Prevalence and natural history of progressive supranuclear palsy

Golbe, Lawrence I.; Davis, Patricia H.; Schoenberg, Bruce S.; Duvoisin, Roger C.

doi:10.1212/wnl.38.7.1031

Cited by 279 publications

(176 citation statements)

References 0 publications

Supporting

Mentioning

158

Contrasting

Unclassified

Order By: Relevance

“…In a very recent large (187 cases) record-based study using the NINDS-SPSP diagnostic criteria [36], it was suggested that older age at disease onset increases mortality risk, irrespective of other prognostic factors. This finding is in agreement with some [23] but not all [42] previous studies. Older age at onset in the group of patients with lower survival duration is consistent with an interpretation that the disease affects older people more severely, leading to earlier death.…”

Section: Discussionsupporting

confidence: 94%

See 1 more Smart Citation

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

Papapetropoulos

Gonzalez

Mash³

2005

Eur Neurol

View full text Add to dashboard Cite

Progressive supranuclear palsy (PSP) is the second most common parkinsonian syndrome, characterized by a ‘diverse’ clinical phenotype. Although several different diagnostic guidelines have been proposed, the early and accurate diagnosis of PSP remains problematic and neuropathology is still considered the ‘gold standard’. In order to contribute to the better clinical characterization of PSP, we conducted a postmortem study in a cohort of 22 consecutive PSP brain donors and compared our results with those of previously published reports. Our results further demonstrate and expand the varied clinical picture of PSP. Furthermore, we report associations between older age at onset, early dementia, early falls and smoking with shorter PSP duration.

show abstract

Section: Discussionsupporting

confidence: 94%

“…The demographic characteristics of our patients were similar to previously published series (table 2) [1, 11, 13, 16,22,23,24,25,26,27,28]. Male gender has been predominant ranging from 54% [16] to 93.7% [22].…”

Section: Discussionsupporting

confidence: 83%

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

Papapetropoulos

Gonzalez

Mash³

2005

Eur Neurol

View full text Add to dashboard Cite

show abstract

“…This was much higher prevalence than those reported by previous studies, which indicated expected rates of 1 5 cases per 100,000 people in the general population, based upon community epidemiological studies 11,12 .…”

Section: Introductioncontrasting

confidence: 55%

Early Stage of Progressive Supranuclear Palsy: A Neuropathological Study of 324 Consecutive Autopsy Cases

et al. 2015

View full text Add to dashboard Cite

Diagnosing clinical progressive supranuclear palsy (PSP) is challenging. We hypothesize that there are more cases of pathological PSP than have been clinically identified, but its diagnosis is challenging because the initial lesions and progression of PSP have not yet been clarified. The purpose of our study was to clarify the incidence of PSP in consecutive autopsy cases and identify pathological characteristics of early PSP. We investigated 324 consecutive autopsy patients from a general geriatric hospital (age, mean±SD=82.5±8.7 years). Paraffin sections of the midbrain were immunostained with anti 4-repeat tau antibodies (RD4). We selected cases showing RD4-positive neurofibrillary tangles and tufted astrocytes in the midbrain sections. Then, we used anti-phosphorylated tau antibody to immunostain sections from the basal ganglia, subthalamic nucleus, midbrain, pons, medulla, and cerebellum. Of the 324 patients, 35 had RD4-positive structures in the midbrain. From these 35 cases, we excluded those for which autopsies confirmed definite PSP (n=5) and cases of corticobasal degeneration (n=1), Alzheimer's disease (n=11), dementia of grain (n=10), and neurofibrillary tangles predominant forms of senile dementia (n=2), leaving 8 cases. We diagnosed these 8 cases as pure PSP-type tauopathy.Pure PSP-type tauopathy was detected in 2.5% of the consecutive autopsy cases, and this incidence was 1.6 times greater than that of neuropathologically definite PSP. This pure PSP-type tauopathy likely indicates preclinical stages of PSP. Furthermore, the novel neuropathological finding, which we term preclinical PSP, is unique and has not previously been reported. In order to elucidate the causes and pathological mechanisms of PSP, preclinical PSP should be investigated further. (J Nippon Med Sch 2015; 82: 266 273)

show abstract

“…Many early cases of PSP present with unexplained falls and only later in the course does the characteristic clinical picture emerge, which features postural instability, vertical gaze palsy, axial rigidity, dysarthria and dementia [37]. PSP is an uncommon disorder, with prevalence rate estimates of about 1.5 per 100,000 [36], compared to 100 to 150 per 100,000 for Parkinson's disease (PD). New cases occur at a rate of about 3 to 4 per million population per year [67].…”

Section: Introductionmentioning

confidence: 99%

Neuropathologic differentiation of progressive supranuclear palsy and corticobasal degeneration

1999

View full text Add to dashboard Cite

Neuropathologic differentiation of progressive supranuclear palsy and corticobasal degeneration atypical cases are described that have overlapping clinical and pathologic features. Both PSP and CBD have similar biochemical alterations in the tau protein, with the abnormal tau protein containing predominantly four-repeat tau. While there is overlap in the pathology in PSP and CBD, there are sufficient differences to continue the present day trend to consider these separate disorders. Several important pathologic features differentiate PSP from CBD. Ballooned neurons are frequent and nearly a sine qua non for CBD, but they are found in PSP at a frequency similar to that of other neurodegenerative diseases, such as Alzheimer's disease. Astrocytic lesions are different, with tufted astrocytes found in motor cortex and striatum in PSP and astrocytic plaques in focal atrophic cortices in CBD. The most characteristic neuronal tau pathology in CBD is wispy, fine filamentous inclusions within neuronal cell bodies, while affected neurons in PSP have compact, dense filamentous aggregates characteristic of globose neurofibrillary tangles. Thread-like processes in gray and white matter are much more numerous and widespread in CBD than in PSP. The brunt of the pathology in CBD is in the cerebrum, while the basal ganglia, diencephalon and brainstem are the targets of PSP. Further clinicopathologic studies will refine our understanding of these disorders and open the possibility that common etiologic factors may be identified for these unusual sporadic tauopathies. Abstract Progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD) are usually sporadic multisystem degenerations associated with filamentous tau inclusions in neurons and glia. As such they can be considered sporadic tauopathies in contrast to familial tauopathies linked to mutations in the tau gene. Mutations have not been found in the tau gene in either PSP or CBD. The clinical syndromes and neuroimaging of typical cases of PSP and CBD are distinct; however, Key words

show abstract

Prevalence and natural history of progressive supranuclear palsy

Cited by 279 publications

References 0 publications

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

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

Early Stage of Progressive Supranuclear Palsy: A Neuropathological Study of 324 Consecutive Autopsy Cases

Neuropathologic differentiation of progressive supranuclear palsy and corticobasal degeneration

Contact Info

Product

Resources

About