Abstract
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.
| Original language | English |
|---|---|
| Pages (from-to) | 935-939 |
| Number of pages | 5 |
| Journal | Neurology |
| Volume | 46 |
| Issue number | 4 |
| Publication status | Published - Apr 1996 |
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ASJC Scopus subject areas
- Neuroscience(all)
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Fatal familial insomnia : Behavioral and cognitive features. / Gallassi, R.; Morreale, A.; Montagna, P.; Cortelli, P.; Avoni, P.; Castellani, R.; Gambetti, P.; Lugaresi, E.
In: Neurology, Vol. 46, No. 4, 04.1996, p. 935-939.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Fatal familial insomnia
T2 - Behavioral and cognitive features
AU - Gallassi, R.
AU - Morreale, A.
AU - Montagna, P.
AU - Cortelli, P.
AU - Avoni, P.
AU - Castellani, R.
AU - Gambetti, P.
AU - Lugaresi, E.
PY - 1996/4
Y1 - 1996/4
N2 - 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.
AB - 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.
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M3 - Article
VL - 46
SP - 935
EP - 939
JO - Neurology
JF - Neurology
SN - 0028-3878
IS - 4
ER -