Progressive supranuclear palsy diagnosis and confounding features: Report on 16 autopsied cases

Abstract

We evaluated 16 (15 men, 1 woman) autopsy-verified progressive supranuclear palsy (PSP) cases during 31 years (1969-2000) for clinical diagnosis and the course of the disease. The onset was gait difficulty or postural instability in 9 (56.3%), general motor slowing in 3 (18.8%), and tremor in 2. One case had onset with cognitive decline and 1 as hemidystonia. Four cases had supranuclear ophthalmoplegia (SNO) at the first assessment and were diagnosed as PSP. By last assessment, PSP diagnosis was made in 4 additional cases, but in 8 (50%) who never manifested ophthalmoplegia (mean 9.8 years after onset), PSP diagnosis was not made. Other manifestations included bulbar symptoms in 13 (81.3%), and cognitive impairment in 10 (62.5%) during the course of illness. Fifteen cases received levodopa, amantadine, anticholinergics, dopamine agonists, and selegiline in different combinations with symptomatic benefit in 9 of 15 (60%). Five had some improvement on levodopa alone and 3 showed more improvement when a dopamine agonist was added to levodopa. In general, the benefit was minimal and occurred only early in the course of illness. The mean age at onset was 63.7 (range, 53-85) years. Mean duration at PSP diagnosis was 4.8 (range, 2-11) years. Mean survival was 8.6 (range, 3-24) years and mean age at death was 72.3 (range, 60-89) years. When the different diagnostic criteria recommended in the literature were used, the accuracy of clinical diagnosis did not improve substantially.

Natural History of Progressive Supranuclear Palsy: A Clinicopathologic Study from a Population of Brain Donors

“…The demographic characteristics of our patients were similar to previously published series (table 2) [1, 11, 13, 16,22,23,24,25,26,27,28]. Male gender has been predominant ranging from 54% [16] to 93.7% [22].…”

“…Male gender has been predominant ranging from 54% [16] to 93.7% [22]. The age at onset also ranges from 62 years [29] to 67.6 (in this series).…”

“…Gait disorder varies from 66.6% [13] to 100% [28] in different series. Similarly, instability also varies from 81.3 [22] to 100% [28]. The prevalence of falls also ranges between 60 [29] and 100% [28].…”

“…The age at onset also ranges from 62 years [29] to 67.6 (in this series). The mean duration of the disease from onset to death ranges from 5 [29] to 8.6 [22]. …”

Parkinson's Disease in the Very Old - Clinicopathological Observations

“…That is similar to another autopsy report [32]. As reported by others, these PSP cases did not manifest ophthalmoplegia which made the clinical diagnosis difficult [32,33]. In our study there were no cases of MSA or CBD -the other two well-known degenerative variants of PS in our study.…”