Imaging in Motor Neuron Diseases & Spinal Muscular Atrophy

Introduction

We are interested in developing novel imaging biomarkers for motor neuron diseases, i. e. amyotrophic lateral sclerosis, primary lateral sclerosis, spinal muscular atrophy, post-polio syndrome.

Currently several prospective imaging studies are ongoing that evaluate novel sequences and MR approaches developed by our partners in MR physic (Prof. Olivier Bieri, Magnetic Resonance Physics & Methodology and PD Dr. Santini, Basel Muscle MRI).

We also conduct post-mortem studies to validate these novel MR-parameters in collaboration with the research groups of Prof. Eva Scheuer and PD Dr. Claudia Lenz, University of Basel.

Selected scientific publication

The Lateral Corticospinal Tract Sign: An MRI Marker for Amyotrophic Lateral Sclerosis read

The lateral corticospinal tract (CST) sign visible on radially sampled averaged magnetization inversion-recovery acquisition (rAMIRA) MRI at the intervertebral disk level C4-5 in a 69-year-old male patient with amyotrophic lateral sclerosis and an upper motor neuron predominant disease type. (Adapted from Wendebourg et al., Radiology 2024)

Two-dimensional axial radially sampled averaged magnetization inversion-recovery acquisition images in a 50-year-old female patient with amyotrophic lateral sclerosis show the lateral corticospinal tract (CST) sign (in-plane resolution 0.5 × 0.5 mm, level C4-5) with (left) positive CST sign (arrowhead) and (right) localization of the CST (marked area). (Adapted from Wendebourg et al., Radiology 2024)

Recent scientific publications and talks

Novel Biomarkers for Spinal muscular atrophy

Kesenheimer EM, Wendebourg MJ, Weidensteiner C, Sander L, Weigel M, Haas T, Fischer D, Neuwirth C, Braun N, Weber M, Granziera C, Sinnreich M, Bieri O, Schlaeger R.
Spinal Cord Gray matter atrophy is associated with disability in Spinal muscular atrophy.
Journal of Neurology. 2024. acc for publication.

Kesenheimer EM, Wendebourg MJ, Sander L, Neuwirth C, Braun N, Weber M, Orleth A, Maleska A, Sinnreich M, Kuhle J, Schlaeger R.
Serum glial fibrilally acidic protein is associated with disability in adult spinal muscular atrophy.
EAN Helsinki 2024, oral

Steiner L, Tscherter A, Henzi B, Branca M, Carda S, Enzmann C, Fluss J, Jacquier D, Neuwirth C, Ripellino P, Scheidegger O, Schlaeger R, Schreiner B, Stettner GM, Klein A; Swiss-Reg-NMD Group.
Chronic Pain in Patients with Spinal Muscular Atrophy in Switzerland: A Query to the Spinal Muscular Atrophy Registry.
J Clin Med. 2024 May 9;13(10):2798. doi: 10.3390/jcm13102798. PMID: 38792340; PMCID: PMC11122245. read

Kesenheimer E, Wendebourg JM, Weidensteiner C, Sander L, Weigel M, Haas T, Fischer D, Neuwirth C, Braun N, Weber M, Granziera C, Sinnreich M, Bieri O, Schlaeger R.
Cervical Spinal Cord Gray Matter Area is Associated with Motor Unit Number Index in Spinal Muscular Atrophy.
Oral Presentation AAN 4/2023, Boston. S33.008

Kesenheimer EM, Wendebourg MJ, Weigel M, Weidensteiner C, Haas T, Richter L, Sander L, Horvath A, Barakovic M, Cattin P, Granziera C, Bieri O, Schlaeger R.
Normalization of Spinal Cord Total Cross-Sectional and Gray Matter Areas as Quantified With Radially Sampled Averaged Magnetization Inversion Recovery Acquisitions.
Front Neurol. 2021 Mar 25;12:637198. doi: 10.3389/fneur.2021.637198. PMID: 33841307; PMCID: PMC8027254. read

Novel Biomarkers for Amyotrophic Lateral Sclerosis

Wendebourg MJ, Kesenheimer E, Sander L, Weigel M, Weidensteiner C, Haas T, Madoerin P, Diebold M, Deigendesch N, Neuhaus D, Naumann N, Neuwirth C, Braun N, Weber M, Granziera C, Scheurer E, Lenz C, Schweikert K, Sinnreich M, Lieb J, Bieri O, Schlaeger R.
The Lateral Corticospinal Tract Sign: An MRI Marker for Amyotrophic Lateral Sclerosis.
Radiology. 2024 Sep;312(3):e231630. doi: 10.1148/radiol.231630. PMID: 39287519. read

Wendebourg MJ, Weigel M, Weidensteiner C, Sander L, Kesenheimer E, Naumann N, Haas T, Madoerin P, Braun N, Neuwirth C, Weber M, Jahn K, Kappos L, Granziera C, Schweikert K, Sinnreich M, Bieri O, Schlaeger R.
Cervical and thoracic spinal cord gray matter atrophy is associated with disability in patients with amyotrophic lateral sclerosis.
Eur J Neurol. 2024 Jun;31(6):e16268. doi:10.1111/ene.16268. Epub 2024 Mar 11. PMID: 38465478; PMCID: PMC11235652. read

SAYN Award, bestes e- Poster: SFCNS for Janina Wendebourg, PhD Student.
The spinal cord lateral tract sign as an rAMIRA based MRI sign for upper motor neuron involvement in Amyotrophic Lateral Sclerosis in a clinical setting.
Clin. Transl. Neurosci. 2022, 6(3), 22;

Weigel M, Haas T, Wendebourg MJ, Schlaeger R, Bieri O.
Imaging of the thoracic spinal cord using radially sampled averaged magnetization inversion recovery acquisitions.
J Neurosci Methods. 2020 Sep 1;343:108825. doi: 10.1016/j.jneumeth.2020.108825. Epub 2020 Jun 21. PMID: 32580062. read

Post mortal-validation of Imaging Biomarkers in Motor Neuron Diseases

Neuhaus D, Rost T, Haas T, Wendebourg MJ, Schulze K, Schlaeger R, Scheurer E, Lenz C.
Comparative analysis of in situ and ex situ postmortem brain MRI: Evaluating volumetry, DTI, and relaxometry.
Magn Reson Med. 2025 Jan;93(1):213-227. doi: 10.1002/mrm.30264. Epub 2024 Sep 9. PMID: 39250425.

Post-Polio Syndrome

Wendebourg MJ, Weigel M, Richter L, Gocheva V, Hafner P, Orsini AL, Crepulja V, Schmidt S, Huck A, Oechtering J, Blatow M, Haas T, Granziera C, Kappos L, Cattin P, Bieri O, Fischer D, Schlaeger R.
Spinal cord gray matter atrophy is associated with functional decline in post-polio syndrome.
Eur J Neurol. 2022 May;29(5):1435-1445. doi: 10.1111/ene.15261. Epub 2022 Mar 3. PMID: 35102676; PMCID: PMC9310958. read

Wendebourg MJ, Nagy S, Derfuss T, Parmar K, Schlaeger R.
Magnetic resonance imaging in immune-mediated myelopathies.
J Neurol. 2020 May;267(5):1233-1244. doi: 10.1007/s00415-019-09206-2. PMID: 30694379. read

Research Group Leader: Prof. Dr. med. Regina Schlaeger
Dr. Dr. Janina Wendebourg
Dr. Eva Kesenheimer
Dr. Laura Sander
Renee Jetzer
Nicola Bohnert
Simone Penker
Valentina Konjevod

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