Laryngeal dystonia in xeroderma pigmentosum.
We report on three patients with xeroderma pigmentosum group A (XPA) who showed laryngeal stridor in their 20s. The stridor appeared on feeding and emotional excitation, was exaggerated during respiratory infection and was life-threatening on some occasions. Adduction of the vocal cords during inspiration, observed by laryngoscopy, confirmed laryngeal dystonia in all cases. This type of focal dystonia may be characteristic in XPA and requires special attention during the management of these patients to avoid serious complications.
Muto
Ayako
A
Department of Pediatrics and Pediatric Neurology, Yokohama Ryo-iku Medical Center, 557-2 Ichizawa-cho, Asahi-ku, Yokohama, and Department of Pediatrics, Tokyo Women's Medical University, Tokyo, Japan.
Matsui
Akira
A
Saito
Yoshiaki
Y
Iwamoto
Hiroko
H
Kaneko
Kaori
K
Masuko
Kaori
K
Chikumaru
Yuri
Y
Saito
Kazuyo
K
Kimura
Seiji
S
D000328:Adult; D004421:Dystonia / Q000209:etiology* / Q000473:pathology; D005260:Female; D006801:Humans; D007818:Laryngeal Diseases / Q000209:etiology* / Q000473:pathology; D007830:Larynx / Q000473:pathology; D008297:Male; D014983:Xeroderma Pigmentosum / Q000150:complications* / Q000473:pathology