Electron microscopic study of neurofibrillary tangles in Steele-Richardson-Olszewski syndrome

Roy, S. C. Dutta; Datta, Chhanda; Hirano, Atsushi; Ghatak, Nitya R.; Zimmerman, H. M.

doi:10.1007/bf00684775

Cited by 61 publications

(15 citation statements)

References 16 publications

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“…34 In PSP, ultrastructural investigation of the neurofibrillary tangles reveals that they are composed of 15-to 18-nm-wide straight filaments, but long periodicity filaments have also been documented. 35,36 In contrast to the three major bands and fourth minor band in AD, abnormal tau in PSP has an electrophoretic migration pattern of two strong bands at 68 and 64 kDa, whereas the minor band at 72 kDa is only variably detectable. In contrast to AD, the tau bands are predominantly composed of fourrepeat tau isoforms in typical cases of PSP.…”

Section: Progressive Supranuclear Palsymentioning

confidence: 88%

Anatamopathological spectrum of tauopathies

Révész

Holton

2003

Mov Disord.

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The presence of tau-positive intraneuronal filamentous inclusions with or without additional inclusions in glial cells has been recognised as a major neuropathological feature in a significant group of neurodegenerative diseases, which are described as tauopathies. In one category of such diseases, the neuronal inclusions occur in association with extracellular deposition of a second aggregated protein (secondary tauopathies), whereas in another, the filamentous inclusions composed of tau are the sole neuropathological abnormality (primary tauopathies). Genetic studies of tauopathies in general, and in frontotemporal dementia with parkinsonism linked to chromosome 17 in particular, have significantly contributed to our knowledge about the pathogenesis not only of rare hereditary conditions but also of other more common diseases such as Alzheimer's disease and progressive supranuclear palsy.

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Section: Progressive Supranuclear Palsymentioning

confidence: 88%

Anatamopathological spectrum of tauopathies

Révész

Holton

2003

Mov Disord.

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“…While NFT in AD are composed mostly of 22-nm diameter paired helical filaments (PHF) and a minor component of 15-to 18-nm diameter straight filaments [79], similar studies of NFT in PSP show mostly straight filaments [10, 65,69,78,83]. In addition astrocytes in PSP contain straight filaments [62].…”

Section: Ultrastructural Differences Between Psp and Cbdmentioning

confidence: 97%

Neuropathologic differentiation of progressive supranuclear palsy and corticobasal degeneration

Dickson

1999

J Neurol

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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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“…OIszewski et al [125], Richardson et al [142] [94] 1 a b e Mano et al [101] 1 a b Morax et al [112] 1 a Powell et al [133] 2 a b c Rizzo [143] 1 a b e Rouzaud et al [146] 1 a b f Roy et al [148] 1 b c…”

Section: Author and Reference Number Of Casesmentioning

confidence: 99%