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Demyelinating Diseases (7):
Recent article
Demyelinating Diseases
Abstract
In the normal brain, cellular types that compose the neurovascular unit, including neurons, astrocytes and endothelial cells express pannexins and connexins, which are protein subunits of two families that form plasma membrane channels. Most available evidence in mammals indicated th...
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PMID: 21288190
PDF is available here.
PDF is available here.
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Managing overly rapid correction of chronic hyponatremia: an ounce of prevention or a pound of cure?
Abstract
Abstract
We studied axonal pathology in the taiep mutant rat, which develops nonimmune oligodendrocyte dysfunction and myelin loss. Using immunohistochemical analysis of several CNS regions, we show that accumulation of dephosphorylated neurofilaments occurs in taiep axons. These changes become more pronounc...
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PMID: 21107138
PDF is available here.
PDF is available here.
Abstract
The myelination of axons by glial cells was the last major step in the evolution of cells in the vertebrate nervous system, and white-matter tracts are key to the architecture of the mammalian brain. Cell biology and mouse genetics have provided insight into axon-glia signalling and the molecular ar...
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PMID: 21068833
PDF is available here.
PDF is available here.
Abstract
Multiple sclerosis may affect both afferent and efferent visual pathways, and sometimes physicians err on ordinary ophthalmologic diagnosis due to overlapping symptoms between demyelinating and nondemyelinating visual diseases. The present article highlights nondemyelinating ocular occurrences due t...
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PMID: 20736835
PDF is available here.
PDF is available here.
Abstract
We suggest these features and encephalopathy may be associated with the age of patients. Furthermore, though persistence of abnormal MRI lesions is significantly more likely in the group of CIS, none of these patients had a subsequent recurrence or developed MS during the follow-up period. The predi...
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PMID: 21077353
PDF is available here.
PDF is available here.
Abstract
We studied the clinical and neuropathologic effects of intravenously administered human MSCs (hMSCs) in C57BL/6 mice with EAE. Human MSCs significantly reduced the clinical disease severity, particularly in later disease. Large numbers of hMSCs migrated into gray and white matter at all levels of th...
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PMID: 20940628
PDF is available here.
PDF is available here.
Abstract
We studied Gjb1-null mice at early stages (i.e. 2-4 months old) of the neuropathy, when there is minimal or no demyelination. The diameters of large myelinated axons were progressively reduced in Gjb1-null mice compared with those in wild-type littermates. Furthermore, neurofilaments were relatively...
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PMID: 20720503
PDF is available here.
PDF is available here.
Abstract
We previously found to be dependent upon capsid serotype. We sought to augment retrograde transduction by providing increased axonal access to peripherally delivered AAV. Others have described utilizing full transection of peripheral nerves to mediate retrograde viral transduction of motor neurons....
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PMID: 20502445
PDF is available here.
PDF is available here.
Abstract
Astrocytic damage reflected by elevated CSF glial fibrillary acidic protein is a clinically relevant, primary pathologic process in neuromyelitis optica, and is far more severe than demyelination....
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PMID: 20644148
PDF is available here.
PDF is available here.
Abstract
We infected Epstein-Barr virus-transformed B cells with JCV and found that the viral genome decreased >1000-fold from days 0 to 20 after infection, which concurred with the absence of viral early and late messenger RNA transcripts and proteins. However, immunofluorescent images of B cells infected w...
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PMID: 20550458
PDF is available here.
PDF is available here.
Abstract
Abstract
We report a patient with Lewis-Sumner syndrome (LSS) who showed an improvement only with plasma exchange (PE). The patient, 32-yr old man, had progressive multifocal motor-sensory deficits with persistent, multiple conduction blocks and marked slowing of NCVs. Nerve pathology supported a diagnosis o...
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PMID: 20592909
PDF is available here.
PDF is available here.
NeuroImage (51)3 2010
Abstract
We compared two genetic mouse models exhibiting relatively selective axonal or myelin deficits using quantitative MRI relaxography of the transverse relaxation times (T2) in vivo and ultrastructural morphometry. In HM-DKO mice, which lack genes encoding the heavy (NF-H) and medium (NF-M) subunits of...
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PMID: 20226865
PDF is available here.
PDF is available here.
Anesthesia & Analgesia (111)1 2010
Abstract
This study demonstrates that, in the rat model, ropivacaine is associated with marked histological abnormality, including edema of the perineurium and axonal destruction with wallerian degeneration, when injected into or extraneurally placed onto a nerve. Extrafascicular injection and extraneural pl...
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PMID: 20442258
PDF is available here.
PDF is available here.
Cell Stem Cell (6)6 2010
Abstract
We have used Cre-lox fate mapping in transgenic mice to show that PDGFRA/NG2-expressing glia, a distributed population of stem/progenitor cells in the adult CNS, produce the remyelinating oligodendrocytes and almost all of the Schwann cells in chemically induced demyelinated lesions. In contrast, th...
