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I型Chiari畸形伴广泛脊髓空洞症表现为进行性双侧足下垂:一例报告

Chiari malformation type I with extensive syringomyelia presenting as progressive bilateral foot drop: a case report.

作者信息

Jamaludin Siti Anisah, Abdul-Razak Suraya, Bhaskar Shalini, Nashra Mimi, Nasir Nik Munirah Nik Mohd, Asri Nordini

机构信息

Department of Primary Care Medicine, Faculty of Medicine, Universiti Teknologi MARA, Kampus Sungai Buloh, Sungai Buloh, Malaysia.

Cardiovascular Advancement and Research Excellence Institute, Pusat Perubatan UiTM, Kampus Sungai Buloh, Sungai Buloh, Malaysia.

出版信息

Korean J Fam Med. 2025 Sep;46(5):369-372. doi: 10.4082/kjfm.25.0020. Epub 2025 Apr 11.

DOI:10.4082/kjfm.25.0020
PMID:40211443
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12463496/
Abstract

Chiari malformation type I (CMI) is a structural abnormality characterized by cerebellar tonsil herniation through the foramen magnum, often leading to disrupted cerebrospinal fluid dynamics and syringomyelia. While CMI commonly presents with occipital headaches and neck pain, atypical manifestations, such as bilateral foot drops, are exceedingly rare. We describe a 37-year-old female patient presenting with bilateral foot drop, which was later established to be caused by syringomyelia secondary to CMI. Magnetic resonance imaging revealed an 8 mm tonsillar descent and a syrinx extending to the conus medullaris. The patient refused surgical intervention and opted for conservative management; partial functional recovery was subsequently observed. This report emphasizes the importance of recognizing rare neurological presentations of CMI. To our knowledge, this is the first documented case of CMI with syringomyelia presenting as a bilateral foot drop.

摘要

I型Chiari畸形(CMI)是一种结构异常,其特征为小脑扁桃体经枕骨大孔疝出,常导致脑脊液动力学紊乱和脊髓空洞症。虽然CMI通常表现为枕部头痛和颈部疼痛,但非典型表现,如双侧足下垂,极为罕见。我们描述了一名37岁女性患者,表现为双侧足下垂,后来确定是由CMI继发的脊髓空洞症所致。磁共振成像显示扁桃体下移8毫米,脊髓空洞延伸至脊髓圆锥。患者拒绝手术干预,选择保守治疗;随后观察到部分功能恢复。本报告强调了认识CMI罕见神经表现的重要性。据我们所知,这是首例记录在案的CMI合并脊髓空洞症表现为双侧足下垂的病例。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4d59/12463496/e3ec61b554f5/kjfm-25-0020f3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4d59/12463496/76525f007bb4/kjfm-25-0020f1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4d59/12463496/db71ecf15ea1/kjfm-25-0020f2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4d59/12463496/e3ec61b554f5/kjfm-25-0020f3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4d59/12463496/76525f007bb4/kjfm-25-0020f1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4d59/12463496/db71ecf15ea1/kjfm-25-0020f2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4d59/12463496/e3ec61b554f5/kjfm-25-0020f3.jpg

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本文引用的文献

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Multicenter comparison of Chiari malformation type I presentation in children versus adults.儿童与成人I型Chiari畸形表现的多中心比较。
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Two differential cavities in syringomyelia of pediatric Chiari I malformation presenting with unilateral foot drop.儿童 Chiari I 畸形伴发脊髓空洞症中的两处差异腔,表现为单侧足下垂。
Brain Dev. 2023 Mar;45(3):191-195. doi: 10.1016/j.braindev.2022.11.007. Epub 2022 Dec 16.
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脊髓空洞症和 Chiari 综合征登记处:基于意大利西北部队列的流行病学、临床表型和自然史的进展。
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