{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,5]],"date-time":"2026-10-05T11:49:23Z","timestamp":1791200963972,"version":"4.2.0"},"reference-count":21,"publisher":"BMJ","issue":"5","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J Med Genet"],"published-print":{"date-parts":[[1976,10]]},"abstract":"<jats:p>A family is described with essential non-progressive chorea occurring in an autosomal dominant inheritance pattern over four generations. A few families with an apparently similar disorder have been reported previously. This condition is characterized by early childhood onset of chorea which is not progressive and is compatible with a long life. It is not associated with dementia, seizures, rigidity, or ataxia. It is a socially embarrassing condition and may, sometimes, be associated with behavioural problems and learning difficulties. For genetic counselling, it is important to distinguish this disorder from Huntington's disease and other hereditary disorders associated with chorea.<\/jats:p>","DOI":"10.1136\/jmg.13.5.357","type":"journal-article","created":{"date-parts":[[2008,11,10]],"date-time":"2008-11-10T14:50:51Z","timestamp":1226328651000},"page":"357-362","source":"Crossref","is-referenced-by-count":32,"title":["Familial essential (\"benign\") chorea."],"prefix":"10.1136","volume":"13","author":[{"given":"T D","family":"Bird","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C B","family":"Carlson","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J G","family":"Hall","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"239","published-online":{"date-parts":[[1976,10,1]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"749","DOI":"10.1093\/brain\/95.4.749","article-title":"Acanthocytosis and neurological disease","volume":"95","author":"Aminoff, M.J.","year":"1972","unstructured":"Aminoff, M. J. (1972). Acanthocytosis and neurological disease. Brain, 95, 749-760.","journal-title":"Brain"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"601","DOI":"10.1212\/WNL.14.7.601","article-title":"The pneumoencephalogram in Huntington's chorea","volume":"14","author":"Blinderman, E.; Weidner, W.; Markham, C.","year":"1964","unstructured":"Blinderman, E., Weidner, W., and Markham, C. (1964). The pneumoencephalogram in Huntington's chorea. Neurology, 14, 601-607.","journal-title":"Neurology"},{"key":"ref_3","first-page":"51","article-title":"Clinical variants and differential diagnosis","volume":"1","author":"Bruyn, G.W.","year":"1973","unstructured":"Bruyn, G. W. (1973). Clinical variants and differential diagnosis. Advances in Neurology, 1, 51-56.","journal-title":"Advances in Neurology"},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"587","DOI":"10.1212\/WNL.17.6.587","article-title":"Huntington's chorea in children","volume":"17","author":"Byers, R.; Dodge, J.","year":"1967","unstructured":"Byers, R. and Dodge, J. (1967). Huntington's chorea in children. Neurology, 17, 587-596.","journal-title":"Neurology"},{"key":"ref_5","author":"Cederbaum, S.; Heywood, D.; Aigner, R.; Motulsky, A.","year":"1971","unstructured":"Cederbaum, S., Heywood, D., Aigner, R., and Motulsky, A. (1971)."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"1603","DOI":"10.1001\/jama.1973.03220410017004","article-title":"Benign familial chorea with onset in childhood","volume":"225","author":"Chun, R.; Daly, R.; Mansheim, B.; Wolcott, G.","year":"1973","unstructured":"Chun, R., Daly, R., Mansheim, B., and Wolcott, G. (1973). Benign familial chorea with onset in childhood. Journal of the American Medical Association, 225, 1603-1607.","journal-title":"Journal of the American Medical Association"},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"134","DOI":"10.1001\/archneur.1968.00470320036004","article-title":"Acanthocytosis and neurological disorder without abetalipoproteinemia","volume":"18","author":"Critchley, E.M.R.; Clark, D.B.; Wikler, A.","year":"1968","unstructured":"Critchley, E. M. R., Clark, D. B., and Wikler, A. (1968). Acanthocytosis and neurological disorder without abetalipoproteinemia. Archives of Neurology, 18, 134-140.","journal-title":"Archives of Neurology"},{"key":"ref_8","first-page":"195","article-title":"Huntington's chorea in relation to heredity and eugenics","volume":"73","author":"Davenport, C.; Muncey, E.","year":"1916","unstructured":"Davenport, C. and Muncey, E. (1916-1917). Huntington's chorea in relation to heredity and eugenics. American journal of Insanity, 73, 195-222.","journal-title":"American journal of Insanity"},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"1220","DOI":"10.1056\/NEJM196706012762202","article-title":"Hereditary nonprogressive chorea of early onset","volume":"276","author":"Haerer, A.; Currier, R.; Jackson, J.","year":"1967","unstructured":"Haerer, A., Currier, R., and Jackson, J. (1967). Hereditary nonprogressive chorea of early onset. New England J'ournal of Medicine, 276, 1220-1224.","journal-title":"New England J'ournal of Medicine"},{"key":"ref_10","first-page":"745","article-title":"Familial paroxysmal choreoathetosis","volume-title":"Progress in Neurogenetics","author":"Homer, F.; Jackson, L.","year":"1969","unstructured":"Homer, F. and Jackson, L. (1969). Familial paroxysmal choreoathetosis. In Progress in Neurogenetics, pp. 745-751. Ed. by A. Barbean and J. Brunette. Excerpta Medica, Amsterdam."