Brain Diseases ; pathology ; Histiocytosis, Sinus ; pathology ; Humans ; Skull ; pathology

Primary familial brain calcification (PFBC) is an inherited neurodegenerative disorder mainly characterized by progressive calcium deposition bilaterally in the brain, accompanied by various symptoms, such as dystonia, ataxia, parkinsonism, dementia, depression, headaches, and epilepsy. Currently, the etiology of PFBC is largely unknown, and no specific prevention or treatment is available. During the past 10 years, six causative genes (SLC20A2, PDGFRB, PDGFB, XPR1, MYORG, and JAM2) have been identified in PFBC. In this review, considering mechanistic studies of these genes at the cellular level and in animals, we summarize the pathogenesis and potential preventive and therapeutic strategies for PFBC patients. Our systematic analysis suggests a classification for PFBC genetic etiology based on several characteristics, provides a summary of the known composition of brain calcification, and identifies some potential therapeutic targets for PFBC.
Animals ; Brain Diseases/therapy* ; Xenotropic and Polytropic Retrovirus Receptor ; Brain/pathology*

We have tracked the response of host and transplanted neural progenitors or stem cells to hypoxic-ischemic (HI) brain injury, and explored the therapeutic potential of neural stem cells (NSCs) injected into mice brains subjected to focal HI injury. Such cells may integrace appropriately into the degenerating central nervous system (CNS), and showed robust engraftment and foreign gene expression within the region of HI inury. They appeared to have migrated preferentially to the site of ischemia, experienced limited proliferation, and differentiated into neural cells lost to injury, trying to repopulate the damaged brain area. The transplantation of exogenous NSCs may, in fact, augment a natural self-repair process in which the damaged CNS "attempts" to mobilize its own pool of stem cells. Providing additional NSCs and trophic factors may optimize this response. Therefore, NSCs may provide a novel approach to reconstituting brains damaged by HI brain injury. Preliminary data in animal models of stroke lends support to these hypotheses.
Animal ; Brain/pathology ; Brain Diseases/therapy* ; Brain Diseases/pathology ; Brain Ischemia/therapy* ; Brain Ischemia/pathology ; Gene Therapy* ; Human ; Nerve Tissue/cytology* ; Stem Cells/transplantation* ; Tissue Therapy*

Cysticercosis, like other parasitic disease, is still endemic in many parts of the world, and is not infrequently found in Korea. The authors reviewed CT findings of 54 cases of the cysticercosis involving the centralnervous system which were proven by pathology and clinical findings. The resuls are as follows; 1. Male to femaleratio is 3:1 and the most prevalent age group is the fifth decade. The most common symptom is seizure(56%). 2.Involved areas in the brain are parenchymal (66%), intraventricular (15%), leptomeningeal (40%) and the mixed(15%). 3. Pre-contrast CT findings are the round low density in 46 cases (85%), multiple pin-point calcificationin 18 cases (33%), variable degrees of hydrocephalus in 20 cases (37%) and peripheral edmatous change in 8 cases(15%). 4. Post
Brain ; Cysticercosis ; Humans ; Hydrocephalus ; Korea ; Male ; Parasitic Diseases ; Pathology

Adult ; Aspergillosis ; pathology ; Brain Diseases ; pathology ; Granuloma ; pathology ; Humans ; Magnetic Resonance Imaging ; Male

Marchiafava-Bignami disease (MBD) is a fatal disorder characterized by demyelination of the corpus callosum. MRI, suggestive of corpus callosum demyelination with associated white matter involvement in both cerebral hemispheres, indicates a diagnosis of MBD. In this case, MR diffusion-weighted findings taken at an acute stage of MBD revealed lesions not only in the corpus callosum but also in the cerebral cortex. Lower apparent diffusion coefficient values of the corpus callosum and cortical lesions were associated with poor clinical outcome.
Middle Aged ; Male ; Humans ; Demyelinating Diseases/*pathology ; Corpus Callosum/*pathology ; Brain/*pathology ; Alcoholism/complications

Atrophy ; Brain ; pathology ; Child ; Female ; Humans ; Muscle Fibers, Slow-Twitch ; pathology ; Neuromuscular Diseases ; congenital ; pathology

Animals ; Brain Diseases/pathology* ; Cell Cycle ; Interneurons/pathology* ; Median Eminence/pathology* ; Mice

Neurodegenerative diseases (NDs) have become a significant threat to an aging human society. Numerous studies have been conducted in the past decades to clarify their pathologic mechanisms and search for reliable biomarkers. Magnetic resonance imaging (MRI) is a powerful tool for investigating structural and functional brain alterations in NDs. With the advantages of being non-invasive and non-radioactive, it has been frequently used in both animal research and large-scale clinical investigations. MRI may serve as a bridge connecting micro- and macro-level analysis and promoting bench-to-bed translational research. Nevertheless, due to the abundance and complexity of MRI techniques, exploiting their potential is not always straightforward. This review aims to briefly introduce research progress in clinical imaging studies and discuss possible strategies for applying MRI in translational ND research.
Animals ; Humans ; Neurodegenerative Diseases/pathology* ; Translational Research, Biomedical ; Magnetic Resonance Imaging/methods* ; Brain/pathology* ; Head/pathology*

The emerging concept of the neurovascular unit may enable a powerful paradigm shift for neuroscience. Instead of a pure focus on the "neurobiology" of disease, an opportunity now exists to return to a more integrative approach. The neurovascular unit emphasizes that signaling between vascular and neuronal compartments comprise the basis for both function and dysfunction in brain. Hence, brain disorders are not just due to death of neurons, but instead manifested as cell signaling perturbations at the neurovascular interface. In this mini-review, we will examine 3 examples of this hypothesis: neurovascular mechanisms involved in the thrombolytic therapy of stroke, the crosstalk between neurogenesis and angiogenesis, and the link between vascular dysfunction and amyloid pathology in Alzheimer's disease. An understanding of cell-cell and cell-matrix signaling at the neurovascular interface may yield new approaches for targeting CNS disorders.
Alzheimer Disease ; Amyloid ; Brain ; Brain Diseases ; Neurogenesis ; Neurons ; Neurosciences ; Pathology ; Stroke* ; Thrombolytic Therapy ; Tissue Plasminogen Activator