Objective: To assess the sensitivity and specificity of the diagnostic criteria for progressive supranuclear palsy (PSP).

Background: The International Parkinson and Movement Disorder Society criteria for the diagnosis of PSP (the MDS criteria) were published in 2017. We retrospectively assessed the sensitivity and specificity of the MDS criteria in autopsy-confirmed patients and compared with the National Institutes of Neurological Disease and Society for Progressive Supranuclear Palsy criteria (the NINDS-SPSP criteria).

Method: We identified patients with a neuropathological diagnosis of PSP, and patients with clinical diagnosis of PSP with non-PSP pathology. We ascertained whether patients met the MDS criteria and assessed their sensitivity and specificity. We also assessed the patients’ early clinical presentation using the MDS criteria.

Results: We identified 16 patients with PSP pathology and seven clinically diagnosed as PSP patients with non-PSP pathology. The sensitivity of the MDS and NINDS-SPSP criteria was 81% and 69%, respectively, while the specificity of the MDS and NINDS-SPSP criteria was 43% and 57%, respectively. All patients with PSP pathology fulfilled, at least, one clinical subtype of the MDS criteria. The leading initial clinical subtype was “suggestive of PSP with predominant postural instability,” followed by “possible PSP with progressive gait freezing.” Overall, 19% of patients with PSP pathology fulfilled some mandatory exclusion criteria of the MDS criteria. Moreover, all clinically diagnosed PSP patients with non-PSP pathology fulfilled, at least, one clinical subtype of the MDS criteria, but no patient corresponded to “probable PSP with progressive gait freezing.” The leading initial clinical subtype was “suggestive of PSP with predominant postural instability,” followed by “suggestive of PSP with the predominant frontal presentation.” The sensitivity elevated to 94% if we excluded prominent appendicular ataxia from the MDS mandatory exclusion criteria, and the specificity elevated to 71% if we added the early prominent abnormal behavior and the limbic atrophy to the MDS exclusion criteria.

Conclusion: Compared with the NINDS-SPSP criteria, the sensitivity of the MDS criteria was higher, but the specificity was lower. Hence, some revisions of exclusion criteria are warranted to enhance the sensitivity and specificity of the MDS criteria.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/validation-of-the-diagnostic-criteria-for-progressive-supranuclear-palsy-in-pathologically-confirmed-patients/