title: Alteration of the serotonergic nervous system in fatal familial insomnia.
abstract: Fatal familial insomnia (FFI) is a unique hereditary prion disease with characteristic disturbances of sleep. We studied the serotonergic system in 8 FFI-affected subjects by immunohistochemistry for the serotonin-synthesizing enzyme, tryptophan hydroxylase (TH). Quantification of neurons in median raphe nuclei showed no total neuronal loss in FFI but a substantial increase of TH+ neurons (approximately 62%) in FFI subjects compared with controls. Our data indicate an alteration of the serotonergic system that might represent the functional substrate of some typical symptoms of FFI.
authors.affiliation: Institute of Neurology, University of Vienna, and Austrian Reference Center of Human Prion Diseases.
authors.forename: J
authors.initials: J
authors.lastname: Wanschitz
authors.forename: S
authors.initials: S
authors.lastname: Klöppel
authors.forename: C
authors.initials: C
authors.lastname: Jarius
authors.forename: P
authors.initials: P
authors.lastname: Birner
authors.forename: H
authors.initials: H
authors.lastname: Flicker
authors.forename: J A
authors.initials: JA
authors.lastname: Hainfellner
authors.forename: P
authors.initials: P
authors.lastname: Gambetti
authors.forename: M
authors.initials: M
authors.lastname: Guentchev
authors.forename: H
authors.initials: H
authors.lastname: Budka
mesh_terms: D000328:Adult; D000368:Aged; D001921:Brain / Q000473:pathology*; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D008875:Middle Aged; D017096:Prion Diseases / Q000473:pathology*; D011985:Receptors, Serotonin / Q000032:analysis*
chemical_list: D011985:Receptors, Serotonin