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December 1927

THE PATHOLOGY OF AMYOTONIA CONGENITA: A DISCUSSION OF ITS RELATION TO INFANTILE PROGRESSIVE MUSCULAR ATROPHY

Arch NeurPsych. 1927;18(6):982-997. doi:10.1001/archneurpsyc.1927.02210060117003
Abstract

Oppenheim,1 who first described amyotonia congenita as a new clinical entity, considered the condition a primary disease either of the muscle or of the lower motor neuron. The first postmortem examination was made by Spiller,2 in 1905, who found the central nervous system entirely normal. To date, the world's literature contains only twenty-two pathologic reports of cases diagnosed clinically as amyotonia congenita. A few authors have corroborated Spiller's first report, whereas others have reported changes within the spinal cord which have been fairly uniform.

Since amyotonia congenita has been linked by some to the myopathies and by others to the infantile type of progressive muscular atrophy, a study of the disease has some importance in a consideration of the degenerative atrophies as a group.

REPORT OF CASE 

Clinical History.  —A girl, aged 6 months, was admitted to the Sarah Morris Hospital on Jan. 15, 1925, with generalized muscular

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