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Published on: October 12, 2017
Keyhole Aqueduct Syndrome
Imad Zak1, Shahram Hadidchi2, Philip Ross3
1Department of Radiology, Wayne State University-John D. Dingell VA Medical Center, 4646 John R Street, Detroit, MI, 48201, USA. izak@wayne.edu.
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.
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