Sensitivity and Specificity of Diagnostic Criteria for Progressive Supranuclear Palsy

Abstract

Background: In 2017, the Movement Disorders Society put forward new clinical criteria for the diagnosis of PSP recognizing diverse PSP phenotypes. Objectives: In this study, we compare the sensitivity and specificity of the new criteria to the National Institutes of Neurological Disease and Society for Progressive Supranuclear Palsy (NINDS-SPSP) criteria at different time points. Methods: Patients with clinical parkinsonism, clinical and/or neuropathological diagnosis of PSP were identified from the Society for Progressive Supranuclear Palsy (SPSP) brain bank. All patients had neuropathologic diagnoses and detailed clinical examination performed by a neurologist at one of the three Mayo Clinic sites located in Florida, Arizona and Minnesota. Clinical symptoms and signs were abstracted retrospectively in a blinded fashion and used to determine whether or not patients met either diagnostic criterion. Patients were divided into early and late disease stage groups using a 3-year cut off. Results: A total of 129 patients were included of which 66 (51%) had PSP pathology. The remainder had other neurodegenerative diseases. The overall sensitivity of the Movement Disorders Society criteria was 87.9% compared to 45.5% for National Institutes of Neurological Disease and Society for Progressive Supranuclear Palsy criteria, whereas the specificity of the Movement Disorders Society criteria probable PSP criteria was 85.7% and 90.5% for National Institutes of Neurological Disease criteria. Individual patients were noted to have features of multiple PSP phenotypes. Conclusion: The Movement Disorders Society criteria recognize several phenotypes of PSP and hence have a higher sensitivity than the previous criteria.

How to apply the movement disorder society criteria for diagnosis of progressive supranuclear palsy

“…Because specificity increases with a higher level of certainty, 5 the allocations to lower levels should be disregarded.…”

“…Postural instability within 3 years after symptom onset (P 1 , P 2 , P 3 ) and ocular motor dysfunction (O 1 , O 2 , O 3 ) have high sensitivity and specificity for PSP. 4,5 Therefore, these domains in combination (PSP-RS), or less so in isolation (PSP-PI, PSP-OM), are considered particularly characteristic of PSP. 4 Thus, PSP-RS shall be ranked higher than PSP-OM and PSP-PI.…”

“…4 A validation study demonstrated higher sensitivity and a small reduction in specificity compared with prior criteria. 5 …”

“…5 Moreover, a patient meeting one category at disease onset may come to meet additional categories as the disease progresses. Although the evolution of different diagnoses through the disease course provides the most accurate description of patients’ syndromes, in many circumstances (therapeutic trials, epidemiology), it is essential to have a single primary diagnosis.…”

Validation of the Movement Disorder Society Criteria for the Diagnosis of 4‐Repeat Tauopathies

“…Although the MDS‐PSP criteria have been retrospectively validated in 2 independent cohorts, the category of “probable 4R‐tauopathy” has not been previously investigated.…”