Fatal familial insomnia in a new Italian kindred

A. Padovani, M. D'Alessandro, P. Parchi, P. Cortelli, G. P. Anzola, P. Montagna, L. A. Vignolo, R. Petraroli, M. Pocchiari, E. Lugaresi, P. Gambetti

Research output: Contribution to journalArticle

Abstract

The authors report a new kindred with fatal familial insomnia (FFI)-an inherited prion disease. The propositus had behavioral, sleep, cognitive, and motor impairment associated with thalamic and olivary atrophy. Spongiosis was confined to the parahippocampal gyrus. Protease-resistant prion protein (PrP(res)) was present with widespread distribution. The propositus fits the histopathology of FFI with similar clinical duration and confirms the role of disease duration in determining histopathology and PrP(res) distribution in FFI.

Original languageEnglish
Pages (from-to)1491-1494
Number of pages4
JournalNeurology
Volume51
Issue number5
Publication statusPublished - Nov 1998

ASJC Scopus subject areas

  • Neuroscience(all)

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    Padovani, A., D'Alessandro, M., Parchi, P., Cortelli, P., Anzola, G. P., Montagna, P., Vignolo, L. A., Petraroli, R., Pocchiari, M., Lugaresi, E., & Gambetti, P. (1998). Fatal familial insomnia in a new Italian kindred. Neurology, 51(5), 1491-1494.