Cataplexy features in childhood narcolepsy
Identifieur interne : 002953 ( Main/Curation ); précédent : 002952; suivant : 002954Cataplexy features in childhood narcolepsy
Auteurs : Leonardo Serra [Italie, Chili] ; Pasquale Montagna [Italie] ; Emmanuel Mignot [États-Unis] ; Elio Lugaresi [Italie] ; Giuseppe Plazzi [Italie]Source :
- Movement Disorders [ 0885-3185 ] ; 2008-04-30.
Descripteurs français
- Pascal (Inist)
- Wicri :
- topic : Enfant.
English descriptors
- KwdEn :
- Adolescent, Adult, Cataplexy, Cataplexy (etiology), Cataplexy (physiopathology), Child, Facial Expression, Female, Humans, Male, Movement Disorders (physiopathology), Narcolepsy, Narcolepsy (complications), Nervous system diseases, Sleep, Speech Disorders (physiopathology), Vision Disorders (physiopathology), cataplectic facies, cataplexy, childhood, narcolepsy, sleep.
- MESH :
- complications : Narcolepsy.
- etiology : Cataplexy.
- physiopathology : Cataplexy, Movement Disorders, Speech Disorders, Vision Disorders.
- Adolescent, Adult, Child, Facial Expression, Female, Humans, Male.
Abstract
Cataplexy, the hallmark of narcolepsy, has been well characterized in adults but not in children. This study systematically used structured clinical assessments and video‐recordings (49 episodes in eight cases) to evaluate cataplexy in 23 patients diagnosed before the age of 18 years. Forty‐three percent of patients had falls as part of their attacks. During cataplexy knees, head, and jaw were the most frequently compromised body segments; eyelids, arms, and trunk being less commonly involved. More rarely, blurred vision, slurred speech, irregular breathing, or a sudden loss of smiling mimics were reported. One‐third of the sample presented with a previously unrecognized description of cataplexy that we coined “cataplectic facies,” consisting of a state of semipermanent eyelid and jaw weakness, on which partial or complete cataplectic attacks were superimposed. The usual triggering emotions, such as laughter, joking, or anger, were not always present, especially when close to an abrupt onset, hampering diagnosis. Video‐recordings of cataplectic attacks may be useful to document the attack, allowing a comparison with archived presentations. © 2008 Movement Disorder Society
Url:
DOI: 10.1002/mds.21965
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<front><div type="abstract" xml:lang="en">Cataplexy, the hallmark of narcolepsy, has been well characterized in adults but not in children. This study systematically used structured clinical assessments and video-recordings (49 episodes in eight cases) to evaluate cataplexy in 23 patients diagnosed before the age of 18 years. Forty-three percent of patients had falls as part of their attacks. During cataplexy knees, head, and jaw were the most frequently compromised body segments; eyelids, arms, and trunk being less commonly involved. More rarely, blurred vision, slurred speech, irregular breathing, or a sudden loss of smiling mimics were reported. One-third of the sample presented with a previously unrecognized description of cataplexy that we coined "cataplectic facies," consisting of a state of semipermanent eyelid and jaw weakness, on which partial or complete cataplectic attacks were superimposed. The usual triggering emotions, such as laughter, joking, or anger, were not always present, especially when close to an abrupt onset, hampering diagnosis. Video-recordings of cataplectic attacks may be useful to document the attack, allowing a comparison with archived presentations.</div>
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<sourceDesc><biblStruct><analytic><title level="a" type="main" xml:lang="en">Cataplexy features in childhood narcolepsy</title>
<author><name sortKey="Serra, Leonardo" sort="Serra, Leonardo" uniqKey="Serra L" first="Leonardo" last="Serra">Leonardo Serra</name>
<affiliation wicri:level="1"><country xml:lang="fr">Italie</country>
<wicri:regionArea>Sleep Disorders Center, Department of Neurological Sciences, University of Bologna</wicri:regionArea>
<wicri:noRegion>University of Bologna</wicri:noRegion>
</affiliation>
<affiliation wicri:level="1"><country xml:lang="fr">Chili</country>
<wicri:regionArea>Sleep Medicine Center, Pontifice Catholic University of Chile, Santiago</wicri:regionArea>
<wicri:noRegion>Santiago</wicri:noRegion>
</affiliation>
</author>
<author><name sortKey="Montagna, Pasquale" sort="Montagna, Pasquale" uniqKey="Montagna P" first="Pasquale" last="Montagna">Pasquale Montagna</name>
