一例慢性脱髓鞘性周围神经病中的亚临床面神经增粗

文献信息

中文摘要

我们报告一例来自约翰斯·霍普金斯Otopathology Archives的颞骨档案病例，患者为一名男性，表现为进行性远端为主的无力、感觉丧失、反射消失和双侧高弓足，其临床病理特征提示慢性脱髓鞘性周围神经病。双侧颞骨组织病理学显示面神经在其颞内全程弥漫性增粗，并伴有舌咽神经和迷走神经增粗。本病例提示，在某些遗传性多神经病中，显著颅神经肥大可能在临床上不明显，并支持未来在确诊病例中将颞骨病理与影像学和基因型进行关联。

英文摘要

We report an archival temporal bone case from the Otopathology Archives at Johns Hopkins of a man with progressive distal-predominant weakness, sensory loss, areflexia, and bilateral pes cavus, with clinicopathologic features suggestive of a chronic demyelinating peripheral neuropathy. Bilateral temporal bone histopathology demonstrated diffuse enlargement of the facial nerve throughout its intratemporal course, with additional enlargement of the glossopharyngeal and vagus nerves. This case suggests that marked cranial nerve hypertrophy may be clinically unapparent in some hereditary polyneuropathies and supports future correlation of temporal bone pathology with imaging and genotype in confirmed cases.