title: Cerebral perfusion abnormality in narcolepsy with cataplexy.
abstract: To investigate abnormal cerebral perfusion in narcoleptics with cataplexy, 25 narcoleptics with cataplexy and 25 normal controls were enrolled in this study. Cerebral perfusion was measured by brain single photon emission computed tomography (SPECT) using 99mTc-ethylcysteinate dimer. Patients and normal controls had not received any medication prior to the SPECT scan. Differences in cerebral perfusion between narcoleptics and normal controls were subjected to statistical parametric mapping (SPM) analysis. Overnight polysomnography and multiple sleep latency test (MSLT) were performed in all patients. Brain SPECT was carried out on all patients and normal controls during the waking state. Clinical symptoms and MSLT results of all patients are in accord with the International Classification of Sleep Disorders criteria for narcolepsy. MSLT showed a short mean sleep latency (1.69 +/- 1.0 min) and 2-5 sleep onset REM periods in individual patient. SPM analysis of brain SPECT showed hypoperfusion of the bilateral anterior hypothalami, caudate nuclei, and pulvinar nuclei of thalami, parts of the dorsolateral/ventromedial prefrontal cortices, parahippocampal gyri, and cingulate gyri in narcoleptics [P < 0.05 by Student's t test with false discovery rate (FDR) correction]. Significant hypoperfusion in the white matter of frontal and parietal lobes was also noted in narcoleptics. This study shows reduced cerebral perfusion in subcortical structures and cortical areas in narcoleptics. The distribution of abnormal cerebral perfusion is concordant with the pathway of the cerebral hypocretin system and may explain the characteristic features of narcolepsy, i.e., cataplexy, emotional lability, and attention deficit.
authors.affiliation: Department of Neurology, Samsung Medical Center and Center for Clinical Medicine, SBRI, Sungkyunkwan University School of Medicine, 50 Irwon-dong, Gangnam-gu, Seoul, Korea.
authors.forename: Eun Yeon
authors.initials: EY
authors.lastname: Joo
authors.forename: Seung Bong
authors.initials: SB
authors.lastname: Hong
authors.forename: Woo Suk
authors.initials: WS
authors.lastname: Tae
authors.forename: Jee Hyun
authors.initials: JH
authors.lastname: Kim
authors.forename: Sun Jung
authors.initials: SJ
authors.lastname: Han
authors.forename: Yong Won
authors.initials: YW
authors.lastname: Cho
authors.forename: Chang Ho
authors.initials: CH
authors.lastname: Yoon
authors.forename: Sung Ik
authors.initials: SI
authors.lastname: Lee
authors.forename: Mann Hyung
authors.initials: MH
authors.lastname: Lee
authors.forename: Kyung Han
authors.initials: KH
authors.lastname: Lee
authors.forename: Myoung-Hee
authors.initials: MH
authors.lastname: Kim
authors.forename: Byung Tae
authors.initials: BT
authors.lastname: Kim
authors.forename: Leen
authors.initials: L
authors.lastname: Kim
mesh_terms: D000293:Adolescent; D000328:Adult; D001921:Brain / Q000000981:diagnostic imaging; D001931:Brain Mapping; D002385:Cataplexy / Q000000981:diagnostic imaging / Q000503:physiopathology*; D002421:Caudate Nucleus / Q000000981:diagnostic imaging; D002561:Cerebrovascular Disorders / Q000000981:diagnostic imaging / Q000503:physiopathology*; D003545:Cysteine / Q000031:analogs & derivatives; D003627:Data Interpretation, Statistical; D005260:Female; D006801:Humans; D007031:Hypothalamus / Q000000981:diagnostic imaging; D007091:Image Processing, Computer-Assisted; D008297:Male; D008875:Middle Aged; D009290:Narcolepsy / Q000000981:diagnostic imaging / Q000503:physiopathology*; D015609:Organotechnetium Compounds; D017286:Polysomnography; D019275:Radiopharmaceuticals; D012890:Sleep / Q000187:drug effects; D015899:Tomography, Emission-Computed, Single-Photon
chemical_list: D015609:Organotechnetium Compounds; D019275:Radiopharmaceuticals; C059659:technetium Tc 99m bicisate; D003545:Cysteine