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卒中患者中福瓦-沙瓦尼-玛丽综合征(FCMS)的非典型表现

Atypical Presentations of Foix-Chavany-Marie Syndrome (FCMS) in Stroke.

作者信息

Sanker Vivek, Srinivasan Aariya, Emara Mohamed, Jagannath Preethi, Mathew Robert

机构信息

General Surgery, Noorul Islam Institute of Medical Science (NIMS), Trivandrum, IND.

Internal Medicine, Saveetha Medical College and Hospital, Tamil Nadu, IND.

出版信息

Cureus. 2023 Apr 23;15(4):e38030. doi: 10.7759/cureus.38030. eCollection 2023 Apr.

Abstract

Foix-Chavany-Marie syndrome (FCMS) presents with anarthria and bilateral (B/L) central facio-linguo-velo-pharyngo-masticatory paralysis with "autonomic voluntary dissociation." The most common cause of FCMS is cerebrovascular disease, while rarer causes include central nervous system infection, developmental disorders, epilepsy, and neurodegenerative disorders. Even though this syndrome is also referred to as (B/L) anterior operculum syndrome, patients with lesion in sites other than (B/L) opercular regions also can develop the syndrome. In this article we describe two such atypical cases. : A 66-year-old man with diabetes and hypertension who is a smoker had right-sided hemiplegia one year back developed the syndrome acutely two days before admission. CT brain showed left perisylvian infarct and right internal capsule anterior limb infarct. : A 48-year-old gentleman, who is a diabetic and hypertensive had right-sided hemiplegia one year back and developed the syndrome acutely two days before admission. CT brain showed (B/L) infarcts in the posterior limb of the internal capsule. Both patients had bifacial, lingual, and pharyngolaryngeal palsy thereby confirming the diagnosis of FCMS. None of them had the classical (B/L) opercular lesions on imaging and one patient did not even have a unilateral opercular lesion. Contrary to the common teaching, (B/L) opercular lesions are not always necessary to produce FCMS and can occur even without opercular lesions at all.

摘要

福瓦-沙瓦尼-玛丽综合征(FCMS)表现为构音障碍以及双侧中枢性面-舌-腭-咽-咀嚼肌麻痹伴“自主-随意分离”。FCMS最常见的病因是脑血管疾病,而罕见病因包括中枢神经系统感染、发育障碍、癫痫和神经退行性疾病。尽管该综合征也被称为双侧岛盖综合征,但双侧岛盖区域以外部位有病变的患者也可能发生该综合征。在本文中,我们描述了两例此类非典型病例。:一名66岁男性,患有糖尿病和高血压,有吸烟史,一年前出现右侧偏瘫,入院前两天急性发生该综合征。脑部CT显示左侧外侧裂周梗死和右侧内囊前肢梗死。:一名48岁男性,患有糖尿病和高血压,一年前出现右侧偏瘫,入院前两天急性发生该综合征。脑部CT显示双侧内囊后肢梗死。两名患者均有双侧面部、舌部和咽喉部麻痹,从而确诊为FCMS。他们在影像学上均无典型的双侧岛盖病变,其中一名患者甚至没有单侧岛盖病变。与通常的观点相反,双侧岛盖病变并非产生FCMS所必需,甚至在完全没有岛盖病变的情况下也可能发生。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0982/10205966/6b09ae7bce0e/cureus-0015-00000038030-i01.jpg

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