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Alteration of the serotonergic nervous system in fatal familial insomnia
Author(s) -
Wanschitz Julia,
Klöppel Stefan,
Jarius Christa,
Birner Peter,
Flicker Helga,
Hainfellner Johannes A.,
Gambetti Pierluigi,
Guentchev Marin,
Budka Herbert
Publication year - 2000
Publication title -
annals of neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 4.764
H-Index - 296
eISSN - 1531-8249
pISSN - 0364-5134
DOI - 10.1002/1531-8249(200011)48:5<788::aid-ana13>3.0.co;2-5
Subject(s) - serotonergic , tryptophan hydroxylase , fatal familial insomnia , serotonin , raphe , insomnia , raphe nuclei , neuroscience , disease , medicine , endocrinology , biology , psychology , psychiatry , prion protein , receptor
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 (∼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. Ann Neurol 2000;48:788–791