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急性呼吸衰竭作为与脊髓空洞症相关的阿诺德-奇亚里畸形的首发症状。

Acute respiratory failure as the first sign of Arnold-Chiari malformation associated with syringomyelia.

作者信息

Alvarez D, Requena I, Arias M, Valdés L, Pereiro I, De la Torre R

机构信息

Service of Pneumology, Hospital Provincial, Santiago de Compostela, Spain.

出版信息

Eur Respir J. 1995 Apr;8(4):661-3.

PMID:7664871
Abstract

We report a rare case of acute respiratory failure in a previously asymptomatic patient showing clinical signs of inferior cranial nerve palsy together with weakness and muscular atrophy of the upper limbs. Magnetic resonance imaging revealed Arnold-Chiari malformation associated with platybasia, basilar impression, syringomyelia and Klippel-Feil syndrome. Episodes of apnoea required tracheostomy and recurred upon tentative closure of the tracheostome, but remitted upon decompression of the posterior fossa. This case involved both obstructive mechanisms and dysfunction of the respiratory centre. Patients with respiratory failure not explained by pulmonary pathology should be checked for underlying neurological disease.

摘要

我们报告了一例罕见的急性呼吸衰竭病例,该患者此前无症状,表现出颅神经麻痹的临床体征,同时伴有上肢无力和肌肉萎缩。磁共振成像显示阿诺德-奇阿利畸形合并扁平颅底、基底凹陷、脊髓空洞症和克利佩尔-费尔综合征。呼吸暂停发作需要进行气管切开术,在尝试关闭气管造口时复发,但在后颅窝减压后缓解。该病例涉及阻塞机制和呼吸中枢功能障碍。对于肺部病理无法解释的呼吸衰竭患者,应检查是否存在潜在的神经系统疾病。

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