Cervical and thoracic spinal cord gray matter atrophy is associated with disability in patients with amyotrophic lateral sclerosis

Author:

Wendebourg Maria Janina1234ORCID,Weigel Matthias12345,Weidensteiner Claudia56,Sander Laura1234,Kesenheimer Eva1234,Naumann Nicole1,Haas Tanja5,Madoerin Philipp5,Braun Nathalie7,Neuwirth Christoph7,Weber Markus7,Jahn Kathleen8,Kappos Ludwig34,Granziera Cristina1234,Schweikert Kathi1,Sinnreich Michael19,Bieri Oliver56,Schlaeger Regina1234ORCID

Affiliation:

1. Department of Neurology University Hospital Basel and University of Basel Basel Switzerland

2. Department of Clinical Research University Hospital Basel, University of Basel Basel Switzerland

3. Department of Biomedical Engineering, Translational Imaging in Neurology (ThINk) University of Basel Basel Switzerland

4. Research Center for Clinical Neuroimmunology and Neuroscience Basel (RC2NB) University of Basel Basel Switzerland

5. Division of Radiological Physics, Department of Radiology University Hospital Basel Basel Switzerland

6. Department of Biomedical Engineering University of Basel Basel Switzerland

7. Neuromuscular Diseases Unit/ALS Clinic Kantonsspital Sankt Gallen St. Gallen Switzerland

8. Clinics of Respiratory Medicine University Hospital Basel and University of Basel Basel Switzerland

9. Department of Biomedicine (DBE) University of Basel Basel Switzerland

Abstract

AbstractBackground and purposeIn amyotrophic lateral sclerosis (ALS), there is an unmet need for more precise patient characterization through quantitative, ideally operator‐independent, assessments of disease extent and severity. Radially sampled averaged magnetization inversion recovery acquisitions (rAMIRA) magnetic resonance imaging enables gray matter (GM) and white matter (WM) area quantitation in the cervical and thoracic spinal cord (SC) with optimized contrast. We aimed to investigate rAMIRA‐derived SC GM and SC WM areas and their association with clinical phenotype and disability in ALS.MethodsA total of 36 patients with ALS (mean [SD] age 61.7 [12.6] years, 14 women) and 36 healthy, age‐ and sex‐matched controls (HCs; mean [SD] age 63.1 [12.1] years, 14 women) underwent two‐dimensional axial rAMIRA imaging at the inter‐vertebral disc levels C2/3–C5/C6 and the lumbar enlargement level Tmax. ALS Functional Rating Scale–revised (ALSFRS‐R) score, muscle strength, and sniff nasal inspiratory pressure (SNIP) were assessed.ResultsCompared to HCs, GM and WM areas were reduced in patients at all cervical levels (p < 0.0001). GM area (p = 0.0001), but not WM area, was reduced at Tmax. Patients with King's Stage 3 showed significant GM atrophy at all levels, while patients with King's Stage 1 showed significant GM atrophy selectively at Tmax. SC GM area was significantly associated with muscle force at corresponding myotomes. GM area at C3/C4 was associated with ALSFRS‐R (p < 0.001) and SNIP (p = 0.0016).ConclusionPatients with ALS assessed by rAMIRA imaging show significant cervical and thoracic SC GM and SC WM atrophy. SC GM area correlates with muscle strength and clinical disability. GM area reduction at Tmax may be an early disease sign. Longitudinal studies are warranted.

Publisher

Wiley

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