An autopsied case of progressive supranuclear palsy presenting with slow progression and unusually prolonged disease duration

It is an unmet need to estimate survival duration for patients with progressive supranuclear palsy (PSP). The objective of this study was to identify factors associated with the survival duration in patients with PSP. We followed up 23 patients with probable PSP-RS (Richardson syndrome) or PSP-P (parkinsonism) in our PSP center until death from 2011 to 2019. We prospectively and quantitatively rated their downgaze palsy whenever first noticed in our clinic. This was utilized along with the disease duration, motor function, medication use for parkinsonism, sex, age at onset of PSP, comorbid pulmonary and cardiovascular diseases, and the total survival duration from the onset of PSP to death for prediction analysis. A well-fitted linear regression model and a multivariant Cox model were applied to identify predicting factors for total survival duration. All patients had the specific hummingbird sign on brain MRI for PSP when downgaze palsy was documented. We found that the severity of downgaze palsy and the disease duration at the assessment were consistently correlated with the total survival duration in both models. The total survival duration could be further estimated by a formed regression equation. We conclude that severity and time to develop downgaze palsy could help to estimate the total survival duration in patients with probable PSP-RS and PSP-P, the major forms of PSP, which has significant clinical applications in clinical counseling and trial enrollment.

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The relationship between histopathological features of progressive supranuclear palsy and disease duration

Parkinsonism & Related Disorders ◽

10.1016/j.parkreldis.2005.08.007 ◽

2006 ◽

Vol 12 (2) ◽

pp. 109-112 ◽

Cited By ~ 9

Author(s):

Keith A. Josephs ◽

Jayawant N. Mandrekar ◽

Dennis W. Dickson

Keyword(s):

Progressive Supranuclear Palsy ◽

Disease Duration ◽

Histopathological Features ◽

The Relationship

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Understanding Fatigue in Progressive Supranuclear Palsy: Prevalence and Associated Factors

10.21203/rs.3.rs-668739/v1 ◽

2021 ◽

Author(s):

Jong Hyeon Ahn ◽

Joomee Song ◽

Dong Yeong Lee ◽

Jinyoung Youn ◽

Jin Whan Cho

Keyword(s):

Progressive Supranuclear Palsy ◽

Associated Factors ◽

Mini Mental State Examination ◽

Disease Duration ◽

Rating Scale ◽

Motor Symptom ◽

Summary Index ◽

State Examination ◽

Insight Into

Abstract Background: Fatigue is a common and disabling non-motor symptom (NMS) of Parkinson’s disease (PD); however, it has been poorly understood in patients with progressive supranuclear palsy (PSP). We investigated the association between fatigue, clinical features, and other NMS in patients with probable PSP.Methods: In 72 probable PSP patients, fatigue was investigated using the PD fatigue scale (PFS). Further, all patients were evaluated using the PSP rating scale (PSPRS), Beck Depression Inventory (BDI), Mini-Mental State Examination (MMSE), Frontal Assessment Battery (FAB), PD Sleep Scale (PDSS), NMS scale (NMSS), PD Questionnaire-39 summary index (PDQ-39 SI), and Scale for Outcomes in PD-Autonomic (SCOPA-AUT). Results: The prevalence of fatigue was 38.9% (28/72) in patients with PSP. Logistic regression analysis showed that depression (BDI) was the factor associated with fatigue. We divided the patients into primary (fatigue without depression, n =16), secondary (fatigue with depression, n = 12), and non-fatigue groups. There were no differences in age, sex, disease duration, and PSPRS, PDSS, MMSE, and FAB scores among the three groups. The primary fatigue group had higher scores in PDQ-39 SI compared to the non-fatigue group. The secondary fatigue group showed higher scores in NMSS, PDQ-39 SI, and SCOPA-AUT compared to the non-fatigue group. PFS was positively correlated with NMSS and PDQ-39 SI and SCOPA-AUT.Conclusions: Fatigue is common in patients with PSP and is associated with the NMS and the quality of life in these patients. The present study provides meaningful insight into fatigue in patients with PSP.

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Understanding fatigue in progressive supranuclear palsy

Scientific Reports ◽

10.1038/s41598-021-96443-z ◽

2021 ◽

Vol 11 (1) ◽

Author(s):

Jong Hyeon Ahn ◽

Joomee Song ◽

Dong Yeong Lee ◽

Jinyoung Youn ◽

Jin Whan Cho

Keyword(s):

Progressive Supranuclear Palsy ◽

Sleep Disturbances ◽

Mini Mental State Examination ◽

Disease Duration ◽

Rating Scale ◽

Motor Symptom ◽

Summary Index ◽

State Examination ◽

Insight Into

AbstractFatigue is a common and disabling non-motor symptom (NMS) of Parkinson’s disease (PD); however, it has been poorly understood in patients with progressive supranuclear palsy (PSP). We investigated the association between fatigue, clinical features, and other NMS in patients with probable PSP. In 72 probable PSP patients, fatigue was investigated using the Parkinson Fatigue Scale (PFS). Further, all patients were evaluated using the PSP rating scale (PSPRS), Beck Depression Inventory (BDI), Mini-Mental State Examination (MMSE), Frontal Assessment Battery (FAB), PD Sleep Scale (PDSS), NMS scale (NMSS), PD Questionnaire-39 summary index (PDQ-39 SI), and Scale for outcomes in PD-Autonomic (SCOPA-AUT). The prevalence of fatigue assessed by PFS was 38.9% (28/72) in patients with PSP. The secondary fatigue was defined as fatigued patients with depression and/or sleep disturbances. We divided the patients into primary (n = 15), secondary (n = 13), and non-fatigue groups. There were no differences in age, sex, disease duration, and PSPRS, PDSS, MMSE, and FAB scores among the three groups. The primary fatigue group had higher scores in PDQ-39 SI compared to the non-fatigue group. The secondary fatigue group showed higher scores in NMSS, PDQ-39 SI, and SCOPA-AUT compared to the non-fatigue group. PFS was positively correlated with NMSS and PDQ-39 SI and SCOPA-AUT. Fatigue is common in patients with PSP and is associated with the NMS and the quality of life in these patients. The present study provides meaningful insight into fatigue in patients with PSP.

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