[18F]FDG PET in fatal familial insomnia: The functional effects of thalamic lesions

D. Perani, P. Cortelli, G. Lucignani, P. Montagna, P. Tinuper, R. Gallassi, P. Gambetti, G. L. Lenzi, E. Lugaresi, F. Fazio

Research output: Contribution to journalArticlepeer-review


We used [18F]2-fluoro-2-deoxy-D-glucose ([18F]FDG) and positron emission tomography (PET) to study regional cerebral glucose utilization (rCMRglc) in four patients with fatal familial insomnia (FFI), a prion disease with a mutation at codon 178 of the prion protein gene. Two patients, presenting only with insomnia and dysautonomia, had a prominent and, in one case, selective thalamic hypometabolism. The remaining two cases presented a more complex clinical picture with multiple neurologic deficits, with both thalamic and widespread brain hypometabolism involving the majority of cortical structures, basal ganglia, and the cerebellum. This widespread pattern was present in the early stage of the disease and showed significant worsening as the disease progressed in one patient examined twice. The thalamic hypometabolism, consistently found with PET in FFI patients, is in agreement with the neuropathologic findings and is a hallmark of the disease. NEUROLOGY 1993;43:2565-2569.

Original languageEnglish
Pages (from-to)2565-2569
Number of pages5
Issue number12
Publication statusPublished - Dec 1993

ASJC Scopus subject areas

  • Neuroscience(all)
  • Arts and Humanities (miscellaneous)
  • Clinical Neurology


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