Hostname: page-component-78c5997874-8bhkd Total loading time: 0 Render date: 2024-11-04T18:58:16.527Z Has data issue: false hasContentIssue false

Alan H. Emery and Marcia L.H. Emery , The History of a Genetic Disease: Duchenne Muscular Dystrophy or Meryon’s Disease, 2nd edn, Oxford Histories of Medicine (Oxford: Oxford University Press, 2011), pp. xxiv $+ $ 231, £80.00/£155.00, hardback, ISBN: 978-0-1995-9147-3.

Published online by Cambridge University Press:  20 August 2012

Irwin M. Siegel*
Affiliation:
Rush University Medical Center
Rights & Permissions [Opens in a new window]

Abstract

Type
Review
Copyright
© The Author 2012 Published by Cambridge University Press

The first edition of this book was published in 1995 and favourably reviewed in this journal by Victor Dubowitz, Medical History, 40, 3 (1996) 409–10. This second edition has been thoughtfully revisited. Information and materials, particularly those relating to current research in the diagnosis and treatment of the muscular dystrophies, have been updated. Illustrations have been modified, references expanded and appended at the end of each chapter rather than all together at the termination of the text, the layout has been changed, even the typeface is different, resulting in a more comfortable format for the reader.

This notable book offers a historical study of muscular dystrophy that reads like an informed novel. Succinct profiles of the numerous clinicians and scientists contributing to this story, from Victorian times to the present (Charles Bell, Giovanni Conte, Richard Partridge, William Little, William Gowers, Kay Davies, Louis Kunkel, Eric Hoffman, to mention but a few) are augmented by fuller biographies of the two major players in the epic – Duchenne deBoulogne (1806–75) and Edward Meryon (1807–80). Their contemporaneous lives, professional and personal, are chronicled at length.

A description of the muscular dystrophies follows, from refining the clinical picture through the latest techniques in molecular genetics. Included are chapters on resolution of heterogenicity, nosology, recognition of other types of muscular dystrophy, biochemical diagnosis in carrier detection, pathogenesis of Duchenne muscular dystrophy and the search for the gene. The book concludes with a thought-provoking review of current trends and the future – all of this painstakingly detailed and referenced.

The authors present a persuasive case for at least appending Meryon’s name to the eponymic Duchenne (Meryon’s) disease. Although Duchenne published a detailed description of the disease in the 1860s, the first systematic study of this condition was presented by Edward Meryon in the 1850s. For a variety of reasons (covered in the book) history has neglected to credit Meryon appropriately. The Emerys have researched and advocated for his recognition. Medical history offers many examples of such linked eponyms where required. In neurology alone, Landry–Guillain–Barré syndrome and Creutzfeldt–Jakob disease come to mind.

The Emerys are to be commended for giving us a scholarly yet readable account of muscular dystrophy that notably improves on their first edition, and for continuing their advocacy for the justified recognition of Edward Meryon and his original description of Duchenne/Meryon disease. It was Sir William Osler who once said ‘In science the credit goes to the man who convinces the world, not to the man to whom the idea first occurs’. I agree with the Emerys that credit is long overdue for Edward Meryon’s contribution to the characterisation of childhood muscular dystrophy and believe you will also agree, should you read this remarkable book.