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@ARTICLE{Kassubek:275878,
      author       = {Kassubek, Jan and Roselli, Francesco and Witzel, Simon and
                      Dorst, Johannes and Ludolph, Albert C and Rasche, Volker and
                      Vernikouskaya, Ina and Müller, Hans-Peter},
      title        = {{H}ypothalamic atrophy in primary lateral sclerosis,
                      assessed by convolutional neural network-based automatic
                      segmentation.},
      journal      = {Scientific reports},
      volume       = {15},
      number       = {1},
      issn         = {2045-2322},
      address      = {[London]},
      publisher    = {Springer Nature},
      reportid     = {DZNE-2025-00113},
      pages        = {1551},
      year         = {2025},
      abstract     = {Primary lateral sclerosis (PLS) is a motor neuron disease
                      (MND) which mainly affects upper motor neurons. Within the
                      MND spectrum, PLS is much more slowly progressive than
                      amyotrophic laterals sclerosis (ALS). `Classical` ALS is
                      characterized by catabolism and abnormal energy metabolism
                      preceding onset of motor symptoms, and previous studies
                      indicated that the disease progression of ALS involves
                      hypothalamic atrophy. Very limited weight loss is observed
                      in patients with PLS, which raises the question of whether
                      there are also less hypothalamic alterations. The purpose of
                      this study was to quantitatively investigate the
                      hypothalamic volume in a group of PLS patients and to
                      compare it with ALS and controls. Recently, we have
                      introduced automatic hypothalamic quantification method
                      based on the use of convolutional neural network (CNN) to
                      reduce human variability and enhance analysis robustness.
                      This CNN of U-Net architecture was applied for automatic
                      segmentation of the hypothalamus and intracranial volume
                      (ICV) to allow adjustments of the hypothalamic volume
                      between subjects with different head sizes respectively.
                      Automatic segmentation and volumetric analysis were
                      performed in high resolution T1 weighted MRI volumes
                      (acquired on a 1.5 T MRI scanner) of 46 PLS patients in
                      comparison to 107 healthy controls and 411 `classical` ALS
                      patients, respectively. Significant hypothalamic volume
                      reduction was observed in PLS (818 ± 73 mm3) when compared
                      to controls (852 ± 77 mm3); significant hypothalamic volume
                      reduction was also confirmed in ALS (823 ± 84 mm3), in
                      support of previous studies. No significant differences were
                      found in normalized hypothalamic volumes between ALS
                      patients and PLS patients at the group level. This unbiased
                      CNN-based hypothalamus volume quantification study
                      demonstrated similarly reduced hypothalamus volume in PLS
                      and ALS patients, despite the clinical phenotypic
                      differences.},
      keywords     = {Humans / Female / Male / Hypothalamus: pathology /
                      Hypothalamus: diagnostic imaging / Middle Aged / Neural
                      Networks, Computer / Magnetic Resonance Imaging: methods /
                      Atrophy: pathology / Aged / Amyotrophic Lateral Sclerosis:
                      pathology / Amyotrophic Lateral Sclerosis: diagnostic
                      imaging / Adult / Motor Neuron Disease: pathology / Motor
                      Neuron Disease: diagnostic imaging / Image Processing,
                      Computer-Assisted: methods / Case-Control Studies /
                      Amyotrophic Lateral Sclerosis (Other) / Hypothalamus (Other)
                      / Magnetic Resonance Imaging (Other) / Metabolism (Other) /
                      Neuronal Networks (Other) / Primary Lateral Sclerosis
                      (Other) / Volumetry (Other)},
      cin          = {AG Roselli / Clinical Study Center (Ulm)},
      ddc          = {600},
      cid          = {I:(DE-2719)1910001 / I:(DE-2719)5000077},
      pnm          = {352 - Disease Mechanisms (POF4-352) / 353 - Clinical and
                      Health Care Research (POF4-353)},
      pid          = {G:(DE-HGF)POF4-352 / G:(DE-HGF)POF4-353},
      typ          = {PUB:(DE-HGF)16},
      pubmed       = {pmid:39789167},
      pmc          = {pmc:PMC11718091},
      doi          = {10.1038/s41598-025-85786-6},
      url          = {https://pub.dzne.de/record/275878},
}