},{"key":"ref_11","first-page":"969991","article-title":"The Lesch-Nyhan syndrome. In The Metabolic Basis of Inherited Disease","volume":"17","author":"Kelley, W.N.; Wyngaarden, J.B.","year":"1972","unstructured":"Kelley, W. N. and Wyngaarden, J. B. (1972). The Lesch-Nyhan syndrome. In The Metabolic Basis of Inherited Disease, pp. 969991. Ed. by J. Stanbury, J. Wyngaarden, and D. Fredrickson. McGraw-Hill, New York. Kertesz, A. (1967). Paroxysmal kinesigenic choreoathetosis. Neurology, 17, 680-690.","journal-title":"Neurology; Paroxysmal kinesigenic choreoathetosis"},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"403","DOI":"10.1001\/archneur.1968.00480040069007","article-title":"Hereditary neurological disorder with acanthocytosis","volume":"19","author":"Levine, I.; Estes, J.W.; Looney, J.","year":"1968","unstructured":"Levine, I., Estes, J. W., and Looney, J. (1968). Hereditary neurological disorder with acanthocytosis. Archives of Neurology, 19, 403-409.","journal-title":"Archives of Neurology"},{"key":"ref_13","first-page":"703","article-title":"Calcification of the striopallidodentate system","volume-title":"Handbook of Clinical Neurology","volume":"6","author":"Lowenthal, A.; Bruyn, G.","year":"1968","unstructured":"Lowenthal, A. and Bruyn, G. (1968). Calcification of the striopallidodentate system. In Handbook of Clinical Neurology, Vol. 6. pp. 703-725. Ed. by P. Vinken and G. Bruyn. North Holland, Amsterdam."},{"key":"ref_14","first-page":"412","article-title":"The Hallervorden-Spatz syndrome","volume-title":"Greenfield's Neuropathology","author":"Meyer, A.","year":"1963","unstructured":"Meyer, A. (1963). The Hallervorden-Spatz syndrome. In Greenfield's Neuropathology, pp. 412-414. Ed. by W. Blackwood, W. H. McMenemey, A. Meyer, and R. Norman. Edward Arnold, London."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"72","DOI":"10.1056\/NEJM197107082850202","article-title":"Familial calcification of the basal ganglians","volume":"285","author":"Moskowitz, M.; Winickoff, R.; Heinz, E.R.","year":"1971","unstructured":"Moskowitz, M., Winickoff, R., and Heinz, E. R. (1971). Familial calcification of the basal ganglians. New England Journal of Medicine, 285, 72-77.","journal-title":"New England Journal of Medicine"},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"408","DOI":"10.1136\/jmg.6.4.408","article-title":"Benign recessively inherited choreo-athetosis of early onset","volume":"6","author":"Nutting, P.; Cole, B.; Schimke, R.N.","year":"1969","unstructured":"Nutting, P., Cole, B., and Schimke, R. N. (1969). Benign recessively inherited choreo-athetosis of early onset. journal of Medical Genetics, 6, 408-410.","journal-title":"journal of Medical Genetics"},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"260","DOI":"10.1111\/j.1528-1157.1967.tb04442.x","article-title":"Paroxysmal choreoathetosis and seizures induced by movement (reflex epilepsy)","volume":"8","author":"Perez-Borja, C.; Tassinari, A.C.; Swanson, A.","year":"1967","unstructured":"Perez-Borja, C., Tassinari, A. C., and Swanson, A. (1967). Paroxysmal choreoathetosis and seizures induced by movement (reflex epilepsy). Epilepsia, 8, 260-270.","journal-title":"Epilepsia"},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"724","DOI":"10.1016\/S0022-3476(67)80322-6","article-title":"Familial benign chorea with intention tremor: A clinical entity","volume":"70","author":"Pincus, J.; Chutorian, A.","year":"1967","unstructured":"Pincus, J. and Chutorian, A. (1967). Familial benign chorea with intention tremor: A clinical entity. journal of Pediatrics, 70, 724-729.","journal-title":"journal of Pediatrics"},{"key":"ref_19","first-page":"93","article-title":"Hereditary nonprogressive involuntary movements of early onset, without epilepsy or dementia","volume":"1","author":"Refsum, S.; Sjaastad, 0","year":"1973","unstructured":"Refsum, S. and Sjaastad, 0. (1973). Hereditary nonprogressive involuntary movements of early onset, without epilepsy or dementia. Advances in Neurology, 1, 93-94.","journal-title":"Advances in Neurology"},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"461","DOI":"10.1212\/WNL.18.5.461","article-title":"Paroxysmal dystonic choreoathetosis: A family study and review of the literature","volume":"18","author":"Richards, R.N.; Barnett, H.J.W.","year":"1968","unstructured":"Richards, R. N. and Barnett, H. J. W. (1968). Paroxysmal dystonic choreoathetosis: A family study and review of the literature. Neurology, 18, 461-469.","journal-title":"Neurology"},{"key":"ref_21","first-page":"79","article-title":"Hereditary nonprogressive chorea of early onset-a new entity?","volume":"1","author":"Sadjapour, K.; Amato, R.","year":"1973","unstructured":"Sadjapour, K. and Amato, R. (1973). Hereditary nonprogressive chorea of early onset-a new entity? Advances in Neurology, 1, 79-91.","journal-title":"Advances in Neurology"}],"container-title":["Journal of Medical Genetics"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1136\/jmg.13.5.357","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,6]],"date-time":"2025-10-06T03:26:58Z","timestamp":1759721218000},"score":1,"resource":{"primary":{"URL":"https:\/\/jmg.bmj.com\/lookup\/doi\/10.1136\/jmg.13.5.357"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1976,10]]},"references-count":21,"journal-issue":{"issue":"5","published-online":{"date-parts":[[1976,10,1]]},"published-print":{"date-parts":[[1976,10]]}},"alternative-id":["10.1136\/jmg.13.5.357"],"URL":"https:\/\/doi.org\/10.1136\/jmg.13.5.357","relation":{},"ISSN":["0022-2593","1468-6244"],"issn-type":[{"value":"0022-2593","type":"print"},{"value":"1468-6244","type":"electronic"}],"subject":[],"published":{"date-parts":[[1976,10]]}}}