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Kinnier Wilson’s puzzling features of amyotrophic lateral sclerosis
  1. Martin R Turner1,
  2. Andrew Eisen2,
  3. Matthew C Kiernan3,
  4. John Ravits4,
  5. Michael Swash5
  1. 1 Nuffield Department of Clinical Neurosciences, Oxford University, Oxford, UK
  2. 2 Department of Neurology, University of British Columbia, Vancouver, British Columbia, Canada
  3. 3 Brain and Mind Centre, Sydney Medical School, The University of Sydney; Department of Neurology, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia
  4. 4 Department of Neurosciences, University of California San Diego, La Jolla, California, USA
  5. 5 The Royal London Hospital, London, UK
  1. Correspondence to Professor Martin R Turner, Nuffield Department of Clinical Neurosciences, Oxford University, John Radcliffe Hospital, OX - OX3 9DU, UK; martin.turner{at}ndcn.ox.ac.uk

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Introduction

It is more than 150 years since Jean-Martin Charcot (1825–1893) published the first descriptions of the adult neurodegenerative disorder amyotrophic lateral sclerosis (ALS).1 Though an unmistakable clinical syndrome in its classical form, ALS has been increasingly understood as representing a complex clinical and molecular syndrome that overlaps with frontotemporal dementia (FTD).2 Samuel Alexander Kinnier Wilson (1878–1937) (figure 1) is perhaps best known for his eponymous hepatolenticular degenerative syndrome linked to copper dysregulation.3 He is also distinguished as the founding editor, in 1920, of the Journal of Neurology & Psychopathology, which then became the Journal of Neurology, Neurosurgery and Psychiatry.4 It is, however, his two-volume textbook Neurology published posthumously in 1940 that stands as the greatest testament to the breadth of his clinical and scientific insight. Chapter 64 demonstrates his extraordinary understanding of the clinical heterogeneity of ALS, in which he highlights five ‘puzzling features’:

Figure 1

Samuel Alexander Kinnier Wilson (1878–1937) was the founding editor, in 1920, of what became the Journal of Neurology, Neurosurgery and Psychiatry.

  1. implication of certain afferent tracts and of ventro-lateral columns in the cord;

  2. irregular degree of morbid change as between spinal nerve cell, nerve fibre and muscle, respectively;

  3. similar disparity as regards Betz cell and pyramidal fibre lesions;

  4. the acuteness of many cases, suggesting a toxic and diffuse process, yet the lesions appear to be systematised;

  5. occasional discord between clinical and pathological findings.

Intrigued by the issues raised by each of his five comments, we here discuss them in light of developments in understanding of the syndrome of ALS during the subsequent 75 years.5

Implication of afferent tracts and ventro-lateral columns in the cord

Kinnier Wilson set out his thoughts on ALS nearly half a century after Charcot’s description. The latter recognised ALS as what he considered to be a uniquely ‘deuteropathic’ condition, one characterised by combined lower motor neuron (LMN) and …

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