MovDisordV3, Ncbi, Merge, bibRecord, 002057

Cataplexy features in childhood narcolepsy.

Identifieur interne : 002057 ( Ncbi/Merge ); précédent : 002056; suivant : 002058

Cataplexy features in childhood narcolepsy.

Auteurs : Leonardo Serra [Italie] ; Pasquale Montagna ; Emmanuel Mignot ; Elio Lugaresi ; Giuseppe Plazzi

Source :

Movement disorders : official journal of the Movement Disorder Society [ 1531-8257 ] ; 2008.

RBID : pubmed:18307264

English descriptors

KwdEn :
- Adolescent, Adult, Cataplexy (etiology), Cataplexy (physiopathology), Child, Facial Expression, Female, Humans, Male, Movement Disorders (physiopathology), Narcolepsy (complications), Speech Disorders (physiopathology), Vision Disorders (physiopathology).
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.

DOI: 10.1002/mds.21965
PubMed: 18307264

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Le document en format XML

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<author><name sortKey="Serra, Leonardo" sort="Serra, Leonardo" uniqKey="Serra L" first="Leonardo" last="Serra">Leonardo Serra</name>
<affiliation wicri:level="1"><nlm:affiliation>Sleep Disorders Center, Department of Neurological Sciences, University of Bologna, Italy.</nlm:affiliation>
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<author><name sortKey="Montagna, Pasquale" sort="Montagna, Pasquale" uniqKey="Montagna P" first="Pasquale" last="Montagna">Pasquale Montagna</name>
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<author><name sortKey="Mignot, Emmanuel" sort="Mignot, Emmanuel" uniqKey="Mignot E" first="Emmanuel" last="Mignot">Emmanuel Mignot</name>
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<author><name sortKey="Lugaresi, Elio" sort="Lugaresi, Elio" uniqKey="Lugaresi E" first="Elio" last="Lugaresi">Elio Lugaresi</name>
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<author><name sortKey="Plazzi, Giuseppe" sort="Plazzi, Giuseppe" uniqKey="Plazzi G" first="Giuseppe" last="Plazzi">Giuseppe Plazzi</name>
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<author><name sortKey="Lugaresi, Elio" sort="Lugaresi, Elio" uniqKey="Lugaresi E" first="Elio" last="Lugaresi">Elio Lugaresi</name>
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<term>Facial Expression</term>
<term>Female</term>
<term>Humans</term>
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<term>Movement Disorders (physiopathology)</term>
<term>Narcolepsy (complications)</term>
<term>Speech Disorders (physiopathology)</term>
<term>Vision Disorders (physiopathology)</term>
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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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<Title>Movement disorders : official journal of the Movement Disorder Society</Title>
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<Abstract><AbstractText>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.</AbstractText>
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	Movement Disorders (revue)
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Movement Disorders (revue)

Cataplexy features in childhood narcolepsy.

Cataplexy features in childhood narcolepsy.

Source :

English descriptors

Abstract

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