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

Abstract

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.

• Authors: D Perani, P Cortelli, G Lucignani, P Montagna, P Tinuper, R Gallassi, P Gambetti, G L Lenzi, E Lugaresi, F Fazio
• Journal: Neurology (Neurology) Vol. 43 Issue 12 Pg. 2565-9 (Dec 1993) ISSN: 0028-3878 [Print] United States
• PMID: 8255458 (Publication Type: Journal Article)

Chemical References

• Fluorine Radioisotopes
• Fluorodeoxyglucose F18
• Deoxyglucose
• Glucose

Topics

• Adult
• Brain (metabolism)
• Deoxyglucose (analogs & derivatives)
• Female
• Fluorine Radioisotopes
• Fluorodeoxyglucose F18
• Glucose (metabolism)
• Humans
• Male
• Middle Aged
• Sleep Initiation and Maintenance Disorders (diagnostic imaging, genetics, metabolism)
• Thalamus (diagnostic imaging, metabolism)
• Tomography, Emission-Computed