Louter Maartje, Pelleboer Roel H, van den Broek Guido B, Post Bart, Pevernagie Dirk A A, Overeem Sebastiaan
UMC St Radboud, Afd. Neurologie, Nijmegen, the Netherlands.
Ned Tijdschr Geneeskd. 2011;155(46):A3621.
Multiple system atrophy is a neurodegenerative disorder with parkinsonism, cerebellar ataxia and autonomic dysfunction. The occurrence of nocturnal stridor in patients with multiple system atrophy is associated with a decreased life expectancy. This is what makes adequate treatment so important. Nevertheless, stridor goes often unrecognized and there is frequent uncertainty about the therapeutic options.
We present a 58-year-old woman with multiple system atrophy and nocturnal stridor. Direct laryngoscopy showed bilateral paresis of the vocal cords and glottic stenosis. Since the outcome of treatment with continuous positive airway pressure (CPAP) was unsatisfactory, a tracheotomy was performed after which the stridor disappeared.
Polysomnography and laryngoscopy are the important diagnostic tools for this condition, after the clinical interview. Tracheotomy used to be the only therapeutic option for stridor. However, recent studies show a comparable effectiveness of non-invasive treatment with CPAP, provided there is a correct indication. The choice of therapy is a multidisciplinary process in which the neurologist, the sleep medicine specialist, the otolaryngologist, and the patient are involved.
多系统萎缩是一种伴有帕金森综合征、小脑共济失调和自主神经功能障碍的神经退行性疾病。多系统萎缩患者夜间喘鸣的出现与预期寿命缩短有关。这使得充分治疗至关重要。然而，喘鸣常常未被识别，治疗方案也常常存在不确定性。
我们报告一名58岁患有多系统萎缩和夜间喘鸣的女性。直接喉镜检查显示双侧声带麻痹和声门狭窄。由于持续气道正压通气（CPAP）治疗效果不佳，遂行气管切开术，术后喘鸣消失。
在临床问诊后，多导睡眠图和喉镜检查是诊断该病的重要工具。气管切开术曾是治疗喘鸣的唯一选择。然而，最近的研究表明，只要有正确的适应症，CPAP无创治疗具有相似的疗效。治疗方案的选择是一个多学科过程，涉及神经科医生、睡眠医学专家、耳鼻喉科医生和患者。
