Brain imaging signatures in amyotrophic lateral sclerosis: Correlation with peripheral motor degeneration

Author:

Hsueh Sung‐Ju12ORCID,Chao Chi‐Chao2ORCID,Chen Ta‐Fu2ORCID,Chen Ya‐Fang3ORCID,Hsueh Hsueh‐Wen24ORCID,Tsai Li‐Kai25ORCID,Wu Wen‐Chau36ORCID,Hsieh Sung‐Tsang2478ORCID

Affiliation:

1. Department of Neurology National Taiwan University Hospital Yunlin Branch Douliu City Yunlin County Taiwan

2. Department of Neurology National Taiwan University Hospital Taipei Taiwan

3. Department of Medical Imaging National Taiwan University Hospital Taipei Taiwan

4. Department of Anatomy and Cell Biology National Taiwan University College of Medicine Taipei Taiwan

5. Department of Neurology National Taiwan University Hospital Hsinchu Branch Zhubei City Hsinchu County Taiwan

6. Graduate Institute of Medical Device and Imaging College of Medicine National Taiwan University Hospital Taipei Taiwan

7. Graduate Institute of Clinical Medicine National Taiwan University College of Medicine Taipei Taiwan

8. Center of Precision Medicine National Taiwan University College of Medicine Taipei Taiwan

Abstract

AbstractObjectiveThis study aimed to explore the clinical significance of brain imaging signatures in the context of clinical neurological deficits in association with upper and lower motor neuron degeneration in amyotrophic lateral sclerosis (ALS).MethodsWe performed brain MRI examinations to quantitatively evaluate (1) gray matter volume and (2) white matter tract fractional anisotropy (FA), axial diffusivity (AD), radial diffusivity (RD), and mean diffusivity (MD). Image‐derived indices were correlated with (1) global neurological deficits of MRC muscle strength sum score, revised amyotrophic lateral sclerosis functional rating scale (ALSFRS‐R), and forced vital capacity (FVC), and (2) focal scores of University of Pennsylvania Upper motor neuron score (Penn score) and the summation of compound muscle action potential Z scores (CMAP Z sum score).ResultsThere were 39 ALS patients and 32 control subjects matched for age and gender. Compared to controls, ALS patients had a lower gray matter volume in the precentral gyrus of the primary motor cortex, which was correlated with FA of corticofugal tracts. The gray matter volume of the precentral gyrus was correlated with FVC, MRC sum score, and CMAP Z sum score, while the FA of the corticospinal tract was linearly associated with CMAP Z sum score and Penn score on multivariate linear regression model.InterpretationThis study indicated that clinical assessment of muscle strength and routine measurements on nerve conduction studies provided surrogate markers of brain structural changes for ALS. Furthermore, these findings suggested parallel involvement of both upper and lower motor neurons in ALS.

Funder

Ministry of Science and Technology, Taiwan

Publisher

Wiley

Subject

Neurology (clinical),General Neuroscience

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