HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS		QUICK SEARCH:   [advanced] Author: Keyword(s): Year:  Vol:  Page:

This Article Full Text (PDF) Correspondence: Submit a response Alert me when this article is cited Alert me when Correspondence are posted Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Perani, D. Articles by Fazio, F. Search for Related Content PubMed PubMed Citation Articles by Perani, D. Articles by Fazio, F.

[18F]FDG PET in fatal familial insomnia

The functional effects of thalamic lesions

Institute of Neurosciences and Bioimages (Dr. Perani), CNR, Milan; the University of Milan (Drs. Lucignani and Fazio), Institute H. S. Raffaele, Milan; and the Institute of Neurology (Drs. Cortelli, Montagna, Tinuper, Gallassi, and Lugaresi), University of Bologna, Bologna, Italy; the Department of Neuropathology (Dr. Gambetti), Case Western Reserve University, Cleveland, OH; and the Department of Neurological Sciences (Dr. Lenzi), University of Rome, Rome, Italy.

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 hypo-metabolism 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.

Address correspondence and reprint requests to Dr. Ferruccio Fazio, Department of Nuclear Medicine, University of Milan, Institute H. S. Raffaele, Via Olgettina 60, I-20132 Milano, Italy.

Received February 12, 1993. Accepted for publication in final form June 7, 1993.

This article has been cited by other articles:

				S. Haik, D. Galanaud, M. G. Linguraru, K. Peoc'h, N. Privat, B. A. Faucheux, N. Ayache, J.-J. Hauw, D. Dormont, and J.-P. Brandel In Vivo Detection of Thalamic Gliosis: A Pathoradiologic Demonstration in Familial Fatal Insomnia Arch Neurol, April 1, 2008; 65(4): 545 - 549. [Abstract] [Full Text] [PDF]

				P. Cortelli, D. Perani, P. Montagna, R. Gallassi, P. Tinuper, P. Federica, P. Avoni, F. Ferrillo, D. Anchisi, R. M. Moresco, et al. Pre-symptomatic diagnosis in fatal familial insomnia: serial neurophysiological and 18FDG-PET studies Brain, March 1, 2006; 129(3): 668 - 675. [Abstract] [Full Text] [PDF]

				T. Hamaguchi, T. Kitamoto, T. Sato, H. Mizusawa, Y. Nakamura, M. Noguchi, Y. Furukawa, C. Ishida, I. Kuji, K. Mitani, et al. Clinical diagnosis of MM2-type sporadic Creutzfeldt-Jakob disease Neurology, February 22, 2005; 64(4): 643 - 648. [Abstract] [Full Text] [PDF]

				K. H. Taber, C. Wen, A. Khan, and R. A. Hurley The Limbic Thalamus J Neuropsychiatry Clin Neurosci, May 1, 2004; 16(2): 127 - 132. [Full Text] [PDF]

				K.-J. Bar, F. Hager, I. Nenadic, T. Opfermann, M. Brodhun, R. F. Tauber, S. Patt, W. Schulz-Schaeffer, D. Gottschild, and H. Sauer Serial Positron Emission Tomographic Findings in an Atypical Presentation of Fatal Familial Insomnia Arch Neurol, November 1, 2002; 59(11): 1815 - 1818. [Abstract] [Full Text] [PDF]

				J. A. Mastrianni, R. Nixon, R. Layzer, G. C. Telling, D. Han, S. J. DeArmond, and S. B. Prusiner Prion Protein Conformation in a Patient with Sporadic Fatal Insomnia N. Engl. J. Med., May 27, 1999; 340(21): 1630 - 1638. [Full Text] [PDF]