scholarly journals Hyperexcitability as a potential cause for diffuse lower motor neuron loss in Isaacs’ syndrome

2013 ◽  
Vol 1 (5) ◽  
pp. 179-181 ◽  
Author(s):  
Takahiro Furukawa ◽  
Hiroyuki Nodera ◽  
Yoshimitsu Shimatani ◽  
Osamu Watanabe ◽  
Ai Miyashiro ◽  
...  
Keyword(s):  
Motor Neuron ◽  
Lower Motor Neuron ◽  
Neuron Loss ◽  
Motor Neuron Loss ◽  
Isaacs Syndrome

scholarly journals Blood-spinal cord barrier leakage is independent of motor neuron pathology in ALS

2019 ◽  
Author(s):  
Sarah Waters ◽  
Birger V. Dieriks ◽  
Molly E. V. Swanson ◽  
Yibin Zhang ◽  
Natasha L. Grimsey ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Motor Neuron ◽  
Lower Motor Neuron ◽  
Spinal Cord Tissue ◽  
Thoracic Spinal Cord ◽  
Neuron Loss ◽  
Thoracic Spinal ◽  
Potential Biomarker ◽  
Motor Neuron Loss ◽  
Blood Spinal Cord Barrier

AbstractBackgroundAmyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease involving progressive degeneration of upper and lower motor neurons. Both lower motor neuron loss and the deposition of phosphorylated TDP-43 inclusions display regional patterning along the spinal cord. The blood-spinal cord barrier (BSCB) ordinarily restricts entry into the spinal cord parenchyma of blood components that are neurotoxic, but in ALS there is evidence for barrier breakdown. Here we sought to examine whether BSCB breakdown, motor neuron loss, and TDP-43 proteinopathy display the same regional patterning across and along the spinal cord.MethodsWe measured cerebrospinal fluid (CSF) hemoglobin in living ALS patients (n=87 controls, n=236 ALS) as a potential biomarker of BSCB and blood-brain barrier leakage. We then immunostained cervical, thoracic, and lumbar post mortem spinal cord tissue (n=5 controls, n=13 ALS) and employed semi-automated imaging and analysis to quantify and map lower motor neuron loss and phosphorylated TDP-43 inclusion load against hemoglobin leakage.ResultsMotor neuron loss and TDP-43 proteinopathy were seen at all three levels of the ALS spinal cord, with most abundant TDP-43 deposition in the ventral grey (lamina IX) of the cervical and lumbar cord. In contrast, hemoglobin leakage was observed along the ALS spinal cord axis but was most severe in the dorsal grey and white matter in the thoracic spinal cord.ConclusionsOur data show that leakage of the blood-spinal cord barrier occurs during life but at end-stage its distribution is independent from the major motor neuron pathology and is unlikely to be a major contributor to pathogenesis in ALS.

Lower motor neuron loss in multiple sclerosis and experimental autoimmune encephalomyelitis

2009 ◽  
Vol 66 (3) ◽  
pp. 310-322 ◽  
Author(s):  
Johannes Vogt ◽  
Friedemann Paul ◽  
Orhan Aktas ◽  
Kathrin Müller-Wielsch ◽  
Jan Dörr ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Experimental Autoimmune Encephalomyelitis ◽  
Motor Neuron ◽  
Lower Motor Neuron ◽  
Autoimmune Encephalomyelitis ◽  
Neuron Loss ◽  
Motor Neuron Loss ◽  
Experimental Autoimmune

scholarly journals Blood-spinal cord barrier leakage is independent of motor neuron pathology in ALS

2021 ◽  
Vol 9 (1) ◽  
Author(s):  
Sarah Waters ◽  
Molly E. V. Swanson ◽  
Birger V. Dieriks ◽  
Yibin B. Zhang ◽  
Natasha L. Grimsey ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Motor Neuron ◽  
Lower Motor Neuron ◽  
Spinal Cord Tissue ◽  
Thoracic Spinal Cord ◽  
Neuron Loss ◽  
Thoracic Spinal ◽  
Potential Biomarker ◽  
Motor Neuron Loss ◽  
Blood Spinal Cord Barrier

AbstractAmyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease involving progressive degeneration of upper and lower motor neurons. The pattern of lower motor neuron loss along the spinal cord follows the pattern of deposition of phosphorylated TDP-43 aggregates. The blood-spinal cord barrier (BSCB) restricts entry into the spinal cord parenchyma of blood components that can promote motor neuron degeneration, but in ALS there is evidence for barrier breakdown. Here we sought to quantify BSCB breakdown along the spinal cord axis, to determine whether BSCB breakdown displays the same patterning as motor neuron loss and TDP-43 proteinopathy. Cerebrospinal fluid hemoglobin was measured in living ALS patients (n = 87 control, n = 236 ALS) as a potential biomarker of BSCB and blood–brain barrier leakage. Cervical, thoracic, and lumbar post-mortem spinal cord tissue (n = 5 control, n = 13 ALS) were then immunolabelled and semi-automated imaging and analysis performed to quantify hemoglobin leakage, lower motor neuron loss, and phosphorylated TDP-43 inclusion load. Hemoglobin leakage was observed along the whole ALS spinal cord axis and was most severe in the dorsal gray and white matter in the thoracic spinal cord. In contrast, motor neuron loss and TDP-43 proteinopathy were seen at all three levels of the ALS spinal cord, with most abundant TDP-43 deposition in the anterior gray matter of the cervical and lumbar cord. Our data show that leakage of the BSCB occurs during life, but at end-stage disease the regions with most severe BSCB damage are not those where TDP-43 accumulation is most abundant. This suggests BSCB leakage and TDP-43 pathology are independent pathologies in ALS.

Clinicopathological characteristics of FTLD-TDP showing corticospinal tract degeneration but lacking lower motor neuron loss

2010 ◽  
Vol 298 (1-2) ◽  
pp. 70-77 ◽  
Author(s):  
Zen Kobayashi ◽  
Kuniaki Tsuchiya ◽  
Tetsuaki Arai ◽  
Osamu Yokota ◽  
Mari Yoshida ◽  
...  
Keyword(s):  
Motor Neuron ◽  
Corticospinal Tract ◽  
Clinicopathological Characteristics ◽  
Lower Motor Neuron ◽  
Neuron Loss ◽  
Motor Neuron Loss

Implications of ALS focality: Rostral-caudal distribution of lower motor neuron loss postmortem

Neurology ◽  
2007 ◽  
Vol 68 (19) ◽  
pp. 1576-1582 ◽  
Author(s):  
J. Ravits ◽  
P. Laurie ◽  
Y. Fan ◽  
D. H. Moore
Keyword(s):  
Motor Neuron ◽  
Lower Motor Neuron ◽  
Neuron Loss ◽  
Motor Neuron Loss

Use of CMAP, MScan fit-MUNE, and MUNIX in understanding neurodegeneration pattern of ALS and detection of early motor neuron loss in daily practice

2021 ◽  
Vol 741 ◽  
pp. 135488
Author(s):  
Taskin Gunes ◽  
Nermin Gorkem Sirin ◽  
Sevki Sahin ◽  
Ercan Kose ◽  
Baris Isak
Keyword(s):  
Motor Neuron ◽  
Daily Practice ◽  
Neuron Loss ◽  
Motor Neuron Loss

scholarly journals Bisperoxovanadium promotes motor neuron survival and neuromuscular innervation in amyotrophic lateral sclerosis

2021 ◽  
Vol 14 (1) ◽  
Author(s):  
Junmei Wang ◽  
Lydia Tierney ◽  
Ranjeet Mann ◽  
Thomas Lonsway ◽  
Chandler L. Walker
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Motor Neuron ◽  
Lumbar Spinal Cord ◽  
Therapeutic Effects ◽  
Spinal Cord Tissue ◽  
Neuron Loss ◽  
Neuroprotective Therapy ◽  
Motor Neuron Loss ◽  
Lateral Sclerosis

AbstractAmyotrophic lateral sclerosis (ALS) is the most common motor neuron (MN) disease, with no present cure. The progressive loss of MNs is the hallmark of ALS. We have previously shown the therapeutic effects of the phosphatase and tensin homolog (PTEN) inhibitor, potassium bisperoxo (picolinato) vanadium (bpV[pic]), in models of neurological injury and demonstrated significant neuroprotective effects on MN survival. However, accumulating evidence suggests PTEN is detrimental for MN survival in ALS. Therefore, we hypothesized that treating the mutant superoxide dismutase 1 G93A (mSOD1G93A) mouse model of ALS during motor neuron degeneration and an in vitro model of mSOD1G93A motor neuron injury with bpV(pic) would prevent motor neuron loss. To test our hypothesis, we treated mSOD1G93A mice intraperitoneally daily with 400 μg/kg bpV(pic) from 70 to 90 days of age. Immunolabeled MNs and microglial reactivity were analyzed in lumbar spinal cord tissue, and bpV(pic) treatment significantly ameliorated ventral horn motor neuron loss in mSOD1G93A mice (p = 0.003) while not significantly altering microglial reactivity (p = 0.701). Treatment with bpV(pic) also significantly increased neuromuscular innervation (p = 0.018) but did not affect muscle atrophy. We also cultured motor neuron-like NSC-34 cells transfected with a plasmid to overexpress mutant SOD1G93A and starved them in serum-free medium for 24 h with and without bpV(pic) and downstream inhibitor of Akt signaling, LY294002. In vitro, bpV(pic) improved neuronal viability, and Akt inhibition reversed this protective effect (p < 0.05). In conclusion, our study indicates systemic bpV(pic) treatment could be a valuable neuroprotective therapy for ALS.

