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Biochemical and Electron Microscopic Studies on Myotonia Congenita and Myotonia Dystrophica

Frederick J. Samaha, MD; J. Michael Schroeder, MD; Jean Rebeiz, MD; Raymond D. Adams, MD

Arch Neurol. 1967;17(1):22-33.

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

MYOTONIA, the denominating characteristic of three genetically determined diseases of muscle (myotonia congenita, myotonia dystrophica, and paramyotonia), and dystrophy of muscle fibers, a feature of one of these diseases (myotonia dystrophica), remain obscure.

Concerning the localization of myotonia, a number of investigators believe it might reside in the muscle cell. Grund¹ and later Schaffer² noted the persistence of this phenomenon after complete block of the nerve fibers supplying the muscle; and Gregor and Schilder³ and later Lindsley and Curnen⁴ observed it during the relaxation phase of contraction, when few nerve impulses were reaching the muscle. Brown and Harvey⁵ in a myotonic disease of goats also demonstrated that complete denervation of muscle failed to abolish the myotonia. Landau⁶ and Floyd et al⁷ have shown that curare has no effect on human myotonia. Denny-Brown and Nevin⁸ confirmed this localization but also obtained evidence . . . [Full Text PDF of this Article]

Author Affiliations
Boston

From the Kennedy Memorial and Experimental Neuropathology Laboratories, Massachusetts General Hospital, the Department of Neurology, Haevard Medical School; and the Retina Foundation, Institute of Biological and Medical Sciences, Boston. Dr. Schroeder is a fellow of the Max-Kade Foundation, New York.

Footnotes
Submitted for publication Dec 2, 1966; accepted Jan 14, 1967.

Reprint requests to Department of Neurology, Massachusetts General Hospital, Boston 02114 (Dr. Samaha).

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