Cerebral metabolism in fatal familial insomnia: Relation to duration, neuropathology, and distribution of protease-resistent prion protein

@article{Cortelli1997CerebralMI,
  title={Cerebral metabolism in fatal familial insomnia: Relation to duration, neuropathology, and distribution of protease-resistent prion protein},
  author={P. Cortelli and D. Perani and Piero Parchi and F. Grassi and P. Montagna and M. De Martin and R. Castellani and P. Tinuper and P. Gambetti and E. Lugaresi and F. Fazio},
  journal={Neurology},
  year={1997},
  volume={49},
  pages={126 - 133}
}
We used [18F]-2-fluoro-2-deoxy-D-glucose (FDG) and PET to study regional cerebral glucose utilization in seven patients with fatal familial insomnia (FFI), an inherited prion disease with a mutation at codon 178 of the prion protein gene. Four patients were methionine/methionine homozygotes at codon 129 (symptom duration, 8.5 ± 1 months) and three were methionine/valine (MET/VAL129) heterozygotes (symptom duration, 35± 11 months). A severely reduced glucose utilization of the thalamus and a… Expand
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