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55 yr old male presents with h/o difficulty getting out of tub after bathing and difficulty climbing stairs.This has been progressive over last 6 months.He also complains of pain in shoulders and hip.Recently he has developed some difficulty in swallowing as wel and is bothered by it.Onexam he has mild atrophy of shoulder and hip girdle muscles,his DTRs are depressed.CPK is 528.An EMG shows mixed myopathic and neuropathic pattern.
Whats is the likely diagnosis?
What investigations u wud do?
What is the prognosis?

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I think this is a case of polymyositis with polyneuropathy due to paraneoplastic manifestation(once more!).The reasons for this asumption is the typical manifestations of polymyositis with diminished tendon reflexes and mixed pattern in EMG.I dont think this is Eaton -lambert because of absence of autonomic nervous system manifestations,sparing of external occular muscles,involvment of pharynx and elevated CPK.
I would ask for cxr and colonoscopy to investigate for lung and large bowel cancer,plasma protein electrophoresis for multiple myeloma,muscle biopsy and electrodiagnostic examination(conduction velocity etc)
I think prognosis is unfavorable.
Nice qs.

Guest · Credits: 79379 My Scrapbook My Reading List 262569 Books

typically polymyositis definitely the EMG pattern is not of eaton lamberts. eaton improves with continuing work or activity.
would do muscle biopsy. if eaton....antibodies to voltage gated Ca channels are positive
prognosis is generally good. if it is pure polymyositis. but if it is sec to malignancy...prognosis depends on primary condition.

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I still believe that this is not clearly polymyositis but there is additional neuropathy too.The pattern of the EMG is leading me to this conclusion.Thats why I am in favor of paraneoplastic polymyositis more.
To Tz,when are you going for P2?I am interested in exchanging views upon preparation.

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The likely diagnosis is INCLUSION BODY MYOSITIS.Although PM is very close D/D,there r some clues to inclusion body myositis.The progression is rather slow(over 6/12),CPK is less than 10 times raised which usually occurs in PM and EMG shows mixed pattern whilke in PM myopathic pattern is seen.
Patrient need muscle Bx
Prognosis is poor with little response to therapy

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I am sorry but I dont really agree with your answer.Inclusion body myositis is characterized by frequent focal and distal myositis and early involvement of finger and forarm flexors and leg extensors as well.As far as I searched in Harrison I couldnt find a combined pattern in EMG as a feature of this disease.The data given imply proximal muscle myositis and they dont give any clue for the rest of the features I refered above.For these reasons I still believe that the right answer is polymyositis with combined polyneuropathy due to a paraneoplastic manifestation.This could explain both proximal muscle involvment and mixed pattern in EMG.If this case was a real one of course I would ask for a muscle biopsy but I would always have superimposed malignacy in mind.I would really like to read your answer.Thank you for your contribution. 8)

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inclusion body myositis Can Be Distal but is more commonly proximal.PM usually occurs in 40s while IBM in 50's and 60's.CPK is above 1000 typically in PM but less in IBM.Patient has dysphagia which occurs much more commonly in IBM than in PM and i have mentioned EMG difference too.
Moreover u cannot expect to find all features written in textbook in all cases and general rule is to lokk for one diagnosis that can explain everything rather than more than one like PM,with underkying malignany with polyneuopathy.

Guest · Credits: 79379 My Scrapbook My Reading List 262569 Books

1)What I propose as a right diagnosis is not two different diagnosis but one,malignacy which can result to both polymyositis and polyneuropathy,two features that according to Harrison can be present together resulting in disproportionately decreased tendon reflexes and mixed pattern in EMG.
2)I couldnt find dysphagia as a characteristic feature of IBM in Harrison while polymyositis due to malignacy is a recognized one.So please give me your source.
3)You refer to the patient's age in order to support that IBM is more possible than polymyositis.I agree with you as far as idiopathic polymyositis.The situation is quite different regarding polymyositis with neoplasia.
4)What you write about levels of CPK is not at all essential for establishing a diagnosis of IBM.Polymyositis can also manifest without very high CPK levels.
5)You also stress upon the duration of the disease for supporting your point.I agree with you that IBM can be more insidious but this cannot rule out polymyositis with malignacy at all.
6)There is no clue in data given such as distal muscle involvment,characteristic finger and forearm flexors involvement etc that could help us even to guess IBM.On the other hand what I propose as right diagnosis is more common in clinical practice and can be fully supported by the given data.
For all the above reasons I am not convinced neither by your answer nor by your farther explanation.This of course doesnt mean that I question your Knowledge or good purposes.Anyway right diagnosis can be established only with muscle biopsy something in which we both agree.I will be glad if you inform me of your sources since Harrison cant be adequate for everything.Thank you in advance.

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i actually pointed that proximal weakness is more common in IBM than distal weaknes.
Source is uptodate,the best source of medical literature i have seen,but is bit pricey
The website is utdol.com

Guest · Credits: 79379 My Scrapbook My Reading List 262569 Books

thanks a lot 8)

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