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First experimental transmission of fatal familial insomnia

Abstract

ORIGINALLY described by Lugaresi et al. in 1986 (ref. 1), fatal familial insomnia (FFI) is a rare inherited neurological disease characterized by the subacute progression of intractable insomnia and other autonomic abnormalities, cerebellar and pyramidal signs, myoclonus and dementia; neuropathologically, the major feature is severe neuronal loss with associated gliosis in the ventral and mediodorsal thalamic nuclei. The disease has been related to the group of spongiform encephalopathies by virtue of the presence of low levels of proteinase-resistant amyloid protein (PrPres) in the brain2á¤-4, and of a pathogenic single-allele mutation at codon 178 of the PRNP gene that encodes PrF68 (refs 2, 5). Here we report the successful transmission of the disease to experimental animals, placing FFI within the group of infectious cerebral amyloidoses.

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References

  1. Lugaresi, E. et al. New Engl. J. Med. 315, 997–1003 (1986).

    Article  CAS  Google Scholar 

  2. Medori, R. et al. New Engl. J. Med. 326, 444–449 (1992).

    Article  CAS  Google Scholar 

  3. Monari, L. et al. Proc. natn. Acad. Sci. U.S.A. 91, 2839–2842 (1994).

    Article  ADS  CAS  Google Scholar 

  4. Brown, P. et al. Ann. Neurol. 38, 245–253 (1995).

    Article  CAS  Google Scholar 

  5. Goldfarb, L. G. et al. Science 258, 806–808 (1992).

    Article  ADS  CAS  Google Scholar 

  6. Bosque, P. J., Vnencak-Jones, C. L., Johnson, M. D., Whitlock, J. A. & McLean, M. J. Neurology 42, 1864–1870 (1992).

    Article  CAS  Google Scholar 

  7. Reder, A. T. et al. Neurology 45, 1068–1075 (1995).

    Article  CAS  Google Scholar 

  8. Kitamoto, T. et al. Am. J. Path. 140, 1285–1294 (1992).

    CAS  PubMed  Google Scholar 

  9. Muramoto, T., Kitamoto, T., Tateishi, J. & Goto, I. Brain Res. 599, 309–316 (1992).

    Article  CAS  Google Scholar 

  10. Tateishi, J. et al. Acta neuropath. 53, 161–163 (1981).

    Article  CAS  Google Scholar 

  11. Muramoto, T., Kitamoto, T., Tateishi, J. & Goto, l. Am. J. Path. 140, 1411–1420 (1992).

    CAS  PubMed  Google Scholar 

  12. Little, B., Brown, P. W., Rodgers-Johnson, P., Perl, D. P. & Gajdusek, D. C. Ann. Neurol. 20, 231–239 (1986).

    Article  CAS  Google Scholar 

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Tateishi, J., Brown, P., Kitamoto, T. et al. First experimental transmission of fatal familial insomnia. Nature 376, 434–435 (1995). https://doi.org/10.1038/376434a0

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  • DOI: https://doi.org/10.1038/376434a0

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