{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,13]],"date-time":"2026-03-13T10:07:42Z","timestamp":1773396462271,"version":"3.50.1"},"reference-count":18,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2004,10,12]],"date-time":"2004-10-12T00:00:00Z","timestamp":1097539200000},"content-version":"vor","delay-in-days":4668,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Movement Disorders"],"published-print":{"date-parts":[[1992,1]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Experience is described in 25 patients from southern New England with Machado\u2010Joseph Disease, examined serially at annual screening clinics. The disorder is dominantly inherited, with a wide range of phenotypic variation. Core clinical features described include ataxia, nystagmus, dysarthria, facial fasciculations, and lid retraction, producing a characteristic staring expression. In addition, young onset patients have spasticity, extrapyramidal rigidity, and dystonic manifestations. Late onset patients often have distal atrophy and sensory loss. Postural instability is often an early feature. We discuss the distinction of this entity from the olivopontocerebellar atrophies.<\/jats:p>","DOI":"10.1002\/mds.870070303","type":"journal-article","created":{"date-parts":[[2005,1,1]],"date-time":"2005-01-01T07:56:07Z","timestamp":1104566167000},"page":"204-208","source":"Crossref","is-referenced-by-count":20,"title":["Machado\u2010Joseph disease in New England: Clinical description and distinction from the olivopontocerebellar atrophies"],"prefix":"10.1002","volume":"7","author":[{"given":"Lewis","family":"Sudarsky","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lee","family":"Corwin","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"David M.","family":"Dawson","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2004,10,12]]},"reference":[{"key":"e_1_2_1_2_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.22.1.49"},{"key":"e_1_2_1_3_2","doi-asserted-by":"publisher","DOI":"10.1016\/0022-510X(72)90137-2"},{"key":"e_1_2_1_4_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.26.8.703"},{"key":"e_1_2_1_5_2","doi-asserted-by":"crossref","first-page":"397","DOI":"10.1017\/S0317167100028122","article-title":"Dominantly inherited ataxias in Portugal","volume":"15","author":"Cunha L","year":"1988","journal-title":"Can J Neurol Sci"},{"key":"e_1_2_1_6_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.33.1.74"},{"key":"e_1_2_1_7_2","doi-asserted-by":"publisher","DOI":"10.1001\/archneur.1984.04050200027013"},{"key":"e_1_2_1_8_2","doi-asserted-by":"publisher","DOI":"10.1001\/archneur.1989.00520400085024"},{"key":"e_1_2_1_9_2","unstructured":"SequerosJ.Origins and dissemination of MJD as another marker of Portuguese migration. Presented at the NIH: Research Initiatives on Machado Joseph Disease Bethesda June 3\u20104 1991."},{"key":"e_1_2_1_10_2","doi-asserted-by":"publisher","DOI":"10.1017\/S0317167100034983"},{"key":"e_1_2_1_11_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.28.7.703"},{"key":"e_1_2_1_12_2","series-title":"Advances in neurology","volume-title":"The olivopontocerebellar atrophies","author":"Rosenberg RN.","year":"1984"},{"key":"e_1_2_1_13_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM197706302962606"},{"key":"e_1_2_1_14_2","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410190207"},{"key":"e_1_2_1_15_2","first-page":"1203","article-title":"A clinicopathological study of Machado\u2010Joseph disease","volume":"27","author":"Riku S","year":"1987","journal-title":"Rinsho Shinkeigaku"},{"key":"e_1_2_1_16_2","unstructured":"CoutinhoP Review of neuropathological findings in Machado\u2010Joseph disease: clinico\u2010pathological correlations. Presented at the NIH: Research Initiatives on Machado Joseph Disease Bethesda June 3\u20104 1991."},{"key":"e_1_2_1_17_2","series-title":"Advances in neurology","volume-title":"The olivopontocerebellar atrophies","author":"Koeppen AH","year":"1984"},{"key":"e_1_2_1_18_2","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/94.2.349"},{"key":"e_1_2_1_19_2","unstructured":"WakisakaA SasakiJ TakadaA et al.Molecular genetic studies in OPCA and MJD in Japan. Presented at the NIH: Research Initiatives on Machado Joseph Disease Bethesda June 3\u20104 1991."}],"container-title":["Movement Disorders"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fmds.870070303","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/movementdisorders.onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/mds.870070303","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,20]],"date-time":"2025-10-20T14:17:30Z","timestamp":1760969850000},"score":1,"resource":{"primary":{"URL":"https:\/\/movementdisorders.onlinelibrary.wiley.com\/doi\/10.1002\/mds.870070303"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1992,1]]},"references-count":18,"journal-issue":{"issue":"3","published-print":{"date-parts":[[1992,1]]}},"alternative-id":["10.1002\/mds.870070303"],"URL":"https:\/\/doi.org\/10.1002\/mds.870070303","archive":["Portico"],"relation":{},"ISSN":["0885-3185","1531-8257"],"issn-type":[{"value":"0885-3185","type":"print"},{"value":"1531-8257","type":"electronic"}],"subject":[],"published":{"date-parts":[[1992,1]]}}}