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Bilateral eyelid ptosis without extraocular weak - okt3 - Printable Version

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Bilateral eyelid ptosis without extraocular weak - okt3 - ArchivalUser - 04-25-2009

A 46-year-old manpresents with difficulties of gait and weakness of the face, neck, and hands. On examination, the patient has a œhatchet-face appearance, with obvious wasting of the temporalis and masseter muscles. Bilateral eyelid ptosis without extraocular weakness is noted. There is prominent neck flexion but not extension, weakness, and atrophy. Interosseous atrophy and a bilateral footdrop are noted.

The patient has prominent frontal baldness and testicular atrophy. When asked to grip the examiner™s hand, the patient has difficulty relaxing the grip quickly. Percussion of the thenar eminence leads to slow relaxation. The serum creatine phosphokinse (CPK) level is normal.

Which of the following is the most likely diagnosis for this patient?

A. Becker muscular dystrophy

B. Duchenne muscular dystrophy

C. Autosomal recessive sarcoglycanopathy

D. Autosomal dominant fascioscapulohumeral dystrophy

E. Myotonic dystrophy (MD)


0 - ArchivalUser - 04-25-2009

d i think for exclusion approach


0 - ArchivalUser - 04-25-2009

E- MD


0 - ArchivalUser - 04-25-2009

After you read a question, what do you do:

A-post an answer
B-keep your answer until the right answer get posted
C-Go back and look-up in your First Aid, wiki, or google.
D-Make an educated guess and learn from the explanation.
E-Dont trust the source and ignore the whole post.


0 - ArchivalUser - 04-25-2009

D.....


0 - ArchivalUser - 04-25-2009

d to ur 2nd question...........lol


0 - ArchivalUser - 04-25-2009

I like your answer D for my second qs drtaher, sometime you see more than 10 views but only few people post answer, maybe I dont know....


0 - ArchivalUser - 04-25-2009

testicular atropy and frontal baldness are features of MD....muscle weakness will be tre ....

not sure about the vast majority of muscles involved as mentioned



0 - ArchivalUser - 04-25-2009

(Answer: E”Myotonic dystrophy [MD])

This patient likely has MD, the most common muscular dystrophy in adults. MD is an
autosomal dominant disorder; patients present with a unique constellation of clinical features:
ptosis, temporal and masseter atrophy, atrophy of the sternocleidomastoid muscles
(with sparing of other posterior neck muscles), atrophy of distal musculature, dysarthria,
and dysphagia. Myotonia (the inability to quickly relax a firm hand grip) should be specifically
sought; its presence is characteristic. Most patients have disorders of the cardiac conduction
system. Other features include frontal baldness, testicular atrophy, cataracts, mild
mental dysfunction, GI motility disorders, and hypersomnia. The CPK level is normal or
only mildly increased. EMG is diagnostic, revealing myotonic discharges.


0 - ArchivalUser - 04-25-2009

some probably just look at the question and they have reaction that is something like :

----- " oh shit they ask about that in exam toooo......".....lol