Central Nervous System ; pathology ; Humans ; Infant ; Leigh Disease ; pathology ; physiopathology

Synapsins serve as flagships among the presynaptic proteins due to their abundance on synaptic vesicles and contribution to synaptic communication. Several studies have emphasized the importance of this multi-gene family of neuron-specific phosphoproteins in maintaining brain physiology. In the recent times, increasing evidence has established the relevance of alterations in synapsins as a major determinant in many neurological disorders. Here, we give a comprehensive description of the diverse roles of the synapsin family and the underlying molecular mechanisms that contribute to several neurological disorders. These physiologically important roles of synapsins associated with neurological disorders are just beginning to be understood. A detailed understanding of the diversified expression of synapsins may serve to strategize novel therapeutic approaches for these debilitating neurological disorders.
Animals ; Central Nervous System Diseases ; physiopathology ; Humans ; Synapsins ; physiology

Central Nervous System ; physiopathology ; Facial Nerve Injuries ; physiopathology ; Humans ; Neuronal Plasticity ; Synapses ; physiology

Embolization therapy has been used as the initial treatment for spinal dural arteriovenous fistula (SDAVF) only for certain patients or in certain medical institutions due to its minimal invasiveness, but the recurrence of embolization remains a clinical challenge. The recurrent patient usually exhibits a gradual onset of symptoms and progressive deterioration of neurological function. Developing paraplegia several hours after embolization is commonly seen in patients with venous thrombosis-related complications, for which anticoagulation therapy is often administered. This article reports on a SDAVF patient who had weakness of both lower extremities before embolization and developed complete paraplegia several hours after embolization therapy, later confirmed by angiography as fistula recurrence. The symptoms were relieved gradually after second embolization. The pathophysiology of this patient is also discussed.
Aged ; Central Nervous System Vascular Malformations ; physiopathology ; therapy ; Embolization, Therapeutic ; methods ; Humans ; Paraplegia ; diagnosis

A large number of cancer patients suffer from pain. Growing evidence suggested that pain might be a serious risk factor for cancer patients. The shared modulators and modulation pathways between neural system and tumor cells, such as various neurotransmitters and neurogenic cytokines, provide essential basis for the effect of pain on tumor. In this article, we reviewed some possible mechanism of this process from two aspects: the systematic regulation of central nervous system on endocrine and immunity, and the regional regulation of peripheral nerves on tumor cells. The aim of this review is to provide more innovative knowledge about pain and cancer and to emphasize the importance of anti-pain in the therapy of cancer.
Cancer Pain ; physiopathology ; Central Nervous System ; Humans ; Memory ; Neurotransmitter Agents ; Pain ; Peripheral Nerves

G-protein-coupled receptor 17 (GPR17), an originally orphan receptor, was identified as a new uracil nucleotides/cysteinyl leukotriene receptor. However, whether GPR17 is really classified as a leukotriene receptor is a matter deserving further investigation. GPR17 is involved in many physiological and pathological processes including brain injury, spinal cord injury, and oligodendrocyte differentiation. GPR17 may become a new therapeutic target in these diseases. In this article, the research progress on the pharmacology and pathophysiological roles of GPR17 is reviewed.
Central Nervous System ; injuries ; physiopathology ; Humans ; Neurogenesis ; physiology ; Receptors, G-Protein-Coupled ; metabolism ; physiology

Microglia are resident macrophages of central nervous system (CNS), and thus act as the crucial stuff of immune response and play very important roles in the progress of various CNS diseases. There are two different polarization statuses of activated microglia, M1 and M2 phenotypes. M1 polarized microglia are important for eradicating bacterial and promoting inflammation, whereas M2 cells are characterized by anti-inflammation and tissue remodeling. Recently, more and more evidence indicated that different polarized microglia showed diverse microRNA (miRNA) expression profiles. MiRNAs regulate microglia polarization, and thus affect the progress of CNS diseases. Fully exploring the polarization status of microglia during CNS diseases and the role of miRNAs in microglia polarization will be very helpful for a deep understanding of the roles of microglia in immunopathologic mechanism of different CNS diseases and offer the theoretical foundation of searching more effective therapies for these disorders.
Central Nervous System Diseases ; physiopathology ; Humans ; Inflammation ; Macrophages ; physiology ; MicroRNAs ; physiology ; Microglia ; physiology

INTRODUCTIONThis study aims to assess the early tumour outcome and morbidity associated with radiation therapy (RT) in tumours of the central nervous system (CNS).

