NR AMFL
AU Wanschitz,J.; Klöppel,S.; Jarius,C.; Birner,P.; Flicker,H.; Hainfellner,J.A.; Gambetti,P.; Guentchev,M.; Budka,H.
TI Alteration of the serotonergic nervous system in fatal familial insomnia
QU Annals of Neurology 2000 Nov; 48(5): 788-91
KI Ann Neurol. 2001 Sep;50(3):421-2. PMID: 11558802
PT journal article
AB 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.
MH Adult; Aged; Brain/*pathology; Female; Human; Immunohistochemistry; Male; Middle Age; Prion Diseases/*pathology; Receptors, Serotonin/*analysis; Support, Non-U.S. Gov't
AD Institute of Neurology, University of Vienna, and Austrian Reference Center of Human Prion Diseases.
SP englisch
PO USA