Cerebral Metabolic Changes Induced by an Unconventional Agent: Experimental Model for Some Human Degenerative Diseases of the Central Nervous System

2015 ◽  
pp. 193-203
Author(s):  
N. Gr�goire ◽  
C. Nezri ◽  
J. M. Gorde-Durand ◽  
C. Bouras ◽  
J. Bert ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Experimental Model ◽  
Metabolic Changes ◽  
Degenerative Diseases ◽  
The Central Nervous System

scholarly journals Merosin-positive congenital muscular dystrophy: neuroimaging findings

2007 ◽  
Vol 65 (1) ◽  
pp. 167-169
Author(s):  
André Palma da Cunha Matta ◽  
Márcia de Castro Diniz Gonsalves
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Autosomal Recessive ◽  
Congenital Muscular Dystrophy ◽  
Metabolic Changes ◽  
Resonance Spectroscopy ◽  
Muscle Dystrophy ◽  
Metabolic Disturbances ◽  
The Central Nervous System ◽  
The Brain

Congenital muscle dystrophy (CMD) is a heterogeneous group of autosomal recessive myopathies. It is known that CMD may affect the central nervous system (CNS). Some authors have shown that merosin-negative CMD patients may have encephalic metabolic disturbances. In order to study metabolic changes within the brain, the authors performed a magnetic resonance spectroscopy (MRS) study in a 1-year-old girl with merosin-positive CMD (MP-CMD). MRS of brain demonstrated that NAA/Cr ratio was decreased (1.52), while Cho/Cr ratio was increased (1.78). These findings suggest that metabolic changes in CNS can also be found in patients with MP-CMD.

scholarly journals Are astrocytes executive cells within the central nervous system?

2016 ◽  
Vol 74 (8) ◽  
pp. 671-678 ◽  
Author(s):  
Roberto E. Sica ◽  
Roberto Caccuri ◽  
Cecilia Quarracino ◽  
Francisco Capani
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Synaptic Activity ◽  
Neural Cells ◽  
Degenerative Diseases ◽  
Human Species ◽  
Cerebellar Ataxias ◽  
The Central Nervous System ◽  
Experimental Findings ◽  
Lateral Sclerosis

ABSTRACT Experimental evidence suggests that astrocytes play a crucial role in the physiology of the central nervous system (CNS) by modulating synaptic activity and plasticity. Based on what is currently known we postulate that astrocytes are fundamental, along with neurons, for the information processing that takes place within the CNS. On the other hand, experimental findings and human observations signal that some of the primary degenerative diseases of the CNS, like frontotemporal dementia, Parkinson’s disease, Alzheimer’s dementia, Huntington’s dementia, primary cerebellar ataxias and amyotrophic lateral sclerosis, all of which affect the human species exclusively, may be due to astroglial dysfunction. This hypothesis is supported by observations that demonstrated that the killing of neurons by non-neural cells plays a major role in the pathogenesis of those diseases, at both their onset and their progression. Furthermore, recent findings suggest that astrocytes might be involved in the pathogenesis of some psychiatric disorders as well.

KINKY HAIR DISEASE

PEDIATRICS ◽  
1972 ◽  
Vol 50 (2) ◽  
pp. 181-183
Author(s):  
John H. Menkes
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
In Utero ◽  
Degenerative Disease ◽  
Degenerative Diseases ◽  
Kinky Hair Disease ◽  
The Central Nervous System ◽  
Almost All ◽  
Enzymatic Defect ◽  
Kinky Hair

Despite many recent advances in our understanding of progressive degenerative diseases of the nervous system which have permitted us in some instances to define the underlying enzymatic defect and to detect the disease in utero, treatment for affected children has been nonexistent in almost all instances. The paper by Danks et al.1 in this issue of Pediatrics is, therefore, of considerable importance. It not only demonstrates the underlying cause for one of these disorders, Kinky Hair disease, but also suggests a relatively simple course of treatment. Ten years ago a group of Residents from the Departments of Neurology, Pediatric Neurology, Neuropathology, and Dermatology described in this journal2 what appeared to be a new degenerative disease of the central nervous system.

Sign in / Sign up

Export Citation Format

Share Document