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Comparison With Childhood X-Linked Pseudohypertrophic Muscular Dystrophy

Paul W. Hathaway, MD; W. King Engel, MD; Hans Zellweger, MD

Arch Neurol. 1970;22(4):365-378.

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

THE ETIOLOGY and pathogenesis of childhood x-linked pseudohypertrophic muscular dystrophy ("Duchenne dystrophy") remains obscure, even though the histopathology and clinical picture were described by Duchenne¹ in 1868, and Erb² formalized the concept of a primary dystrophic disease of muscle in 1891. Meryon³ in 1864 suggested that it may be "... an idiopathic disease of the muscle, dependent perhaps on defective nutrition." Recent emphasis has centered on multiple possibilities, including a metabolic defect intrinsic to the muscle fiber or muscle-fiber membrane,^4-7 a functional vascular abnormality,^8-11 an autoimmune process,¹² an abnormal circulating serum factor,¹³ an endocrine abnormality,^10,14,15 and a primary defect involving connectivetissue proliferation.¹⁶

The earliest histological changes seen in minimally weak, or even clinically normal, muscles of patients with Duchenne muscular dystrophy are small foci of grouped muscle fibers undergoing necrosis or regeneration, all fibers of the group being in about the . . . [Full Text PDF of this Article]

Author Affiliations
Bethesda, Md

From the Medical Neurology Branch, National Institute of Neurological Diseases and Stroke, Bethesda, Md.

Footnotes
Submitted for publication Aug 11, 1969; accepted Sept 29.

Reprint requests to Medical Neurology Branch, National Institute of Neurological Diseases and Stroke, Bethesda, Md 20014 (Dr. Engel).

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