Involvement of the external ocular muscles in classical examples of progressive muscular dystrophy is rare. However, an isolated dystrophy of these muscles, inappropriately designated as chronic progressive nuclear atrophy, has long been recognized. Excellent histological studies have convincingly revealed its myopathic nature.1,2 However, that some instances of this form of dystrophy may be related to classical muscular dystrophy with involvement of other skeletal musculature is apparent from reports revealing alteration of the latter subsequent to the ocular disease.1,2
Although the exact etiology of progressive muscular dystrophy is unknown, it is commonly held that hereditary influence may play a prominent role in its development. Adams, Denny-Brown, and Pearson3 have considered the defect to represent an inborn metabolic fault affecting certain muscles, particularly those developing early in fetal life, which cannot be sustained in a normal state. Recently, Beckett and Bourne4 have observed an increase of alkaline phosphatase
SENITA GR, FISHER ER. Progressive Dystrophic External Ophthalmoplegia Following Trauma. AMA Arch Ophthalmol. 1958;60(3):422–426. doi:10.1001/archopht.1958.00940080440011
Monkeypox Resource Center
Customize your JAMA Network experience by selecting one or more topics from the list below.