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@ARTICLE{Lehto:278793,
      author       = {Lehto, Annaliis and Schumacher, Julia and Kurth, Jens and
                      Krause, Bernd J and Kasper, Elisabeth and Teipel, Stefan and
                      Prudlo, Johannes},
      collaboration = {Initiative, Alzheimer’s Disease Neuroimaging},
      title        = {{I}nconsistent primary motor cortex glucose hypometabolism
                      in primary lateral sclerosis.},
      journal      = {Journal of neurology},
      volume       = {272},
      number       = {6},
      issn         = {0367-004X},
      address      = {[Darmstadt]},
      publisher    = {Steinkopff},
      reportid     = {DZNE-2025-00629},
      pages        = {410},
      year         = {2025},
      abstract     = {Primary lateral sclerosis (PLS) is a rare neurodegenerative
                      disorder and a model for upper motor neuron degeneration,
                      which is believed to begin in the primary motor cortex.
                      However, clinical observation suggests that not all PLS
                      cases show primary motor cortex glucose hypometabolism on
                      2-deoxy-2-[18F]fluoro-D-glucose positron emission tomography
                      (FDG-PET). We aimed to assess the reliability of FDG-PET in
                      identifying motor cortex hypometabolism over disease course
                      in a sample of patients with PLS.Baseline FDG-PET data from
                      nine consecutive PLS patients were analyzed. At least one
                      follow up assessment was available for five patients. We
                      extracted the average FDG-PET signal in the primary motor
                      cortex and other motor regions and calculated the
                      covariate-corrected z scores based on data of healthy
                      controls from the Alzheimer's Disease Neuroimaging
                      Initiative (ADNI) cohort.Among the nine patients evaluated,
                      only four demonstrated glucose hypometabolism in the primary
                      motor cortex across available baseline and follow up
                      assessments. Glucose metabolism of motor regions declined
                      over time in some patients, whereas others maintained stable
                      levels despite a worsening in symptom severity.Primary motor
                      cortex hypometabolism in PLS patients is less consistent
                      than previously reported, and the absence of this
                      hypometabolic sign should not be considered as irrefutable
                      evidence against PLS in the diagnostic process. The findings
                      of our study underline the heterogeneity of PLS, indicating
                      that more precise diagnostic tools would be beneficial to
                      confirm a PLS diagnosis at an earlier stage.},
      keywords     = {Humans / Motor Cortex: metabolism / Motor Cortex:
                      diagnostic imaging / Male / Female / Positron-Emission
                      Tomography / Middle Aged / Aged / Fluorodeoxyglucose F18 /
                      Glucose: metabolism / Motor Neuron Disease: diagnostic
                      imaging / Motor Neuron Disease: metabolism / Follow-Up
                      Studies / Cerebral glucose metabolism (Other) / FDG-PET
                      (Other) / Primary lateral sclerosis (Other) / Primary motor
                      cortex (Other) / Fluorodeoxyglucose F18 (NLM Chemicals) /
                      Glucose (NLM Chemicals)},
      cin          = {AG Teipel / AG Storch},
      ddc          = {610},
      cid          = {I:(DE-2719)1510100 / I:(DE-2719)5000014},
      pnm          = {353 - Clinical and Health Care Research (POF4-353)},
      pid          = {G:(DE-HGF)POF4-353},
      typ          = {PUB:(DE-HGF)16},
      pmc          = {pmc:PMC12092561},
      pubmed       = {pmid:40392424},
      doi          = {10.1007/s00415-025-13089-x},
      url          = {https://pub.dzne.de/record/278793},
}