Cerebral perfusion abnormality in narcolepsy with cataplexy.
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.
Joo
Eun Yeon
EY
Department of Neurology, Samsung Medical Center and Center for Clinical Medicine, SBRI, Sungkyunkwan University School of Medicine, 50 Irwon-dong, Gangnam-gu, Seoul, Korea.
Hong
Seung Bong
SB
Tae
Woo Suk
WS
Kim
Jee Hyun
JH
Han
Sun Jung
SJ
Cho
Yong Won
YW
Yoon
Chang Ho
CH
Lee
Sung Ik
SI
Lee
Mann Hyung
MH
Lee
Kyung Han
KH
Kim
Myoung-Hee
MH
Kim
Byung Tae
BT
Kim
Leen
L
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
D015609:Organotechnetium Compounds; D019275:Radiopharmaceuticals; C059659:technetium Tc 99m bicisate; D003545:Cysteine