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Progressive supranuclear palsy (PSP) is a neurodegenerative disease, classified pathologically as a tauopathy, which all neurologists encounter from time to time. Andrew Larner made the intriguing suggestion that Charles Dickens may have been the first to describe someone with PSP in 1857 in his novel The lazy tour of two idle apprentices:
“A chilled, slow, earthy, fixed old man. A cadaverous man of measured speech. An old man who seemed as unable to wink, as if his eyelids had been nailed to his forehead. An old man whose eyes—two spots of fire—had no more motion than [sic] if they had been connected with the back of his skull by screws driven through it, and riveted and bolted outside, among his grey hair.”
“He had come in and shut the door, and now he sat down. He did not bend himself to sit, as other people do, but seemed to sink bolt upright, as if in water, until the chair stopped him.”
These quotes are certainly suggestive of a character with several features with more than a passing resemblance to PSP.1 From the medical perspective, Dutil, one of Charcot’s pupils, may have described and illustrated the first documented case of PSP in 1889.2 Another early description of the clinical syndrome in 1904 later transpired to be due to a mesencephalic tumour rather than PSP.3 In the early 20th century, cases of PSP were probably misdiagnosed as either arteriosclerotic or post-encephalitic parkinsonism.4 PSP (also known as the Steele-Richardson-Olszewski syndrome) was first described as a clinicopathological entity at the American Neurological Association by Dr J Clifford Richardson in 1963. Because there appeared to be an unusual concentration of cases from the Toronto …