This Article  • References  • Full text PDF  • Reply to article  • Send to a friend  • Save in My Folder  • Save to citation manager  • Permissions  Citing Articles  • Citation map  • Citing articles on HighWire  • Contact me when this article is cited  Related Content  • Similar articles in this journal  Social Bookmarking   What's this?

George H. Klinkerfuss, MD

Arch Neurol. 1967;16(2):181-193.

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

FAMILIAL myotonic dystrophy usually has its onset in adult life and shows myotonia and muscle wasting as its principal characteristics. A high incidence of cataracts, frontal baldness, hypothyroidism, testicular or ovarian atrophy, and diabetes mellitus support the multisystem nature of the disease.

The skeletal muscle manifestations have been of principal interest to neurologists. MacDermot¹ and Coers² describe abnormalities of the neuromuscular junction similar to those seen in denervation of muscle. Daniel and Strich³ report proliferation of intrafusal fibers in muscle spindles. However, the main histopathology lies in the skeletal muscle fibers themselves. Variation in individual fiber size, loss of fibers with rare

Fig 1.—Left, A portion of hypertrophic fiber with usual cross striations fills the upper field. In the lower portion of the figure, a large area of degeneration has replaced fibrils with alternating zones of dense and light material. To the right of the . . . [Full Text PDF of this Article]

Author Affiliations
St. Louis

From the Department of Neurology, Washington University School of Medicine, and the Beaumont May Institute of Neurology, St. Louis.

Footnotes
Submitted for publication Sept 2, 1966; accepted Oct 1.

Read in part before the 91st Annual Meeting of the American Neurological Association, Washington, DC, June 15, 1966.

Reprint requests to Washington University School of Medicine, 660 S Euclid, St. Louis 63110.

CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    Twitter     What's this?