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Sleep. 2014 Jun 1;37(6):1035-42. doi: 10.5665/sleep.3756.

Challenges in diagnosing narcolepsy without cataplexy: a consensus statement.

Author information

1
Department of Neurology, University Hospital Zurich, Zurich, Switzerland ; Department of Neurology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA.
2
Stanford Center for Sleep Sciences, Stanford University, Stanford, CA.
3
Department of Neurology, Leiden University Medical Center, Leiden, The Netherlands.
4
Department of Neurology, Radboud University Medical Center, Nijmegen, The Netherlands.
5
Hôpital Pitié-Salpêtrière, Pierre and Marie Curie University, Paris, France.
6
Department of Neurology and Program in Sleep, Emory University School of Medicine, Atlanta.
7
National Reference Network for Orphan Diseases (Narcolepsy), Department of Neurology, Hôpital Gui de Chauliac, CHU Montpellier, INSERM U1061, France.
8
Tokyo Metropolitan Institute of Sleep Science, Tokyo, Japan.
9
Sleep Medicine Children's National Med Center, Washington, DC.
10
Department of Biomedical and Neuromotor Neurological Sciences, University of Bologna, and IRCCS Istituto delle Scienze Neurologiche, Bologna, Italy.
11
Department of Neurology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA.

Abstract

BACKGROUND:

Diagnosing narcolepsy without cataplexy is often a challenge as the symptoms are nonspecific, current diagnostic tests are limited, and there are no useful biomarkers. In this report, we review the clinical and physiological aspects of narcolepsy without cataplexy, the limitations of available diagnostic procedures, and the differential diagnoses, and we propose an approach for more accurate diagnosis of narcolepsy without cataplexy.

METHODS:

A group of clinician-scientists experienced in narcolepsy reviewed the literature and convened to discuss current diagnostic tools, and to map out directions for research that should lead to a better understanding and more accurate diagnosis of narcolepsy without cataplexy.

RECOMMENDATIONS:

To aid in the identification of narcolepsy without cataplexy, we review key indicators of narcolepsy and present a diagnostic algorithm. A detailed clinical history is mainly helpful to rule out other possible causes of chronic sleepiness. The multiple sleep latency test remains the most important measure, and prior sleep deprivation, shift work, or circadian disorders should be excluded by actigraphy or sleep logs. A short REM sleep latency (≤ 15 minutes) on polysomnography can aid in the diagnosis of narcolepsy without cataplexy, although sensitivity is low. Finally, measurement of hypocretin levels can helpful, as levels are low to intermediate in 10% to 30% of narcolepsy without cataplexy patients.

KEYWORDS:

insomnia; multiple sleep latency test; narcolepsy; polysomnography

PMID:
24882898
PMCID:
PMC4015377
DOI:
10.5665/sleep.3756
[Indexed for MEDLINE]
Free PMC Article

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