Objective: To examine the evolution of diagnostic certainty and clinical predominance types as defined by the movement disorder society (MDS) criteria for the clinical diagnosis of PSP (Höglinger et al, 2017) during the course of disease in autopsy-confirmed patients with PSP.

Background: Three degrees of diagnostic certainty (“suggestive of” = s.o., “possible”, or “probable”), and definition of clinical predominance types (PSP-RS, PSP-PGF, PSP-P, PSP-F, PSP-OM, PSP-SL, PSP-CBS, PSP-PI) were implemented into the new clinical diagnostic criteria for PSP (MDS criteria for the clinical diagnosis of PSP, Höglinger et al, 2017). The evolution of diagnostic certainty and predominance types over the course of disease in PSP patients according to the MDS-criteria has not been studied so far. However, this is relevant for further understanding the natural history and clinical spectrum of PSP.

Methods: Features relevant for the diagnosis of PSP according to the MDS-diagnostic criteria were collected in 187 autopsy-confirmed PSP patients by chart review. Diagnostic certainty and PSP-predominance types according to the MDS-diagnostic criteria were determined for each patient and each year.

Results: According to the MDS-PSP diagnostic criteria, 62% (n=115) of patients had a clinical diagnosis of PSP in the first year of disease, as opposed to 14% (n=26) according to the NINDS SPSP criteria, and 98% (n=183) at final record, as opposed to 79% (n=147) according to the NINDS/SPSP criteria. A diagnosis of s.o. PSP was present in 44% (n=83) of patients in the first year. Of these, 77% (n=64) had a diagnosis of “probable” PSP at final record. In the first year of disease, the variability of predominance types was greatest, and PSP-RS represented only 11% of patients. At final record, 56% patients had a PSP-RS predominance type.

Conclusions: The conversion of s.o. PSP into possible and probable PSP in 72% of the cases shows that the concept of s.o. PSP indeed allows identification of PSP patients at a very early clinical stage. Furthermore, our data suggest that many initially variant PSP presentations converge to a PSP-RS phenotype during the course of the disease.

References: MDS criteria for the clinical diagnosis of PSP, Höglinger et al, 2017.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/evolution-of-diagnostic-certainty-and-psp-predominance-types-in-187-pathologically-confirmed-psp-patients/