{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2024,9,5]],"date-time":"2024-09-05T10:02:55Z","timestamp":1725530575769},"reference-count":48,"publisher":"Cambridge University Press (CUP)","issue":"2","license":[{"start":{"date-parts":[[2015,9,18]],"date-time":"2015-09-18T00:00:00Z","timestamp":1442534400000},"content-version":"unspecified","delay-in-days":12193,"URL":"https:\/\/www.cambridge.org\/core\/terms"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Can. j. neurol. sci."],"published-print":{"date-parts":[[1982,5]]},"abstract":"<jats:title>ABSTRACT:<\/jats:title><jats:p>We present a working classification of recessively inherited ataxic syndromes based on the use of simple tools available to every clinician: a good history (particularly pinpointing the age of onset) and a good neurological examination (simplified to the verification of the presence of ataxia, deep tendon reflexes in the knee, optic nerve, retinal and\/or 8th nerve signs). In the three groups of disorders (non progressive, intermittent or progressive) patients can be hyper\/normo reflexic, or they can be hypolareflexic. Six principal types of progressive ataxic disorders are further delineated by the age of onset. Sub-types depend on the presence or absence of eye and ear signs, whereas eponymic or regional denominations are used only for simplicity while awaiting exact delineation of the biochemical defects.<\/jats:p>","DOI":"10.1017\/s0317167100043766","type":"journal-article","created":{"date-parts":[[2016,7,28]],"date-time":"2016-07-28T11:34:31Z","timestamp":1469705671000},"page":"95-98","source":"Crossref","is-referenced-by-count":11,"title":["A Tentative Classification of Recessively Inherited Ataxias"],"prefix":"10.1017","volume":"9","author":[{"given":"Andr\u00e9","family":"Barbeau","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"56","published-online":{"date-parts":[[2015,9,18]]},"reference":[{"key":"S0317167100043766_ref194","doi-asserted-by":"publisher","DOI":"10.1111\/j.1651-2227.1969.tb04729.x"},{"key":"S0317167100043766_ref193","volume-title":"The spinocerebellar degenerations","author":"Greenfield","year":"1954"},{"key":"S0317167100043766_ref214","first-page":"573","article-title":"Metabolic ataxias","volume":"2","author":"Salam","year":"1975","journal-title":"Handbook of Clinical Neurology"},{"key":"S0317167100043766_ref186","doi-asserted-by":"publisher","DOI":"10.1111\/j.1600-0404.1977.tb05638.x"},{"key":"S0317167100043766_ref185","doi-asserted-by":"publisher","DOI":"10.1017\/S0317167100119651"},{"key":"S0317167100043766_ref184","doi-asserted-by":"crossref","first-page":"61","DOI":"10.1017\/S0317167100024793","article-title":"Autosomal recessive spastic ataxia of Charlevoix-Saguenay","volume":"5","author":"Bouchard","year":"1978","journal-title":"Can. 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