Abstract | Study Objectives: To follow the temporal changes of cerebrospinal fluid (CSF) biomarker levels in narcoleptic patients with unexpected hypocretin level at referral. Methods: Results: CSF hypocretin levels decreased from normal/intermediate to undetectable levels in three of the four patients with definite cataplexy and remained stable in the other (>250 pg/mL). Hypocretin level decreased from 106 to 27 pg/mL in one patient with atypical cataplexy, and remained stable in the other (101 and 106 pg/mL). CSF histamine and tele-methylhistamine levels remained stable, but for one patient showing increased frequency of cataplexy and a strong decrease (-72.5%) of tele-methylhistamine levels several years after disease onset. No significant association was found between relative or absolute change in hypocretin level and demographic/clinical features. Conclusions: These findings show that in few patients with narcolepsy with cataplexy, symptoms and CSF marker levels can change over time. In these rare patients with cataplexy without baseline hypocretin deficiency, CSF markers should be monitored over time with potential for immune therapies in early stages to try limiting hypocretin neuron loss.
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Authors | Régis Lopez, Lucie Barateau, Elisa Evangelista, Sofiene Chenini, Philippe Robert, Isabelle Jaussent, Yves Dauvilliers |
Journal | Sleep
(Sleep)
Vol. 40
Issue 1
(Jan 01 2017)
ISSN: 1550-9109 [Electronic] United States |
PMID | 28364477
(Publication Type: Journal Article)
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Copyright | © Sleep Research Society 2017. Published by Oxford University Press [on behalf of the Sleep Research Society]. |
Chemical References |
- Biomarkers
- HLA-DQ beta-Chains
- HLA-DQB1 antigen
- Methylhistamines
- Orexins
- Histamine
- tele-methylhistamine
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Topics |
- Adolescent
- Adult
- Biomarkers
(cerebrospinal fluid)
- Cataplexy
(cerebrospinal fluid)
- Child
- Female
- HLA-DQ beta-Chains
(immunology)
- Histamine
(cerebrospinal fluid)
- Histocompatibility Testing
- Humans
- Male
- Methylhistamines
(cerebrospinal fluid)
- Middle Aged
- Narcolepsy
(cerebrospinal fluid)
- Orexins
(cerebrospinal fluid, deficiency)
- Time Factors
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