Upper cervical spinal cord atrophy as a sensitive marker of amyotrophic lateral sclerosis

2017 ◽  
Vol 381 ◽  
pp. 717
Author(s):  
Y. Tsuzuki ◽  
T. Takeda ◽  
M. Iijima ◽  
Y. Shimizu ◽  
Y. Hiroshi ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Amyotrophic Lateral Sclerosis ◽  
Cervical Spinal Cord ◽  
Spinal Cord Atrophy ◽  
Upper Cervical ◽  
Lateral Sclerosis ◽  
Sensitive Marker

scholarly journals The possible meaning of fractional anisotropy measurement of the cervical spinal cord in correct diagnosis of amyotrophic lateral sclerosis

2015 ◽  
Vol 37 (3) ◽  
pp. 417-421 ◽  
Author(s):  
Slawomir Budrewicz ◽  
Pawel Szewczyk ◽  
Joanna Bladowska ◽  
Ryszard Podemski ◽  
Ewa Koziorowska-Gawron ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Amyotrophic Lateral Sclerosis ◽  
Fractional Anisotropy ◽  
Cervical Spinal Cord ◽  
Correct Diagnosis ◽  
Anisotropy Measurement ◽  
Lateral Sclerosis

Spinal cord atrophy correlates with disease severity in amyotrophic lateral sclerosis

2013 ◽  
Vol 333 ◽  
pp. e447-e448
Author(s):  
H.M.T. Andrade ◽  
L.M.T. Branco ◽  
M. Albuquerque ◽  
F.P.G. Bergo ◽  
A. Nucci ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Amyotrophic Lateral Sclerosis ◽  
Disease Severity ◽  
Spinal Cord Atrophy ◽  
Lateral Sclerosis

Higher incidence of cervical spinal cord compression in amyotrophic lateral sclerosis: a single-institute cohort study

2021 ◽  
Author(s):  
Kosuke Matsuzono ◽  
Masayuki Suzuki ◽  
Kumiko Miura ◽  
Tadashi Ozawa ◽  
Takafumi Mashiko ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Amyotrophic Lateral Sclerosis ◽  
Cohort Study ◽  
Spinal Cord Compression ◽  
Cervical Spinal Cord ◽  
Cord Compression ◽  
Lateral Sclerosis

scholarly journals Microglia RAGE exacerbates the progression of neurodegeneration within the SOD1G93A murine model of amyotrophic lateral sclerosis in a sex-dependent manner

2021 ◽  
Vol 18 (1) ◽  
Author(s):  
Michael MacLean ◽  
Judyta Juranek ◽  
Swetha Cuddapah ◽  
Raquel López-Díez ◽  
Henry H. Ruiz ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Amyotrophic Lateral Sclerosis ◽  
Murine Model ◽  
Intercellular Communication ◽  
Cervical Spinal Cord ◽  
Disease Onset ◽  
Lumbar Spinal Cord ◽  
Dependent Manner ◽  
Als Patients ◽  
Lateral Sclerosis

Abstract Background Burgeoning evidence highlights seminal roles for microglia in the pathogenesis of neurodegenerative diseases including amyotrophic lateral sclerosis (ALS). The receptor for advanced glycation end products (RAGE) binds ligands relevant to ALS that accumulate in the diseased spinal cord and RAGE has been previously implicated in the progression of ALS pathology. Methods We generated a novel mouse model to temporally delete Ager from microglia in the murine SOD1G93A model of ALS. Microglia Ager deficient SOD1G93A mice and controls were examined for changes in survival, motor function, gliosis, motor neuron numbers, and transcriptomic analyses of lumbar spinal cord. Furthermore, we examined bulk-RNA-sequencing transcriptomic analyses of human ALS cervical spinal cord. Results Transcriptomic analysis of human cervical spinal cord reveals a range of AGER expression in ALS patients, which was negatively correlated with age at disease onset and death or tracheostomy. The degree of AGER expression related to differential expression of pathways involved in extracellular matrix, lipid metabolism, and intercellular communication. Microglia display increased RAGE immunoreactivity in the spinal cords of high AGER expressing patients and in the SOD1G93A murine model of ALS vs. respective controls. We demonstrate that microglia Ager deletion at the age of symptomatic onset, day 90, in SOD1G93A mice extends survival in male but not female mice. Critically, many of the pathways identified in human ALS patients that accompanied increased AGER expression were significantly ameliorated by microglia Ager deletion in male SOD1G93A mice. Conclusions Our results indicate that microglia RAGE disrupts communications with cell types including astrocytes and neurons, intercellular communication pathways that divert microglia from a homeostatic to an inflammatory and tissue-injurious program. In totality, microglia RAGE contributes to the progression of SOD1G93A murine pathology in male mice and may be relevant in human disease.

Spinal cord atrophy correlates with disease duration and severity in amyotrophic lateral sclerosis

2013 ◽  
Vol 15 (1-2) ◽  
pp. 93-97 ◽  
Author(s):  
Lucas M. T. Branco ◽  
Milena De Albuquerque ◽  
Helen Maia T. De Andrade ◽  
Felipe P. G. Bergo ◽  
Anamarli Nucci ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Amyotrophic Lateral Sclerosis ◽  
Disease Duration ◽  
Spinal Cord Atrophy ◽  
Lateral Sclerosis

scholarly journals Cervical spinal cord and motor unit pathology in a canine model of SOD1-associated amyotrophic lateral sclerosis

2017 ◽  
Vol 378 ◽  
pp. 193-203 ◽  
Author(s):  
Martin L. Katz ◽  
Cheryl A. Jensen ◽  
Jeffrey T. Student ◽  
Gayle C. Johnson ◽  
Joan R. Coates
Keyword(s):  
Spinal Cord ◽  
Amyotrophic Lateral Sclerosis ◽  
Motor Unit ◽  
Cervical Spinal Cord ◽  
Canine Model ◽  
Lateral Sclerosis
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