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000275878 1001_ $$0P:(DE-2719)9001967$$aKassubek, Jan$$b0
000275878 245__ $$aHypothalamic atrophy in primary lateral sclerosis, assessed by convolutional neural network-based automatic segmentation.
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000275878 520__ $$aPrimary lateral sclerosis (PLS) is a motor neuron disease (MND) which mainly affects upper motor neurons. Within the MND spectrum, PLS is much more slowly progressive than amyotrophic laterals sclerosis (ALS). `Classical` ALS is characterized by catabolism and abnormal energy metabolism preceding onset of motor symptoms, and previous studies indicated that the disease progression of ALS involves hypothalamic atrophy. Very limited weight loss is observed in patients with PLS, which raises the question of whether there are also less hypothalamic alterations. The purpose of this study was to quantitatively investigate the hypothalamic volume in a group of PLS patients and to compare it with ALS and controls. Recently, we have introduced automatic hypothalamic quantification method based on the use of convolutional neural network (CNN) to reduce human variability and enhance analysis robustness. This CNN of U-Net architecture was applied for automatic segmentation of the hypothalamus and intracranial volume (ICV) to allow adjustments of the hypothalamic volume between subjects with different head sizes respectively. Automatic segmentation and volumetric analysis were performed in high resolution T1 weighted MRI volumes (acquired on a 1.5 T MRI scanner) of 46 PLS patients in comparison to 107 healthy controls and 411 `classical` ALS patients, respectively. Significant hypothalamic volume reduction was observed in PLS (818 ± 73 mm3) when compared to controls (852 ± 77 mm3); significant hypothalamic volume reduction was also confirmed in ALS (823 ± 84 mm3), in support of previous studies. No significant differences were found in normalized hypothalamic volumes between ALS patients and PLS patients at the group level. This unbiased CNN-based hypothalamus volume quantification study demonstrated similarly reduced hypothalamus volume in PLS and ALS patients, despite the clinical phenotypic differences.
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000275878 650_7 $$2Other$$aAmyotrophic Lateral Sclerosis
000275878 650_7 $$2Other$$aHypothalamus
000275878 650_7 $$2Other$$aMagnetic Resonance Imaging
000275878 650_7 $$2Other$$aMetabolism
000275878 650_7 $$2Other$$aNeuronal Networks
000275878 650_7 $$2Other$$aPrimary Lateral Sclerosis
000275878 650_7 $$2Other$$aVolumetry
000275878 650_2 $$2MeSH$$aHumans
000275878 650_2 $$2MeSH$$aFemale
000275878 650_2 $$2MeSH$$aMale
000275878 650_2 $$2MeSH$$aHypothalamus: pathology
000275878 650_2 $$2MeSH$$aHypothalamus: diagnostic imaging
000275878 650_2 $$2MeSH$$aMiddle Aged
000275878 650_2 $$2MeSH$$aNeural Networks, Computer
000275878 650_2 $$2MeSH$$aMagnetic Resonance Imaging: methods
000275878 650_2 $$2MeSH$$aAtrophy: pathology
000275878 650_2 $$2MeSH$$aAged
000275878 650_2 $$2MeSH$$aAmyotrophic Lateral Sclerosis: pathology
000275878 650_2 $$2MeSH$$aAmyotrophic Lateral Sclerosis: diagnostic imaging
000275878 650_2 $$2MeSH$$aAdult
000275878 650_2 $$2MeSH$$aMotor Neuron Disease: pathology
000275878 650_2 $$2MeSH$$aMotor Neuron Disease: diagnostic imaging
000275878 650_2 $$2MeSH$$aImage Processing, Computer-Assisted: methods
000275878 650_2 $$2MeSH$$aCase-Control Studies
000275878 7001_ $$0P:(DE-2719)2812851$$aRoselli, Francesco$$b1
000275878 7001_ $$aWitzel, Simon$$b2
000275878 7001_ $$0P:(DE-2719)9001951$$aDorst, Johannes$$b3$$udzne
000275878 7001_ $$0P:(DE-2719)2812633$$aLudolph, Albert C$$b4$$udzne
000275878 7001_ $$aRasche, Volker$$b5
000275878 7001_ $$aVernikouskaya, Ina$$b6
000275878 7001_ $$aMüller, Hans-Peter$$b7
000275878 773__ $$0PERI:(DE-600)2615211-3$$a10.1038/s41598-025-85786-6$$gVol. 15, no. 1, p. 1551$$n1$$p1551$$tScientific reports$$v15$$x2045-2322$$y2025
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