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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 histopa...

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Bibliographic Details
Published in:Neuroradiology 2024-09, Vol.66 (9), p.1577-1580
Main Authors: Zak, Imad, Hadidchi, Shahram, Ross, Philip
Format: Article
Language:English
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Summary: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.
ISSN:0028-3940
1432-1920
1432-1920
DOI:10.1007/s00234-024-03408-w