Gerstmann-Sträussler-Scheinker disease showing β-protein type cerebellar and cerebral amyloid angiopathy

Biomedical and Life Sciences

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https://doi.org/10.1007/BF00293403
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Keywords

Aged, Amyloid beta-Peptides, Amyloidosis, Brain, Cerebellum, Cerebrovascular Disorders, Gerstmann-Straussler-Scheinker Disease, Humans, Immunohistochemistry, Male, Prions

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Gerstmann-Sträussler-Scheinker disease showing β-protein type cerebellar and cerebral amyloid angiopathy. / Ikeda, S I; Yanagisawa, N; Allsop, D et al.
In: Acta Neuropathologica, Vol. 88, No. 3, 09.1994, p. 262-266.

Research output: Contribution to Journal/Magazine › Journal article › peer-review

Bibtex

@article{a420dcc6b7cd496a9a13dca64175fa48,

title = "Gerstmann-Str{\"a}ussler-Scheinker disease showing β-protein type cerebellar and cerebral amyloid angiopathy",

abstract = "Cerebral amyloid angiopathy is observed in several brain degenerative disorders, but this pathological condition has received little attention in Gerstmann-Str{\"a}ussler-Scheinker disease (GSS). We report a 69-year-old man who showed the cardinal features of GSS together with typical and extensive congophilic angiopathy. Immunohistochemical studies revealed that the vast majority of the amyloid plaques present in the brain of this patient were consistently labeled by anti-prion protein (PrP) antibody. Double immunostaining disclosed many additional beta-protein immunoreactive plaque-like lesions, including a special type of {"}hybrid{"} plaque with colocalization of PrP and beta-protein (beta-PrP). The vascular amyloid deposits seen in both the cerebellum and cerebrum were immunoreactive only to anti-beta-protein antibody. It seems likely that the extensive deposition of beta-protein amyloid (including brain vascular amyloidosis) seen in this and other similar cases is part of pathology of GSS, although the possibility that this finding is due to ageing or concomitant Alzheimer's disease cannot be completely ruled out.",

keywords = "Aged, Amyloid beta-Peptides, Amyloidosis, Brain, Cerebellum, Cerebrovascular Disorders, Gerstmann-Straussler-Scheinker Disease, Humans, Immunohistochemistry, Male, Prions",

author = "Ikeda, {S I} and N Yanagisawa and D Allsop and Glenner, {G G}",

year = "1994",

month = sep,

doi = "10.1007/BF00293403",

language = "English",

volume = "88",

pages = "262--266",

journal = "Acta Neuropathologica",

issn = "0001-6322",

publisher = "Springer Verlag",

number = "3",

}

RIS

TY - JOUR

T1 - Gerstmann-Sträussler-Scheinker disease showing β-protein type cerebellar and cerebral amyloid angiopathy

AU - Ikeda, S I

AU - Yanagisawa, N

AU - Allsop, D

AU - Glenner, G G

PY - 1994/9

Y1 - 1994/9

N2 - Cerebral amyloid angiopathy is observed in several brain degenerative disorders, but this pathological condition has received little attention in Gerstmann-Sträussler-Scheinker disease (GSS). We report a 69-year-old man who showed the cardinal features of GSS together with typical and extensive congophilic angiopathy. Immunohistochemical studies revealed that the vast majority of the amyloid plaques present in the brain of this patient were consistently labeled by anti-prion protein (PrP) antibody. Double immunostaining disclosed many additional beta-protein immunoreactive plaque-like lesions, including a special type of "hybrid" plaque with colocalization of PrP and beta-protein (beta-PrP). The vascular amyloid deposits seen in both the cerebellum and cerebrum were immunoreactive only to anti-beta-protein antibody. It seems likely that the extensive deposition of beta-protein amyloid (including brain vascular amyloidosis) seen in this and other similar cases is part of pathology of GSS, although the possibility that this finding is due to ageing or concomitant Alzheimer's disease cannot be completely ruled out.

AB - Cerebral amyloid angiopathy is observed in several brain degenerative disorders, but this pathological condition has received little attention in Gerstmann-Sträussler-Scheinker disease (GSS). We report a 69-year-old man who showed the cardinal features of GSS together with typical and extensive congophilic angiopathy. Immunohistochemical studies revealed that the vast majority of the amyloid plaques present in the brain of this patient were consistently labeled by anti-prion protein (PrP) antibody. Double immunostaining disclosed many additional beta-protein immunoreactive plaque-like lesions, including a special type of "hybrid" plaque with colocalization of PrP and beta-protein (beta-PrP). The vascular amyloid deposits seen in both the cerebellum and cerebrum were immunoreactive only to anti-beta-protein antibody. It seems likely that the extensive deposition of beta-protein amyloid (including brain vascular amyloidosis) seen in this and other similar cases is part of pathology of GSS, although the possibility that this finding is due to ageing or concomitant Alzheimer's disease cannot be completely ruled out.

KW - Aged

KW - Amyloid beta-Peptides

KW - Amyloidosis

KW - Brain

KW - Cerebellum

KW - Cerebrovascular Disorders

KW - Gerstmann-Straussler-Scheinker Disease

KW - Humans

KW - Immunohistochemistry

KW - Male

KW - Prions

U2 - 10.1007/BF00293403

DO - 10.1007/BF00293403

M3 - Journal article

C2 - 7810298

VL - 88

SP - 262

EP - 266

JO - Acta Neuropathologica

JF - Acta Neuropathologica

SN - 0001-6322

IS - 3

ER -

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