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The dogs appear normal during the first 6 months of life, but betwen 6 and 12 months of age they develop a broad\u2010based gait and dysmetria, followed by truncal ataxia. Nystagmus occurs late. The disorder is slowly and insidiously progressive. Neuropathological examination shows atrophy of the cerebellar cortex, particularly the pars in termedia. There is loss of Purkinje and granule cells, more severe in older dogs. Other parts of the neuraxis appear normal. These pathological changes resemble those of the familial human cerebellar cortical atrophies.<\/jats:p>","DOI":"10.1002\/ana.410090512","type":"journal-article","created":{"date-parts":[[2005,1,1]],"date-time":"2005-01-01T13:25:41Z","timestamp":1104585941000},"page":"492-498","source":"Crossref","is-referenced-by-count":20,"title":["Canine inherited ataxia"],"prefix":"10.1002","volume":"9","author":[{"given":"Linda C.","family":"Cork","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Juan C.","family":"Troncoso","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Donald L.","family":"Price","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2004,10,7]]},"reference":[{"key":"e_1_2_1_2_2","doi-asserted-by":"publisher","DOI":"10.1176\/ajp.94.5.1115"},{"key":"e_1_2_1_3_2","first-page":"239","article-title":"The mammalinan cerebellum and its contribution to movement control","volume":"7","author":"Armstrong DM","year":"1978","journal-title":"Int Rev Physiol"},{"key":"e_1_2_1_4_2","first-page":"203","article-title":"Pyruvate dehydrogenase complex in Friedreich's ataxia","volume":"21","author":"Barbeau A","year":"1978","journal-title":"Adv Neurol"},{"key":"e_1_2_1_5_2","first-page":"10","article-title":"Genetic approaches to the nosology of nervous system defects","volume":"7","author":"Becker PE","year":"1971","journal-title":"Birth Defects"},{"key":"e_1_2_1_6_2","first-page":"1","article-title":"Ueber eine ungew\u00f6hnliche Form von cerebellaren Heredo\u2010ataxie","volume":"51","author":"Bielschowsky M","year":"1934","journal-title":"Jahrb Psychiatr Neurol"},{"key":"e_1_2_1_7_2","first-page":"181","article-title":"The Inherited Ataxias","volume":"21","author":"Blass JP","year":"1978","journal-title":"Adv Neurol"},{"key":"e_1_2_1_8_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM197607082950202"},{"key":"e_1_2_1_9_2","article-title":"Huntington's Disease","volume":"23","author":"Chase TN","year":"1979","journal-title":"Adv Neurol"},{"key":"e_1_2_1_10_2","first-page":"1119","article-title":"Hereditary cerebellar cortical and extrapyramidal nuclear abiotrophy in Kerry blue terriers","volume":"168","author":"De Lahunta A","year":"1976","journal-title":"J Am Vet Med Assoc"},{"key":"e_1_2_1_11_2","doi-asserted-by":"publisher","DOI":"10.1016\/0006-8993(74)90627-1"},{"key":"e_1_2_1_12_2","first-page":"403","volume-title":"Handbook of Clinical Neurology, Vol 21, Systems Disorders and Atrophies, Part I","author":"Eadie MJ","year":"1975"},{"key":"e_1_2_1_13_2","doi-asserted-by":"publisher","DOI":"10.1007\/978-3-662-13147-3"},{"key":"e_1_2_1_14_2","first-page":"319","article-title":"Uber degenerative Atrophie die spinalen Hinterstr\u00e4nge","volume":"36","author":"Friedreich N","year":"1863","journal-title":"Arch Pathol Anat"},{"key":"e_1_2_1_15_2","first-page":"1003","article-title":"Abiotrophy","volume":"1","author":"Gowers WR","year":"1902","journal-title":"Lancet"},{"key":"e_1_2_1_16_2","volume-title":"The Spino\u2010cerebellar Degenerations","author":"Greenfeld JG","year":"1954"},{"key":"e_1_2_1_17_2","doi-asserted-by":"publisher","DOI":"10.5694\/j.1326-5377.1945.tb54494.x"},{"key":"e_1_2_1_18_2","doi-asserted-by":"publisher","DOI":"10.1007\/BF00235446"},{"key":"e_1_2_1_19_2","unstructured":"HerndonRM: Selective vulnerability in the cerebellum. 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