<affiliation wicri:level="1"><country xml:lang="fr">Italie</country>
<wicri:regionArea>Sleep Disorders Center, Department of Neurological Sciences, University of Bologna</wicri:regionArea>
<wicri:noRegion>University of Bologna</wicri:noRegion>
</affiliation>
</author>
<author><name sortKey="Mignot, Emmanuel" sort="Mignot, Emmanuel" uniqKey="Mignot E" first="Emmanuel" last="Mignot">Emmanuel Mignot</name>
<affiliation wicri:level="2"><country xml:lang="fr">États-Unis</country>
<wicri:regionArea>Center for Narcolepsy, Stanford University, California</wicri:regionArea>
<placeName><region type="state">Californie</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Lugaresi, Elio" sort="Lugaresi, Elio" uniqKey="Lugaresi E" first="Elio" last="Lugaresi">Elio Lugaresi</name>
<affiliation wicri:level="1"><country xml:lang="fr">Italie</country>
<wicri:regionArea>Sleep Disorders Center, Department of Neurological Sciences, University of Bologna</wicri:regionArea>
<wicri:noRegion>University of Bologna</wicri:noRegion>
</affiliation>
</author>
<author><name sortKey="Plazzi, Giuseppe" sort="Plazzi, Giuseppe" uniqKey="Plazzi G" first="Giuseppe" last="Plazzi">Giuseppe Plazzi</name>
<affiliation wicri:level="1"><country xml:lang="fr">Italie</country>
<wicri:regionArea>Sleep Disorders Center, Department of Neurological Sciences, University of Bologna</wicri:regionArea>
<wicri:noRegion>University of Bologna</wicri:noRegion>
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<series><title level="j">Movement Disorders</title>
<title level="j" type="abbrev">Mov. Disord.</title>
<idno type="ISSN">0885-3185</idno>
<idno type="eISSN">1531-8257</idno>
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<pubPlace>Hoboken</pubPlace>
<date type="published" when="2008-04-30">2008-04-30</date>
<biblScope unit="vol">23</biblScope>
<biblScope unit="issue">6</biblScope>
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<profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Adolescent</term>
<term>Adult</term>
<term>Cataplexy (etiology)</term>
<term>Cataplexy (physiopathology)</term>
<term>Child</term>
<term>Facial Expression</term>
<term>Female</term>
<term>Humans</term>
<term>Male</term>
<term>Movement Disorders (physiopathology)</term>
<term>Narcolepsy (complications)</term>
<term>Speech Disorders (physiopathology)</term>
<term>Vision Disorders (physiopathology)</term>
<term>cataplectic facies</term>
<term>cataplexy</term>
<term>childhood</term>
<term>narcolepsy</term>
<term>sleep</term>
</keywords>
<keywords scheme="MESH" qualifier="complications" xml:lang="en"><term>Narcolepsy</term>
</keywords>
<keywords scheme="MESH" qualifier="etiology" xml:lang="en"><term>Cataplexy</term>
</keywords>
<keywords scheme="MESH" qualifier="physiopathology" xml:lang="en"><term>Cataplexy</term>
<term>Movement Disorders</term>
<term>Speech Disorders</term>
<term>Vision Disorders</term>
</keywords>
<keywords scheme="MESH" xml:lang="en"><term>Adolescent</term>
<term>Adult</term>
<term>Child</term>
<term>Facial Expression</term>
<term>Female</term>
<term>Humans</term>
<term>Male</term>
</keywords>
</textClass>
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<front><div type="abstract" xml:lang="en">Cataplexy, the hallmark of narcolepsy, has been well characterized in adults but not in children. This study systematically used structured clinical assessments and video‐recordings (49 episodes in eight cases) to evaluate cataplexy in 23 patients diagnosed before the age of 18 years. Forty‐three percent of patients had falls as part of their attacks. During cataplexy knees, head, and jaw were the most frequently compromised body segments; eyelids, arms, and trunk being less commonly involved. More rarely, blurred vision, slurred speech, irregular breathing, or a sudden loss of smiling mimics were reported. One‐third of the sample presented with a previously unrecognized description of cataplexy that we coined “cataplectic facies,” consisting of a state of semipermanent eyelid and jaw weakness, on which partial or complete cataplectic attacks were superimposed. The usual triggering emotions, such as laughter, joking, or anger, were not always present, especially when close to an abrupt onset, hampering diagnosis. Video‐recordings of cataplectic attacks may be useful to document the attack, allowing a comparison with archived presentations. © 2008 Movement Disorder Society</div>
</front>
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