Neurológia pre prax 5/2021

Samuel Alexander Kinnier Wilson (1878–1937), or copper, basal ganglia, chelates, and a new textbook concept

Samuel Alexander Kinnier Wilson (1878–1937) was a British neurologist of American (actually of Scottish-Irish) ancestry. He was born in New Jersey, but when one year old his mother brought him to Edinburgh in her native Scotland. Here, he completed both secondary and medical school. After graduation in 1902, he worked as a house officer at the Edinburgh Royal Infirmary where he spent one year; afterwards, he worked in neurology wards in Paris and Leipzig for three years. In 1905, upon his return to Britain, he joined the National Hospital for the Paralysed and Epileptic, Queen Square, London. In 1912, Wilson was appointed assistant physician at the Westminster Hospital, and a year later he was promoted to assistant physician at Queen Square as well. During World War I, he worked at Queen Square, and right after the war, he was offered a consultant post at King’s College Hospital. He was appointed to the same post at Queen Square in 1921, and for the rest of his active life, he worked as consultant in these two leading London hospitals. He died of coronary causes in 1937. Wilson is remembered primarily for the discovery and description of hepatolenticular degeneration, a disease named after him. The disease was first described in detail in Wilson’s doctoral thesis, defended in Edinburgh in 1911 (for which he won a gold medal from the University of Edinburgh); in 1912, it was submitted as original article in Brain. This original article was published in Brain on 214 pages and has so far been the longest paper to have been published in Brain. However, an effective treatment of Wilson’s disease had remained unclear until the 1950s when Schouwink and Walshe, Jr. succeeded in finding a treatment with penicillamine. The inheritance of Wilson’s disease was clarified only in 1993, more than eighty years after its clinical description. Wilson devoted his final years to compiling a textbook which appeared posthumously in 1940. It is an extraordinary, almost 2,000-page piece of work which served as a reference neurology textbook all over the world for more than thirty years; moreover, it is the last “big” neurology textbook to be compiled by a single author.

Keywords: Samuel Kinnier Wilson, hepatolenticular degeneration, copper, ceruloplasmin