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Fatal familial insomnia

Behavioral and cognitive features

From the Institute of Neurology (Drs. Gallassi, Morreale, Montagna, Cortelli, Avoni, and Lugaresi), University of Bologna, Bologna, Italy; Department of Neuropathology (Drs. Gambetti and Castellani), Case Western Reserve University, Cleveland, OH.
Supported in part by University of Bologna, grant no. 12.6.C, 95/02416.
Received June 16, 1995. Accepted in final form August 6, 1995.
Address correspondence and reprint requests to Dr. Roberto Gallassi, Istituto di Clinica Neurologica, via U.Foscolo 7, I-40123 Bologna, Italy.

Fatal familial insomnia (FFI) is a familial prion disease linked to a mutation of the prion protein gene.Neuropsychological investigations in seven patients with FFI belonging to two different families showed that the main behavioral and neuropsychological features are (1) early impairment of attention and vigilance, (2) memory deficits, mainly of the working memory, (3) impairment of temporal ordering of events, and (4) a progressive dream-like state with neuropsychological and behavioral features of a confusional state. Neuropathologic examination of six patients showed prominent neuronal loss and gliosis involving the anterior ventral and mediodorsal thalamic nuclei, with additional cerebral cortical involvement in two cases. Clinicopathologic correlations indicate that FFI is associated with a neuropsychological and behavioral syndrome that is distinct from the cortical and subcortical dementias, and Wernicke-Korsakoff syndrome. These findings offer insights into the function of the thalamic nuclei and challenge the notion of thalamic dementia.

NEUROLOGY 1996;46: 935-939

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