Sleep Medicine 15 (2014) 273–275
Contents lists available at ScienceDirect
Sleep Medicine journal homepage: www.elsevier.com/locate/sleep
Video-Clinical Corners
Introduction to Video-Clinical Corners This section is a new feature of Sleep Medicine allowing readers to submit unique sleep related cases with video segments as a way to enhance educational value in a manner unattainable through paper format. Please see our web site’s (http://ees.elsevier.com/sleep/)
Guide for Authors for instructions. We hope this section will be enriched by the contributions of our colleagues who wish to offer stimulating opportunities for discussion and new insights into the field of sleep.
Cataplectic attacks during rapid eye movement sleep behavior disorder episodes in a narcoleptic patient Simone Baiardi a, Fabio Pizza a, Stefano Vandi a, Christian Franceschini a, Alessandro Vigo b, Carlo Cipolli c, Giovanni Tuozzi d, Rocco Liguori a, Giuseppe Plazzi a,⇑ a
DIBINEM, Alma Mater Studiorum University of Bologna and IRCCS Istituto delle Scienze Neurologiche, Bologna, Italy Centro S.I.D.S., A.O. Città della Salute e della Scienza, Torino, Italy c DIMES, Alma Mater Studiorum University of Bologna, Bologna, Italy d QUVI, Alma Mater Studiorum University of Bologna, Bologna, Italy b
a r t i c l e
i n f o
a b s t r a c t
Article history: Received 14 June 2013 Received in revised form 31 July 2013 Accepted 2 August 2013 Available online 22 November 2013 Keywords: Narcolepsy Cataplexy REM sleep behavior disorder Status dissociates REM sleep atonia Video-polysomnography
1. Introduction Narcolepsy with cataplexy (NC) is characterized by a loss of boundaries between wake and sleep and altered manifestations of rapid eye movement (REM) sleep [1]. In particular, cataplexy, a sudden loss of muscle tone during wakefulness triggered by strong emotions and the main motor feature of NC, is considered an incomplete REM sleep manifestation (REM sleep muscle atonia surfacing during wakefulness) [1,2]. The other typical symptoms of NC (sleep paralysis and hallucinations) also are interpreted as REM sleep–wakefulness dissociated states which frequently overlap [2].
⇑ Corresponding author. Address: DIBINEM, Alma Mater Studiorum, University of Bologna, Via Ugo Foscolo 7, 40123 Bologna, Italy. Tel.: +39 051 4966926. E-mail address: [email protected] (G. Plazzi). 1389-9457/$ - see front matter Ó 2013 Elsevier B.V. All rights reserved. http://dx.doi.org/10.1016/j.sleep.2013.08.794
REM sleep behavior disorder (RBD), characterized by intermittent loss of REM sleep muscle atonia and intense motor or verbal dream enacting activity [1], also is a dissociated REM sleep–wakefulness 2013 Elsevier B.V. All rights reserved. state common in NC [3] and Ó may occur at the sleep-onset REM period (SOREMP) [4]. Although RBD in NC has a different pathophysiology from idiopathic RBD and it does not seem to represent a tell-tale of Parkinsonism or dementia [5], it can be an important concern (i.e., leading to injuries [6]) and also the heralding sign of NC [7]. Herein, we report one NC patient who presented cataplexy and RBD close to disease onset during the same REM sleep period.
2. Case report A 14-year-old boy presented with excessive daytime sleepiness (EDS) and cataplexy, characterized by head drops and occasional knee weakness, which was elicited by laughing; multisensory hypnagogic hallucinations; and sleep paralysis since the age of 12 years (since 2011). The patient never took stimulants, sodium oxybate, hypnotic agents, or antidepressant agents; however, he exhibited pathologic EDS, scoring 17 at the Epworth Sleepiness Scale; facial hypotonia; eyelid narrowing; and a tendency of protrusion of his tongue on neurologic examination (cataplectic facies [8,9]). Brain magnetic resonance imaging revealed no abnormalities. He carried the HLA DQB1⁄06:02 allele and his cerebrospinal hypocretin-1 level was 23.1 pg/mL (hypocretin-1 deficiency defined as
Contents lists available at ScienceDirect
Sleep Medicine journal homepage: www.elsevier.com/locate/sleep
Video-Clinical Corners
Introduction to Video-Clinical Corners This section is a new feature of Sleep Medicine allowing readers to submit unique sleep related cases with video segments as a way to enhance educational value in a manner unattainable through paper format. Please see our web site’s (http://ees.elsevier.com/sleep/)
Guide for Authors for instructions. We hope this section will be enriched by the contributions of our colleagues who wish to offer stimulating opportunities for discussion and new insights into the field of sleep.
