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Paul Slotwiner, MD; Sun K. Song, MD; Howard S. Maker, MD

Arch Neurol. 1969;20(6):586-598.

	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 30-year-old man who suffered from severe muscle cramps and weakness following exertion. The symptoms were relieved by rest, and results of neurological examination were entirely normal. A disorder of glycogen breakdown was presumed on the basis of the failure of the patient's blood lactate level to rise following exercise under ischemicconditions. It was shown in subsequent similar cases^2-5 that there was an absence of muscle phosphorylase, the initial enzyme of glycolysis reacting directly with branched glycogen. Findings from muscle biopsies^2-5 demonstrated abnormal aggregates of glycogen within the muscle, both in the form of subsarcolemmal "blebs" and in more central regions of the fibers. These findings were confirmed by electronmicroscopy.^6,7 Subsequently, other examples of myopathy associated with different abnormalities of glycogen metabolism have been presented. These have included the absence of the enzymes -glucosidase (amylo-1,4-glucosidase),^8-11 amylo-1,6-glucosidase (debrancher),^12-14 phosphohexoisomerase,¹⁵ . . . [Full Text PDF of this Article]

Author Affiliations
New York

From the departments of neurology (Drs. Slotwiner; Song; and Maker) and neuropathology (Drs. Slotwiner and Song), Mount Sinai School of Medicine, New York.

Footnotes
Submitted for publication Nov 8, 1968.

Read in part before the New York Neurological Society, New York, Jan 10, 1967.

Reprint requests to the Department of Neuropathology, Maimonides Medical Center, 4802 Tenth Ave, Brooklyn, NY 11219 (Dr. Slotwiner).

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