Campisi Raffaele, Ciancio Nicola, Bivona Laura, Di Maria Annalisa, Maria Giuseppe Di
Pneumology Unit Policlinico "G, Rodolico", University of Catania, Via Santa Sofia 78, 95123, Catania, Italy.
Multidiscip Respir Med. 2013 Feb 22;8(1):15. doi: 10.1186/2049-6958-8-15.
Arnold Chiari Malformation (ACM) is defined as a condition where part of the cerebellar tissue herniates into the cervical canal toward the medulla and spinal cord resulting in a number of clinical manifestations. Type I ACM consists of variable displacement of the medulla throughout the formamen magnum into the cervical canal, with prominent cerebellar herniation.Type I ACM is characterized by symptoms related to the compression of craniovertebral junction, including ataxia, dysphagia, nistagmus, headache, dizziness, and sleep disordered breathing. We report a case of a life-long non-smoker, 54 years old woman who presented these symptoms associated with bronchiectasis secondary to recurrent inhalation pneumonia, hypercapnic respiratory failure, and central sleep apnea (CSA).CSA was first unsuccessfully treated with nocturnal c-PAP. The subsequent treatment with low flow oxygen led to breathing pattern stabilization with resolution of CSA and related clinical symptoms during sleep. We suggest that in patients with type I ACM the presence of pulmonary manifestations aggravating other respiratory disturbances including sleep disordered breathing (SDB) should be actively investigated. The early diagnosis is desirable in order to avoid serious and/or poorly reversible damages.
阿诺德-奇阿里畸形（ACM）被定义为一种小脑组织的一部分向延髓和脊髓方向疝入颈椎管的病症，会导致多种临床表现。I型ACM包括延髓在整个枕骨大孔处向颈椎管的不同程度移位，伴有明显的小脑疝。I型ACM的特征是与颅颈交界处受压相关的症状，包括共济失调、吞咽困难、眼球震颤、头痛、头晕和睡眠呼吸紊乱。我们报告了一例54岁终身不吸烟女性的病例，她出现了这些症状，伴有反复吸入性肺炎继发的支气管扩张、高碳酸血症呼吸衰竭和中枢性睡眠呼吸暂停（CSA）。CSA最初采用夜间持续气道正压通气（c-PAP）治疗未成功。随后采用低流量吸氧治疗，使呼吸模式稳定，CSA及相关睡眠期间的临床症状得到缓解。我们建议，对于I型ACM患者，应积极调查是否存在加重包括睡眠呼吸紊乱（SDB）在内的其他呼吸障碍的肺部表现。早期诊断是可取的，以避免严重和/或难以逆转的损害。
