Muscle & Neurotransmitter Case Study (CS2)Name:
Case Study: Muscle Weakness
Chief Complaint: A 26-year-old woman with muscleweakness in the face.
Patient Presentation:
A 26-year-old woman with muscle weakness in theface.
Patient History:
Jill Rothman, a 26-year-old gymnastics instructor, presents withcomplaints of muscle weakness in her face that comes and goes, andhas been getting worse over the past two months. Her symptoms getworse by the end of an active day. Most notably, she complains thather \"jaw gets tired\" as she chews and that swallowing has becomedifficult.
She also notes diplopia (\"double vision\") which seems tocome on late in the evening, particularly after reading for a fewminutes. At work, it has become increasingly difficult to \"spot\"her gymnasts during acrobatic moves because of upper armweakness.
Physical Exam:
- she has ptosis (\"drooping\") of both eyelids after repeatedblinking exercises.
- When smiling, she appears to be snarling – indicatingasymmetry in contraction of facial muscles.
Tests:
- Single fiber electromyography (EMG or muscle test)testing revealed progressive muscle weakness, and decreasedstrength of contraction, of the distal arm flexor muscles uponrepeated mild shocks (5 shocks per second) of the ulnar and mediannerves.
- Her symptoms and EMG findings were reversed within 40 seconds ofintravenous administration of edrophonium (Tensilon) - anacetylcholinesterase inhibitor (ACh-EI).
- A CT scan of Jill’s chest reveals thymus hyperplasia.
- Blood testing also revealed high levels of an ACh receptorantibody in her plasma.
Instructions: Use what you’ve learned about musclephysiology, and searching Google, to come up with:
A) A reasonable diagnosis for Jill’s condition: (HINT –if you type in Jill’s symptoms (in bold), and edrophonium, intogoogle, you’ll quickly find out what the disorder is most likely tobe.)
Diagnosis? ________Myasthenia gravis_________________________________________________
and
B) A reasonable course of pharmacological treatment, and WHY thattreatment helps her, based on the diagnosis: (Google search forthis disorder)
Treatment(s)? Medications such as Cholinesteraseinhibitors,Corticosteroids and Immunosuppressants some of thesemedications can work together to help improve muscle contractionand muscle strength. Intravenous therapy could also be a treatmentuse such as Plasmapheresis,Intravenous immunoglobulin, andMonoclonal antibody these a blood transfusion that could be used itwhere you exchange plasma to filtrare overactive immune cells inthe blood signaling your muscles to move. Lastly, surgery couldalso an option to remove the thymus gland.
Questions:
1. Why is this young woman experiencing difficultychewing and double vision (diplopia)?
Answer 1.
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2. How are the anti-acetylcholine receptor antibodiesinterfering with her normal skeletal muscle activity?
(Why are ACh receptors important for muscles to workcorrectly? What happens to muscle function when antibodies blockACh receptors?)
Answer 2.
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3. What is the significance of Jill’s distal arm flexormuscles not responding well to relatively low frequencystimulation? Why does repetitive nerve stimulation result indecreased strength of the muscle contractions?
Answer 3.
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4. How do the anticholinesterase drugs act to improveJill's skeletal muscle function?
Answer 4.
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5. Why are nausea, abdominal cramps, diarrhea, andexcessive salivation all side effects of the anticholinesterasedrug she is taking?
Answer 5.
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6. Why is atropine beneficial in treating thegastrointestinal side effects mentioned in question#5?
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7. What is the significance of the enlarged thymus glandin diagnosing Jill with Myasthenia gravis? What is the thymus gland(what is its function)?
Answer 7.
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8. How will the corticosteroid prednisone benefit thispatient?
Answer 8.
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9. Why must Jill undergo “plasmapheresis†when hersymptoms become especially severe?
Answer 9.
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10. Jill's doctor advises her that she is at increasedrisk for respiratory failure. Explain why this is so.
Answer 10.
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11. In addition to being aware of respiratory problems,Jill's doctor also advises her to take her time when eating,especially when swallowing food, to avoid aspiration pneumonia. Whyis this?
Answer 11.
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12. Jill’s doctor recommends that she stay on treatmentfor 6 months and then come back in for a re-check of her thymuswith another CT scan of her chest. Why is this?
Answer 12.
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