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Clinical significance of fatal laryngeal stridor in fatal familial insomnia
Author(s) -
Fukuoka Takuya,
Kawasaki Hitoshi,
Nakazato Yoshihiko,
Takahashi Kazushi,
Ikeda Kei,
Furuya Tomotsugu,
Miyake Akifumi,
Mitsufuji Takashi,
Ito Yasuo,
Araki Nobuo,
Yamamoto Toshimasa
Publication year - 2019
Publication title -
neurology and clinical neuroscience
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.125
0ISSN - 2049-4173
DOI - 10.1111/ncn3.12293
Subject(s) - medicine , stridor , dysphagia , dysautonomia , swallowing , pediatrics , anesthesia , surgery , airway , disease
Abstract Background Fatal familial insomnia ( FFI ) is characterized by numerous signs and symptoms, including progressive insomnia; dysautonomia and endocrine disturbances; and motor disturbances such as myoclonus, ataxia, parkinsonism, spasticity, dysarthria, and dysphagia. Mental health and behavioral changes include impaired concentration and memory, as well as confusion and hallucinations. FFI patients frequently die due to secondary infections and sudden insomnia‐induced death. To date, laryngeal stridor has not been fully investigated in FFI patients. Aim This study aims to clarify the mechanism of laryngeal stridor in patients with FFI . Methods We retrospectively analyzed the clinical records of two FFI patients. Results Two men (age: 58 and 61 years) were diagnosed with FFI . They showed laryngeal stridor, swallowing dysfunction, and respiratory failure. They required mechanical ventilation, followed by a tracheostomy. Conclusion Laryngeal stridor in FFI was not previously investigated in detail. If laryngeal stridor occurs in awake FFI patients, emergency tracheostomy should be performed.