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February 1950

COURSE AND SYMPTOMS OF PROGRESSIVE INFANTILE MUSCULAR ATROPHY: A Follow-Up Study of One Hundred and Twelve Cases in Denmark

Author Affiliations

COPENHAGEN, DENMARK

Arch NeurPsych. 1950;63(2):218-228. doi:10.1001/archneurpsyc.1950.02310200026003
Abstract

PROGRESSIVE infantile muscular atrophy was first described by Werdnig1 and Hoffmann2 about fifty years ago. This disorder is defined as a hereditary familial degeneration of the motor cells in the anterior horns of the spinal cord causing progressive atrophy and paralysis of the skeletal muscles. The atrophy begins in the muscles of the back and in the proximal muscles of the limbs and spreads gradually to the whole body. Life is threatened when the nerve cells supplying the respiratory muscles are affected, particularly when this involvement is followed by infection of the respiratory tract. Up to the time of this report, approximately 200 cases, occurring in about 120 families, have been reported. However, the reader will still find the best description of the disease in Hoffmann's papers. Many later single observations and short series of cases have contributed to our knowledge of the disease and its relation to

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