scholarly journals Case of hereditary neuropathy with liability to pressure palsies presenting progressive muscular atrophy with lower motor neuron degeneration in the spinal cord and the brainstem

2015 ◽  
Vol 4 (1) ◽  
pp. 19-21 ◽  
Author(s):  
Rie Tohge ◽  
Yuya Shinoto ◽  
Makio Takahashi
Keyword(s):  
Spinal Cord ◽  
Motor Neuron ◽  
Muscular Atrophy ◽  
Lower Motor Neuron ◽  
Hereditary Neuropathy ◽  
Motor Neuron Degeneration ◽  
Neuron Degeneration ◽  
Pressure Palsies ◽  
Progressive Muscular Atrophy

Decreased Spinal Cord cGMP in Murine (Wobbler) Spontaneous Lower Motor Neuron Degeneration

1978 ◽  
Vol 35 (9) ◽  
pp. 590-591 ◽  
Author(s):  
B. R. Brooks ◽  
W. D. Lust ◽  
J. M. Andrews ◽  
W. K. Engel
Keyword(s):  
Spinal Cord ◽  
Motor Neuron ◽  
Lower Motor Neuron ◽  
Motor Neuron Degeneration ◽  
Neuron Degeneration

scholarly journals Rapid generation of ventral spinal cord-like astrocytes from human iPSCs for modeling non-cell autonomous mechanisms of lower motor neuron disease

2021 ◽  
Author(s):  
Vincent Soubannier ◽  
Mathilde Chaineau ◽  
Lale Gursu ◽  
Ghazal Haghi ◽  
Anna Kristyna Franco Flores ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Motor Neuron ◽  
Biological Properties ◽  
Calcium Transients ◽  
Lower Motor Neuron ◽  
Motor Neuron Degeneration ◽  
Neuron Degeneration ◽  
Human Ipscs ◽  
Human Ipsc ◽  
Ventral Spinal Cord

Astrocytes play important roles in the function and survival of neuronal cells. Dysfunctions of astrocytes are associated with numerous disorders and diseases of the nervous system, including motor neuron diseases such as amyotrophic lateral sclerosis (ALS). Human induced pluripotent stem cell (iPSC)-based approaches are becoming increasingly important for the study of the mechanisms underlying the involvement of astrocytes in non-cell autonomous processes of motor neuron degeneration in ALS. These studies must account for the molecular and functional diversity among astrocytes in different regions of the brain and spinal cord. It is essential that the most pathologically-relevant astrocyte preparations are used when investigating non-cell autonomous mechanisms of either upper or lower motor neuron degeneration in ALS. In this context, the main aim of this study was to establish conditions enabling rapid and robust generation of physiologically-relevant ventral spinal cord-like astrocytes that would provide an enhanced experimental model for the study of lower motor neuron degeneration in ALS. Neural progenitor cells with validated caudal and ventral features were derived from human iPSCs and differentiated into astrocytes, which were then characterized by examining morphology, markers of ventral spinal cord astrocytes, spontaneous and induced calcium transients, and astrogliosis markers. Efficient and streamlined generation of human iPSC-derived astrocytes with molecular and biological properties similar to physiological astrocytes in the ventral spinal cord was achieved. These induced astrocytes express markers of mature ventral spinal cord astrocytes, exhibit spontaneous and ATP-induced calcium transients, and lack signs of overt activation. Human iPSC-derived astrocytes with ventral spinal features offer advantages over more generic astrocyte preparations for the study of both ventral spinal cord astrocyte biology and the involvement of astrocytes in mechanisms of lower motor neuron degeneration in ALS.

Case 19

2018 ◽  
Author(s):  
Bashar Katirji
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Motor Neuron ◽  
Motor Neuron Disease ◽  
Muscular Atrophy ◽  
Multifocal Motor Neuropathy ◽  
Lower Motor Neuron ◽  
Motor Neuropathy ◽  
Motor Neuron Degeneration ◽  
Neuron Degeneration ◽  
Lateral Sclerosis

Amyotrophic lateral sclerosis is a fatal neurological disorder, classically presenting with signs of upper motor neuron and lower motor neuron degeneration. Several motor neuron disease variants with purely upper or lower motor neuron degeneration exist. These includes primary lateral sclerosis, progressive muscular atrophy and progressive bulbar palsy. The diagnostic criteria, including El-Escorial criteria and its most recent Awaji revision, are not used in clinical practice and for research purposes. This case highlights the clinical features and electrodiagnostic characteristics of amyotrophic lateral sclerosis. The findings on nerve conduction studies and needle electromyography are emphasized in detail. The role of electrodiagnostic studies in the diagnosis of amyotrophic lateral sclerosis is to establish evidence of lower motor neuron degeneration, confirm its diffuse nature, and exclude treatable causes (such as multifocal motor neuropathy and mimickers of motor neuron disease such as chronic myopathies).

Motor neuron degeneration due to aluminium deposition in the spinal cord: a light microscopical study

1999 ◽  
Vol 101 (2) ◽  
pp. 193-201 ◽  
Author(s):  
T. Tanridag ◽  
T. Coskun ◽  
C. Hürdag ◽  
S. Arbak ◽  
S. Aktan ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Motor Neuron ◽  
Microscopical Study ◽  
Motor Neuron Degeneration ◽  
Neuron Degeneration

scholarly journals Resolution of pathogenic R-loops rescues motor neuron degeneration in spinal muscular atrophy

Brain ◽  
2019 ◽  
Vol 143 (1) ◽  
pp. 2-5
Author(s):  
Niko Hensel ◽  
Nora Tula Detering ◽  
Lisa Marie Walter ◽  
Peter Claus
Keyword(s):  
Spinal Muscular Atrophy ◽  
Motor Neuron ◽  
Muscular Atrophy ◽  
Motor Neuron Degeneration ◽  
Neuron Degeneration

This scientific commentary refers to ‘ZPR1 prevents R-loop accumulation, upregulates SMN2 expression and rescues spinal muscular atrophy’, by Kannan et al. (doi: 10.1093/brain/awz373).

scholarly journals Neuroprotective Effects of Toll-Like Receptor 4 Antagonism in Spinal Cord Cultures and in a Mouse Model of Motor Neuron Degeneration

2012 ◽  
Vol 18 (6) ◽  
pp. 971-981 ◽  
Author(s):  
Massimiliano De Paola ◽  
Alessandro Mariani ◽  
Paolo Bigini ◽  
Marco Peviani ◽  
Giovanni Ferrara ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Mouse Model ◽  
Motor Neuron ◽  
Toll Like Receptor ◽  
Motor Neuron Degeneration ◽  
Toll Like Receptor 4 ◽  
Neuron Degeneration ◽  
Neuroprotective Effects
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