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March 30, 2018Article

Neurocognition, sleep, and PET findings in type 2 vs type 1 narcolepsy

Yu-Shu Huang, Ing-Tsung Hsiao, Feng-Yuan Liu, Fang-Ming Hwang, Kuang-Lin Lin, Wen-Cheng Huang and Christian Guilleminault
First published March 30, 2018, DOI: https://doi.org/10.1212/WNL.0000000000005346
Yu-Shu Huang
From the Department of Child Psychiatry, Sleep Center and Craniofacial Research Center (Y.-S.H.), Department of Nuclear Medicine and Molecular Imaging Center (I.-T.H., F.-Y.L., W.-C.H.), and Division of Pediatric Neurology (K.-L.L.), Chang Gung Memorial Hospital and Chang Gung University College of Medicine; Department of Medical Imaging and Radiological Sciences, College of Medicine (I.-T.H., F.-Y.L.), Chang Gung University, Taoyuan; Department of Education (F.-M.H.), National Chia-Yi University, Chiayi, Taiwan; and Stanford University Sleep Medicine Division (C.G.), Redwood City, CA.
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Ing-Tsung Hsiao
From the Department of Child Psychiatry, Sleep Center and Craniofacial Research Center (Y.-S.H.), Department of Nuclear Medicine and Molecular Imaging Center (I.-T.H., F.-Y.L., W.-C.H.), and Division of Pediatric Neurology (K.-L.L.), Chang Gung Memorial Hospital and Chang Gung University College of Medicine; Department of Medical Imaging and Radiological Sciences, College of Medicine (I.-T.H., F.-Y.L.), Chang Gung University, Taoyuan; Department of Education (F.-M.H.), National Chia-Yi University, Chiayi, Taiwan; and Stanford University Sleep Medicine Division (C.G.), Redwood City, CA.
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Feng-Yuan Liu
From the Department of Child Psychiatry, Sleep Center and Craniofacial Research Center (Y.-S.H.), Department of Nuclear Medicine and Molecular Imaging Center (I.-T.H., F.-Y.L., W.-C.H.), and Division of Pediatric Neurology (K.-L.L.), Chang Gung Memorial Hospital and Chang Gung University College of Medicine; Department of Medical Imaging and Radiological Sciences, College of Medicine (I.-T.H., F.-Y.L.), Chang Gung University, Taoyuan; Department of Education (F.-M.H.), National Chia-Yi University, Chiayi, Taiwan; and Stanford University Sleep Medicine Division (C.G.), Redwood City, CA.
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Fang-Ming Hwang
From the Department of Child Psychiatry, Sleep Center and Craniofacial Research Center (Y.-S.H.), Department of Nuclear Medicine and Molecular Imaging Center (I.-T.H., F.-Y.L., W.-C.H.), and Division of Pediatric Neurology (K.-L.L.), Chang Gung Memorial Hospital and Chang Gung University College of Medicine; Department of Medical Imaging and Radiological Sciences, College of Medicine (I.-T.H., F.-Y.L.), Chang Gung University, Taoyuan; Department of Education (F.-M.H.), National Chia-Yi University, Chiayi, Taiwan; and Stanford University Sleep Medicine Division (C.G.), Redwood City, CA.
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Kuang-Lin Lin
From the Department of Child Psychiatry, Sleep Center and Craniofacial Research Center (Y.-S.H.), Department of Nuclear Medicine and Molecular Imaging Center (I.-T.H., F.-Y.L., W.-C.H.), and Division of Pediatric Neurology (K.-L.L.), Chang Gung Memorial Hospital and Chang Gung University College of Medicine; Department of Medical Imaging and Radiological Sciences, College of Medicine (I.-T.H., F.-Y.L.), Chang Gung University, Taoyuan; Department of Education (F.-M.H.), National Chia-Yi University, Chiayi, Taiwan; and Stanford University Sleep Medicine Division (C.G.), Redwood City, CA.
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Wen-Cheng Huang
From the Department of Child Psychiatry, Sleep Center and Craniofacial Research Center (Y.-S.H.), Department of Nuclear Medicine and Molecular Imaging Center (I.-T.H., F.-Y.L., W.-C.H.), and Division of Pediatric Neurology (K.-L.L.), Chang Gung Memorial Hospital and Chang Gung University College of Medicine; Department of Medical Imaging and Radiological Sciences, College of Medicine (I.-T.H., F.-Y.L.), Chang Gung University, Taoyuan; Department of Education (F.-M.H.), National Chia-Yi University, Chiayi, Taiwan; and Stanford University Sleep Medicine Division (C.G.), Redwood City, CA.
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Christian Guilleminault
From the Department of Child Psychiatry, Sleep Center and Craniofacial Research Center (Y.-S.H.), Department of Nuclear Medicine and Molecular Imaging Center (I.-T.H., F.-Y.L., W.-C.H.), and Division of Pediatric Neurology (K.-L.L.), Chang Gung Memorial Hospital and Chang Gung University College of Medicine; Department of Medical Imaging and Radiological Sciences, College of Medicine (I.-T.H., F.-Y.L.), Chang Gung University, Taoyuan; Department of Education (F.-M.H.), National Chia-Yi University, Chiayi, Taiwan; and Stanford University Sleep Medicine Division (C.G.), Redwood City, CA.
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Citation
Neurocognition, sleep, and PET findings in type 2 vs type 1 narcolepsy
Yu-Shu Huang, Ing-Tsung Hsiao, Feng-Yuan Liu, Fang-Ming Hwang, Kuang-Lin Lin, Wen-Cheng Huang, Christian Guilleminault
Neurology Mar 2018, 10.1212/WNL.0000000000005346; DOI: 10.1212/WNL.0000000000005346

