UCL Queen Square Institute of Neurology


Samuel Alexander Kinnier Wilson

In 1905, Wilson (1878 - 1937) applied successfully for the post of resident medical officer at the National, he was much influenced by Hughlings Jackson, and described the eponymous Wilson's Disease

Wilson was born in Cedarville, New Jersey. After the death of his father, his bereaved mother brought him to Edinburgh, where his formative years were spent. He graduated cum laude from Edinburgh University in 1902 and, after a house officer post working for Sir Byron Bramwell, professor of medicine at the Royal Infirmary, won a Carnegie Research Fellowship to study neurology in Paris with Pierre Marie and Joseph Babinski. During this Wanderjahre, he immersed himself not only in the advanced anatomo-clinical methods of the Salpêtrière but also in the richness of French culture. After this two-year stage in his neurological training, Wilson moved to Germany where he worked in Leipzig with Paul Fleschig. This influential part of his training allowed Wilson to keep abreast of the extensive German literature throughout the rest of his career.

On his return to the UK in 1905, Wilson applied successfully for the post of resident medical officer at the National Hospital, where, like many others, he was much influenced by Hughlings Jackson, who had retired but continued to visit the hospital and ask the trainees to show him patients of interest.

In 1912, Wilson was appointed assistant physician at the Westminster Hospital, and a year later was promoted to assistant physician at the National Hospital. With a grant from the British Medical Association, he now embarked on a research project in Victor Horsley’s laboratory examining the effect of lesions in the lenticular nucleus in monkeys. After the First World War, he was offered a post at King’s College Hospital and a lectureship in neurology at King’s Medical School, at which point he resigned from the Westminster Hospital. In 1921, Wilson was promoted physician to outpatients at the National Hospital, and a year later became a physician with admission rights.

At the age of 30, in 1908, Wilson wrote ‘A Contribution to the Study of Apraxia’, with a scholarly review of the literature, and, in 1928, a short but highly praised monograph on aphasia. In time, Wilson became the expert on disorders of the basal ganglia. After passing the MRCP examination, Wilson had translated Meige and Feindel’s monograph Les tics et leur traitement (1902; English edition, 1907).

A few years later, he defended his doctoral thesis at the University of Edinburgh entitled ‘A Fatal Familial Nervous Disease Associated with Cirrhosis of the Liver’ whilst working as a junior doctor carrying out post-mortem examinations at the National Hospital. In the dissertation, he described four children or young adults with a rapidly progressive neurological syndrome characterised by tremor, difficulty in swallowing and speaking, muscular weakness, spasticity and fixed dystonic contractures, which he named ‘progressive lenticular degeneration’.

This outstanding piece of work was awarded the Gold Medal by the university in 1911. A brief version appeared in the Lancet, but the following year Wilson published the definitive account in Brain, emphasising the clinical relevance of damage to the lenticular nucleus and the important coexistence of liver cirrhosis. Wilson included an addendum relating to a patient already described by Völsch that he felt sure was an example of progressive lenticular degeneration, and he also unearthed a typical case in Friedrich Theodor von Frerichs’ 'Klinik der Leberkrankheiten' (1858–61). Wilson also acknowledged the earlier reports by his senior colleagues at Queen Square. The 1912 paper rehearsed Gowers’ previous case, with further details of another affected family member provided to Wilson by the index case’s mother. Details of Ormerod’s patient were supplemented by access to the case notes at Queen Square.

Following the review of prior examples, Wilson then reported in detail on the four cases personally observed, of whom three came to autopsy. Wilson provided the most authoritative account of what later came to be recognised as the first treatable metabolic disorder of the brain, in this instance caused by a disorder of copper transport. The paper also offered a new approach to research on motor function of the basal ganglia and demonstrated conclusively that lesions of the lenticular nucleus result in abnormal involuntary movements.

Quite when his Greco-Latin rubric lost favour and became ‘Wilson’s disease’ is uncertain but the eponym was probably adopted by German neurologists who, along with the French, were the first to appreciate the importance of Wilson’s work. In the UK, Greenfield and Denny-Brown were using the eponym by the mid-1930s and, in later life, Wilson was only too happy to refer to ‘my disease’. He vehemently rejected von Strumpell and Westphal’s claims to precedence, arguing that they had failed to emphasise the importance of hepatic pathology. However, Wilson did not like use of the term ‘abdominal Wilson’s disease’ by liver specialists. What Wilson had missed, and continued to deny through most of his career, was the distinctive green ring of copper deposited on Descemet’s membrane of the cornea, first described by Kaiser (1902) and by Fleischer (1903).

His Croonian lectures (1925) were on disorders of motility and muscle tone, in which Wilson speculated that the tremor of Parkinsonism must originate in the cerebral cortex through the influence of the striatum and pallidum. The presentation was illustrated with some of his own cinematographic films recorded in Queen Square. Wilson was among the first to demonstrate persistence of the glabellar tap, which for a while was known at Queen Square as ‘Wilson’s sign’.

In 1920, Wilson founded the Journal of Neurology and Psychopathology, and the contents of the journal reflect Wilson’s interest in psychiatry and medical psychology, perhaps stemming from his continental training.

He read and spoke German and French fluently, travelled widely and had many influential friends in continental Europe and America, explaining why Wilson commanded a far greater reputation overseas than at home. He became a friend of Charlie Chaplin, and on one of his many American trips was invited to Chaplin’s Californian ranch. This encounter may have also led to his early interest in the use of cinematography as an aid to the teaching of neurology.

The other major achievement for which Wilson is remembered in the UK is his encyclopaedic two volume 1,838-page textbook Neurology (1940) with its 276 illustrations and 16 plates.

Queen Square: A History of the National Hospital and its Institute of Neurology https://www.nationalbrainappeal.org/product/queen-square-history-book/