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伴有感觉神经元病的纵向广泛背柱脊髓损伤。

Longitudinally extensive dorsal column spinal cord lesion with sensory ganglionopathy.

机构信息

Department of Neurology, Division of Neuromuscular Medicine, Yale School of Medicine, New Haven, CT, USA.

出版信息

Neurol Sci. 2022 Aug;43(8):5157-5159. doi: 10.1007/s10072-022-06003-0. Epub 2022 Apr 28.

Abstract

We present a case of a 23-year-old woman with a history of celiac disease who presented with a 2-month history of progressive gait unsteadiness and falls. Neurologic examination exhibited preserved motor strength, diffuse areflexia, and ataxic gait. Autoimmune and infectious workups were unremarkable, including vitamin B12. Electrodiagnostic testing showed absent diffuse sensory responses, consistent with sensory ganglionopathy. Total spine magnetic resonance imaging (MRI) revealed a non-enhancing, posterior cord, hyperintense signal from C1-T11. Partial improvement in her sensory ataxia was noted after 6 months of high-dose steroids without dorsal cord signals change on repeat MRI that suggests Wallerian degeneration of sensory axons.

摘要

我们报告一例 23 岁女性,既往有乳糜泻病史,表现为进行性步态不稳和跌倒 2 个月。神经系统检查显示运动力量正常、广泛反射消失和共济失调步态。自身免疫和感染方面的检查无明显异常,包括维生素 B12。电诊断测试显示弥漫性感觉反应缺失,符合感觉神经元病。全脊柱磁共振成像(MRI)显示 C1-T11 后索无强化、高信号。在接受高剂量类固醇治疗 6 个月后,她的感觉共济失调部分改善,而重复 MRI 未见后索信号改变,提示感觉轴突的瓦勒变性。

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