Mucopolysaccharidosis in Adults

2016 ◽  
Author(s):  
Christian J. Hendriksz ◽  
Francois Karstens
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Autosomal Recessive ◽  
Clinical Symptoms ◽  
Cell Types ◽  
Type Ii ◽  
System P ◽  
Different Types ◽  
The Central Nervous System ◽  
Different Cell Types

There are 8 different types of diseases of the mucopolysaccharides, each caused by a deficiency in one of 10 different enzymes involved in the degradation of glycosaminoglycans (GAGs). Partially degraded GAGs accumulate within the lysosomes of many different cell types and lead to clinical symptoms and excretion of large amounts of GAGs in the urine. Heritability is autosomal recessive except for MPS type II, which is X-linked. The disorders are chronic and progressive and, although the specific types all have their individual features, they share an abundance of clinical similarities. All involve the musculoskeletal, the cardiovascular, the pulmonary and the central nervous system.

Neuroanatomy and neurophysiology

2021 ◽  
pp. 725-852
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Cervical Vertebrae ◽  
Lymphatic Vessels ◽  
Cell Types ◽  
Cellular Level ◽  
Inhibitory Synapses ◽  
The Central Nervous System ◽  
Emotion Memory ◽  
Different Cell Types

‘Neuroanatomy and neurophysiology’ covers the anatomy and organization of the central nervous system, including the skull and cervical vertebrae, the meninges, the blood and lymphatic vessels, muscles and nerves of the head and neck, and the structures of the eye, ear, and central nervous system. At a cellular level, the different cell types and the mechanism of transmission across synapses are considered, including excitatory and inhibitory synapses. This is followed by a review of the major control and sensory systems (including movement, information processing, locomotion, reflexes, and the main five senses of sight, hearing, touch, taste, and smell). The integration of these processes into higher functions (such as sleep, consciousness and coma, emotion, memory, and ageing) is discussed, along with the causes and treatments of disorders of diseases such as depression, schizophrenia, epilepsy, addiction, and degenerative diseases.

Perspectives on the central nervous system toxicity of methylmercury

1982 ◽  
Vol 60 (7) ◽  
pp. 1037-1045 ◽  
Author(s):  
William J. Racz ◽  
Laurie J. S. Vandewater
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Membrane Integrity ◽  
Cell Types ◽  
Environmental Pollutant ◽  
Central Nervous System Toxicity ◽  
Central Nervous System Damage ◽  
Rate Of Absorption ◽  
The Central Nervous System ◽  
Different Cell Types

Methylmercury is a widespread and highly toxic environmental pollutant. The source of the substance in the environment is industrial and agricultural use. Chronic methylmercury poisoning is characterized by peripheral and central nervous system damage. The rate of absorption and distribution of this organomercurial into neural tissue determines the rate of development and the severity of the neural lesion. Furthermore, the rate of metabolism and excretion of an organomercurial will greatly influence its neural toxicity. There are differences in the accumulation of methylmercury in different regions of the brain, as well as by the different cell types in these regions. The significance of this variable accumulation of methylmercury is not known. Methylmercury influences a large number of neurocellular functions ranging from inhibition of membrane integrity to alteration in the synthesis and release of transmitter substances.

Acquired Diseases of the Nervous System

2018 ◽  
pp. 219-243
Author(s):  
Leila Chimelli ◽  
Françoise Gray
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Metabolic Diseases ◽  
Cell Types ◽  
Brain Regions ◽  
Vitamin Deficiencies ◽  
System P ◽  
Wide Range ◽  
The Central Nervous System ◽  
Anatomic Region

This chapter describes and illustrates the different neuropathological changes observed in a wide range of systemic acquired metabolic diseases that affect the central or peripheral nervous systems: hypoxia, hypoglycemia, hyperthermia, disorders of serum electrolytes, vitamin deficiencies, and exogenous intoxications, particularly alcoholism and intoxications by drugs, methanol, and heavy metals. In the central nervous system, lesions may find expression via selective involvement of some brain regions, with simultaneous complete preservation of others. The pathogenesis of the predisposition to injury for a particular anatomic region or for some specific set of cell types (neurons mostly) varies considerably form one disease to another and is undoubtedly multifactorial. The chapter also considers central nervous system abnormalities secondary to systemic diseases, including respiratory encephalopathies, hepatic encephalopathy, multifocal necrotizing leukoencephalopathy, and paraneoplastic encephalomyelopathies.

