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@ARTICLE{Preler:282604,
      author       = {Preßler, Hannah and Haddy, Imène and Daugherty, Claire
                      and Postila, Ville and Meisel, Andreas},
      title        = {{P}araneoplastic {L}ambert-{E}aton myasthenic syndrome
                      associated with non-small cell lung cancer: data from the
                      {E}uropean {LEMS} registry and systematic review.},
      journal      = {Neurological research and practice},
      volume       = {7},
      number       = {1},
      issn         = {2524-3489},
      address      = {[London]},
      publisher    = {BioMed Central},
      reportid     = {DZNE-2025-01362},
      pages        = {95},
      year         = {2025},
      abstract     = {Paraneoplastic Lambert-Eaton myasthenic syndrome (pLEMS) is
                      well-established in small-cell lung cancer (SCLC), but data
                      on other malignancies are limited. We aimed to define the
                      clinical phenotype of pLEMS in non-SCLC cancers
                      (non-SCLC-pLEMS) relative to SCLC-associated LEMS
                      (SCLC-pLEMS) and autoimmune LEMS (aiLEMS).Retrospective
                      analysis was conducted to compare patients with SCLC-pLEMS,
                      aiLEMS and non-SCLC-pLEMS from the European LEMS registry,
                      and further non-SCLC-pLEMS cases were identified by a
                      systematic review following the Preferred Reporting Items
                      for Systematic Reviews and Meta-Analyses (PRISMA)
                      guidelines.The registry included 72 aiLEMS, 12 SCLC-pLEMS,
                      and 11 non-SCLC-pLEMS patients. LEMS preceded cancer
                      diagnosis in $33\%$ of SCLC-pLEMS (median 2 months) and
                      $30\%$ of non-SCLC-pLEMS (median 15 months), was concurrent
                      in $25\%$ and $30\%$ and followed tumor diagnosis in the
                      remainder. At study enrollement, Quantitative Myasthenia
                      Gravis (QMG) scores were higher in SCLC-pLEMS (median 12,
                      range 1-24) with recent tumor therapy initiation (median 3
                      months), and lower in non-SCLC-pLEMS (median 6, range 0-19),
                      with longer (median 12 months) or completed tumor therapy,
                      and aiLEMS (median 5, range 0-23). During follow-up, QMG
                      improved with tumor therapy, and worsened with
                      recurrence/progression in pLEMS groups. After completion of
                      cancer treatment, QMG values in SCLC-pLEMS (median 6, range
                      0-19) and non-SCLC-pLEMS (median 5, range 1-22) were
                      comparable to each other and to aiLEMS (median 7, range
                      0-29). Ataxia was significantly more frequent in SCLC-pLEMS
                      $(64\%)$ and non-SCLC-pLEMS $(55\%)$ than in aiLEMS $(19\%,$
                      p = 0.006 and p = 0.024). Another 115 literature-reported
                      non-SCLC-pLEMS cases were identified (total n = 126,
                      comprising 137 tumors). Most common were non-small cell lung
                      cancer (NSCLC) (n = 25, $18\%),$ Merkel cell carcinoma (n =
                      18, $13\%)$ and lymphoproliferative disorders (n = 15,
                      $11\%).$ In 52 literature-reported LEMS patients with
                      outcome data, $88\%$ partially or fully recovered after
                      tumor therapy, leaving the paraneoplastic origin uncertain
                      in many.Our results suggest that, beyond SCLC, other tumors
                      can trigger pLEMS. Compared with aiLEMS, non-SCLC-pLEMS and
                      SCLC-pLEMS showed a higher frequency of ataxia, and LEMS
                      severity tended to reflect tumor treatment status, while
                      disease severity becomes comparable across subtypes after
                      cancer therapy. The frequent improvement of symptoms with
                      tumor-directed treatment supports extended screening beyond
                      SCLC and timely management.},
      keywords     = {Ataxia (Other) / Cancer treatment (Other) / Lambert-Eaton
                      myasthenic syndrome (Other) / Paraneoplastic (Other) / Tumor
                      (Other)},
      cin          = {AG Prüß},
      ddc          = {610},
      cid          = {I:(DE-2719)1810003},
      pnm          = {353 - Clinical and Health Care Research (POF4-353)},
      pid          = {G:(DE-HGF)POF4-353},
      typ          = {PUB:(DE-HGF)16},
      pubmed       = {pmid:41361909},
      pmc          = {pmc:PMC12687528},
      doi          = {10.1186/s42466-025-00453-5},
      url          = {https://pub.dzne.de/record/282604},
}