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Features of Ocular Myopathy, Distal Myopathy, and Myotonic Dystrophy

DONALD L. SCHOTLAND, MD; LEWIS P. ROWLAND, MD

Arch Neurol. 1964;10(5):433-445.

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

Introduction

Because of their hereditary nature, human muscular dystrophies are assumed to be inborn errors of metabolism. The ultimate classification of these diseases will depend upon recognition of the specific metabolic fault in each. At the present time, however, McArdle's disease (deficiency of muscle phosphorylase) is the only inherited myopathy in which the enzymatic abnormality has been identified. Classification, therefore, depends upon clinical grounds, and these are sometimes imprecise.

The myopathy of myotonic dystrophy is different from most other dystrophies for three reasons: (1) Limb weakness is primarily distal; (2) cranial muscles (especially facial, masticatory, and oropharyngeal) are frequently affected; and (3) myotonia is present. If myotonia were not present, the syndrome would resemble "distal myopathy." Furthermore, the ocular muscles are said to be involved in some cases of myotonic dystrophy, and in some cases of "ocular myopathy," other cranial muscles and limb muscles may be involved. Transitional forms of . . . [Full Text PDF of this Article]

Author Affiliations
NEW YORK

Departments of neurology and pathology (neuropathology), College of Physicians and Surgeons, Columbia University and the Neurological Clinical Research Center, Columbia-Presbyterian Medical Center.

Footnotes
Submitted for publication Jan 13, 1964; accepted Jan 27.

Visiting Fellow in Neuropathology, College of Physicians and Surgeons, Columbia University, USPHS grants BT-815 and 2B-5062 (C7), National Institute of Neurological Diseases and Blindness, National Institutes of Health (Dr. Schotland); Associate Professor of Neurology, College of Physicians and Surgeons, Columbia University, Co-Director, Neurological Clinical Research Center (Dr. Rowland).

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