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Coexisting narcolepsy (with and without cataplexy) and multiple sclerosis

Six new cases and a literature review

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Abstract

Background

There are increasing data suggesting the involvement of the immune system in narcolepsy. The co-occurrence of narcolepsy with other autoimmune disorders (including multiple sclerosis, MS) is rare.

Patients and methods

International multicenter sleep center survey and literature review on narcolepsy with (NC) and without (NwC) cataplexy.

Results

A total of 26 patients (pts), 6 in the survey and 20 in the literature were found. Two different types of association were identified: (1) Symptomatic type (5 pts): MS preceding the onset of narcolepsy, which was always without cataplexy (NwC); sleep onset REM episodes (SOREM) and hypocretin deficiency were observed in some, and lesions in the hypothalamus in all patients. (2) Coexisting type (18 pts): MS preceding or following the appearance of NC with SOREM, hypocretin deficiency but no lesions in the hypothalamus. A positive effect of steroids, immunoglobulins or natalizumab on narcolepsy symptoms was observed in four patients.

Discussion

Narcolepsy and MS are rarely associated. In addition to NwC secondary to hypothalamic demyelination, some patients present a coexistence of MS with NC without detectable hypothalamic lesions. The rarity of reports on this association probably reflects underrecognition. The elucidation of underlying genetic and immune mechanisms needs further studies.

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Author notes

  1. Ulf Kallweit and Claudio L. A. Bassetti are co-shared first authors.

Authors and Affiliations

  1. Department of Neurology, University Hospital of Bern (Inselspital), Freiburgstrasse 18, 3010, Bern, Switzerland

    Ulf Kallweit, Claudio L. A. Bassetti, Michael Oberholzer & Mathieu Béguin

  2. Institute for Immunology, University Witten/Herdecke, Witten, Germany

    Ulf Kallweit

  3. Leiden Medical University Center and University of Leiden, Leiden, The Netherlands

    Rolf Fronczek & Gert Jan Lammers

  4. Practice for Neurology, Basel, Switzerland

    Matthias Strub

  5. Sleep-Wake Center SEIN, Heemstede, The Netherlands

    Gert Jan Lammers

Authors
  1. Ulf Kallweit
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  2. Claudio L. A. Bassetti
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  3. Michael Oberholzer
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  4. Rolf Fronczek
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  5. Mathieu Béguin
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  6. Matthias Strub
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  7. Gert Jan Lammers
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Corresponding author

Correspondence to Claudio L. A. Bassetti.

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The authors declare that they have no conflict of interest.

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Kallweit, U., Bassetti, C.L.A., Oberholzer, M. et al. Coexisting narcolepsy (with and without cataplexy) and multiple sclerosis. J Neurol 265, 2071–2078 (2018). https://doi.org/10.1007/s00415-018-8949-x

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  • DOI: https://doi.org/10.1007/s00415-018-8949-x

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