scholarly journals Tumors of the Central Nervous System: An Update

Cancers ◽  
2020 ◽  
Vol 12 (9) ◽  
pp. 2507
Author(s):  
Carla Mucignat-Caretta
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Brain Structure ◽  
Cell Types ◽  
Structure And Function ◽  
Brain Structure And Function ◽  
The Central Nervous System ◽  
And Function ◽  
Different Cell Types ◽  
The Brain

The brain may be affected by a variety of tumors of different grade, which originate from different cell types at distinct locations, thus impacting on the brain structure and function [...]

scholarly journals Co-Deregulated miRNA Signatures in Childhood Central Nervous System Tumors: In Search for Common Tumor miRNA-Related Mechanics

Cancers ◽  
2021 ◽  
Vol 13 (12) ◽  
pp. 3028
Author(s):  
George I. Lambrou ◽  
Apostolos Zaravinos ◽  
Maria Braoudaki
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Cns Tumors ◽  
Normal Brain ◽  
Cns Tumor ◽  
Different Types ◽  
Receiver Operator Curve Analysis ◽  
The Central Nervous System ◽  
Tumor Types ◽  
Operator Curve

Despite extensive experimentation on pediatric tumors of the central nervous system (CNS), related to both prognosis, diagnosis and treatment, the understanding of pathogenesis and etiology of the disease remains scarce. MicroRNAs are known to be involved in CNS tumor oncogenesis. We hypothesized that CNS tumors possess commonly deregulated miRNAs across different CNS tumor types. Aim: The current study aims to reveal the co-deregulated miRNAs across different types of pediatric CNS tumors. Materials: A total of 439 CNS tumor samples were collected from both in-house microarray experiments as well as data available in public databases. Diagnoses included medulloblastoma, astrocytoma, ependydoma, cortical dysplasia, glioblastoma, ATRT, germinoma, teratoma, yoc sac tumors, ocular tumors and retinoblastoma. Results: We found miRNAs that were globally up- or down-regulated in the majority of the CNS tumor samples. MiR-376B and miR-372 were co-upregulated, whereas miR-149, miR-214, miR-574, miR-595 and miR-765 among others, were co-downregulated across all CNS tumors. Receiver-operator curve analysis showed that miR-149, miR-214, miR-574, miR-595 and miR765 could distinguish between CNS tumors and normal brain tissue. Conclusions: Our approach could prove significant in the search for global miRNA targets for tumor diagnosis and therapy. To the best of our knowledge, there are no previous reports concerning the present approach.

Effects of an injectable functionalized self-assembling nanopeptide hydrogel on angiogenesis and neurogenesis for regeneration of the central nervous system

Nanoscale ◽  
2017 ◽  
Vol 9 (42) ◽  
pp. 16281-16292 ◽  
Author(s):  
Tzu-Wei Wang ◽  
Kai-Chieh Chang ◽  
Liang-Hsin Chen ◽  
Shih-Yung Liao ◽  
Chia-Wei Yeh ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Brain Tissue ◽  
Tissue Regeneration ◽  
Self Assembling ◽  
System P ◽  
Injured Brain ◽  
The Central Nervous System

Functionalised self-assembling nanopeptide hydrogel mediates angiogenesis and neurogenesis for injured brain tissue regeneration.

