title: A case of Creutzfeldt-Jacob disease with bilateral vocal fold abductor paralysis.
abstract: Bilateral vocal fold abductor paralysis was seen in a patient with Creutzfeldt-Jacob disease. After tracheotomy, the patient showed disappearance of reduced oxygen saturation with high-pitched inspiratory stridor and pulling phenomenon of the supraclavicular region and larynx. Electromyographic examinations of the intrinsic laryngeal muscles, including the thyroarytenoid and posterior cricoarytenoid muscles, demonstrated that there was no apparent action potential in those muscles during spontaneous respiratory movements, and there was no abnormal potential for those muscles at rest. By pushing the infrasternal region of the patient on the expiration, normal motor unit action potential could be seen in the posterior cricoarytenoid muscle on the next inspiration. Based on those findings, we concluded that bilateral vocal fold abductor paralysis in this case of Creutzfeldt-Jacob disease was not induced by disorders of the degeneration of motor nucleus in the ambiguus as in multiple system atrophy, but by a disorder of the upper motor neuron.
authors.affiliation: Department of Otolaryngology, Nippon Medical School, Tokyo 113-8603, Japan.
authors.forename: Lishu
authors.initials: L
authors.lastname: Li
authors.forename: Hideto
authors.initials: H
authors.lastname: Saigusa
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Nagayama
authors.forename: Tsuyoshi
authors.initials: T
authors.lastname: Nakamura
authors.forename: Iichirou
authors.initials: I
authors.lastname: Aino
authors.forename: Taro
authors.initials: T
authors.lastname: Komachi
authors.forename: Satoshi
authors.initials: S
authors.lastname: Yamaguchi
mesh_terms: D000200:Action Potentials; D001921:Brain / Q000473:pathology; D007562:Creutzfeldt-Jakob Syndrome / Q000150:complications* / Q000503:physiopathology / Q000601:surgery; D004576:Electromyography; D005260:Female; D006801:Humans; D007821:Laryngeal Muscles / Q000473:pathology / Q000503:physiopathology* / Q000601:surgery; D007828:Laryngoscopy; D008279:Magnetic Resonance Imaging; D008875:Middle Aged; D012119:Respiration; D016896:Treatment Outcome; D014826:Vocal Cord Paralysis / Q000209:etiology* / Q000503:physiopathology* / Q000601:surgery