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Abstract

Objective To analyze differences in functional brain images collected in patients with type 2 and type 1 narcolepsy compared to normal controls and the relationship among brain images, objective Neuropsychologic tests, and sleep findings.

Methods Data collection included comprehensive clinical investigation, study of sleep/wake with actigraphy, polysomnography, Multiple Sleep Latency Test, human leukocyte antigen typing, 18F-fluorodeoxyglucose PET, and cognitive tests obtained from 29 patients with type 2 narcolepsy, 104 patients with type 1 narcolepsy, and 26 sex- and age-matched normal control individuals. Conners’ Continuous Performance Test (CPT II) and Wisconsin Card-Sorting Task were performed simultaneously with the FDG-PET examination. After analyses of variance, data between patients with type 1 and type 2 narcolepsy were compared by post hoc analysis, and correlation between functional brain imaging findings and results of neurocognitive tests was obtained.

Results All patients with narcolepsy presented with at least 2 sleep-onset REM periods (SOREMP) and subjective sleepiness. Patients with type 2 narcolepsy compared to patients with type 1 narcolepsy had significantly less SOREMP, longer mean sleep latencies, and lower body mass indexes, apnea-hypopnea indexes, and frequency of human leukocyte antigen DQ-Beta1*0602. In patients with type 2 narcolepsy, FDG-PET studies showed significantly less hypermetabolism in the fusiform gyrus, striatum, hippocampus, thalamus, basal ganglia, and cerebellum than in patients with type 1 narcolepsy, and significantly less hypometabolism in the regions of frontal lobe, posterior cingulum, angular gyrus, and part of the parietal lobe; these changes were associated with fewer errors on the CPT.

Conclusion Young patients with type 2 narcolepsy have fewer clinical impairments and less distinct brain functional abnormalities than patients with type 1 narcolepsy, who are significantly more affected.

Footnotes

  • ↵* These authors contributed equally to this work.

  • Received December 3, 2016.
  • Accepted in final form January 4, 2018.
  • © 2018 American Academy of Neurology

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  • Narcolepsy
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  • PET
  • Neuropsychological assessment

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