Mechanisms and prevention of sudden death in multiple system atrophy.
Sudden death in multiple system atrophy (MSA) usually occurs during sleep and was therefore attributed to suffocation resulting from vocal cord abductor paralysis, a characteristic laryngeal finding of MSA. This led to the use of tracheostomy and noninvasive positive pressure ventilation (NPPV) for the prevention of sudden death. However, neither method has been able to prevent sudden death, and both have occasionally precipitated treatment-related complications, including central sleep apneas and exacerbation of floppy epiglottis. Therefore, it is important to determine the mechanisms and prevention of sudden death in MSA.



We reviewed the literature on the mechanisms and prevention of sudden death in patients with MSA.



Sudden death in MSA is hypothesized to be a consequence of disordered central respiration, suffocation caused by sputum and food, upper airway obstruction from NPPV acting on a floppy epiglottis, cardiac autonomic disturbance, or a combination of these factors.



Various factors may be involved in the mechanism of sudden death in MSA. A multidisciplinary approach is needed to prevent sudden death, and this requires an organized system of several medical specialties. Neurologists require a cooperative network that includes experts in otorhinolaryngology, sleep medicine, dysphagia rehabilitation, and cardiology.
Department of Neurology, Brain Research Institute, Niigata University, Aahimachi-dori 1-757, Chuo-ku, 951-8585, Niigata City, Niigata, Japan. Electronic address: t-shimo@bri.niigata-u.ac.jp.
Takayoshi
T
Shimohata
Department of Otorhinolaryngology, Uonuma Institute of Community Medicine, Niigata University Medical and Dental Hospital, Minami-uonuma, Japan.
Naotaka
N
Aizawa
Department of Respiratory Medicine, Tokyo Medical University, Tokyo, Japan.
Hideaki
H
Nakayama
Department of Dentistry, School of Medicine, Fujita Health University, Toyoake, Japan.
Hiroshige
H
Taniguchi
Department of Respiratory Medicine and Infectious Diseases, Niigata University Graduate School of Medical and Dental Sciences, Niigata, Japan.
Yasuyoshi
Y
Ohshima
Division of Otolaryngology, Niigata University Graduate School of Medical and Dental Sciences, Niigata, Japan.
Hitoshi
H
Okumura
Department of Neurology, Brain Research Institute, Niigata University, Aahimachi-dori 1-757, Chuo-ku, 951-8585, Niigata City, Niigata, Japan.
Tetsuya
T
Takahashi
Department of Molecular Neuroscience, Resource Branch for Brain Disease Research, Brain Research Institute, Niigata University, Niigata, Japan.
Akio
A
Yokoseki
Division of Dysphagia Rehabilitation, Niigata University Graduate School of Medical and Dental Sciences, Niigata, Japan.
Makoto
M
Inoue
Department of Neurology, Brain Research Institute, Niigata University, Aahimachi-dori 1-757, Chuo-ku, 951-8585, Niigata City, Niigata, Japan.
Masatoyo
M
Nishizawa
D003645:Death, Sudden / Q000517:prevention & control*; D006801:Humans; D019578:Multiple System Atrophy / Q000150:complications*; D017286:Polysomnography / Q000379:methods; D012890:Sleep / Q000502:physiology*; D014139:Tracheostomy / Q000379:methods; D014826:Vocal Cord Paralysis / Q000150:complications*
Abnormal control of breathing; Floppy epiglottis; Multiple system atrophy; Prevention; Sudden death