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December 1951

Werdnig-Hoffmann's Infantile Progressive Muscular Atrophy.

Author Affiliations

By Sven Brandt. Price, 20 Danish kronen. Pp. 328, with 39 figures. Ejnar Munksgaard, 6, Nörregade, Copenhagen, Denmark, 1950.

AMA Am J Dis Child. 1951;82(6):785-786. doi:10.1001/archpedi.1951.02040040806013

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The book is an exhaustive review of the subject of generalized muscular flabbiness in infancy and childhood, with special reference to the well-defined syndrome, Werdnig-Hoffmann infantile progressive muscular atrophy, to the more heterogeneous group that, in the author's opinion, falls into the category of amyotonia congenita (Oppenheim's disease), and to the rarer examples of what appears to be muscular dystrophy. The first chapter is a review of the literature, following which the writer presents a survey of 131 Danish cases in which these diagnoses, or equivalent ones, had been recently made in various clinics.

The writer investigated by personal interview with the families the 71 cases in which death had occurred and personally examined 60 patients. During this undertaking 25 secondary cases were found, making a total material of 156 cases.

Diagnostic criteria are discussed and the importance of properly performed biopsy stressed as the most important single laboratory aid.

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