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May 1968

The Spectrum of Rod Myopathies

Author Affiliations

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

Arch Neurol. 1968;18(5):529-542. doi:10.1001/archneur.1968.00470350087008

TWO REPORTS appeared almost simultaneously, in 1963, describing a new myopathy. The condition was characterized pathologically by nuṃerous 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 reports3-8 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

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