It has been well established that the recovery ability of central nervous system (CNS) is very poor in adult mammals. As a result, CNS trauma generally leads to severe and persistent functional deficits. Thus, the investigation in this field becomes a "hot spot". Up to date, accumulating evidence supports the hypothesis that the failure of CNS neurons to regenerate is not due to their intrinsic inability to grow new axons, but due to their growth state and due to lack of a permissive growth environment. Therefore, any successful approaches to facilitate the regeneration of injured CNS axons will likely include multiple steps: keeping neurons alive in a certain growth-state, preventing the formation of a glial scar, overcoming inhibitory molecules present in the myelin debris, and giving direction to the growing axons. This brief review focused on the recent progress in the neuron regeneration of CNS in adult mammals.
Animals ; Axons ; physiology ; Central Nervous System Diseases ; complications ; metabolism ; pathology ; Humans ; Mammals ; physiology ; Nerve Regeneration ; physiology

von Economo neuron (VEN) is a bipolar neuron characterized by a large spindle-shaped soma. VEN is generally distributed in the layer V of anterior insular lobe and anterior cingulate cortex. Fork neuron is another featured bipolar neuron. In recent years,many studies have illustrated that VEN and fork neurons are correlated with complicated cognition such as self-consciousness and social emotion. Studies in the development and morpholigies of these two neurons as well as their pathological changes in various neurological and psychiatric disorders have found that the abnormal number and functions of VEN can cause corresponding dysfunctions in social recognition and emotions both during the neuro-developmental stages of childhood and during the nerve degeneration in old age stage. Therefore, more attentions should be paid on the research of VEN and fork neurons in neuropsychiatric diseases.
Central Nervous System Diseases ; pathology ; Cerebral Cortex ; Humans ; Mental Disorders ; Neurons

Neurogenesis occurs in the adult brain, and neural stem cells (NSCs) reside in the adult central nervous system (CNS). In the adult brain, newly generated neuronal cells would originate from a population of glial cells with stem cells properties, and be involved in processes such as learning and memory, depression, and in regenerative attempts in the diseased brain and after injuries. In human, a recent study reported no evidence of migrating neural progenitor cells along the subventricular zone (SVZ) to the olfactory bulb (OB), contrary to other species, highlighting the particularity of adult neurogenesis in human. Though the origin and contribution of newly generated neuronal cells to CNS pathophysiology remain to be fully understood, the discovery that NSCs reside in the adult CNS force us to re-evaluate our knowledge and understanding of brain functioning, and suggest that the adult CNS may be amenable to repair. In this manuscript,we will review the recent data, debates and controversies on the identification, origin and function of newly generated neuronal cells in the adult brain, in human and in other species. We will discuss their contribution and significance to CNS pathophysiology, and for cellular therapy.
Adult Stem Cells ; cytology ; transplantation ; Aging ; Animals ; Brain ; cytology ; Central Nervous System ; cytology ; growth & development ; Central Nervous System Diseases ; pathology ; surgery ; Humans ; Stem Cell Transplantation ; methods

Clinical and postmortem findings of a case that had combined alcoholic pellagra encephalopathy and Wernicke disease are described. This 51-year-old malnourished and chronic alcoholic man presented with progressive mental deterioration, pellagra dermatitis, hypertonus of the neck and other musculatures, myoclonic jerks with bizarre involuntary movements, in addition to total external ophthalmoplegia and gait disturbance. After administration of multivitamins, including thiamine and nicotinamide, these neurologic abnormalities were dramatically improved in a few days. However, the patient died thereafter because of sepsis associated with pneumonia. Postmortem examination revealed marked abnormalities in CNS, characterized by diffuse atrophy of gray matter and widespread neuronal degeneration and characteristic central chromatolysis in pontine nuclei, dentate nuclei, cranial nerve nuclei in the brain stem, Betz cells of the cerebral cortex, and Clarke's column and anterior horn cells of the spinal cord. There were also atrophy and gliosis of the mammillary bodies, degeneration and vascular proliferation of periaqueductal gray matter, and massive gliosis around the third ventricle. These neuropathological changes were compatible with symptoms of both alcoholic pellagra encephalopathy and Wernicke's disease, but they were also strongly suspected on clinical grounds.
Alcoholism/*complications ; Central Nervous System Diseases/complications/pathology ; Humans ; Male ; Middle Aged ; Pellagra/*complications/pathology ; Wernicke Encephalopathy/*complications/pathology

The aggregation of α-synuclein (α-syn) has been implicated in the pathogenesis of many neurodegenerative disorders, including Parkinson's disease (PD), dementia with Lewy bodies (DLB), and multiple system atrophy (MSA). Postmortem analyses of α-syn pathology, especially that of PD, have suggested that aggregates progressively spread from a few discrete locations to wider brain regions. The neuron-to-neuron propagation of α-syn has been suggested to be the underlying mechanism by which aggregates spread throughout the brain. Many cellular and animal models has been created to study cell-to-cell propagation. Recently, it has been shown that a single injection of preformed fibrils (PFFs) made of recombinant α-syn proteins into various tissues and organs of many different animal species results in widespread α-syn pathology in the central nervous system (CNS). These PFF models have been extensively used to study the mechanism by which aggregates spread throughout the brain. Here, we review what we have learned from PFF models, describe the nature of PFFs and the neuropathological features, neurophysiological characteristics, and behavioral outcomes of the models.
alpha-Synuclein ; Animals ; Brain ; Central Nervous System ; Dementia ; Lewy Bodies ; Models, Animal ; Multiple System Atrophy ; Neurodegenerative Diseases ; Parkinson Disease ; Pathology

