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Progressive supranuclear palsy (PSP) is uncommon and often overshadowed by PD in public attention and as a target for experimental therapeutics. However, patients with PSP know it is an inexorably progressive and largely untreatable condition. Three articles in this issue of Neurology shed new light on PSP and may facilitate efforts to test treatments for this disorder.
In the first of these reports, Goetz et al.1 describe the natural history of 50 patients with clinically probable PSP followed at their movement disorders clinic. The study aimed to establish the rate at which patients reached five clinical disability milestones: (1) unintelligible speech; (2) inability to walk independently; (3) inability to stand unassisted; (4) being wheelchair bound; and (5) requirement for a feeding tube. Remarkably, 88% of subjects reached at least one of the milestones during the 4 to 4.5 year period of observation. Median survival from first symptom averaged 7.9 years and was 6.5 years in the 21 patients followed to death. This study shows that gait and speech impairments develop rapidly in PSP and can be monitored using standard clinical rating scales. Goetz et al. suggest that these indices could measure the impact of a putative …
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