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Exercise tolerance in McArdle's is often not that bad. Classically, they get pain and stiffness early, relieved by rest, but if they carry on they move from getting muscle energy from glycogen to getting it from blood-borne substrate (the "second-wind" phenomenon) and can do quite strenuous exercise in some cases. Our patient was quite a fit guy and did regular sports training. He had got used to "feeling knackered at the start", but after resting for a short time could exercise for quite long periods.
 
What did for him was being challenged to a swimming race by his 14 year old son... Extreme exercise from a standing start, in the sea in Scotland so your peripheral circulation is shut down! Wasn't a good idea...
 
Mike
 
 

From: Clinical biochemistry discussion list [mailto:[log in to unmask]] On Behalf Of Mohammad Al-Jubouri
Sent: 26 August 2009 14:04
To: [log in to unmask]
Subject: Re: High CK - ?further investigation

Within the scenario context given by Rob "....... having spent all day laying slabs in his garden", I am not sure that a McArdle's patient has the exercise tolerance to do such a heavy duty job without being stopped very early by muscle pain and stiffness. I still think that exertional rhabdomyolysis due to unaccustomed exercise load is the more likely explanation. However, you could do the ischaemic forearm exercise test while at it and let us know the result.
 
Mohammad

From: Hallworth Mike (RLZ) <[log in to unmask]>
To: [log in to unmask]
Sent: Wednesday, 26 August, 2009 13:35:46
Subject: Re: High CK - ?further investigation

I have seen McArdle's present like this, and agree with those who
suggest it needs following up. Our chap went into ARF, and on
questioning had had a number of episodes of muscle pain and dark urine
after exercise in the past which had been ignored. At the very least,
see if his CK is normal now.

Mike



-----Original Message-----
From: Clinical biochemistry discussion list
[mailto:[log in to unmask]] On Behalf Of Robert Lord
Sent: 26 August 2009 10:33
To: [log in to unmask]
Subject: High CK - ?further investigation

Reviewing a set of medical notes today on a 24 yr old male who was
admitted in July with a CK of 35,825. Swelling of both arms having spent
all day laying slabs in his garden.
Apparently had similar episode in Jan following exercise at gym.
Otherwise well. Medication = inhalers for asthma.
O/E muscles in both arms swollen and tender. Other tests - TSH = normal,
CRP = normal, U+E = normal, FBC = normal, ALT = 165, LDH = 1243.
Elevated urine myoglobin = 124 ug/L. Treated with iv fluids. CK = 7203
when discharged 3 days later. No follow up has been arranged.
Do you see this kind of self induced increase in CK due to strenuous
exercise or should I be suggesting follow up regarding investigation of
a possible metabolic myopathy?

Many thanks

Rob

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------ACB discussion List Information-------- This is an open discussion list for the academic and clinical community working in clinical biochemistry. Please note, archived messages are public and can be viewed via the internet. Views expressed are those of the individual and they are responsible for all message content. ACB Web Site http://www.acb.org.uk List Archives http://www.jiscmail.ac.uk/lists/ACB-CLIN-CHEM-GEN.html List Instructions (How to leave etc.) http://www.jiscmail.ac.uk/ ------ACB discussion List Information-------- This is an open discussion list for the academic and clinical community working in clinical biochemistry. Please note, archived messages are public and can be viewed via the internet. Views expressed are those of the individual and they are responsible for all message content. ACB Web Site http://www.acb.org.uk List Archives http://www.jiscmail.ac.uk/lists/ACB-CLIN-CHEM-GEN.html List Instructions (How to leave etc.) http://www.jiscmail.ac.uk/