NR AEXZ
AU Guentchev,M.; Wanschitz,J.; Voigtländer,T.; Flicker,H.; Budka,H.
TI Selective neuronal vulnerability in human prion diseases. Fatal familial insomnia differs from other types of prion diseases.
QU American Journal of Pathology 1999 Nov; 155(5): 1453-7
IA http://ajp.amjpathol.org/cgi/reprint/155/5/1453.pdf
PT journal article
AB Human transmissible spongiform encephalopathies (TSEs) or prion diseases are neurodegenerative disorders of infectious, inherited or sporadic origin and include Creutzfeldt-Jakob disease (CJD), Gerstmann-Sträussler-Scheinker disease (GSS), kuru and fatal familial insomnia (FFI). Clinicopathologic features of FFI differ markedly from other human TSEs. Previous studies demonstrated selective neuronal vulnerability of parvalbumin positive (PV+) GABAergic inhibitory interneurons in sporadic CJD and experimental TSEs. In this report we show uniform severe loss of PV+ neurons also in other TSEs such as GSS, kuru, new variant and familial CJD. In contrast, these neurons are mostly well preserved, or only moderately reduced, in FFI. Only PV+ neurons surrounded by isolectin-B4 positive perineuronal nets were severely affected in TSEs, suggesting a factor residing in this type of extracellular matrix around PV+ neurons as modulator for the selective neuronal vulnerability.
MH Adult; Brain/metabolism/pathology; Female; Human; Immunohistochemistry; Male; Middle Age; Neurons/pathology; Parvalbumins/*biosynthesis; *Prion Diseases/classification/genetics/metabolism/pathology; *Sleep Initiation and Maintenance; Disorders/classification/genetics/metabolism/pathology; Support, Non-U.S. Gov't; gamma-Aminobutyric Acid/metabolism
AD Institute of Neurology, University of Vienna, Vienna, Austria.
SP englisch
PO USA