Cataplectic attacks during rapid eye movement sleep behavior disorder episodes in a narcoleptic patient Simone Baiardi a, Fabio Pizza a, Stefano Vandi a, Christian Franceschini a, Alessandro Vigo b, Carlo Cipolli c, Giovanni Tuozzi d, Rocco Liguori a, Giuseppe Plazzi a,⇑ a
DIBINEM, Alma Mater Studiorum University of Bologna and IRCCS Istituto delle Scienze Neurologiche, Bologna, Italy Centro S.I.D.S., A.O. Città della Salute e della Scienza, Torino, Italy c DIMES, Alma Mater Studiorum University of Bologna, Bologna, Italy d QUVI, Alma Mater Studiorum University of Bologna, Bologna, Italy b
a r t i c l e
i n f o
a b s t r a c t
Article history: Received 14 June 2013 Received in revised form 31 July 2013 Accepted 2 August 2013 Available online 22 November 2013 Keywords: Narcolepsy Cataplexy REM sleep behavior disorder Status dissociates REM sleep atonia Video-polysomnography
1. Introduction Narcolepsy with cataplexy (NC) is characterized by a loss of boundaries between wake and sleep and altered manifestations of rapid eye movement (REM) sleep [1]. In particular, cataplexy, a sudden loss of muscle tone during wakefulness triggered by strong emotions and the main motor feature of NC, is considered an incomplete REM sleep manifestation (REM sleep muscle atonia surfacing during wakefulness) [1,2]. The other typical symptoms of NC (sleep paralysis and hallucinations) also are interpreted as REM sleep–wakefulness dissociated states which frequently overlap [2].
⇑ Corresponding author. Address: DIBINEM, Alma Mater Studiorum, University of Bologna, Via Ugo Foscolo 7, 40123 Bologna, Italy. Tel.: +39 051 4966926. E-mail address: [email protected] (G. Plazzi). 1389-9457/$ - see front matter Ó 2013 Elsevier B.V. All rights reserved. http://dx.doi.org/10.1016/j.sleep.2013.08.794
REM sleep behavior disorder (RBD), characterized by intermittent loss of REM sleep muscle atonia and intense motor or verbal dream enacting activity [1], also is a dissociated REM sleep–wakefulness 2013 Elsevier B.V. All rights reserved. state common in NC [3] and Ó may occur at the sleep-onset REM period (SOREMP) [4]. Although RBD in NC has a different pathophysiology from idiopathic RBD and it does not seem to represent a tell-tale of Parkinsonism or dementia [5], it can be an important concern (i.e., leading to injuries [6]) and also the heralding sign of NC [7]. Herein, we report one NC patient who presented cataplexy and RBD close to disease onset during the same REM sleep period.
2. Case report A 14-year-old boy presented with excessive daytime sleepiness (EDS) and cataplexy, characterized by head drops and occasional knee weakness, which was elicited by laughing; multisensory hypnagogic hallucinations; and sleep paralysis since the age of 12 years (since 2011). The patient never took stimulants, sodium oxybate, hypnotic agents, or antidepressant agents; however, he exhibited pathologic EDS, scoring 17 at the Epworth Sleepiness Scale; facial hypotonia; eyelid narrowing; and a tendency of protrusion of his tongue on neurologic examination (cataplectic facies [8,9]). Brain magnetic resonance imaging revealed no abnormalities. He carried the HLA DQB1⁄06:02 allele and his cerebrospinal hypocretin-1 level was 23.1 pg/mL (hypocretin-1 deficiency defined as
