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Gerstmann‐Sträussler‐Scheinker disease: Immunohistological and experimental studies
Author(s) -
Tateishi June,
Kitamoto Tetsuyuki,
Hashiguchi Hideyuki,
Shii Hirofumi
Publication year - 1988
Publication title -
annals of neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 4.764
H-Index - 296
eISSN - 1531-8249
pISSN - 0364-5134
DOI - 10.1002/ana.410240108
Subject(s) - kuru , pathology , immunostaining , senile plaques , disease , creutzfeldt jakob syndrome , amyloid (mycology) , scrapie , biology , prion protein , virology , medicine , alzheimer's disease , immunohistochemistry
Abstract The older brother of the patient from whom the Fukuoka‐l strain was isolated was found to have numerous kuru plaques, the main finding common to both siblings. Other clinicopathological features including spongiform change were absent in the older brother. Immunostaining using anti‐kuru plaque core protein and anti‐ β ‐protein peptide revealed many kuru plaques and a few senile plaques in the older brother. Experimental transmission of the disease to laboratory animals was successful, using tissues from both siblings, through inoculation of fresh brain homogenates, purified prion protein, and formalin‐fixed brain homogenates. Prion protein fractions from the patient's brain shortened the incubation periods and formalin‐fixed mouse brains did not lengthen the periods. The disease in the two brothers can be classified as Gerstmann‐Sträussler‐Scheinker disease, a familial variant of Creutzfeldt‐Jakob disease. Gerstmann‐Sträussler‐Scheinker disease manifests a variety of clinicopathological features. Immunohistological verification of kuru plaques has major diagnostic value in assessing dementia.