Natural History of Progressive Supranuclear Palsy: A Clinicopathologic Study from a Population of Brain Donors

Papapetropoulos, Spiridon; Gonzalez, Jocely; Mash, Deborah C.

doi:10.1159/000086754

Cited by 41 publications

(28 citation statements)

References 85 publications

(98 reference statements)

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“…Although our PSP-P group consists of only seven cases, due to the strict use of NINDS-SPSP criteria, the difference in survival compared with our RS cases was striking as well. The effect of higher onset-age on survival in the present study was also found in retrospective studies,20 21 35 whereas our observed predictive value of gender contrasted to a weak or absent effect on survival in several other studies,18 20 21 but not all 35. The prognostic significance of older onset-age in PSP resembles observations made in Alzheimer disease36 and Parkinson disease (PD), whereas there is conflicting evidence regarding effect on prognosis of male gender in PD 37.…”

Section: Discussionsupporting

confidence: 84%

Survival in progressive supranuclear palsy and frontotemporal dementia

Chiu

Kaat

Seelaar

et al. 2010

Journal of Neurology, Neurosurgery & Psychiatry

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International audienceAbstract Objective: To compare survival and to identify prognostic predictors for progressive supranuclear palsy and frontotemporal dementia. Background: PSP and FTD are related disorders. Homozygosity for H1 haplotype is associated with PSP, whereas several MAPT mutations have been identified in FTLD-tau. Survival duration probably reflects underlying pathophysiology or disease. Methods: Patients with PSP and FTD were recruited by nation-wide referral. Survival of 354 FTD patients was compared to that of 197 PSP patients. Cox regression analysis was performed to identify prognostic predictors. FTLD-tau was defined as Pick's disease and FTDP-17 with MAPT mutations. Semiquantitative evaluation of tau-positive pathology was performed on all pathologically proven cases. Results: Median survival of PSP patients (8.0 years; 95% CI 7.3 to 8.7) was significantly shorter than of FTD patients (9.9 years; 95% CI 9.2 to 10.6). Corrected for demographic differences, PSP patients were still significantly more at risk of dying than FTD patients. In PSP, male gender, older onset-age, and higher PSP Rating Scale score were identified as independent predictors for shorter survival, whereas in FTD a positive family history and an older onset-age were associated with a poor prognosis. The difference in hazard rate was even more pronounced when comparing pathologically proven cases of PSP with FTLD-tau. Conclusion: Survival of PSP patients is shorter than of FTD patients, and probably reflects a more aggressive disease process in PSP. Independent predictors of shorter survival in PSP were male gender, older onset-age and higher PSP rating scale score, whereas in FTD a positive family history and higher onset-age were predictors for worse prognosis

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Section: Discussionsupporting

confidence: 84%

Survival in progressive supranuclear palsy and frontotemporal dementia

Chiu

Kaat

Seelaar

et al. 2010

Journal of Neurology, Neurosurgery & Psychiatry

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“…6,16,17 Interestingly, the latency of the urogenital dysfunction is associated with the disease duration in our patients. The urogenital dysfunction might well represent frontal lobe dysfunction in PSP, given the lack of orthostatic hypotension and the absence of urogenital dysfunction before the disease onset in our study and another report.…”

Section: Discussionmentioning

confidence: 55%

Comparison of clinical features in pathologically confirmed PSP and MSA patients followed at a tertiary center

Xie

Kang

Kuo

et al. 2015

npj Parkinson's Disease

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Background/Objectives:The clinical diagnosis of progressive supranuclear palsy (PSP) and multiple system atrophy (MSA) remains challenging due to heterogeneity of the diseases.AIMS:Here we compared the clinical features of PSP and MSA to gain insight into their diagnosis and prognosis, particularly the prognostic value of down-gaze palsy latency in PSP progression.Methods:We reviewed clinical features of pathologically confirmed 10 PSP and 13 MSA patients, incidentally matched in age-at-onset, gender, and disease duration, followed at Columbia University Medical Center during 1994–2009.Results:The final antemortem diagnosis was incorrect in 30% of PSP (all lacking down-gaze palsy) and 23% of MSA patients. Falls in the first year of the disease, pyramidal involvement and freezing of gait during the course were similar between PSP and MSA. Ataxia and apraxia were in 50% of the PSP patients. Parkinsonism responsive to levodopa treatment was in 30% of the PSP (all with resting tremor) and 50% of the MSA patients. Dysautonomia in MSA could occur as early as 3 years preceding the motor symptoms, with 46% within the first year of the motor onset, but 15% did not have dysautonomia in life. The latency of down-gaze palsy and urogenital dysfunction and MMSE scores at first visit in PSP, and the latency of falls and wheelchair confinement in MSA were all associated with the disease progression.Conclusions:We confirmed most of the previously published characterizations, and identified for the first time the prognostic value of down-gaze palsy latency in PSP progression.

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“…The disorder has a prevalence of 6.4/100 000 of the population [2,3] and is the second most common syndrome in which 'parkinsonism' is a prominent symptom [4]. The first description of PSP was based on nine cases that exhibited supranuclear ophthalmoplegia, pseudobulbar palsy, dysarthria, and dystonic rigidity of the upper neck and trunk [5,6].…”

Section: Introductionmentioning

confidence: 99%