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[福-恰-马综合征的异质性地形学]

[Heterogeneous topography of Foix-Chavany-Marie syndrome].

作者信息

Moragas Garrido M, Cardona Portela P, Martínez-Yélamos S, Rubio Borrego F

机构信息

Servicio de Neurología, IDIBELL, Hospital Universitario de Bellvitge, L'Hospitalet de Llobregat (Barcelona).

出版信息

Neurologia. 2007 Jun;22(5):333-6.

Abstract

INTRODUCTION

Bilateral facio-pharyngo-laryngo-glosso-masticatory palsy with automatic-voluntary dissociation is known as Foix-Chavany-Marie (FCM) syndrome. It is usually due to bilateral cortical lesions involving both anterior opercula (biopercular syndrome). We describe three patients with FCM syndrome associated with ischemic lesions in two atypical localizations: a) bilateral subcortical infarcts, and b) unilateral opercular infarct.

CASES REPORT

Patient 1, a 66 year old male, was admitted for a sudden onset of anarthria and facial, lingual and masticatory paralysis. Neurological examination revealed automatic-voluntary dissociation of facial motility. MRI showed an acute left subcortical infarct and multiple bilateral subcortical ischemic lesions. Patient 2, a 61 year old male, also suffered a sudden onset of anarthria, with bilateral facial and lingual paralysis and automatic-voluntary dissociation together with sudden onset swallowing alteration. MRI showed a single ischemic lesion involving the left operculum. Patient 3, a 36 year old male, presented sudden onset of dysarthria, dysphagia and bilateral facial palsy with automatic-voluntary dissociation and loss of force in left upper limb. MRI showed an acute right opercular infarct and no contralateral lesions.

CONCLUSIONS

FCM syndrome is not only due to bilateral opercular lesions but can also be seen in bilateral subcortical and even unilateral opercular lesions.

摘要

引言

伴有自主 - 随意分离的双侧面 - 咽 - 喉 - 舌 - 咀嚼肌麻痹被称为福瓦 - 沙瓦尼 - 玛丽(FCM)综合征。它通常是由于双侧皮质病变累及双侧岛盖前部(双侧岛盖综合征)。我们描述了三例FCM综合征患者,其与两个非典型部位的缺血性病变相关:a)双侧皮质下梗死,以及b)单侧岛盖梗死。

病例报告

患者1,一名66岁男性,因突然出现构音障碍、面部、舌部和咀嚼肌麻痹入院。神经系统检查发现面部运动存在自主 - 随意分离。MRI显示急性左侧皮质下梗死以及多个双侧皮质下缺血性病变。患者2,一名61岁男性,也突然出现构音障碍,伴有双侧面部和舌部麻痹、自主 - 随意分离以及突然出现的吞咽改变。MRI显示单个缺血性病变累及左侧岛盖。患者3,一名36岁男性,突然出现构音障碍、吞咽困难和双侧面瘫,伴有自主 - 随意分离以及左上肢无力。MRI显示急性右侧岛盖梗死且对侧无病变。

结论

FCM综合征不仅由双侧岛盖病变引起,也可见于双侧皮质下甚至单侧岛盖病变。

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