{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,5]],"date-time":"2026-10-05T15:40:44Z","timestamp":1791214844929,"version":"4.1.0"},"reference-count":20,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2014,2,12]],"date-time":"2014-02-12T00:00:00Z","timestamp":1392163200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Euro J of Neurology"],"published-print":{"date-parts":[[2014,4]]},"abstract":"<jats:sec>\n                    <jats:title>Background and purpose<\/jats:title>\n                    <jats:p>\n                      To assess, through systematic review, distinctive or common clinical signs of autosomal dominant cerebellar ataxias (\n                      <jats:styled-content style=\"fixed-case\">ADCA<\/jats:styled-content>\n                      s), also referred to as spinocerebellar ataxias (\n                      <jats:styled-content style=\"fixed-case\">SCA<\/jats:styled-content>\n                      s) in genetic nomenclature.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>\n                      This was a structured search of electronic databases up to September 2012 conducted by two independent reviewers. Publications containing proportions or descriptions of\n                      <jats:styled-content style=\"fixed-case\">ADCA<\/jats:styled-content>\n                      clinical features written in several languages were selected. Gray literature was included and a back\u2010search was conducted of retrieved publication reference lists. Initial selection was based on title and abstract screening, followed by full\u2010text reading of potentially relevant publications. Clinical findings and demographic data from genetically confirmed patients were extracted. Data were analyzed using the chi\u2010squared test and controlled for alpha\u2010error inflation by applying the\n                      <jats:styled-content style=\"fixed-case\">H<\/jats:styled-content>\n                      olms step\u2010down procedure.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      In all, 1062 publications reviewing 12\u00a0141 patients (52% male) from 30\n                      <jats:styled-content style=\"fixed-case\">SCA<\/jats:styled-content>\n                      s were analyzed. Mean age at onset was 35\u00a0\u00b1\u00a011\u00a0years. Onset symptoms in 3945 patients revealed gait ataxia as the most frequent sign (68%), whereas overall non\u2010ataxia symptom frequency was 50%. Some\n                      <jats:styled-content style=\"fixed-case\">ADCA<\/jats:styled-content>\n                      s often presented non\u2010ataxia symptoms at onset, such as\n                      <jats:styled-content style=\"fixed-case\">SCA<\/jats:styled-content>\n                      7 (visual impairment),\n                      <jats:styled-content style=\"fixed-case\">SCA<\/jats:styled-content>\n                      14 (myoclonus) and\n                      <jats:styled-content style=\"fixed-case\">SCA<\/jats:styled-content>\n                      17 (parkinsonism). Therefore a categorization into two groups was established: pure ataxia and mainly non\u2010ataxia forms. During overall disease course, dysarthria (90%) and saccadic eye movement alterations (69%) were the most prevalent non\u2010ataxia findings. Some\n                      <jats:styled-content style=\"fixed-case\">ADCA<\/jats:styled-content>\n                      s were clinically restricted to cerebellar dysfunction, whilst others presented additional features.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>\n                      Autosomal dominant cerebellar ataxias encompass a broad spectrum of clinical features with high prevalence of non\u2010ataxia symptoms. Certain features distinguish different genetic subtypes. A new algorithm for\n                      <jats:styled-content style=\"fixed-case\">ADCA<\/jats:styled-content>\n                      classification at disease onset is proposed.\n                    <\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/ene.12350","type":"journal-article","created":{"date-parts":[[2014,2,12]],"date-time":"2014-02-12T06:48:28Z","timestamp":1392187708000},"page":"607-615","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":122,"title":["Autosomal dominant cerebellar ataxias: a systematic review of clinical features"],"prefix":"10.1111","volume":"21","author":[{"given":"M.","family":"Rossi","sequence":"first","affiliation":[{"name":"Movement Disorders Section Department of Neuroscience Raul Carrea Institute for Neurological Research (FLENI) Buenos Aires 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