This looks like a carefully-done study for 23 PSP patients though I don’t think there’s pathological confirmation of the diagnosis. Of the 23, 14 had the Richardson’s syndrome (RS) type of PSP, called “classic PSP.” Nine had PSP-parkinsonism, the second most common type of PSP.
Previous studies suggest “that the clinical presentation of the two subtypes differs especially in the first 2 years of disease and then converges.” This German study attempted to confirm that view.
They found that “RS and PSP-P show considerable symptoms overlap during the disease course when using routine assessments, with persisting differences regarding non-motor symptoms.” Those non-motor symptoms include psychological, cognitive, and behavioral deficits.
We can glean two other points from the abstract:
* “RS patients showed shorter time from disease onset to diagnosis…”
This is certainly because RS patients have “classic PSP” so neurologists are better able to recognize it as being PSP.
” Shorter disease duration of the comparably affected RS patients indicates that this subtype has an accelerated disease progression at early disease stages.”
We know from previous studies that those with the RS type of PSP have a shorter survival time than those with the PSP-P type.
Robin
Journal of Neural Transmission. 2011 Jan 5. [Epub ahead of print]
In vivo comparison of Richardson’s syndrome and progressive supranuclear palsy-parkinsonism.
Srulijes K, Mallien G, Bauer S, Dietzel E, Gröger A, Ebersbach G, Berg D, Maetzler W.
Department of Neurodegeneration, Hertie Institute for Clinical Brain Research and German Center for Neurodegenerative Diseases, University of Tuebingen, Tuebingen, Germany
Abstract
Richardson’s syndrome (RS) and progressive supranuclear palsy-parkinsonism (PSP-P) are the most common subtypes of PSP.
Post-mortem data suggests that the clinical presentation of the two subtypes differs especially in the first 2 years of disease and then converges. This hypothesis has, to our knowledge, never been confirmed in a living cohort.
Medical history was used to define subtypes retrospectively in 23 consecutive PSP patients from our outpatient clinic specialized in movement disorders. 14 patients suffered from RS, and 9 from PSP-P.
Using a prospective cross-sectional approach, clinical, cognitive, behavioral, speech and biochemical (cerebrospinal fluid tau levels) features were compared.
RS patients showed shorter time from disease onset to diagnosis and more neuropsychological and neurobehavioral deficits than PSP-P patients, but differed not significantly with regard to clinical and biochemical features.
RS and PSP-P show considerable symptoms overlap during the disease course when using routine assessments, with persisting differences regarding non-motor symptoms.
Shorter disease duration of the comparably affected RS patients indicates that this subtype has an accelerated disease progression at early disease stages.
PubMed ID#: 21207078