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@ARTICLE{Hansen:153294,
      author       = {Hansen, Niels and Hirschel, Sina and Stöcker, Winfried and
                      Manig, Anja and Falk, Hannah Sönne and Ernst, Marielle and
                      Vukovich, Ruth and Zerr, Inga and Wiltfang, Jens and
                      Bartels, Claudia},
      title        = {{F}igural {M}emory {I}mpairment in {C}onjunction {W}ith
                      {N}europsychiatric {S}ymptoms in {I}g{LON}5
                      {A}ntibody-{A}ssociated {A}utoimmune {E}ncephalitis},
      journal      = {Frontiers in psychiatry},
      volume       = {11},
      issn         = {1664-0640},
      address      = {Lausanne},
      publisher    = {Frontiers Research Foundation},
      reportid     = {DZNE-2020-01291},
      pages        = {576},
      year         = {2020},
      abstract     = {Background: IgLON5 disease is an autoimmune disorder that
                      shares neuropathological aspects with a tauopathy. Its
                      clinical spectrum is heterogeneous, and figural memory
                      impairment as an initial phenomenon of IgLON5 syndrome has
                      not yet been described. The rationale of this report is to
                      highlight symptoms related to IgLON5 disease that have not
                      been reported to date. This case report will thereby
                      emphasize how important it is to initiate thorough
                      diagnostic methods including cerebrospinal fluid analysis
                      (CSF) before starting early immunotherapy.Methods: We
                      examined a 65-year-old Caucasian male via neuropsychological
                      tests, magnetic resonance imaging (MRI),
                      electroencephalography (EEG), neurography and
                      polysomnography. He also underwent two lumbar punctures from
                      which we determined specific autoantibodies in cerebrospinal
                      (CSF) and peripheral blood (PB).Results: The patient
                      presented initially complaining of memory loss, gradual
                      dysphagia and sleeping dysfunction. Neuropsychological
                      testing at first presentation and follow-up revealed subtle
                      figural and working memory impairment. At onset and at his
                      6-month follow-up, we detected IgLON5 antibodies in CSF and
                      PB. Furthermore, we identified in the CSF a blood–brain
                      barrier disturbance at disease onset and follow-up, and
                      markers of neuroaxonal damage such as mildly elevated
                      phosphorylated Tau-181 protein with 86 pg/ml (normal range
                      ≤ 61 pg/ml) at onset. Three months after his initial
                      presentation, he was suffering from axonal neuropathy and
                      transient ataxia in the extremities. Assuming a definitive
                      autoimmune encephalitis-associated with anti-IgLON5
                      antibodies, we applied high-dose steroids monthly (1g
                      methylprednisolone i.v. for five consecutive days) and his
                      memory complaints, ataxia of extremities and peripheral
                      neuropathy as well as sleeping dysfunction
                      decreased.Conclusions: Our findings broaden IgLON5
                      disease’s clinical spectrum to include predominant and
                      discrete figural memory impairment together with sleeping
                      dysfunction at disease onset. In addition, our report
                      illustrates how important taking an elaborated diagnostic
                      approach is to assuring an accurate diagnosis and the
                      appropriate therapy if a patient presents with a persisting
                      figural memory impairment and sleeping abnormalities so as
                      to avoid overlooking IgLON5 disease and a potentially poor
                      outcome.},
      cin          = {Göttingen Pre 2020 / AG Zerr / AG Wiltfang},
      ddc          = {610},
      cid          = {I:(DE-2719)6000014 / I:(DE-2719)1440011-1 /
                      I:(DE-2719)1410006},
      pnm          = {342 - Disease Mechanisms and Model Systems (POF3-342) / 344
                      - Clinical and Health Care Research (POF3-344)},
      pid          = {G:(DE-HGF)POF3-342 / G:(DE-HGF)POF3-344},
      typ          = {PUB:(DE-HGF)16},
      pmc          = {pmc:PMC7351505},
      pubmed       = {pmid:32714214},
      doi          = {10.3389/fpsyt.2020.00576},
      url          = {https://pub.dzne.de/record/153294},
}