Primary Leptomeningeal Gliomatosis in Children and Adults: A Morphological and Molecular Comparative Study With Literature Review

Neurosurgery ◽  
2015 ◽  
Vol 78 (3) ◽  
pp. 343-352 ◽  
Author(s):  
Arnault Tauziede-Espariat ◽  
Andre Maues de Paula ◽  
Melanie Pages ◽  
Annie Laquerriere ◽  
Emilie Caietta ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Overall Survival ◽  
Cerebrospinal Fluid ◽  
Nervous System ◽  
Clinical Symptoms ◽  
Malignant Gliomas ◽  
Leptomeningeal Gliomatosis ◽  
Molecular Features ◽  
The Central Nervous System ◽  
The Mean

Abstract BACKGROUND: Primary leptomeningeal gliomatosis (PLG) is a poorly recognized tumor of the central nervous system. OBJECTIVE: To describe the histopathological, immunohistochemical, and molecular features of PLG. METHODS: Results of our multicentric retrospective study of 6 PLG cases (3 pediatric and 3 adult) were compared with literature data. RESULTS: The mean age was 54.7 years for adults and 8.7 years for children, with 3 males and 3 females. Clinical symptoms were nonspecific. Cerebrospinal fluid analyses showed a high protein level often associated with pleocytosis but without neoplastic cells. On neuroimaging, diffuse leptomeningeal enhancement and hydrocephalus were observed, except in 1 case. PLG was mostly misinterpreted as infectious or tumoral meningitis. The first biopsy was negative in 50% of cases. Histopathologically, PLG cases corresponded to 1 oligodendroglioma without 1p19q codeletion and 5 astrocytomas without expression of p53. No immunostaining for IDH1R132H and no mutations of IDH1/2 and H3F3A genes were found. Overall survival was highly variable (2-82 months) but seems to be increased in children treated with chemotherapy. CONCLUSION: This study shows the difficulties of PLG diagnosis. The challenge is to achieve an early biopsy to establish a diagnosis and to begin a treatment, but the prognosis remains poor. PLG seems to have a different molecular and immunohistochemical pattern compared with intraparenchymal malignant gliomas.

A practical pH-compatible fluorescent sensor for hydrazine in soil, water and living cells

The Analyst ◽  
2020 ◽  
Vol 145 (22) ◽  
pp. 7380-7387 ◽  
Author(s):  
Huming Yan ◽  
Fangjun Huo ◽  
Yongkang Yue ◽  
Jianbin Chao ◽  
Caixia Yin
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Respiratory Tract ◽  
Human Body ◽  
Water Solubility ◽  
Fluorescent Sensor ◽  
Living Cells ◽  
Human Organs ◽  
System P ◽  
The Central Nervous System

The excellent water solubility of hydrazine (N2H4) allows it to easily invade the human body through the skin and respiratory tract, thereby damaging human organs and the central nervous system.

scholarly journals Elucidating the Neuropathologic Mechanisms of SARS-CoV-2 Infection

2021 ◽  
Vol 12 ◽  
Author(s):  
Mar Pacheco-Herrero ◽  
Luis O. Soto-Rojas ◽  
Charles R. Harrington ◽  
Yazmin M. Flores-Martinez ◽  
Marcos M. Villegas-Rojas ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Molecular Mechanisms ◽  
Cell Types ◽  
Public Health Emergency ◽  
Converting Enzyme ◽  
Neurological Manifestations ◽  
Brain Areas ◽  
Angiotensin Converting Enzyme 2 ◽  
The Central Nervous System

The current pandemic caused by the new severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) has become a public health emergency. To date, March 1, 2021, coronavirus disease 2019 (COVID-19) has caused about 114 million accumulated cases and 2.53 million deaths worldwide. Previous pieces of evidence suggest that SARS-CoV-2 may affect the central nervous system (CNS) and cause neurological symptoms in COVID-19 patients. It is also known that angiotensin-converting enzyme-2 (ACE2), the primary receptor for SARS-CoV-2 infection, is expressed in different brain areas and cell types. Thus, it is hypothesized that infection by this virus could generate or exacerbate neuropathological alterations. However, the molecular mechanisms that link COVID-19 disease and nerve damage are unclear. In this review, we describe the routes of SARS-CoV-2 invasion into the central nervous system. We also analyze the neuropathologic mechanisms underlying this viral infection, and their potential relationship with the neurological manifestations described in patients with COVID-19, and the appearance or exacerbation of some neurodegenerative diseases.

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