{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,21]],"date-time":"2025-10-21T00:44:39Z","timestamp":1761007479842,"version":"build-2065373602"},"reference-count":38,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2004,11,4]],"date-time":"2004-11-04T00:00:00Z","timestamp":1099526400000},"content-version":"vor","delay-in-days":2318,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Movement Disorders"],"published-print":{"date-parts":[[1998,7]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>A family with two members who had early\u2010onset cerebellar ataxia (EOCA) with retained tendon reflexes had, in addition to their motor deficits, a progressive impairment of cognitive and visuospatial abilities. We used positron emission tomography (PET) with <jats:sup>11<\/jats:sup>C\u2010flumazenil to study gammaaminobutyric type A\/benzodiazepine receptor binding (BZR) and <jats:sup>18<\/jats:sup>F\u20102\u2010fluoro\u20102\u2010deoxy\u2010D\u2010glucose to analyze longitudinally regional cerebral glucose metabolism. Flumazenil\u2010PET demonstrated loss of BZR binding that has not been shown in Friedreich's ataxia and olivopontocerebellar atrophy. These findings may be useful for differentiation of EOCA from other types of cerebellar ataxia. In comparison to age\u2010matched control subjects, these patients showed a global metabolic decline and predominant hypometabolism in the thalamus and cerebellum. The progressive metabolic derangement may be explainable by a disturbed integrity of cognition\u2010related networks resulting from secondary degeneration of cerebello\u2010thalamo\u2010cortical projections.<\/jats:p>","DOI":"10.1002\/mds.870130423","type":"journal-article","created":{"date-parts":[[2005,5,28]],"date-time":"2005-05-28T20:46:41Z","timestamp":1117313201000},"page":"739-745","source":"Crossref","is-referenced-by-count":8,"title":["Early\u2010onset cerebellar ataxia (EOCA) with retained reflexes: Reduced cerebellar benzodiazepine\u2010receptor binding, progressive metabolic and cognitive impairment"],"prefix":"10.1002","volume":"13","author":[{"given":"R\u00dcDiger","family":"Mielke","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"R\u00dcDiger","family":"Hilker","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gerald","family":"Weber\u2010Luxenburger","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Josef","family":"Kessler","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Wolf\u2010Dieter","family":"Heiss","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2004,11,4]]},"reference":[{"key":"e_1_2_1_2_2","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.44.6.503"},{"key":"e_1_2_1_3_2","first-page":"26","article-title":"Zur Differentialdiagnostik der Friedreich Ataxie","volume":"60","author":"Claus D.","year":"1989","journal-title":"Nervenarzt"},{"key":"e_1_2_1_4_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1600-0404.1989.tb03933.x"},{"key":"e_1_2_1_5_2","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.53.8.667"},{"key":"e_1_2_1_6_2","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/114.4.1559"},{"key":"e_1_2_1_7_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1399-0004.1993.tb04450.x"},{"key":"e_1_2_1_8_2","doi-asserted-by":"publisher","DOI":"10.1016\/0022-510X(92)90263-K"},{"key":"e_1_2_1_9_2","first-page":"1014","article-title":"Early onset hereditary spinocerebellar ataxia: an autosomal recessive disorder distinct from Friedreich's ataxia","volume":"26","author":"Kumar D","year":"1989","journal-title":"Indian Pediatr"},{"key":"e_1_2_1_10_2","doi-asserted-by":"publisher","DOI":"10.1007\/BF01629292"},{"key":"e_1_2_1_11_2","doi-asserted-by":"publisher","DOI":"10.1016\/0022-3956(75)90026-6"},{"volume-title":"Leistungs\u2010Pr\u00fcf\u2010System","year":"1962","author":"Horn W.","key":"e_1_2_1_12_2"},{"volume-title":"Ein verbaler Ged\u00e4chtnistest nach der Methode des selektiven Erinnerns","year":"1992","author":"Schaaf A","key":"e_1_2_1_13_2"},{"key":"e_1_2_1_14_2","first-page":"157","volume-title":"A Compendium of Neuropsychological Tests","author":"Spreen