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Human Prion Diseases: Creutzfeldt‐Jakob Disease, Gerstmann‐Sträussler Syndrome, and Unknown Dementia
Author(s) -
Kitamoto Tetsuyuki
Publication year - 1993
Publication title -
neuropathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.701
H-Index - 61
eISSN - 1440-1789
pISSN - 0919-6544
DOI - 10.1111/j.1440-1789.1993.tb00218.x
Subject(s) - myoclonus , dementia , pathology , prion protein , pathological , disease , central nervous system , biology , medicine , neuroscience
Recent molecular genetic and immunohistochemical studies have revealed that various dementias belong to the group of prion disease. The clinicopathological variabilities of these disorders can be explained by prion protein (PrP) gene polymorphisms. Here we report on the usefulness of classifying prion diseases into plaque types and non‐plaque types, based on the distribution of PrP in the central nervous system. The non‐plaque type prion diseases are characterized by rapidly progressing dementia, myoclonus, and periodic synchronous discharges in the electroencephalogram, and by the presence of diffuse gray matter accumulations of PrP, including the synaptic strutures. The plaque type prion diseases have a long clinical course without myoclonus and periodic synchronous discharges, and the major PrP accumulation sites are extracellular PrP plaques. The distribution of abnormal PrP accumulations, either plaque type or non‐plaque type is influenced by the polymorphism of the PrP gene and in turn the distribution of PrP deposits within the central nervous system influences the clinical and pathological features of prion diseases.