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Fifty years of progressive supranuclear palsy
  1. Carlo Colosimo1,
  2. Thomas H Bak2,
  3. Matteo Bologna3,
  4. Alfredo Berardelli1,3
  1. 1Department of Neurology and Psychiatry, “Sapienza” University, Rome, Italy
  2. 2School of Philosophy, Psychology and Language Sciences (PPLS) & Centre for Clinical Brain Sciences (CCBS), University of Edinburgh, Edinburgh, UK
  3. 3Neuromed Institute IRCCS, Pozzilli (IS), Italy
  1. Correspondence to Dr Carlo Colosimo, Dipartimento di Neurologia e Psichiatria, Sapienza Università di Roma, Viale dell'Università 30, Roma 00185, Italy; carlo.colosimo{at}uniroma1.it

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Introduction

Fifty years ago, in 1963, at the American Neurological Association annual meeting in Atlantic City, John Clifford Richardson, John Steele and Jerzy Olszewski presented eight patients seen in Toronto who had gaze paresis, nuchal rigidity, gait difficulties, dysphagia, dysarthria and dementia (figure 1). The description of these cases was published in the Transactions of the American Neurological Association,1 ,2 and led in the following year to what is considered the first comprehensive description of progressive supranuclear palsy (PSP) published.3 Here, Richardson and coworkers described the clinical features in nine patients and detailed the neuropathological changes in seven of them. In this report, the authors fended off arguments that the condition in the patients they described reflected a genetic mutation or environmental toxin geographically confined to the Toronto area.

Figure 1

First page of the original description of progressive supranuclear palsy by Richardson, Steele and Oszewski.1

Some speculate that earlier descriptions might exist,4 given that Richardson et al explicitly mention a case described by Chavany et al5 Much earlier, in 1889, Dutil cited by Goetz6 also reported a case of abnormal posture in a parkinsonian patient (‘attitude anormale dans la paralysie agitante’). What is most intriguing, however, is that the first description possibly came not from a physician but from a novelist. In ‘The Lazy Tour of Two Idle Apprentices’ Charles Dickens describes a memorable appearance of a man

A chilled, slow, earthy, fixed man. A cadaverous man of measured speech. A man who seemed as unable to wink, as if his eyelids had been nailed to his forehead. A man whose eyes—two spots of fire—had no more motion than if they had been connected with the back of his skull by screws driven through them, and riveted and bolted outside among his gray hair. …

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