Arm Injuries ; etiology ; Autonomic Pathways ; pathology ; Central Nervous System ; pathology ; China ; Humans ; Occupational Diseases ; etiology ; Temperature ; Vibration ; adverse effects

We report the case of a 52-year-old woman who developed vertical diplopia of 1-days duration. Neuro-ophthalmological testing revealed left trochlear nerve palsy, and sellar MRI revealed a 1.5 cm-sized pituitary mass lesion, a Rathke's cleft cyst. The diplopia disappeared spontaneously after 6 days.
Central Nervous System Cysts/*complications/pathology ; Female ; Human ; Magnetic Resonance Imaging ; Middle Aged ; Trochlear Nerve Diseases/*etiology/pathology

Compared with biochemical information available about the diseases in the central nervous system, that for peripheral neuropathy is quite limited primarily due to the difficulties in obtaining samples. Characterization of the core pathology is a prerequisite to the development of personalized medicine for genetically heterogeneous diseases, such as hereditary motor and sensory neuropathy (HMSN). Here, we first documented the transcriptome profile of distal sural nerve obtained from HMSN patients. RNA-seq analysis revealed that over 12,000 genes are expressed in distal sural nerve. Among them 4,000 transcripts are novel and 10 fusion genes per sample were observed. Comparing dataset from whole exome sequencing revealed that over 1,500 transcriptional base modifications occur during transcription. These data implicate that dynamic alterations are generated when genetic information are transitioned in distal sural nerve. Although, we could not find significant alterations associated with HMSN, these data might provide crucial information about the pathophysiology of HMSN. Therefore, next step in the development of therapeutic strategy for HMSN might be unveiling biochemical and biophysical abnormalities derived from those potent variation.
Central Nervous System ; Dataset ; Exome ; Gene Expression Profiling* ; Hereditary Sensory and Motor Neuropathy ; Humans ; Pathology ; Peripheral Nervous System Diseases ; Sural Nerve* ; Transcriptome ; Precision Medicine

PURPOSE: The importance of trace elements in their effect on the physiology and pathology of the central nervous system is well recognized. Changes in the concentrations of these elements in the brain could take place in pathological states. Recently, a greater emphasis has been given to the role of trace elements in the function of the nervous system both in normal and pathological conditions. The past experiments from animal demonstrate that Na+-K+-ATPase inhibition, particularly in the hippocampus, is involved in epileptogenicity. Zinc is the most potent inhibitor of Na+-K+-ATPase followed closely by copper. Zinc modulates the activity of glutamic acid decarboxylase, the rate limiting enzyme in the synthesis of -aminobutyric acid (GABA), which is a major inhibitory neurotransmitter. There are few reports of zinc and copper concentrations in normal CSF and in CSF from patients with neurological diseases. The aim of this study was designed to determine the zinc and copper concentrations and their correlation with protein in CSF of pediatric patients with neurologic disorders. METHODS: The study population was 43 patients who had admitted to Kang Nam Sacred Heart Hospital of Hallym University from March to June, 1996 due to high fever, headache, vomiting, and seizure. All patients were examined CSF study, 32 patients (group I) were showed abnormal CSF and seizure disorders including febrile convulsion and 11 patients (group II) were showed normal CSF and clinical symptoms of febrile illness. Zinc and copper concentrations in CSF were determined with flame atomic absorption spectrophotometry. In addition, CSF zinc and copper concentrations in normal CSF proteingroup (group A) and in increased CSF protein group (group B) were determined to investigate probability that the damaged blood-brain-barrier permits the passage of zinc and copper into the subarachnoid space. RESULTS: 1) The CSF zinc concentrations in group I and II were 9.40+/-6.18 and 7.39+/-5.48microgram/dl, and the CSF copper concentrations in group I and II were 4.86+/-7.07 and 2.93+/-1.45microgram/dl, respectively. There was no statistically significant difference in the CSF zinc and copper concentrations between the two groups. 2) The CSF zinc concentrations in group A and B were 7.21+/-4.96 and 11.24+/-7.32microgram/ dl, and the copper concentrations in group A and B were 3.31+/-2.15 and 5.59+/-9.46microgram/dl, respectively. There was no statistically significant difference in the CSF zinc and copper concentrations between the two groups. 3) There was a significant positive correlation between the CSF zinc and copper concentrations as well as between the CSF zinc and protein concentrations. But there was no significant correlation between the CSF copper and protein concentrations. CONCLUSIONS: There was no statiscally significant defference in the CSF zinc and copper concentrations between neurologic disorders and febrile diseases. Increased CSF zinc and copper concentrations in increased CSF protein groups were not found. But there were some correlation between zinc, copper, protein levels in CSF. These results do not support assumption that damaged BBB permits the passage of the zinc, copper into the subarachnoid space.
Animals ; Brain ; Central Nervous System ; Cerebrospinal Fluid* ; Child* ; Copper* ; Epilepsy ; Fever ; Glutamate Decarboxylase ; Headache ; Heart ; Hippocampus ; Humans ; Nervous System ; Nervous System Diseases* ; Neurotransmitter Agents ; Pathology ; Physiology ; Seizures ; Seizures, Febrile ; Spectrophotometry, Atomic ; Subarachnoid Space ; Trace Elements ; Vomiting ; Zinc*

Brain ; pathology ; Central Nervous System Diseases ; congenital ; diagnosis ; pathology ; physiopathology ; Diagnosis, Differential ; Humans ; Infant, Newborn ; Magnetic Resonance Imaging ; Male ; Mobius Syndrome ; diagnosis ; pathology ; physiopathology