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Hypothalamic atrophy in primary lateral sclerosis, assessed by convolutional neural network-based automatic

Jan Kassubek¹, Francesco Roselli^1,2, Simon Witzel¹

¹Dept. of Neurology, University of Ulm, Oberer Eselsberg 45, 89081, Ulm, Germany.

Scientific Reports

|January 9, 2025

Summary

This summary is machine-generated.

Primary lateral sclerosis (PLS) and amyotrophic lateral sclerosis (ALS) patients exhibit reduced hypothalamus volume compared to controls. This study found similar hypothalamic volume reductions in both PLS and ALS, despite their differing clinical presentations.

Keywords:

Amyotrophic Lateral Sclerosis Hypothalamus Magnetic Resonance Imaging Metabolism Neuronal Networks Primary Lateral Sclerosis Volumetry

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Area of Science:

Neuroscience
Radiology
Medical Imaging

Background:

Primary lateral sclerosis (PLS) is a slowly progressive motor neuron disease (MND) primarily affecting upper motor neurons.
Amyotrophic lateral sclerosis (ALS), another MND, involves catabolism and abnormal energy metabolism, with hypothalamic atrophy noted in its progression.
The limited weight loss in PLS suggests potentially less severe hypothalamic alterations compared to ALS, prompting further investigation.

Purpose of the Study:

To quantitatively assess hypothalamic volume in patients with PLS.
To compare hypothalamic volumes in PLS patients against both healthy controls and patients with classical ALS.
To investigate potential differences in hypothalamic alterations between PLS and ALS, given their distinct clinical phenotypes.

Main Methods:

Utilized a convolutional neural network (CNN) with a U-Net architecture for automated segmentation of the hypothalamus and intracranial volume (ICV).
Performed volumetric analysis on high-resolution T1-weighted MRI scans from 46 PLS patients, 107 healthy controls, and 411 classical ALS patients.
Normalized hypothalamic volumes by ICV to account for variations in head size between subjects.

Main Results:

Significant hypothalamic volume reduction was observed in PLS patients (818 ± 73 mm³) compared to controls (852 ± 77 mm³).
Significant hypothalamic volume reduction was also confirmed in ALS patients (823 ± 84 mm³), consistent with prior research.
No significant differences in normalized hypothalamic volumes were found between ALS and PLS patient groups at the group level.

Conclusions:

This study demonstrates that both PLS and ALS patients exhibit reduced hypothalamic volumes.
The findings indicate similar hypothalamic volume reductions in PLS and ALS, irrespective of their distinct clinical characteristics.
Automated CNN-based quantification provides an unbiased method for assessing hypothalamic volume in neurodegenerative diseases.