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...

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Bibliographic Details
Published in:Annals of neurology 2000-11, Vol.48 (5), p.788-791
Main Authors: Wanschitz, Julia, Klöppel, Stefan, Jarius, Christa, Birner, Peter, Flicker, Helga, Hainfellner, Johannes A., Gambetti, Pierluigi, Guentchev, Marin, Budka, Herbert
Format: Article
Language:English
Subjects:
Adult
Aged
Biological and medical sciences
Brain - pathology
Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases
Female
Human viral diseases
Humans
Immunohistochemistry
Infectious diseases
Male
Medical sciences
Middle Aged
Neurology
Prion Diseases - pathology
Receptors, Serotonin - analysis
Viral diseases
Viral diseases of the lymphoid tissue and the blood. Aids
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Summary: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
ISSN:0364-5134
1531-8249
DOI:10.1002/1531-8249(200011)48:5<788::AID-ANA13>3.0.CO;2-5