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@ARTICLE{Jiang:141523,
      author       = {Jiang, Xueyan and Faber, J. and Giordano, I. and Machts, J.
                      and Kindler, Ch and Dudesek, A. and Speck, O. and Kamm, Ch
                      and Düzel, E. and Jessen, F. and Spottke, A. and Vielhaber,
                      St and Boecker, H. and Klockgether, T. and Scheef, L.},
      title        = {{C}haracterization of {C}erebellar {A}trophy and {R}esting
                      {S}tate {F}unctional {C}onnectivity {P}atterns in {S}poradic
                      {A}dult-{O}nset {A}taxia of {U}nknown {E}tiology ({SAOA}).},
      journal      = {The Cerebellum},
      volume       = {18},
      number       = {5},
      issn         = {1473-4222},
      address      = {London},
      publisher    = {Dunitz},
      reportid     = {DZNE-2020-07847},
      pages        = {873-881},
      year         = {2019},
      abstract     = {Sporadic adult-onset ataxia of unknown etiology (SAOA) is a
                      non-genetic neurodegenerative disorder of the cerebellum of
                      unknown cause which manifests with progressive ataxia
                      without severe autonomic failure. Although SAOA is
                      associated with cerebellar degeneration, little is known
                      about the specific cerebellar atrophy pattern in SAOA.
                      Thirty-seven SAOA patients and 49 healthy controls (HCs)
                      were included at two centers. We investigated the structural
                      and functional characteristics of SAOA brains using
                      voxel-based morphometry (VBM) and resting-state functional
                      imaging (rs-fMRI). In order to examine the functional
                      consequence of structural cerebellar alterations, the
                      amplitude of low-frequency fluctuation (ALFF) and degree
                      centrality (DC) were analyzed, and then assessed their
                      relation with disease severity, disease duration, and age of
                      onset within these regions. Group differences were
                      investigated using two-sample t tests, controlling for age,
                      gender, site, and the total intracranial volume. The VBM
                      analysis revealed a significant, mostly bilateral reduction
                      of local gray matter (GM) volume in lobules I-V, V, VI, IX,
                      X, and vermis VIII a/b in SAOA patients, compared with HCs.
                      The GM volume loss in these regions was significantly
                      associated with disease severity, disease duration, and age
                      of onset. The disease-related atrophy regions did not show
                      any functional alternations compared with HCs but were
                      functionally characterized by high ALFF and poor DC compared
                      with intact cerebellar regions. Our data revealed volume
                      reduction in SAOA in cerebellar regions that are known to be
                      involved in motor and somatosensory processing,
                      corresponding with the clinical phenotype of SAOA. Our data
                      suggest that the atrophy occurs in those cerebellar regions
                      which are characterized by high ALFF and poor DC. Further
                      studies have to show if these findings are specific for
                      SAOA, and if they can be used to predict disease
                      progression.},
      keywords     = {Adult / Aged / Atrophy: diagnostic imaging / Atrophy:
                      physiopathology / Cerebellar Ataxia: diagnostic imaging /
                      Cerebellar Ataxia: physiopathology / Cerebellum: diagnostic
                      imaging / Cerebellum: physiopathology / Female / Humans /
                      Magnetic Resonance Imaging: methods / Male / Middle Aged /
                      Nerve Net: diagnostic imaging / Nerve Net: physiopathology /
                      Rest: physiology},
      cin          = {Patient Studies Bonn / AG Speck / Bonn common / AG Düzel /
                      AG Boecker / Delcode},
      ddc          = {610},
      cid          = {I:(DE-2719)1011101 / I:(DE-2719)1340009 /
                      I:(DE-2719)6000011 / I:(DE-2719)5000006 / I:(DE-2719)1011202
                      / I:(DE-2719)5000034},
      pnm          = {344 - Clinical and Health Care Research (POF3-344)},
      pid          = {G:(DE-HGF)POF3-344},
      experiment   = {EXP:(DE-2719)DELCODE-20140101},
      typ          = {PUB:(DE-HGF)16},
      pubmed       = {pmid:31422550},
      doi          = {10.1007/s12311-019-01072-y},
      url          = {https://pub.dzne.de/record/141523},
}