Alteration of the serotonergic nervous system in fatal familial insomnia.
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.
Wanschitz
J
J
Institute of Neurology, University of Vienna, and Austrian Reference Center of Human Prion Diseases.
Klöppel
S
S
Jarius
C
C
Birner
P
P
Flicker
H
H
Hainfellner
J A
JA
Gambetti
P
P
Guentchev
M
M
Budka
H
H
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*
D011985:Receptors, Serotonin