{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T08:25:49Z","timestamp":1791275149457,"version":"4.1.0"},"reference-count":49,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2006,5,11]],"date-time":"2006-05-11T00:00:00Z","timestamp":1147305600000},"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":[[2006,8]]},"abstract":"<jats:p>Despite much evidence of cognitive and affective disorders in Friedreich's ataxia (FRDA), the nature of mental status in FRDA has received little systematic attention. It has been proposed that the cerebellum may interfere indirectly with cognition through the cerebello\u2010cortical loops, whereas the role of pathological changes in different areas of the central nervous system is still undetermined. In the present study, 13 patients with molecularly determined FRDA and a group of matched controls were evaluated by a comprehensive battery of neuropsychological tests and the Minnesota Multiphasic Personality Inventory. A repetitive task of simple visual\u2010reaction times was used to investigate implicit learning in all subjects. Pathological changes in cortical areas were explored comparing cerebral activations of patients and controls during finger movements (functional MRI). The intelligence profile of FRDA patients is characterized by concrete thinking, poor capacity in concept formation and visuospatial reasoning. FRDA patients show reduced speed of information processing. The learning effect seen in controls was notably absent in patients with FRDA. The patients\u2019 personality is characterized by some pathological aspects and reduced defensiveness. Patterns of cortical activation during finger movements are heterogeneous in patients compared to controls. Cognitive impairment, mood disorders and motor deficits in FRDA patients may be the result of the cumulative damage caused by frataxin deficiency not only in the cerebellum and spinal cord but also in other brain areas.<\/jats:p>","DOI":"10.1111\/j.1468-1331.2006.01363.x","type":"journal-article","created":{"date-parts":[[2006,5,11]],"date-time":"2006-05-11T08:59:34Z","timestamp":1147337974000},"page":"827-835","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":67,"title":["Exploring mental status in Friedreich's ataxia: a combined neuropsychological, behavioral and neuroimaging study"],"prefix":"10.1111","volume":"13","author":[{"given":"M. C.","family":"Mantovan","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A.","family":"Martinuzzi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"F.","family":"Squarzanti","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A.","family":"Bolla","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"I.","family":"Silvestri","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"G.","family":"Liessi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C.","family":"Macchi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"G.","family":"Ruzza","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C. P.","family":"Trevisan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C.","family":"Angelini","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2006,7,20]]},"reference":[{"key":"e_1_2_6_2_2","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/121.4.561"},{"key":"e_1_2_6_3_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.44.11.2047"},{"key":"e_1_2_6_4_2","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/115.1.155"},{"key":"e_1_2_6_5_2","doi-asserted-by":"publisher","DOI":"10.1016\/0278-2626(90)90057-U"},{"key":"e_1_2_6_6_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.43.8.1536"},{"key":"e_1_2_6_7_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.42.8.1493"},{"key":"e_1_2_6_8_2","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/113.1.103"},{"key":"e_1_2_6_9_2","doi-asserted-by":"publisher","DOI":"10.1126\/science.271.5254.1423"},{"key":"e_1_2_6_10_2","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410170615"},{"key":"e_1_2_6_11_2","doi-asserted-by":"publisher","DOI":"10.1159\/000116970"},{"key":"e_1_2_6_12_2","doi-asserted-by":"publisher","DOI":"10.1076\/jcen.24.5.677.1014"},{"key":"e_1_2_6_13_2","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.12.1.34"},{"key":"e_1_2_6_14_2","first-page":"202","article-title":"Psychometric studies in Friedreich's ataxia","volume":"85","author":"Leclercq M","year":"1985","journal-title":"Acta Neurologica Belgica"},{"key":"e_1_2_6_15_2","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.12.3.246"},{"key":"e_1_2_6_16_2","doi-asserted-by":"publisher","DOI":"10.1034\/j.1600-0404.2000.102004222.x"},{"key":"e_1_2_6_17_2","doi-asserted-by":"crossref","first-page":"217","DOI":"10.33588\/rn.3803.2003539","article-title":"Friedreich's ataxia: analysis of magnetic resonance imaging parameters and their correlates with cognitive and motor slowing","volume":"38","author":"Wollmann