Comparison of Two Clinical Upper Motor Neuron Burden Rating Scales in ALS Using Quantitative Brain Imaging

摘要: Several clinical upper motor neuron burden scales (UMNSs) variably measure brain dysfunction in amyotrophic lateral sclerosis (ALS). Here, we compare relationship of two widely used UMNSs ALS (Penn and MGH UMNSs) with (a) neuroimaging markers (b) neurological impairment status using the gold-standard functional measure, revised Functional Rating Scale (ALSFRS-R). UMNS measures hyperreflexia alone, Penn hyperreflexia, spasticity, pseudobulbar affect. Twenty-eight participants underwent both UMNSs, at a matching time-point as simultaneous [11C]PBR28 positron emission tomography (PBR28-PET)/Magnetic Resonance scan ALSFRS-R. The were compared for localization strength association of: neuroinflammation, PBR28-PET MR Spectroscopy metabolites (myo-inositol choline) corticospinal axonal loss, fractional anisotropy (FA), metabolite (N-acetylaspartate). Among UMN manifestations, segmental affect occurred 100, 43, 18% participants, respectively. Pseudobulbar did not map to any specific regional dysfunction, while spasticity subdomains significantly correlated colocalized neurobiological changes pathways on whole voxel-wise analyses. Both total scores showed significant similar (PBR28-PET, FA, metabolites) primary cortices severity decline Hyperreflexia is most frequent manifestation correlates best molecular imaging ALS. UMNS's subdomains, carries weight biological A scale comprising items alone clinically relevant sufficient predict highest yield abnormalities