China ; Humans ; Nervous System Diseases ; pathology

A wide variety of neurologic complications associated with human immunodeficiency virus-type 1 (HIV-1) infection result from HIV-1 itself or secondarily related to immunosuppression. In Korea, the number of HIV-1 seropositive populations is increasing, but little has been known about the neurologic complications of HIV-1 infection. To investigate the neurologic complications in HIV-1 infected Korean patients, we performed a cross-sectional study in consecutive admissions to the Seoul National University Hospital between March 1998 and June 1999. Thirty-four HIV-1 seropositive patients were included. As a result, a total of 26 HIV-1 related neurologic complications were identified from 17 patients. Among them, 10 patients showed cognitive/motor abnormalities: 3 HIV-1-associated dementia and 7 possible HIV-1-associated minor cognitive/motor disorder. Neuromuscular complications were found in 10 patients: 9 distal symmetric polyneuropathy, and 1 possible chronic inflammatory demyelinating polyradiculoneuropathy. In 3 patients with focal brain lesions, 2 were presumptively diagnosed as having primary CNS lymphoma, and 1 as having progressive multifocal leukoencephalopathy in the posterior fossa, based on history, clinical findings, serology, radiological appearances, and response to empirical therapy. Other complications included cryptococcal meningitis and only soft neurologic signs without any neurologic disease. Most of these complications (88%) occurred in the advanced stage of infection.
Adult ; Brain/pathology ; HIV Infections/complications* ; HIV Seropositivity ; HIV-1* ; Human ; Korea ; Male ; Nervous System Diseases/etiology* ; Nervous System Diseases/pathology ; Nervous System Diseases/physiopathology

Perivascular space (PVS) is a crevice between two slices of cerebral pia maters, filled with tissue fluid, which be formed by pia mater emboling in the surrounding of cerebral perforating branch (excluding micrangium). Normal PVS (diameter < 2 mm) can be found in almost all healthy adults; however enlarged PVS (diameter > 2 mm) has correlation with neurological disorders probably. The article reviews the formation mechanism, imageology characteristics and the relation with neurological disorders of PVS, which is beneficial to the research of some neurological disorders etiopathogenesis and treatment.
Animals ; Blood Vessels ; pathology ; Humans ; Nervous System Diseases ; pathology ; Pia Mater ; pathology

In this review, combined post-mortem brain magnetic resonance imaging (MRI) and histology studies are highlighted, illustrating the relevance of translational approaches to define novel MRI signatures of neuropathological lesions in neuroinflammatory and neurodegenerative disorders. Initial studies combining post-mortem MRI and histology have validated various MRI sequences, assessing their sensitivity and specificity as diagnostic biomarkers in neurologic disease. More recent studies have focused on defining new radiological (bio)markers and implementing them in the clinical (research) setting. By combining neurological and neuroanatomical expertise with radiological development and pathological validation, a cycle emerges that allows for the discovery of novel MRI biomarkers to be implemented in vivo. Examples of this cycle are presented for multiple sclerosis, Alzheimer's disease, Parkinson's disease, and traumatic brain injury. Some applications have been shown to be successful, while others require further validation. In conclusion, there is much to explore with post-mortem MRI and histology studies, which can eventually be of high relevance for clinical practice.
Animals ; Brain ; diagnostic imaging ; pathology ; Humans ; Magnetic Resonance Imaging ; methods ; Nervous System Diseases ; diagnostic imaging ; pathology

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

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

Peripheral neuroepithelioma is a rare tumor, comprising less than 1% of all soft tissue malignancies arising from the peripheral nonautonomic nervous system. Most peripheral neuroepitheliomas reported were located in the extremities, thoraco-pulmonary region, and pelvic areas, and as many as 30% of cases were associated with peripheral nerve. We report one case of peripheral neuroepithelioma arising in the kidney, mimicking renal cell carcinoma on the CT scan.
Adult ; Case Report ; Female ; Human ; Kidney Neoplasms/*pathology ; Neuroectodermal Tumors, Primitive, Peripheral/*pathology ; Peripheral Nervous System Diseases/*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

Autonomic Nervous System Diseases ; metabolism ; pathology ; surgery ; Colon ; metabolism ; pathology ; surgery ; Diagnosis, Differential ; Enteric Nervous System ; abnormalities ; pathology ; Hirschsprung Disease ; pathology ; Humans ; Infant, Newborn ; Intestinal Diseases ; metabolism ; pathology ; surgery ; Male ; Phosphopyruvate Hydratase ; metabolism ; S100 Proteins ; metabolism