Please use this identifier to cite or link to this item: https://ahro.austin.org.au/austinjspui/handle/1/16475
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dc.contributor.authorHarding, Ian H-
dc.contributor.authorCorben, Louise A-
dc.contributor.authorStorey, Elsdon-
dc.contributor.authorEgan, Gary F-
dc.contributor.authorStagnitti, Monique R-
dc.contributor.authorPoudel, Govinda R-
dc.contributor.authorDelatycki, Martin B-
dc.contributor.authorGeorgiou-Karistianis, Nellie-
dc.date2015-10-
dc.date.accessioned2017-01-02T23:23:52Z-
dc.date.available2017-01-02T23:23:52Z-
dc.date.issued2016-01-
dc.identifier.citationHuman Brain Mapping 2016; 37(1): 338-350en_US
dc.identifier.urihttps://ahro.austin.org.au/austinjspui/handle/1/16475-
dc.description.abstractFriedreich ataxia (FRDA) is a progressive neurodegenerative disorder defined by pathology within the cerebellum and spinal tracts. Although FRDA is most readily linked to motor and sensory dysfunctions, reported impairments in working memory and executive functions indicate that abnormalities may also extend to associations regions of the cerebral cortex and/or cerebello-cerebral interactions. To test this hypothesis, 29 individuals with genetically confirmed FRDA and 34 healthy controls performed a verbal n-back working memory task while undergoing functional magnetic resonance imaging. No significant group differences were evident in task performance. However, individuals with FRDA had deficits in brain activations both in the lateral cerebellar hemispheres, principally encompassing lobule VI, and the prefrontal cortex, including regions of the anterior insular and rostrolateral prefrontal cortices. Functional connectivity between these brain regions was also impaired, supporting a putative link between primary cerebellar dysfunction and subsequent cerebral abnormalities. Disease severity and genetic markers of disease liability were correlated specifically with cerebellar dysfunction, while correlations between behavioural performance and both cerebral activations and cerebello-cerebral connectivity were observed in controls, but not in the FRDA cohort. Taken together, these findings support a diaschisis model of brain dysfunction, whereby primary disease effects in the cerebellum result in functional changes in downstream fronto-cerebellar networks. These fronto-cerebellar disturbances provide a putative biological basis for the nonmotor symptoms observed in FRDA, and reflect the consequence of localized cerebellar pathology to distributed brain function underlying higher-order cognition.en_US
dc.subjectFriedreich ataxiaen_US
dc.subjectCerebellumen_US
dc.subjectCognitionen_US
dc.subjectfMRIen_US
dc.subjectFunctional connectivityen_US
dc.subjectPsychophysiological interactionen_US
dc.subjectWorking memoryen_US
dc.titleFronto-cerebellar dysfunction and dysconnectivity underlying cognition in friedreich ataxia: the IMAGE-FRDA studyen_US
dc.typeJournal Articleen_US
dc.identifier.journaltitleHuman Brain Mappingen_US
dc.identifier.affiliationSchool of Psychological Sciences, Monash University, Melbourne, Victoria, Australiaen_US
dc.identifier.affiliationBruce Lefroy Centre, Murdoch Childrens Research Institute, Melbourne, Victoria, Australiaen_US
dc.identifier.affiliationFriedreich Ataxia Clinic, Monash Medical Centre, Monash Health, Melbourne, Victoria, Australiaen_US
dc.identifier.affiliationDepartment of Medicine, Monash University, Melbourne, Victoria, Australiaen_US
dc.identifier.affiliationMonash Biomedical Imaging, Monash University, Melbourne, Victoria, Australiaen_US
dc.identifier.affiliationDepartment of Clinical Genetics, Austin Health, Heidelberg, Victoria, Australiaen_US
dc.identifier.pubmedurihttps://pubmed.ncbi.nlm.nih.gov/26502936en_US
dc.identifier.doi10.1002/hbm.23034en_US
dc.type.contentTexten_US
dc.type.austinJournal Articleen_US
local.name.researcherDelatycki, Martin B
item.openairetypeJournal Article-
item.openairecristypehttp://purl.org/coar/resource_type/c_18cf-
item.grantfulltextnone-
item.fulltextNo Fulltext-
item.cerifentitytypePublications-
crisitem.author.deptClinical Genetics-
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