No to shinkei = Brain and nerve最新文献

Progressive multifocal leukoencephalopathy (PML) is a fetal demyelinating disease in the central nervous system. PML was once a rare disease, but it is now relatively common among AIDS (acquired immunodeficiency syndrome) patients. The immunological state of patients mainly contributes to the pathogenesis of PML. Genetic changes of the etiological agent, however, may also be involved in the development and progression of the disease. The major genetic changes possibly associated with PML include the regulatory region rearrangement and the VP1 loop mutation. Both changes have been identified as genetic changes usually occurring in JCV (JCvirus) DNAs from the brain and cerebrospinal fluid of PML patients. Although it remained to be clarified how these changes are involved in the pathogenesis of PML, accumulating evidence suggests that the VP1 loop mutation is associated with the progression of PML. Here we overview studies (mainly those performed by ourselves) on these genetic changes.

Progressive multifocal leukoencephalopathy (PML) is a demyelinating disease of the central nervous system caused by the reactivation of a ubiquitous polyomavirus JC (JCV). PML was for many years a rare disease occurring only in patients with underlying severe impaired immunity. Over the past three decades, the incidence of PML has significantly increased related to the AIDS (acquired immunodeficiency syndrome) pandemic and, more recently, to the growing use of immunosuppressive drugs. The clinical presentation of PML is variable with neurological symptoms corresponding to affected cerebral areas. Usually, the clinical outcome of patients with PML is poor with an inexorable progression to death within 6 months of symptom onset. Although PML usually requires a brain biopsy or autopsy for confirmation, radiological imaging and a demonstration of JCV-DNA in the CSF (cerebrospinal fluid) provide supportive evidence for the diagnosis. Although there is no proven effective therapy for PML, patients with HIV (human immunodeficeincy virus)-related PML may benefit significantly from HAART (highly active antiretroviral therapy). In this article the author reviews the epidemiology, especially in Japan, current challenges in the diagnosis and the treatment guidelines of patients with PML based on recent advances in the understanding of the JC virus biology.

For five years, a 56-year-old woman had undergone "Shiatsu" (a technique that uses fingers and the palm of the hand to apply pressure to particular sections of the body's surface to correct neck stiffness and body imbalances in order to maintain and promote health). She suddenly developed neck pain, dizziness, dysphagia, and speech and gait disturbances during treatment. A neurological examination detected bradylalia and truncal and mild bilateral limb ataxia of the cerebellar type. Diffusion-weighted brain MRI showed multiple hyperintense signal lesions at the bilateral cerebellar hemisphere in the posterior inferior cerebellar artery territory. Three-dimensional computed tomographic angiography (3D-CTA) revealed irregular stenosis of the intracranial right vertebral artery (string sign). Dissection of the intracranial portion of the vertebral artery owing to trauma is rare. Physicians need to be aware of patients who have acute dissecting infarction after long periods of repeated trivial pressure such as "Shiatsu". 3D-CTA is a very useful diagnostic procedure for arterial dissection.

Progressive multifocal leukoencephalopathy (PML) is caused by opportunistic infection of JC virus which belongs to Papovavirus, and presents with progressive demyelinating lesion in the central nervous system. PML was originally reported as a rare complication of hematologic disorders, but later greatly increased in number in association with acquired immunodeficeincy syndrome (AIDS). Original neuropathological features of PML consist of demyelination lacking inflammatory reaction or necrosis, accompanying oligodendroglial nuclear inclusions in the periphery of demyelination. The lesion is preferentially localized to gray-white junction of the cerebral hemisphere and manifests as characteristic demyelinating lesion, called scallopping. Detection of JC virus is essential for the final diagnosis of PML and is achieved via immunohistochemical detection of JC virus with antibodies raised against their components, ultrastructural demonstration of virions characteristic of JC virus, or detection with in-situ hybridization of the genome of JC virus. JC virus can replicate only in oligodendroglial cells, but astrocytes are frequently infected by the virus. The resume of immunological function through therapeutic intervention develops new pathology in PML, exhibiting severe inflammatory reaction with edema and necrosis. This new pathological feature is called immune reconstruction syndrome and clinically presents with severe progression in symptoms of the central nervous system. Nevertheless, treatment of PML is directed for the elimination of the viruses by host immune system. The modification of the above immune reconstruction syndrome is essential for successful outcome of such therapeutic trial.

It was previously reported that Mato cells (Mato's fluorescent granular perithelial cells) were frequently localized in the bifurcating areas of cerebral arterioles and occasionally, collagen fibers appeared close to Mato cells of aged rats. It has also been established that Mato cells were scavenger cells in the cerebral tissue and provided with MHC-class II antigen. The present paper deals with the relationship between the distribution of collagen fibers and Mato cells in the bifurcating area of cerebral arterioles. 6 Wistar rats (3 rats aged 4 months and 3 rats aged 16 months) were employed for this electron microscopical study. They were perfused with the mixture of paraform-glutaraldehyde solution, and cerebral cortices were excised and fixed with 1% osmic solution and embedded in Epon 812. In order to observe the bifurcating area of cerebral arterioles, serial semithin sections cutting with the diamond knife were stained with toluidine blue, and checked under the light microscope. After obtaining available regions, serial thin sections were stained with uranyl acetate and lead nitrate, and observed with Hitach H7600 electron microscope. In order to survey the distribution of collagen fibers, 2 groups of the specimens of 4 months old (2 rats) and 5 groups of specimens of 16 months old rats (2 rats) were prepared. Each group consisted of serial 10 thin sections. Other rats of young and aged were used for complementary use. From the observation, it is confirmed that in the cerebral arteriole, collagen fibers are localized only in the interstices around Mato cells, and the fibers appear in a small quantity in the rats aged 4 months, while a certain amounts of the fibers are arranged sporadically in the rats aged 16 months. However, no collagen fibers can be detected in the subendothelial space and in the interstices among smooth muscle cells of cerebral arteriole. If Mato cells lack in some regions of cerebral arterioles, collagen fibers cannot be recognized. The following is also to be stressed that no fibroblastoid cells do appear in any serial sections of cerebral arterioles. From these findings, it seems possible that Mato cells play a principal role in the formation of collagen fibers in the cerebral arterioles, and associate with the sclerosis of cerebral arterioles.

The authors studied patients who presented depression and apathy following intracerebral hemorrhage (ICH). Twelve patients who were admitted in our hospital were divided into two groups according to the presence of post-stroke depression (PSD). Five patients with PSD are in group A, and another seven patients without PSD are in group B. Zung-self depression scale (SDS) and apathy scale were used for screening of depression and apathy. PSD was recognized in 5 (42%) of patients following ICH. Single photon emission tomography (SPECT) suggested the reduction of cerebral blood flow (CBF) in the frontal lobe in all patients of the group A (100%), whereas only 29% of patients of the group B. The reduction of CBF in the frontal lobe might be involved in the mechanism of depression following ICH in subacute stage.