MATERIALS AND METHODSPatients receiving RT with radical intent were entered on a prospective database. Tumour types were categorised into glioma, base of skull, pituitary, germ cell or primitive neuroectodermal tumour (PNET) and other malignant CNS tumours. Study endpoints were overall survival and progression free survival. Acute and late toxicity endpoints included Common Terminology Criteria version 3.0 (CTC) grade 3 or 4 events, need for admission during RT and change in performance status at 12 months.

RESULTSOne hundred and fifty-two patients with CNS tumours were managed with radical intent over the 4-year period. The median age was 49 years and 68.4% were Eastern Co-operative Group (ECOG) 0-1 performance status. The major pathology groups were glioma (59.9%) and base of skull tumours (17.1%). Gross total resection was performed in 28.3% only and RT was delayed after diagnosis until time of progression in 19.7%. For the 91 patients with glioma, the median survival and 2-year survival rate was 19.1 months and 44.1%, respectively. The 2-year survival rates for the subgroups of WHO Grade I or II, III and IV were 100%, 52% and 35%, respectively. For the non-glioma tumour groups, the relapse varied with pathology. Toxicity was minimal with only 3 acute and 3 late CTC grade 3 or 4 events occurring. Overall, 47 or 31% of patients required some inpatient hospitalisation during RT, although this was determined to have some causative relationship to RT in only 12 or 8% of patients. In the 12 months post-RT, performance status was stable or improved in 76.2% of patients, and most deterioration was associated with tumour relapse.

CONCLUSIONSRT for CNS tumours using modern techniques was well-tolerated with good tumour outcome and minimal morbidity.

Adolescent ; Adult ; Aged ; Central Nervous System ; physiopathology ; Central Nervous System Neoplasms ; radiotherapy ; Child ; Child, Preschool ; Female ; Humans ; Male ; Middle Aged ; Prospective Studies ; Radiotherapy ; methods ; standards ; Singapore ; Survival Analysis

Activation of interferon (IFN) signaling in the central nervous system (CNS) is usually associated with inflammation. However, a robust activation of type I IFN-stimulated genes (ISGs) at pre-symptomatic stages occurs in the spinal cord of SOD1(G93A) mice, an amyotrophic lateral sclerosis (ALS) animal model, without obvious signs of inflammation. To determine if the same signaling pathway is elevated in other types of neuronal injuries, we examined the protein expression levels of an IFN-stimulated gene, ISG15, in mouse models of acute and chronic neuronal injuries. We found that ISG15 protein was dramatically increased in the brains of mice subjected to global ischemia and traumatic brain injury, and in transgenic mice overexpressing HIV gp120 protein. These results suggest that activation of ISGs is a shared feature of neuronal injuries and that ISG15 may be a suitable biomarker for detecting neuronal injuries in the CNS.
Amyotrophic Lateral Sclerosis ; physiopathology ; Animals ; Brain Injuries ; physiopathology ; Brain Ischemia ; physiopathology ; Central Nervous System ; physiopathology ; Cytokines ; metabolism ; Disease Models, Animal ; Mice ; Mice, Transgenic ; Ubiquitins ; metabolism

Perineuronal nets (PNNs) are reticular structures resulting from the aggregation of extracellular matrix (ECM) molecules around the cell body and proximal neurite of specific population of neurons in the central nervous system (CNS). Since the first description of PNNs by Camillo Golgi in 1883, the molecular composition, developmental formation and potential functions of these specialized extracellular matrix structures have only been intensively studied over the last few decades. The main components of PNNs are hyaluronan (HA), chondroitin sulfate proteoglycans (CSPGs) of the lectican family, link proteins and tenascin-R. PNNs appear late in neural development, inversely correlating with the level of neural plasticity. PNNs have long been hypothesized to play a role in stabilizing the extracellular milieu, which secures the characteristic features of enveloped neurons and protects them from the influence of malicious agents. Aberrant PNN signaling can lead to CNS dysfunctions like epilepsy, stroke and Alzheimer's disease. On the other hand, PNNs create a barrier which constrains the neural plasticity and counteracts the regeneration after nerve injury. Digestion of PNNs with chondroitinase ABC accelerates functional recovery from the spinal cord injury and restores activity-dependent mechanisms for modifying neuronal connections in the adult animals, indicating that PNN is an important regulator of neural plasticity. Here, we review recent progress in the studies on the formation of PNNs during early development and the identification of CSPG receptor - an essential molecular component of PNN signaling, along with a discussion on their unique regulatory roles in neural plasticity.
Animals ; Central Nervous System ; physiology ; physiopathology ; Chondroitin Sulfate Proteoglycans ; Extracellular Matrix ; physiology ; Humans ; Neuronal Plasticity ; Neurons ; Receptors, Cell Surface ; physiology