Measurement of spinal cord atrophy using phase sensitive inversion recovery (PSIR) imaging in motor neuron disease

• Published in: PloS one Vol. 13; no. 11; p. e0208255
• Main Authors: Olney, Nicholas T., Bischof, Antje, Rosen, Howard, Caverzasi, Eduardo, Stern, William A., Lomen-Hoerth, Catherine, Miller, Bruce L., Henry, Roland G., Papinutto, Nico
• Format: Journal Article
• Language: English
• Published: United States Public Library of Science 29.11.2018; Public Library of Science (PLoS)
• Subjects: Adult; Aged; Aging; Amyotrophic lateral sclerosis; Amyotrophic Lateral Sclerosis - diagnostic imaging; Amyotrophic Lateral Sclerosis - pathology; Atrophy; Atrophy - diagnostic imaging; Atrophy - pathology; Automation; Biology and Life Sciences; Correlation; Dementia; Dementia disorders; Diagnosis; Diagnostic imaging; Disease; Female; Frontotemporal dementia; Frontotemporal Dementia - diagnostic imaging; Frontotemporal Dementia - pathology; Gray Matter - diagnostic imaging; Gray Matter - pathology; Humans; Image acquisition; Image Processing, Computer-Assisted; Intervertebral discs; Inversion; Life expectancy; Linear models (Statistics); Magnetic Resonance Imaging; Male; Medical imaging; Medical prognosis; Medicine and Health Sciences; Memory; Middle Aged; Motor neuron disease; Motor Neuron Disease - diagnostic imaging; Motor Neuron Disease - pathology; Motor neuron diseases; Motor neurone disease; Motors; Multiple sclerosis; Muscular atrophy; Neurology; Neurons; Patients; Phenotypes; Prognosis; Recovery; Regression analysis; Research and Analysis Methods; Spinal cord; Spinal Cord - diagnostic imaging; Spinal Cord - pathology; Spinal cord diseases; Substantia alba; Substantia grisea; Transcranial magnetic stimulation; White Matter - diagnostic imaging; White Matter - pathology; United States > US; San Francisco California; California
• ISSN: 1932-6203; 1932-6203
• DOI: 10.1371/journal.pone.0208255

The spectrum of motor neuron disease (MND) includes numerous phenotypes with various life expectancies. The degree of upper and lower motor neuron involvement can impact prognosis. Phase sensitive inversion recovery (PSIR) imaging has been shown to detect in vivo gray matter (GM) and white matter (WM) atrophy in the spinal cord of other patient populations but has not been explored in MND. In this study, total cord, WM and GM areas of ten patients with a diagnosis within the MND spectrum were compared to those of ten healthy controls (HC). Patients' diagnosis included amyotrophic lateral sclerosis (ALS), primary lateral sclerosis, primary muscular atrophy, facial onset sensory and motor neuronopathy and ALS-Frontotemporal dementia. Axial 2D PSIR images were acquired at four cervical disc levels (C2-C3, C3-C4, C5-C6 and C7-T1) with a short acquisition time (2 minutes) protocol. Total cross-sectional areas (TCA), GM and WM areas were measured using a combination of highly reliable manual and semi-automated methods. Cord areas in MND patients were compared with HC using linear regression analyses adjusted for age and sex. Correlation of WM and GM areas in MND patients was explored to gain insights into underlying atrophy patterns. MND patients as a group had significantly smaller cervical cord GM area compared to HC at all four levels (C2-C3: p = .009; C3-C4: p = .001; C5-C6: p = .006; C7-T1: p = .002). WM area at C5-C6 level was significantly smaller (p = .001). TCA was significantly smaller at C3-C4 (p = .018) and C5-C6 (p = .002). No significant GM and WM atrophy was detected in the two patients with predominantly bulbar phenotype. Concomitant GM and WM atrophy was detected in solely upper or lower motor neuron level phenotypes. There was a significant correlation between GM and WM areas at all four levels in this diverse population of MND. Spinal cord GM and WM atrophy can be detected in vivo in patients within the MND spectrum using a short acquisition time 2D PSIR imaging protocol. PSIR imaging shows promise as a method for quantifying spinal cord involvement and thus may be useful for diagnosis, prognosis and for monitoring disease progression.