| McArdle disease: what do neurologists need to know? | |
| | |
MedLine Citation:
|
PMID: 18833216 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
|
McArdle disease (also known as glycogen storage disease type V) is a pure myopathy caused by an inherited deficit of myophosphorylase, the skeletal muscle isoform of the enzyme glycogen phosphorylase. The disease exhibits clinical heterogeneity, but patients typically experience exercise intolerance, that is, reversible, acute crises (early fatigue and contractures, sometimes with rhabdomyolysis and myoglobinuria) triggered by static muscle contractions (e.g. lifting weights) or dynamic exercise (e.g. climbing stairs or running). In this Review, we discuss the main features of McArdle disease, with the aim of providing neurologists with up-to-date, useful information to assist their patients. The topics covered include diagnostic tools-for example, molecular genetic diagnosis, the classic ischemic forearm test and the so-called 'second wind' phenomenon-and current therapeutic options-for example, a carbohydrate-rich diet and carbohydrate ingestion shortly before strenuous exercise, in combination with medically supervised aerobic training of low to moderate intensity. |
| | |
Authors:
|
Alejandro Lucia; Gisela Nogales-Gadea; Margarita Pérez; Miguel A Martín; Antoni L Andreu; Joaquín Arenas |
Related Documents
:
|
3710966 - Epinephrine-induced changes in muscle carbohydrate metabolism during exercise in male s... 9609776 - Effects of high-intensity intermittent swimming on glucose transport in rat epitrochlea... 9363376 - Current issues in exercise metabolism: the crossover concept. 21419356 - Physical and psychological benefits of a 24-week traditional dance program in breast ca... 2022576 - Oxygen uptake dynamics during high-intensity exercise in children and adults. 2022206 - Changes in structure and function of the human left ventricle after acclimatization to ... |
Publication Detail:
|
Type: Journal Article; Research Support, Non-U.S. Gov't; Review |
Journal Detail:
|
Title: Nature clinical practice. Neurology Volume: 4 ISSN: 1745-8358 ISO Abbreviation: Nat Clin Pract Neurol Publication Date: 2008 Oct |
Date Detail:
|
Created Date: 2008-10-03 Completed Date: 2008-12-30 Revised Date: 2011-07-19 |
Medline Journal Info:
|
Nlm Unique ID: 101261799 Medline TA: Nat Clin Pract Neurol Country: England |
Other Details:
|
Languages: eng Pagination: 568-77 Citation Subset: IM |
Affiliation:
|
Department of Physiology, Universidad Europea de Madrid, Madrid, Spain. alejandro.lucia@uem.es |
Export Citation:
|
APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
|
Glycogen Storage Disease Type V* Humans Neurology / education |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
Previous Document: TOF-SIMS analysis of lipid accumulation in the skeletal muscle of ob/ob mice.
Next Document: Challenges in the development of an HIV-1 vaccine.