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(American Journal of Pathology. 1999;155:1453-1457.)
© 1999 American Society for Investigative Pathology

Short Communications

Selective Neuronal Vulnerability in Human Prion Diseases

Fatal Familial Insomnia Differs from Other Types of PrionDiseases

Marin Guentchev, Julia Wanschitz, Till Voigtländer, Helga Flicker and Herbert Budka

From the Institute of Neurology, University of Vienna, Vienna, Austria

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.




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Copyright © 1999 by the American Society for Investigative Pathology.