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Jill , a 26-year-old gymnastics instructor, presents with complaints of muscle w

ID: 86371 • Letter: J

Question

Jill, a 26-year-old gymnastics instructor, presents with complaints of muscle weakness in her face that comes and goes, but has been getting worse over the past two months. Most notably, she complains that her "jaw gets tired" as she chews and that swallowing has become difficult. She also notes diplopia ("double vision") which seems to come on late in the evening, particularly after reading for a few minutes. At work, it has become increasingly difficult to "spot" her gymnasts during acrobatic moves because of upper arm weakness.

On physical examination, she has notable ptosis ("drooping") of both eyelids after repeated blinking exercises. When smiling, she appears to be snarling. Electromyographic (EMG)testing revealed progressive weakness and decreased amplitude of contraction of the distal arm muscles upon repeated mild shocks (5 shocks per second) of the ulnar and median nerves. Both her symptoms and electromyographic (EMG) findings were reversed within 40 seconds of intravenous administration of edrophonium (Tensilon), an acetylcholinesterase inhibitor. Blood testing revealed high levels of an anti-acetylcholine receptor antibody in her plasma, and a diagnosis of myasthenia gravis was made.

Jill was treated with pyridostigmine bromide, which is a long-acting anticholinesterase drug, and was also started on prednisone, which is a corticosteroid drug. She was given a prescription of atropine as needed to reduce the nausea, abdominal cramps, diarrhea, and excessive salivation she experienced as side effects of the anticholinesterase drug.

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Questions (1 point each)

1. Why is this young woman experiencing difficulty chewing and double vision?

2. Describe the mechanism of this particular disease.

3. How do the anticholinesterase drugs act to improve Jill's skeletal muscle function?

4. How will the corticosteroid prednisone benefit this patient?

5. Jill's doctor advises her that she is at increased risk for respiratory failure. Why?

Explanation / Answer

1) this condition is called Myasthenia Graves. It is a condition occurs in this. It is a neuromuscular disorder in which the contact between the voluntary skeletal muscle and neurons is impaired due to which the no impulse is passed from brain thus skeletal muscle can't work properly and hence causing muscle weakness as seen in the case of chewing Jaws and double vision where these muscle gets tired..!!

2) the mechanism of the disease is autoimmunity. The body preapre antibodies against the pathogens and foreign bodies which comes inside the body. Doing this, the body sometimes preapre the antibodies against the own body system I.e actylcholine in this case which acts on this neurotransmitter actylcholine and degrades it. So, there is a impairment of transmission of signal and impulse from nerve to the skeletal muscle hence the no proper transmission occurs to the skeletal muscles hence it gets tired and muscle weakness. As nerve impulse transmission is very much required for the continuous contraction of the muscle..!!

3) anti-cholinesterase drugs mimics the structure as that of the actylcholine which goes and binds to the anti-cholineastrate drugs and block the action of the drugs thus inhibiting the breaking of actylcholine and thus increases the concentration and time duration of the actylcholine in the body during nerve impulse hence initiating the rapid nerve impulse transmission..!!

4) According to the new study It had been observed that the presence of the steroids induces the expression of the more actylcholine receptors on the cell thus helps in the more binding of the actylcholine hence more action..

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