Cataplexy features in childhood nacrolepsy

Leonardo Serra, Pasquale Montagna, Emmanuel Mignot, Elio Lugaresi, Giuseppe Plazzi

Research output: Contribution to journalArticlepeer-review

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.

Original languageEnglish
Pages (from-to)858-865
Number of pages8
JournalMovement Disorders
Volume23
Issue number6
DOIs
Publication statusPublished - Apr 30 2008

Keywords

  • Cataplectic facies
  • Cataplexy
  • Childhood
  • Narcolepsy
  • Sleep

ASJC Scopus subject areas

  • Clinical Neurology
  • Neurology
  • Medicine(all)

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