French consensus. Type 1 and type 2 narcolepsy: Investigations and follow-up

C. Monaca, P. Franco, P. Philip, Y. Dauvilliers
Revue Neurologique. 2017-01-01; 173(1-2): 25-31
DOI: 10.1016/j.neurol.2016.09.016

Read on PubMed

1. Rev Neurol (Paris). 2017 Jan – Feb;173(1-2):25-31. doi:
10.1016/j.neurol.2016.09.016. Epub 2016 Nov 9.

French consensus. Type 1 and type 2 Narcolepsy: Investigations and follow-up.

[Article in French]

Monaca C(1), Franco P(2), Philip P(3), Dauvilliers Y(4).

Author information:
(1)Unité des troubles du sommeil, neurophysiologie clinique, CHRU de Lille, 2,
avenue Oscar-Lambret, 59000 Lille, France; Inserm U1171, 59000 Lille, France.
(2)Centre de référence national narcolepsie et hypersomnie idiopathique, France;
Unités d’explorations fonctionnelles neurologiques, hôpital Mère-Couple-Enfant,
hospices civils de Lyon, 69500 Lyon, France.
(3)Centre de référence Hôtel Dieu Paris, CHU de Bordeaux, 33076 Bordeaux, France;
USR SANPSY 3413 CNRS, université de Bordeaux, 33076 Bordeaux, France.
(4)Centre de référence national narcolepsie et hypersomnie idiopathique, France;
Sleep disorders centre, hôpital Gui-De-Chauliac, 80, avenue Augustin-Fliche,
34295 Montpellier cedex 5, France; Inserm U1061, 34295 Montpellier, France.
Electronic address: .

In the new international classification of sleep disorders (ICSD-3), narcolepsy
is differentiated into two distinct pathologies: type 1 narcolepsy (NT1) and type
2 narcolepsy (NT2). NT1 is characterised by periods of an irrepressible need to
sleep, cataplexy (a sudden loss of muscle tone triggered by emotion) and in some
cases the presence of symptoms such as hypnagogic hallucinations, sleep paralysis
and disturbed night-time sleep. Its physiopathology is based on the loss of
hypocretin neurons in the hypothalamus, seemingly connected to an auto-immune
process. By definition, cataplexy is absent and the hypocretin levels in the CSF
are normal in NT2. Confirming the diagnosis requires polysomnography and multiple
sleep latency tests. The choice of further investigations is based on the
presence or absence of typical cataplexy. Further investigations include HLA
typing, lumbar puncture to measure the hypocretin level in the CSF, or even brain
imagery in the case of narcolepsy suspected to be secondary to an underlying
pathology. In this consensus we propose recommendations for the work-up to be
carried out during diagnosis and follow-up for patients suffering from

Copyright © 2016 Elsevier Masson SAS. All rights reserved.

DOI: 10.1016/j.neurol.2016.09.016
PMID: 27838095 [Indexed for MEDLINE]

Know more about