Variant Gerstmann-Straussler syndrome with the P105L prion gene mutation: An unusual case with nigral degeneration and widespread neurofibrillary tangles

Acta Neuropathologica

Volumn 98, Issue 5, 1999, Pages 506-511

  Yamazaki, Mineo ^a,b,c   Oyanagi, Kiyomitsu ^b   Mori, Osamu ^a   Kitamura, Shin ^a   Ohyama, Masashi ^c   Terashi, Akiro ^a   Kitamoto, Tetsuyuki ^d   Katayama, Yasuo ^a  

^a NIPPON MEDICAL SCHOOL   (Japan)

^b TOKYO METROPOLITAN INSTITUTE OF MEDICAL SCIENCE   (Japan)

^c Hatsuishi Hospital   (Japan)

^d TOHOKU UNIVERSITY SCHOOL OF MEDICINE   (Japan)

Author keywords

Gait apraxia; Gerstmann Straussler syndrome; Neurofibrillary tangles; Prion protein; Substantia nigra

Indexed keywords

AMYLOID; PRION PROTEIN;

ADULT; ARTICLE; BRAIN ATROPHY; BRAIN DEGENERATION; BRAIN SPONGIOSIS; BRAIN STEM; BRAIN WEIGHT; CASE REPORT; DISEASE COURSE; DISEASE DURATION; FEMALE; FRONTAL CORTEX; FRONTAL LOBE; GAIT; GENE MUTATION; GERSTMANN STRAUSSLER SCHEINKER SYNDROME; GLIOSIS; GLOBUS PALLIDUS; HUMAN; HUMAN TISSUE; LOCUS CERULEUS; NERVE CELL; NEUROFIBRILLARY TANGLE; OLIVARY NUCLEUS; PARIETAL LOBE; PERIAQUEDUCTAL GRAY MATTER; PRIORITY JOURNAL; RAPHE NUCLEUS; SPASTIC PARAPLEGIA; SUBSTANTIA NIGRA; TEMPORAL CORTEX; THALAMUS; BRAIN; GENETICS; IMMUNOHISTOCHEMISTRY; JAPAN; MIDDLE AGED; NUCLEOTIDE SEQUENCE; PATHOLOGY; PEDIGREE; PRION; SENILE PLAQUE;

BRAIN; DNA MUTATIONAL ANALYSIS; FEMALE; GERSTMANN-STRAUSSLER-SCHEINKER DISEASE; HUMANS; IMMUNOHISTOCHEMISTRY; JAPAN; MIDDLE AGED; NEUROFIBRILLARY TANGLES; PEDIGREE; PRIONS; SENILE PLAQUES; SUBSTANTIA NIGRA;

EID: 0033228346     PISSN: 00016322     EISSN: None     Source Type: Journal    
DOI: 10.1007/s004010051116     Document Type: Article

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