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L. P. Heffernan, B Sc, MD; N. B. Rewcastle, MB; ChB; J. G. Humphrey, MD, FRCP(C)

Arch Neurol. 1968;18(5):529-542.

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

TWO REPORTS appeared almost simultaneously, in 1963, describing a new myopathy. The condition was characterized pathologically by nuerous inclusions in muscle fibers referred to variously as myogranules, nemaline (thread-like) structures, or rod-like bodies.^1,2

These initial cases together with other subsequent reports^3-8 were considered to represent a disease entity with identical pathological findings and many of the following clinical features: "floppy infant"; delayed motor development; proximal limb weakness, usually with hypotonia; reduced muscle bulk; reflexes usually absent; onset from birth, most noticeable on initial attempt to walk; associated deformities, particularly pes cavus, high-arched palate, kyphosis or scoliosis; possibly (autosomal) dominant inheritance; and extremely slow or nonprogressive course.

Since this disorder was first brought to light, Engel and Resnick⁹ and Engel¹⁰ described a disorder in which there were rodlike structures but this disorder was of late onset, noninherited, and progressive. The question has therefore been raised whether . . . [Full Text PDF of this Article]

Author Affiliations
Toronto

From the Division of Neuropathology, Banting Institute, Toronto, Canada.

Footnotes
Submitted for publication June 22, 1967; accepted Nov 13.

Reprint requests to Division of Neuropathology, the Banting Institute, 100 College St, Toronto 2, Ontario, Canada (Dr. Rewcastle).

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