[Skip to Navigation]
September 1971

Infantile Spinal Muscular Atrophy

Author Affiliations

Durham, NC
From the Divisions of Neurosurgery and Neurology, Duke University Medical Center, and the Durham Veterans Administration Hospital, Durham, NC.

Arch Neurol. 1971;25(3):276. doi:10.1001/archneur.1971.00490030102013

WHEN Johann Hoffmann (1857 to 1919), professor of neurology at Heidelberg and discoverer of the digital reflex that bears his name,1,2 published the first of his papers on infantile progressive muscular atrophy in 1893,3-6 Guido Werdnig (1844 to 1919),7 a neurologist in Graz, Austria, had already reported two such cases.8,9 Both men described the same basic features of this familial degenerative disease, which usually leads to death by the age of 4 years.10,11

Hermann Oppenheim12 then complicated the subject with his brief report in 1900 of a benign form of congenital muscular hypotonia (amyotonia congenita), and for many years, it was believed that amyotonia congenita began in the neonatal period, whereas progressive muscular atrophy began later in infancy. We now know that this distinction in terms of age of onset is not valid.13 The term amyotonia congenita has generally been discarded in

Add or change institution