptosis, especially on looking over one's head. Abnormal eye
25 muscle movement, w hich cannot be attributed to one nerve or muscle, may be seen on examination;
26 how ever, unlike botulism, the pupils are always spared in MG. Bulbar muscle involvement can lead to
27 "m yasthenic snarl", nasal speech, and difficulty in chew ing or swallow ing.
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Myasthenia gravis is an autoimmune disorder. The basic pathology lies at the neuromuscular junction and is
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mediated by auto-antibodies against the acetylcholine receptor, leading to a decrease in the number of
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available acetylcholine receptors (AchRs) at the neuromuscular junctions. The histological hallmarks include
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decreased numbers of acetylcholine receptors, simplification of the postsynaptic clefts, and w idening of the
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synaptic space w ith a normal presynaptic nerve terminal.
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34 Educational objective:
35 The basic pathology in m yasthenia gravis lies at the neuromuscular junction (that's w hy it's called
36 m yasthenia) and is mediated by auto-antibodies against the acetylcholine receptor.
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Time Spent: 6 seconds Copyright © UW orld Last updated: [1 2/29/2014]
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