Progressive supranuclear palsy

Gearing, Maria; Olson, David A.; Watts, Ray L.; Mirra, Suzanne S.

doi:10.1212/wnl.44.6.1015

Cited by 117 publications

(56 citation statements)

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“…PSP has been reported with coexisting AD [33,34,401, but that diagnostic possibility in our patient is unlikely in the absence of neuritic plaques required for the diagnosis of AD [41]. It is noted, however, that allocortical NFT-predominant dementia (so-called limbic AD), with minimal or no neuritic plaques, has been reported in rare demented elderly individuals .…”

Section: Fig G Electron Microscopy Demonstrates Paired Helical Fibmecontrasting

confidence: 47%

Autosomal dominant dementia with widespread neurofibrillary tangles

Reed

Schelper

Solodkin

et al. 1997

Annals of Neurology

173

158

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Several familial dementing conditions with atypical features have been characterized, but only rarely is the neuropathology dominated solely by neurofibrillary lesions. We present a Midwestern American pedigree spanning four generations in which 15 individuals were affected by early-onset dementia with long disease duration, with an autosomal dominant inheritance pattern, and with tau-rich neurofibrillary pathology found in the brain post mortem. The average age at presentation was 55 years with gradual onset and progression of memory loss and personality change. After 30 years' disease duration, the proband's neuropathologic examination demonstrated abundant intraneuronal neurofibrillary tangles (NFTs) involving the hippocampus, pallidum, subthalamic nucleus, substantia nigra, pons, and medulla. Only sparse neocortical tangles were present and amyloid plaques were absent. The tangles were recognized by antibodies specific for phosphorylation-independent (Tau-2, T46, 133, and Alz-50) and phosphorylation-dependent epitopes (AT8, T3P, PHF-1, 12E8, AT6, AT18, AT30) in tau proteins. Electron microscopy of NFTs in the dentate gyrus and midbrain demonstrated paired helical filaments. Although the clinical phenotype resembles Alzheimer's disease, and the neuropathologic phenotype resembles progressive supranuclear palsy, an alternative consideration is that this familial disorder may be a new or distinct disease entity.

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Section: Fig G Electron Microscopy Demonstrates Paired Helical Fibmecontrasting

confidence: 47%

Autosomal dominant dementia with widespread neurofibrillary tangles

Reed

Schelper

Solodkin

et al. 1997

Annals of Neurology

173

158

View full text Add to dashboard Cite

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“…In the same study, fine resting tremor was noted in 3 patients. In another series [10] of 13 patients, tremor was present in 4, only one of whom had PD changes along with PSP pathology. Three of these patients exhibited action/postural tremor; the fourth displayed tremor at rest and postural tremor.…”

Section: Discussionmentioning

confidence: 99%

“…Clinicopathologic studies have highlighted the ‘diverse’ clinical phenotype of the disease. Although several different diagnostic guidelines have been proposed [3,4,5,6,7,8,9,10,11,12,13,14], the early and accurate diagnosis of PSP remains problematic [15, 16], and neuropathology is considered the gold standard.…”

Section: Introductionmentioning

confidence: 99%

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

Papapetropoulos

Gonzalez

Mash³

2005

Eur Neurol

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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.

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“…While the majority of clinically well-characterized patients with PSP have distinctive postmortem findings, there have been several reports suggesting that the match is not always perfect [13,19,33]. Gross examination of the brain may be unrevealing in PSP, but the most common pathological findings are midbrain atrophy, with dilation of the aqueduct of Sylvius and depigmentation of the substantia nigra.…”

Section: Neuropathology Of Pspmentioning

confidence: 99%

Neuropathologic differentiation of progressive supranuclear palsy and corticobasal degeneration

1999

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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

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Progressive supranuclear palsy

Cited by 117 publications

References 0 publications

Autosomal dominant dementia with widespread neurofibrillary tangles

Autosomal dominant dementia with widespread neurofibrillary tangles

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

Neuropathologic differentiation of progressive supranuclear palsy and corticobasal degeneration

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