T","year":"2004","journal-title":"Revista de Neurologia"},{"key":"e_1_2_6_18_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.49.2.606"},{"key":"e_1_2_6_19_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.55.11.1719"},{"key":"e_1_2_6_20_2","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.57.1.51"},{"key":"e_1_2_6_21_2","doi-asserted-by":"publisher","DOI":"10.1007\/s004150050253"},{"key":"e_1_2_6_22_2","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/114.4.1559"},{"key":"e_1_2_6_23_2","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/104.3.589"},{"key":"e_1_2_6_24_2","first-page":"554","article-title":"The relationship between trinucleotide (GAA) repeat length and clinical features in Friedreich ataxia","volume":"59","author":"Filla A","year":"1996","journal-title":"American Journal of Human Genetics"},{"key":"e_1_2_6_25_2","doi-asserted-by":"publisher","DOI":"10.1148\/116.1.85"},{"key":"e_1_2_6_26_2","doi-asserted-by":"crossref","first-page":"405","DOI":"10.1212\/WNL.31.4_Part_2.405","article-title":"Cerebellar atrophy demonstrated by computed tomography","volume":"31","author":"Koller WC","year":"1981","journal-title":"Neurology"},{"key":"e_1_2_6_27_2","first-page":"267","volume-title":"Handbook of Intelligence","author":"Horn JL","year":"1985"},{"key":"e_1_2_6_28_2","doi-asserted-by":"publisher","DOI":"10.2466\/pms.1958.8.3.271"},{"key":"e_1_2_6_29_2","doi-asserted-by":"publisher","DOI":"10.1098\/rstb.1982.0082"},{"key":"e_1_2_6_30_2","doi-asserted-by":"crossref","unstructured":"NavonD.Forest before trees: The precedence of global features in visual perception. Cognitive Psychology1977;9:353\u2013383.","DOI":"10.1016\/0010-0285(77)90012-3"},{"key":"e_1_2_6_31_2","doi-asserted-by":"publisher","DOI":"10.1016\/0010-0285(87)90002-8"},{"key":"e_1_2_6_32_2","volume-title":"MMPI\u20102 Inventario Multifasico della Personalit\u00e0 Minnesota","author":"Hathawa SR","year":"1999"},{"key":"e_1_2_6_33_2","doi-asserted-by":"publisher","DOI":"10.1080\/00207450390200927"},{"key":"e_1_2_6_34_2","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1097-4679(199609)52:5<569::AID-JCLP12>3.0.CO;2-C"},{"key":"e_1_2_6_35_2","doi-asserted-by":"publisher","DOI":"10.1002\/hbm.460010306"},{"key":"e_1_2_6_36_2","volume-title":"Co\u2010planar Stereotaxic Atlas of the Human Brain","author":"Talairach J","year":"1988"},{"key":"e_1_2_6_37_2","volume-title":"Wechsler Adult Intelligence Scale Revised (WAIS\u2010R)","author":"Wechsler D","year":"1998"},{"key":"e_1_2_6_38_2","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/123.5.1051"},{"key":"e_1_2_6_39_2","doi-asserted-by":"publisher","DOI":"10.1126\/science.7939688"},{"key":"e_1_2_6_40_2","doi-asserted-by":"publisher","DOI":"10.1007\/BF01613351"},{"key":"e_1_2_6_41_2","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/120.10.1753"},{"key":"e_1_2_6_42_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0028-3932(99)00138-4"},{"key":"e_1_2_6_43_2","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/122.1.87"},{"key":"e_1_2_6_44_2","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/123.5.1041"},{"key":"e_1_2_6_45_2","doi-asserted-by":"publisher","DOI":"10.1176\/ajp.131.8.858"},{"key":"e_1_2_6_46_2","doi-asserted-by":"publisher","DOI":"10.1097\/00005053-197711000-00002"},{"key":"e_1_2_6_47_2","volume-title":"Wechsler Intelligence Scale for Children Revised (WISC\u2010R)","author":"Wechsler D","year":"1998"},{"key":"e_1_2_6_48_2","volume-title":"Boston Naming Test","author":"Kaplan E","year":"1983"},{"key":"e_1_2_6_49_2","first-page":"1","article-title":"Standardizzazione e taratura italiana di test neuropsicologici","volume":"8","author":"Spinnler H","year":"1987","journal-title":"The Italian Journal of Neurological Sciences"},{"key":"e_1_2_6_50_2","volume-title":"Wechsler Memory Scale","author":"Wechsler D","year":"1999"}],"container-title":["European Journal of Neurology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fj.1468-1331.2006.01363.x","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/j.1468-1331.2006.01363.x","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,10]],"date-time":"2023-10-10T13:36:44Z","timestamp":1696945004000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/j.1468-1331.2006.01363.x"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2006,7,20]]},"references-count":49,"journal-issue":{"issue":"8","published-print":{"date-parts":[[2006,8]]}},"alternative-id":["10.1111\/j.1468-1331.2006.01363.x"],"URL":"https:\/\/doi.org\/10.1111\/j.1468-1331.2006.01363.x","archive":["Portico"],"relation":{},"ISSN":["1351-5101","1468-1331"],"issn-type":[{"value":"1351-5101","type":"print"},{"value":"1468-1331","type":"electronic"}],"subject":[],"published":{"date-parts":[[2006,7,20]]},"assertion":[{"value":"2006-07-20","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}