Cataplexy features in childhood narcolepsy.
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.
Serra
Leonardo
L
Sleep Disorders Center, Department of Neurological Sciences, University of Bologna, Italy.
Montagna
Pasquale
P
Mignot
Emmanuel
E
Lugaresi
Elio
E
Plazzi
Giuseppe
G
D000293:Adolescent; D000328:Adult; D002385:Cataplexy / Q000209:etiology* / Q000503:physiopathology; D002648:Child; D005149:Facial Expression; D005260:Female; D006801:Humans; D008297:Male; D009069:Movement Disorders / Q000503:physiopathology; D009290:Narcolepsy / Q000150:complications*; D013064:Speech Disorders / Q000503:physiopathology; D014786:Vision Disorders / Q000503:physiopathology