de Mello Ramon Andrade Bezerra, Ferreira Diana, Dias da Costa José Manuel, Rosas Maria José, Quinaz João Manuel
Department of Internal Medicine, Hospital São João, EPE, 4200-319 Porto, Portugal.
Case Rep Med. 2010;2010. doi: 10.1155/2010/351239. Epub 2010 Sep 1.
Background. MSA (Multiple System Atrophy) may be associated either with Parkinsonism or with cerebellar ataxia (MSA-c subtype). It is considered a rare disease, but many patients are misdiagnosed as suffering from idiopathic Parkinson's disease. In this paper, we report a case of a patient admitted with respiratory failure and vocal cords paralysis due to MSA-c. Case Report. A 79-year-old Caucasian woman was admitted in March 2010 with dyspnea, asthenia, stridor, and respiratory failure needing noninvasive ventilation. She had orthostatic blood pressure decline, constipation, insomnia, daytime sleepiness, and snoring. The neurologic examination revealed cerebellar ataxia. A laryngoscopy revealed vocal cord paralysis in midline position and tracheostomy was performed. The Brain Magnetic Resonance Imaging revealed atrophy of middle cerebellar peduncles and pons with the "hot cross bun sign." Conclusion. Although Multiple-system atrophy is a rare disease, unexplained respiratory failure, bilateral vocal cord paralysis, or stridor should lead to consider MSA as diagnosis.
背景。多系统萎缩(MSA)可能与帕金森综合征或小脑共济失调(MSA-c亚型)相关。它被认为是一种罕见疾病,但许多患者被误诊为特发性帕金森病。在本文中,我们报告了一例因MSA-c导致呼吸衰竭和声带麻痹而入院的患者。病例报告。一名79岁的白种女性于2010年3月因呼吸困难、乏力、喘鸣和呼吸衰竭需要无创通气而入院。她有体位性血压下降、便秘、失眠、日间嗜睡和打鼾症状。神经系统检查发现小脑共济失调。喉镜检查显示声带麻痹处于中线位置,并进行了气管切开术。脑部磁共振成像显示小脑中间脚和脑桥萎缩伴有“热十字面包征”。结论。尽管多系统萎缩是一种罕见疾病,但不明原因的呼吸衰竭、双侧声带麻痹或喘鸣应考虑将MSA作为诊断。