Longitudinal study of functional spinal alpha motor neuron loss in amyotrophic lateral sclerosis

2002 ◽  
Vol 25 (4) ◽  
pp. 520-526 ◽  
Author(s):  
K. Arasaki ◽  
Y. Kato ◽  
A. Hyodo ◽  
R. Ushijima ◽  
M. Tamaki
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Longitudinal Study ◽  
Motor Neuron ◽  
Neuron Loss ◽  
Motor Neuron Loss ◽  
Alpha Motor Neuron ◽  
Lateral Sclerosis

scholarly journals Stress exacerbates neuron loss and microglia proliferation in a rat model of excitotoxic lower motor neuron injury

2015 ◽  
Vol 49 ◽  
pp. 246-254 ◽  
Author(s):  
Denise A. Puga ◽  
C. Amy Tovar ◽  
Zhen Guan ◽  
John C. Gensel ◽  
Matthew S. Lyman ◽  
...  
Keyword(s):  
Motor Neuron ◽  
Rat Model ◽  
Lower Motor Neuron ◽  
Neuron Loss ◽  
Neuron Injury ◽  
Motor Neuron Injury

scholarly journals Diaphragm muscle function following midcervical contusion injury in rats

2019 ◽  
Vol 126 (1) ◽  
pp. 221-230 ◽  
Author(s):  
Obaid U. Khurram ◽  
Matthew J. Fogarty ◽  
Sabhya Rana ◽  
Pangdra Vang ◽  
Gary C. Sieck ◽  
...  
Keyword(s):  
Motor Neuron ◽  
Motor Neurons ◽  
Force Generation ◽  
Diaphragm Muscle ◽  
Neuron Loss ◽  
Motor Behaviors ◽  
Contusion Injury ◽  
Motor Neuron Loss ◽  
The Impact ◽  
Over Time

Midcervical spinal cord contusion injury results in tissue damage, disruption of spinal pathways, and motor neuron loss. Unilateral C4 contusion results in loss of 40%–50% of phrenic motor neurons ipsilateral to the injury (~25% of the total phrenic motor neuron pool). Over time after unilateral C4 contusion injury, diaphragm muscle (DIAm) electromyogram activity increases both contralateral and ipsilateral to the side of injury in rats, suggesting compensation because of increased activation of the surviving motor neurons. However, the impact of contusion injury on DIAm force generation is less clear. Transdiaphragmatic pressure (Pdi) was measured across motor behaviors over time after unilateral C4 contusion injury in adult male Sprague-Dawley rats. Maximum Pdi (Pdimax) was elicited by bilateral phrenic nerve stimulation at 7 days postinjury. We hypothesized that Pdimax is reduced following unilateral C4 contusion injury, whereas ventilatory behaviors of the DIAm are unimpaired. In support of our hypothesis, Pdimax was reduced by ~25% after unilateral C4 contusion, consistent with the extent of phrenic motor neuron loss following contusion injury. One day after contusion injury, the Pdi amplitude during airway occlusion was reduced from ~30 to ~20 cmH2O, but this reduction was completely reversed by 7 days postinjury. Ventilatory behaviors (~10 cmH2O), DIAm-specific force, and muscle fiber cross-sectional area did not differ between the laminectomy and contusion groups. These results indicate that the large reserve capacity for DIAm force generation allows for higher-force motor behaviors to be accomplished despite motor neuron loss, likely reflecting changes in motor unit recruitment. NEW & NOTEWORTHY Respiratory muscles such as the diaphragm generate the pressures necessary to accomplish a variety of motor behaviors ranging from ventilation to near-maximal expulsive behaviors. However, the impact of contusion injury on diaphragm pressure generation across behaviors is not clear. The present study shows that contusion injury impairs maximal pressure generation while preserving the ability of the diaphragm to accomplish lower-force motor behaviors, likely reflecting changes in diaphragm motor unit recruitment.

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