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.

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. …