Article

This historical article describes the life and work of the British physician
Samuel Alexander Kinnier Wilson (1878-1937), who was one of the world's greatest
neurologists of the first half of the 20th century. Early in his career, Wilson
spent one year in Paris in 1903 where he learned from Pierre-Marie at Bicetre
Hospital. He subsequently retained uninterrupted links with French neurology. He
also visited in Leipzig the German anatomist Paul Flechsig. In 1904, Wilson
returned to London, where he worked for the rest of his life at the National
Hospital for the Paralysed and Epileptic (later the National Hospital for Nervous
Diseases, and today the National Hospital for Neurology and Neurosurgery) in
Queen Square, and also at Kings' College Hospital. He wrote on 'the old motor
system and the new', on disorders of motility and muscle tone, on the epilepsies,
on aphasia, apraxia, tics, and pathologic laughing and crying, and most
importantly on Wilson's disease. The other objective of our paper is to
commemorate the centenary of Wilson's most important work published in 1912 in
Brain, and also in Revue Neurologique, on an illness newly recognized and
characterized by him entitled "Progressive lenticular degeneration, a familial
nervous disease associated with liver cirrhosis". He analyzed 12 clinical cases,
four of whom he followed himself, but also four cases previously published by
others and a further two that he considered in retrospect had the same disease as
he was describing. The pathological profile combined necrotic damage in the
lenticular nuclei of the brain and hepatic cirrhosis. This major original work is
summarized and discussed in the present paper. Wilson not only delineated what
was later called hepato-lenticular degeneration and Wilson's disease, but also
introduced for the first time the terms extrapyramidal syndrome and
extrapyramidal system, stressing the role of the basal ganglia in motility. The
present historical work emphasizes the special contributions made by Wilson to
the study of movement disorders, including akinesia and bradykinesia in
Parkinson's disease, and their relation to basal ganglia pathology.
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