{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,22]],"date-time":"2026-07-22T04:14:27Z","timestamp":1784693667700,"version":"3.55.0"},"reference-count":41,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2011,9,23]],"date-time":"2011-09-23T00:00:00Z","timestamp":1316736000000},"content-version":"vor","delay-in-days":22,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Annals of the New York Academy of Sciences"],"published-print":{"date-parts":[[2011,9]]},"abstract":"<jats:p>The association of bilateral vestibulopathy with cerebellar ataxia was first reported in 1991 and delineated as a distinct syndrome with a characteristic and measurable clinical sign\u2014an absent visually enhanced vestibulo\u2010ocular reflex\u2014in 2004. We reviewed 27 patients with this syndrome and show that a non\u2010length\u2010dependent sensory deficit with absent sensory nerve action potentials is an integral component of this syndrome, which we now call \u201ccerebellar ataxia with neuropathy and bilateral vestibular areflexia syndrome\u201d (CANVAS). All patients had brain MRI and 22\/27 had evidence of cerebellar atrophy involving anterior and dorsal vermis, as well as the hemispheric crus I. Brain and temporal bone pathology in one patient showed marked loss of Purkinje cells and of vestibular, trigeminal, and facial ganglion cells, but not of spiral ganglion cells. There are two sets of sibling pairs, suggesting CANVAS is a late\u2010onset recessive disorder. The characteristic clinical sign\u2014the visual vestibulo\u2010ocular reflex deficit\u2014can be demonstrated and measured clinically using video\u2010oculography.<\/jats:p>","DOI":"10.1111\/j.1749-6632.2011.06158.x","type":"journal-article","created":{"date-parts":[[2011,9,27]],"date-time":"2011-09-27T19:47:30Z","timestamp":1317152850000},"page":"139-147","source":"Crossref","is-referenced-by-count":146,"title":["Cerebellar ataxia, neuropathy, vestibular areflexia syndrome (CANVAS): a review of the clinical features and video\u2010oculographic diagnosis"],"prefix":"10.1111","volume":"1233","author":[{"given":"David J.","family":"Szmulewicz","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John A.","family":"Waterston","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hamish G.","family":"MacDougall","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stuart","family":"Mossman","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrew M.","family":"Chancellor","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Catriona A.","family":"McLean","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Saumil","family":"Merchant","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peter","family":"Patrikios","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"G. 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