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Anomalous Hypothalamic Responses to Humor in Cataplexy

Reiss, Allan L. and Hoeft, Fumiko and Tenforde, Adam S. and Chen, Wynne and Mobbs, Dean and Mignot, Emmanuel J. (2008) Anomalous Hypothalamic Responses to Humor in Cataplexy. PLoS ONE, 3 (5). Art. No. e2225. ISSN 1932-6203. PMCID PMC2377337. doi:10.1371/journal.pone.0002225.

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Background: Cataplexy is observed in a subset of patients with narcolepsy and affects approximately 1 in 2,000 persons. Cataplexy is most often triggered by strong emotions such as laughter, which can result in transient, yet debilitating, muscle atonia. The objective of this study was to examine the neural systems underlying humor processing in individuals with cataplexy. Methodology/Principal Findings: While undergoing functional Magnetic Resonance Imaging (fMRI), we showed ten narcolepsy-cataplexy patients and ten healthy controls humorous cartoons. In addition, we examined the brain activity of one subject while in a full-blown cataplectic attack. Behavioral results showed that participants with cataplexy rated significantly fewer humorous cartoons as funny compared to controls. Concurrent fMRI showed that patients, when compared to controls and in the absence of overt cataplexy symptoms, showed pronounced activity in the emotional network including the ventral striatum and hypothalamus while viewing humorous versus non-humorous cartoons. Increased activity was also observed in the right inferior frontal gyri -a core component of the inhibitory circuitry. In comparison, the one subject who experienced a cataplectic attack showed dramatic reductions in hypothalamic activity. Conclusions: These findings suggest an overdrive of the emotional circuitry and possible compensatory suppression by cortical inhibitory regions in cataplexy. Moreover, during cataplectic attacks, the hypothalamus is characterized by a marked decrease in activity similar to that observed during sleep. One possible explanation for these findings is an initial overdrive and compensatory shutdown of the hypothalamus resulting in full cataplectic symptoms.

Item Type:Article
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URLURL TypeDescription CentralArticle
Mobbs, Dean0000-0003-1175-3772
Additional Information:© 2008 Reiss et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. Received: January 17, 2008; Accepted: April 3, 2008; Published: May 21, 2008. Editor: Ernest Greene, University of Southern California, United States of America Funded by NIH 23724 and the Howard Hughes Medical Institute. DM is funded by the Medical Research Council. Author Contributions: Conceived and designed the experiments: EM AR DM WC. Performed the experiments: DM AT WC. Analyzed the data: FH AT. Wrote the paper: EM AR FH DM. The authors have declared that no competing interests exist.
Funding AgencyGrant Number
Howard Hughes Medical Institute (HHMI)UNSPECIFIED
Medical Research Council (UK)UNSPECIFIED
Issue or Number:5
PubMed Central ID:PMC2377337
Record Number:CaltechAUTHORS:20180302-153736448
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Official Citation:Reiss AL, Hoeft F, Tenforde AS, Chen W, Mobbs D, Mignot EJ (2008) Anomalous Hypothalamic Responses to Humor in Cataplexy. PLoS ONE 3(5): e2225.
Usage Policy:No commercial reproduction, distribution, display or performance rights in this work are provided.
ID Code:85080
Deposited By: George Porter
Deposited On:05 Mar 2018 22:00
Last Modified:15 Nov 2021 20:25

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