锁孔导水管综合症

IF 2.4 3区 医学 Q2 CLINICAL NEUROLOGY Neuroradiology Pub Date : 2024-09-01 Epub Date: 2024-06-24 DOI:10.1007/s00234-024-03408-w
Imad Zak, Shahram Hadidchi, Philip Ross
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引用次数: 0

摘要

锁孔导水管综合征是一种罕见的进行性神经退行性疾病,尸检时会出现一系列独特的神经眼科、神经影像学和组织病理学发现。在影像学和组织病理学上,可以看到中线间脑裂隙与大脑导水管相通,类似鞘膜积液。文献中已发表了 9 个病例。我们遇到了一名患有垂直性眼球震颤、核间性眼肌麻痹和进行性共济失调的患者,他的中线裂隙连接着大脑导水管和小脑间水囊,这突显了该综合征的显著特征。
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Keyhole Aqueduct Syndrome.

Keyhole aqueduct syndrome is a rare progressive neurodegenerative disorder describing a unique set of neuro-ophthalmologic, neuroimaging, and histopathological findings on autopsy. A midline mesencephalic cleft communicating with the cerebral aqueduct resembling syrinx is seen on imaging and histopathology. There are 9 cases published in the literature. We encountered a patient with vertical nystagmus, internuclear ophthalmoplegia, and progressive ataxia who has a midline cleft connecting the cerebral aqueduct with the interpeduncular cistern highlighting a distinguishing feature of this syndrome.

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来源期刊
Neuroradiology
Neuroradiology 医学-核医学
CiteScore
5.30
自引率
3.60%
发文量
214
审稿时长
4-8 weeks
期刊介绍: Neuroradiology aims to provide state-of-the-art medical and scientific information in the fields of Neuroradiology, Neurosciences, Neurology, Psychiatry, Neurosurgery, and related medical specialities. Neuroradiology as the official Journal of the European Society of Neuroradiology receives submissions from all parts of the world and publishes peer-reviewed original research, comprehensive reviews, educational papers, opinion papers, and short reports on exceptional clinical observations and new technical developments in the field of Neuroimaging and Neurointervention. The journal has subsections for Diagnostic and Interventional Neuroradiology, Advanced Neuroimaging, Paediatric Neuroradiology, Head-Neck-ENT Radiology, Spine Neuroradiology, and for submissions from Japan. Neuroradiology aims to provide new knowledge about and insights into the function and pathology of the human nervous system that may help to better diagnose and treat nervous system diseases. Neuroradiology is a member of the Committee on Publication Ethics (COPE) and follows the COPE core practices. Neuroradiology prefers articles that are free of bias, self-critical regarding limitations, transparent and clear in describing study participants, methods, and statistics, and short in presenting results. Before peer-review all submissions are automatically checked by iThenticate to assess for potential overlap in prior publication.
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