Progressive supranuclear palsy diagnosis and confounding features: report on 16 autopsied cases.
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.
Birdi
Sam
S
Division of Neurology, University of Saskatchewan, Saskatoon, Saskatchewan, Canada.
Rajput
Ali H
AH
Fenton
Mark
M
Donat
Jeffery R
JR
Rozdilsky
Bohdan
B
Robinson
Christopher
C
Macaulay
Rob
R
George
David
D
D000368:Aged; D000369:Aged, 80 and over; D001921:Brain / Q000473:pathology; D003937:Diagnosis, Differential; D018491:Dopamine Agonists / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D005260:Female; D006801:Humans; D007980:Levodopa / Q000627:therapeutic use; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009460:Neurologic Examination / Q000187:drug effects; D011379:Prognosis; D013494:Supranuclear Palsy, Progressive / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology; D016896:Treatment Outcome
D018491:Dopamine Agonists; D007980:Levodopa