Department of Radiology, Wayne State University-John D. Dingell VA Medical Center, 4646 John R Street, Detroit, MI, 48201, USA.
Department of Radiology, Wayne State University-Detroit Medical Center, Detroit, USA.
Neuroradiology. 2024 Sep;66(9):1577-1580. doi: 10.1007/s00234-024-03408-w. Epub 2024 Jun 24.
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.
导水管狭窄综合征是一种罕见的进行性神经退行性疾病，描述了一组独特的神经眼科、神经影像学和尸检组织病理学发现。影像学和组织病理学可见中脑中线裂隙与导水管相通，类似于脊髓空洞症。文献中已有 9 例报道。我们遇到了一位患者，表现为垂直性眼球震颤、核间眼肌麻痹和进行性共济失调，其导水管与脚间池之间有中线裂隙，突出了该综合征的一个显著特征。
