Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2024 Sep;66(9):1577-1580.
doi: 10.1007/s00234-024-03408-w. Epub 2024 Jun 24.

Keyhole Aqueduct Syndrome

Imad Zak  1 , Shahram Hadidchi  2 , Philip Ross  3
Affiliations
Case Reports

Keyhole Aqueduct Syndrome

Imad Zak et al. Neuroradiology. 2024 Sep.

Abstract

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.

Keywords: Aqueduct; Keyhole; Syringomesencephalia.

PubMed Disclaimer

References

    1. Oh AJ, Lanzman BA, Liao YJ (2018) Vertical diplopia and oscillopsia due to midbrain keyhole aqueduct syndrome associated with severe cough. Am J Ophthalmol Case Rep 10:128–131 - DOI - PubMed - PMC
    1. Ahmad O, Reddel S, Lueck CJ (2010) Midbrain cleft as a cause of chronic internuclearophthalmoplegia, progressive ataxia, and facial weakness. J Neuro Ophthalmol 30:145–149 - DOI
    1. Burgett RA, Kawasaki A (1997) Mesencephalic clefts and eye movement disorders. Arch Ophthalmol 115:824 - DOI - PubMed
    1. Lagreze WD, Warner JE, Zamani AA, Gouras GK, Koralnik IJ, Bienfang DC (1996) Mesencephalic clefts with associated eye movement disorders. Arch Ophthalmol 114:429–432 - DOI - PubMed
    1. de la Monte SM, Horowitz SA, Larocque AA, Richardson EP Jr (1986) Keyhole aqueduct syndrome. Arch Neurol 43:926–929 - DOI - PubMed

Publication types

MeSH terms

LinkOut - more resources