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November 1965

Contracture in McArdle's Disease: Stability of Adenosine Triphosphate During Contracture in Phosphorylase-Deficient Human Muscle

Author Affiliations

From the departments of neurology and neurosurgery, College of Physicians and Surgeons, Columbia University, and the Neurological Clinical Research Center, Neurological Institute, Columbia Presbyterian Medical Center. Associate professor of Neurology, College of Physicians and Surgeons, Co-director, Neurological Clinical Research Center (Dr. Rowland); Associate professor of Neurology, Neurological Institute, Faculty of Medicine, Kyushu University, Japan (Dr. Araki); Assistant resident in Neurosurgery (Dr. Carmel).

Arch Neurol. 1965;13(5):541-544. doi:10.1001/archneur.1965.00470050089010

IN 1951, McArdle1 described a patient whose major symptom was limitation of vigorous activity because of cramps after exercise. When electromyographic examination was performed during a cramp, electrical silence was found despite maximum shortening of the affected muscle; this could be considered a contracture (shortening of muscle without propagated action potential2). McArdle recognized the similarity of this event to the classical experiments of Lundsgaard3 in which muscle poisoned by iodoacetate was able to contract without formation of lactic acid. When the patient exercised forearm muscles under ischemic conditions, in contrast to a normal person, there was no rise in venous lactic acid. McArdle therefore postulated that the disorder was due to a defeat of muscle glycogen breakdown.

The nature of the enzymatic abnormality was clearly established by Schmid and his colleagues in one family,4 and by Pearson, Mommaerts, and their colleagues in another.5 Inability

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