O","year":"1991"},{"key":"e_1_2_1_15_2","first-page":"161","volume-title":"A Compendium of Neuropsychological Tests","author":"Taylor LB.","year":"1991"},{"volume-title":"Wechsler Memory Scale\u2013Revised","year":"1987","author":"Wechsler D.","key":"e_1_2_1_16_2"},{"key":"e_1_2_1_17_2","doi-asserted-by":"publisher","DOI":"10.1093\/oxfordjournals.bmb.a070866"},{"key":"e_1_2_1_18_2","first-page":"67","article-title":"Influence of different paths on spatial memory performance in the Block\u2010Tapping test","volume":"111","author":"Smirini P","year":"1983","journal-title":"J Clin Neuropsychol"},{"key":"e_1_2_1_19_2","first-page":"221","volume-title":"A Compendium of Neuropsychological Tests","author":"Spreen O","year":"1991"},{"volume-title":"Der Zahlen\u2010Verbindungs\u2010Test (ZVT)","year":"1987","author":"Oswald WD","key":"e_1_2_1_20_2"},{"issue":"1","key":"e_1_2_1_21_2","first-page":"S794","article-title":"Comparison of kinetic and equilibrium PET measurements of (11C) flumazenil binding to human benzodiazepine receptors","volume":"13","author":"Frey KA","year":"1993","journal-title":"J Cereb Bolld Flow Metab"},{"key":"e_1_2_1_22_2","doi-asserted-by":"publisher","DOI":"10.1038\/jcbfm.1985.15"},{"key":"e_1_2_1_23_2","first-page":"2011","article-title":"An interactive technique for three\u2010dimensional image registration: validation for PET, SPECT, MRI and CT brain studies","volume":"35","author":"Pietrzyk U","year":"1994","journal-title":"J Nucl Med"},{"key":"e_1_2_1_24_2","doi-asserted-by":"publisher","DOI":"10.1001\/archneur.1990.00530090071015"},{"key":"e_1_2_1_25_2","doi-asserted-by":"publisher","DOI":"10.1001\/archneur.1991.00530230086029"},{"key":"e_1_2_1_26_2","doi-asserted-by":"crossref","first-page":"404","DOI":"10.1017\/S0317167100030973","article-title":"Positron emission tomography study of benzodiazepine receptors in Friedreich's ataxia","volume":"17","author":"Chavoix C","year":"1990","journal-title":"Can J Neurol Sci"},{"key":"e_1_2_1_27_2","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410380209"},{"key":"e_1_2_1_28_2","doi-asserted-by":"publisher","DOI":"10.1038\/277397a0"},{"key":"e_1_2_1_29_2","doi-asserted-by":"publisher","DOI":"10.1016\/0014-2999(79)90071-2"},{"key":"e_1_2_1_30_2","doi-asserted-by":"publisher","DOI":"10.1523\/JNEUROSCI.07-06-01866.1987"},{"key":"e_1_2_1_31_2","doi-asserted-by":"publisher","DOI":"10.1016\/0006-8993(89)91165-7"},{"key":"e_1_2_1_32_2","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410240310"},{"key":"e_1_2_1_33_2","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410360208"},{"key":"e_1_2_1_34_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.45.7.1345"},{"key":"e_1_2_1_35_2","doi-asserted-by":"publisher","DOI":"10.1001\/archneur.1995.00540260085021"},{"key":"e_1_2_1_36_2","doi-asserted-by":"publisher","DOI":"10.1016\/0022-510X(94)90242-9"},{"key":"e_1_2_1_37_2","doi-asserted-by":"publisher","DOI":"10.1001\/archneur.1994.00540160043007"},{"key":"e_1_2_1_38_2","doi-asserted-by":"publisher","DOI":"10.1093\/cercor\/3.5.421"},{"key":"e_1_2_1_39_2","first-page":"70","volume-title":"Cerebellar Functions","author":"Sasaki K.","year":"1985"}],"container-title":["Movement Disorders"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fmds.870130423","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/movementdisorders.onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/mds.870130423","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,20]],"date-time":"2025-10-20T13:23:43Z","timestamp":1760966623000},"score":1,"resource":{"primary":{"URL":"https:\/\/movementdisorders.onlinelibrary.wiley.com\/doi\/10.1002\/mds.870130423"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1998,7]]},"references-count":38,"journal-issue":{"issue":"4","published-print":{"date-parts":[[1998,7]]}},"alternative-id":["10.1002\/mds.870130423"],"URL":"https:\/\/doi.org\/10.1002\/mds.870130423","archive":["Portico"],"relation":{},"ISSN":["0885-3185","1531-8257"],"issn-type":[{"type":"print","value":"0885-3185"},{"type":"electronic","value":"1531-8257"}],"subject":[],"published":{"date-parts":[[1998,7]]}}}