We present a 78-year-old lady with a clinical and radiological diagnosis of anterior opercular syndrome or Foix-Chavany-Marie syndrome (FCMS) and an autopsy diagnosis of type E TDP43 pathology. The neurodegenerative form of FCMS presents with articulation difficulty, dysprosody, orofacial and speech apraxia and can mimic but is distinct from primary progressive aphasia (PPA) (1, 2). It was suggested that the progressive form of FCMS should be included with frontotemporal lobar degenerations (FTLD) (3). However, its neuropathological substrates are poorly understood. This case reveals a rare pattern of TDP43 pathology as a neurodegenerative cause of FCMS. This article is protected by copyright. All rights reserved.

中文翻译：

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由 E 型 TDP43 病理引起的 Foix-Chavany-Marie 综合征

我们介绍了一位 78 岁的女士，其临床和放射学诊断为前鳃盖综合征或 Foix-Chavany-Marie 综合征 (FCMS)，尸检诊断为 E 型 TDP43 病理。FCMS 的神经退行性形式表现为发音困难、发音障碍、口面部和言语失用，并且可以模仿但不同于原发性进行性失语症 (PPA) (1, 2)。有人建议 FCMS 的渐进形式应包括额颞叶变性 (FTLD) (3)。然而，对其神经病理学基础知之甚少。该病例揭示了 TDP43 病理学的罕见模式，作为 FCMS 的神经退行性原因。本文受版权保护。版权所有。