Document Detail


A nonischemic forearm exercise test for McArdle disease.
MedLine Citation:
PMID:  12210784     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Ischemic forearm exercise invariably causes muscle cramps and pain in patients with glycolytic defects. We investigated an alternative diagnostic exercise test that may be better tolerated. Nine patients with McArdle disease, one with the partial glycolytic defect phosphoglycerate mutase deficiency, and nine matched, healthy subjects performed the classic ischemic forearm protocol and an identical protocol without ischemia. Blood was sampled in the median cubital vein of the exercised arm. Plasma lactate level increased similarly in healthy subjects during ischemic (Delta5.1 +/- 0.7mmol L(-1)) and non-ischemic (Delta4.4 +/- 0.3) tests and decreased similarly in McArdle patients (Delta-0.10 +/- 0.02 vs Delta-0.40 +/- 0.10mmol L(-1)). Postexercise peak lactate to ammonia ratios clearly separated patients and healthy controls in ischemic (McArdle, 4 +/- 2 [range, 1-12]; partial glycolytic defect phosphoglycerate mutase deficiency, 6; healthy, 33 +/- 4 [range, 17-56]) and non-ischemic (McArdle, 5 +/- 1 [range, 1-10]; partial glycolytic defect phosphoglycerate mutase deficiency, 5; healthy, 42 +/- 3 [range, 35-56]) protocols. Similar differences in lactate to ammonia ratio between patients and healthy subjects were observed in two other work protocols using intermittent handgrip contraction at 50% and static handgrip exercise at 30% of maximal voluntary contraction force. All patients developed pain and cramps during the ischemic test, and four had to abort the test prematurely. No patient experienced cramps in the non-ischemic test, and all completed the test. The findings indicate that the diagnostic ischemic forearm test for glycolytic disorders should be replaced by an aerobic forearm test.
Authors:
Pedram Kazemi-Esfarjani; Elwira Skomorowska; Tina Dysgaard Jensen; Ronald G Haller; John Vissing
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Publication Detail:
Type:  Clinical Trial; Comparative Study; Controlled Clinical Trial; Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Annals of neurology     Volume:  52     ISSN:  0364-5134     ISO Abbreviation:  Ann. Neurol.     Publication Date:  2002 Aug 
Date Detail:
Created Date:  2002-09-04     Completed Date:  2002-09-18     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  7707449     Medline TA:  Ann Neurol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  153-9     Citation Subset:  IM    
Affiliation:
The Copenhagen Muscle Research Center, Department of Neurology, National University Hospital, Rigshospitalet, Copenhagen, Denmark.
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MeSH Terms
Descriptor/Qualifier:
Adult
Ammonia / blood
Exercise*
Exercise Test* / adverse effects
Female
Forearm / blood supply,  physiopathology*
Glycogen Storage Disease Type V / blood,  complications,  diagnosis*
Hand Strength
Humans
Ischemia / physiopathology
Lactic Acid / blood
Male
Metabolism, Inborn Errors / complications,  diagnosis
Muscle Cramp / etiology
Pain / etiology
Phosphoglycerate Mutase / deficiency
Reference Values
Regional Blood Flow
Chemical
Reg. No./Substance:
50-21-5/Lactic Acid; 7664-41-7/Ammonia; EC 5.4.2.1/Phosphoglycerate Mutase

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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