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Rev Neurol (Paris). 2013 Dec;169(12):927-35. doi: 10.1016/j.neurol.2013.04.006. Epub 2013 Oct 11.

Samuel Alexander Kinnier Wilson. Wilson's disease, Queen Square and neurology.

Author information

1
Service de neurologie C, université Claude-Bernard-Lyon I, hospices civils de Lyon, hôpital neurologique Pierre-Wertheimer, 59, boulevard Pinel, 69500 Bron, France; CNRS UMR 5229, centre de neurosciences cognitives, 67, boulevard Pinel, 69675 Bron cedex, France. Electronic address: emmanuel.broussolle@chu-lyon.fr.

Abstract

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 Bicêtre 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.

KEYWORDS:

Dégénérescence hépatolenticulaire; Hepatolenticular degeneration; Histoire; History; Kinnier Wilson; Maladie de Wilson; Mouvements anormaux; Movement disorders; Queen Square; Wilson's disease

PMID:
24125461
DOI:
10.1016/j.neurol.2013.04.006
[Indexed for MEDLINE]

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