I’ve been looking around for journal articles that review autopsied cases of PSP and relate these back to the clinical symptoms seen. This 2002 article doesn’t have a large sample — only 16 cases. In half of these cases, PSP was never diagnosed while the patient was alive. All 16 cases were seen by one neurologist between 1969 and 2000 at a Canadian university. (Incredibly, 30% of all patients seen at this movement disorders clinic in Canada agree to autopsy!)
This was the most interesting info in the article:
“Those with incorrect final diagnosis were mean 64.4 years old at onset (vs. 63 years (for those clinically diagnosed with PSP)), and had longer survival (mean, 9.8 vs. 7.5) than those diagnosed clinically. The development of falls, evolution to H&Y stage >= 3 disability, bulbar symptoms, and cognitive decline were also delayed in the undiagnosed group. Nearly two-thirds of those not diagnosed as PSP benefited from LD (levodopa) alone, but none of those diagnosed as PSP during life improved.”
I view Dr. David Williams’ research from Queen Square Brain Bank in the UK to be a directly connected to this Canadian research. Dr. Williams has found that there are two main clinical types of PSP — cases of Richardson’s syndrome (RS) made up 54% of all cases, and were characterized by the early onset of postural instability and falls, supranuclear vertical gaze palsy and cognitive dysfunction; cases of PSP-parkinsonism (PSP-P) made up 32% of all cases, and were characterized by asymmetric onset, tremor, a moderate initial therapeutic response to levodopa and were frequently confused with Parkinson’s disease.
Another piece of info from the Canadian research that I found distressing was: “Our data indicate that accurate clinical diagnosis of PSP is most likely within 5 years of onset.” That’s a long, frustrating wait.
When the Canadian research abstract says “Mean duration at PSP diagnosis was 4.8 (range, 2-11) years,” I take this to mean that the symptoms of PSP began 4.8 years before a PSP diagnosis was rendered. So I believe that the other 8 who were never given a PSP diagnosis during life were not part of this calculation of “mean duration at PSP diagnosis.”
Note that the PSP diagnostic criteria only became set in 1996. (These are the NINDS-SPSP criteria referred to in the excerpts. The authors are not fans of the ’96 criteria.) Despite the application of different diagnostic criteria, the diagnostic accuracy would not have improved, the researchers said.
The abstract follows.
Robin
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Movement Disorders 2002 Nov;17(6):1255-64.
Progressive supranuclear palsy diagnosis and confounding features: report on 16 autopsied cases.
Birdi S, Rajput AH, Fenton M, Donat JR, Rozdilsky B, Robinson C, Macaulay R, George D.
Division of Neurology, University of Saskatchewan, Saskatoon, Saskatchewan, Canada.
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.
PubMed ID#: 12465065 (see pubmed.gov for abstract)