title: Anomalous hypothalamic responses to humor in cataplexy.
abstract: 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.
  
  
  RESULTS
  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.
authors.affiliation: Center for Interdisciplinary Brain Sciences Research, Department of Psychiatry and Behavioral Sciences, Stanford University School of Medicine, Stanford, California, United States of America. reiss@stanford.edu
authors.forename: Allan L
authors.initials: AL
authors.lastname: Reiss
authors.forename: Fumiko
authors.initials: F
authors.lastname: Hoeft
authors.forename: Adam S
authors.initials: AS
authors.lastname: Tenforde
authors.forename: Wynne
authors.initials: W
authors.lastname: Chen
authors.forename: Dean
authors.initials: D
authors.lastname: Mobbs
authors.forename: Emmanuel J
authors.initials: EJ
authors.lastname: Mignot
mesh_terms: D000328:Adult; D002385:Cataplexy / Q000503:physiopathology*; D006801:Humans; D007031:Hypothalamus / Q000503:physiopathology*; D008279:Magnetic Resonance Imaging; D014925:Wit and Humor as Topic