Autopsy Validation of Progressive Supranuclear Palsy‐Predominant Speech/Language Disorder Criteria

Abstract

if it is related to their disease. 9,10 On the contrary, even if all beekeepers diagnosed with PD were among the responders, the estimated prevalence would be 20/1885 × 100 = 1.06% (20 PD patients among 1885 beekeepers aged 60 years and above), which is within the prevalence expected in the general population of the same age. We did not examine participants to confirm the diagnosis of PD. Because in Slovenia all patients with PD are followed up by a neurologist, misdiagnoses are unlikely. Finally, bee stings might not be an optimal method of BV application for a successful preventive effect. Yet all hitherto-conducted clinical studies used subcutaneous BV application, which is equivalent to envenoming by bee stings.We conclude that regular and extensive exposure to BV in pre-disease state and prodromal stage of PD does not prevent or reduce the neurodegeneration to the extent that it could prevent the expression of the disease.

Brainstem Biomarkers of Clinical Variant and Pathology in Progressive Supranuclear Palsy

“…The findings suggest that these brainstem measures could be useful to help differentiate PSP‐Richardson's from PSP‐speech/language. Also notable is that all PSP‐speech/language patients met at least possible PSP criteria 11 ; we did not include patients who only met suggestive of PSP‐speech/language because they have a lower probability of having underlying PSP pathology 58,59 . We have previously shown that patients with suggestive of PSP‐speech/language do not show midbrain atrophy 45 …”

Clinical Spectrum of Tauopathies

“…Early motor speech disorder has specifically been linked to a PSP pathology ( 149 ). A pathology-based study showed that among patients fulfilling the criteria for PSP-SL, those with probable PSP-SL are more likely to have an underlying PSP pathology, while those with suggestive-of-PSP-SL are more consistently associated with a CBD pathology ( 101 ). In a recent study, survival was about 6 years for patients presenting with primary progressive apraxia of speech, around 5 years for agrammatic aphasia, and about 4 years for combined apraxia of speech and agrammatism ( 150 ).…”

Diffusion tractography of superior cerebellar peduncle and dentatorubrothalamic tracts in two autopsy confirmed progressive supranuclear palsy variants: Richardson syndrome and the speech-language variant

“…We previously found relative sparing of the SCP in PSP-SL, although that study utilized region-of-interest-based analyses rather than tractography, and the cohort lacked autopsy confirmation ( Whitwell et al, 2021 ). Patients with PSP-SL can also have corticobasal degeneration at autopsy ( Hokelekli et al, 2022 ), and hence it would be reasonable to conclude that the diffusion signal in our previous study was weakened by the inclusion of corticobasal degeneration. Brainstem involvement is greater in speech-language patients with PSP compared to those with corticobasal degeneration ( Josephs et al, 2021 ).…”

“…However, no studies have utilized tractography to assess the degree of involvement of the SCP or DRTT in PSP-SL. Furthermore, the pathology underlying PSP-SL is heterogeneous, with PSP pathology only observed in approximately 67% of patients clinically diagnosed with PSP-SL ( Hokelekli et al, 2022 , Josephs et al, 2021 ), and from the Hokelekli study we focus on a subgroup of PSP-SL patients who underwent neuroimaging studies. Hence, autopsy-confirmed cases are needed to assess how SCP and DRTT integrity relates to PSP pathology.…”