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Stability of Adenosine Triphosphate During Contracture in Phosphorylase-Deficient Human Muscle

LEWIS P. ROWLAND, MD; SHKURO ARAKI, MD; PETER CARMEL, MD

Arch Neurol. 1965;13(5):541-544.

	Since this article does not have an abstract, we have provided the first 150 words of the full text PDF and any section headings.

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 . . . [Full Text PDF of this Article]

Author Affiliations
NEW YORK

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).

Footnotes
Submitted for publication April 30, 1965; accepted July 7.

Reprint requests to Neurological Institute, 710 West 168th St., New York, NY 10032 (Dr. Rowland).

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