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首页> 外文期刊>European journal of paediatric neurology: EJPN : official journal of the European Paediatric Neurology Society >Acute VI nerve palsy in a 4 year-old girl with Chiari I malformation and pontomedullary extension of syringomyelia: case report and review of the literature.
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Acute VI nerve palsy in a 4 year-old girl with Chiari I malformation and pontomedullary extension of syringomyelia: case report and review of the literature.

机译：Chiari I畸形和脊髓空洞延髓延髓的4岁女孩的急性VI神经麻痹：病例报告和文献复习。

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摘要

We report the case of a previously healthy 4 year-old African American female who presented to the emergency department with acute onset of unilateral abducens nerve palsy and torticollis. Within 12 h of presentation, the patient's symptoms progressed to include ipsilateral facial nerve palsy and gait ataxia. On exam, the patient demonstrated right cranial nerve VI and VII palsies, ataxic gait with left lateropulsion, spasticity of bilateral lower extremities with clonus, and the presence of bilateral Babinski sign. MRI of the brain and spinal cord revealed severe Chiari I malformation with associated extensive holochord syringomyelia and syringobulbia. The patient underwent successful surgical decompression 72 h after initial presentation. We review the literature on Chiari malformations and syringomyelia, including epidemiology, presentation and neurological manifestations, and treatment recommendations. As our patient had a very acute presentation, we additionally review the previously reported cases of acute and atypical presentation of patients with Chiari I malformation and syringomyelia. The aim of this report is to make practitioners aware of the acuteness with which children with Chiari malformation type I with syringomyelia and syringobulbia can present.
机译：我们报告了一个以前健康的4岁非裔美国女性的病例，该女性因单侧外展神经麻痹和斜颈急性发作而出现在急诊科。在就诊后的12小时内，患者的症状发展为包括同侧面神经麻痹和步态共济失调。在检查中，患者表现出右颅神经VI和VII麻痹，共济失调步态伴左近搏，双侧下肢痉挛性痉挛以及双侧Babinski征象。脑和脊髓的MRI显示严重的Chiari I畸形，并伴有广泛的holochord脊髓空洞症和syringobulbia。初次就诊后72小时，患者接受了成功的手术减压。我们回顾了有关Chiari畸形和脊髓空洞症的文献，包括流行病学，表现和神经系统表现以及治疗建议。由于我们的患者表现非常急性，因此我们还回顾了先前报道的Chiari I畸形和脊髓空洞症患者的急性和非典型表现。这份报告的目的是使从业者意识到患有脊髓空洞症和脊髓空洞症的I型Chiari畸形儿童所表现出的急性性。

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