Article
Absence of sleep EEG markers in fatal familial insomnia healthy carriers: a spectral analysis study.
Clinical neurophysiology : official journal of the International Federation of Clinical Neurophysiology - 1 Oct 2001
Ferrillo F, Plazzi G, Nobili L, Beelke M, De Carli F, Cortelli P, Tinuper P, Avoni P, Vandi S, Gambetti P, Lugaresi E, Montagna P
Abstract excerpt
OBJECTIVES: Fatal familial insomnia (FFI) is linked to a mutation at codon 178 (C178) of the prion protein gene (PRNP). FFI is pathologically characterized by selective atrophy of the anteroventral and mediodorsal thalamic nuclei and clinically by loss of sleep, dysautonomia and motor signs. A key early polysomnographic sign of the disease onset is the loss of sleep spindling (sigma activity, SA). In FFI the loss...
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