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PMID: 20569695
PDF is available here.
PDF is available here.
Abstract
The osmotic demyelination syndrome (ODS) has been a recognized complication of the rapid correction of hyponatremia for decades. However, in recent years, a variety of other medical conditions have been associated with the development of ODS, independent of changes in serum sodium. This finding sugg...
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PMID: 20453633
PDF is available here.
PDF is available here.
Abstract
We evaluated the therapeutic effects of RTL551, specific for T cells reactive to mouse (m)MOG-35-55 peptide, on EAE induced with recombinant human (rh)MOG in C57BL/6 mice. We report that RTL551 therapy can reverse disease progression and reduce demyelination and axonal damage induced by rhMOG withou...
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PMID: 19789980
PDF is available here.
PDF is available here.
Journal of Neuroscience (30)19 2010
Abstract
We investigated whether myelination and lysolecithin-induced demyelination affect axonal mitochondria. Myelination increased the size of axonal stationary mitochondrial sites by 2.3-fold. After demyelination, the size of axonal stationary mitochondrial sites was increased by an additional 2.2-fold a...
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PMID: 20463228
PDF is available here.
PDF is available here.
Abstract
The genetic myelin disorders are a range of diseases that manifest with severe neurological problems, often from infancy. It has been postulated for some time that stem cells might be an effective treatment for these disorders, primarily as agents to restore dysfunctional or lost myelin. Stem cells,...
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PMID: 20455653
PDF is available here.
PDF is available here.
Revue Neurologique (166)5 2010
Abstract
We report here on the case of a 53-year-old woman with a severe phenotype of TD. The patient had a dizygous twin sister who had only asymptomatic corneal opacities and thrombopenia. CONCLUSION: This family demonstrates the wide intrafamilial phenotype diversity of TD.
Copyright 2009 Elsevier Masson...
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PMID: 20070997
PDF is available here.
PDF is available here.
Rinsho Shinkeigaku (50)5 2010
Abstract
We regarded this case as one of chronic progression of Encephalo-myelo-radiculo-neuropathy....
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PMID: 20535982
PDF is available here.
PDF is available here.
Abstract
We compared the perfusion CT parameters of these patients with those of 24 patients with high grade gliomas. TDLs showed lower permeability surface area product (PS) (0.8 +/- 0.2 vs 2.4 +/- 1.4 ml/100 g/min, P-value 0.014) and lower cerebral blood volume (CBV) (1.0 +/- 0.2 vs 2.8 +/- 1.2 ml/100 g, P...
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PMID: 19830525
PDF is available here.
PDF is available here.
Journal of Neurosurgery (112)5 2010
Abstract
Approximately 350,000 individuals in the US are affected annually by severe and moderate traumatic brain injuries (TBI) that may result in long-term disability. This rate of injury has produced approximately 3.3 million disabled survivors in the US alone. There is currently no specific treatment ava...
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PMID: 19499984
PDF is available here.
PDF is available here.
Nitric Oxide (22)3 2010
Abstract
We determined the activation and consequences of neuronal nitric oxide synthase (nNOS) following stimulation with LPS in the MO3.13 cell line. Our studies show that MO3.13 cells induce nNOS following stimulation with LPS. Most importantly, these studies show a susceptibility of MO3.13 cells to NO me...
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PMID: 20005301
PDF is available here.
PDF is available here.
Neurosurgery (66)4 2010
Abstract
OBJECTIVE: To describe the surgical and ultrastructural findings in the trigeminal root of a patient with trigeminal neuralgia (TN) associated with a cerebellopontine epidermoid tumor, and to relate these to literature reports of patients with vascular compression-related TN. CLINICAL PRESENTATION:...
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PMID: 20190659
PDF is available here.
PDF is available here.
Abstract
New England Journal of Medicine (362)10 2010
Abstract
Brain research (1317) 2010
Abstract
We evaluated OECs for the repair of white matter after transplantation in middle cerebral artery occlusion (MCAO) rat. OECs were cultured from the olfactory bulbs of adult green fluorescent protein (GFP)-expressing transgenic rats and transplanted into peri-infarct basal ganglia imminently after rep...
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PMID: 20043890
PDF is available here.
PDF is available here.
Abstract
Nature Neuroscience (13)3 2010
Abstract
We show that ablation of the prion protein PrP(C) triggers a chronic demyelinating polyneuropathy (CDP) in four independently targeted mouse strains. Ablation of the neighboring Prnd locus, or inbreeding to four distinct mouse strains, did not modulate the CDP. CDP was triggered by depletion of PrP(...
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PMID: 20098419
PDF is available here.
PDF is available here.
Nature Neuroscience (13)3 2010
Abstract
We found that mice lacking the type 2 CXC chemokine receptor (CXCR2) were relatively resistant to cuprizone-induced demyelination and that circulating CXCR2-positive neutrophils were important for cuprizone-induced demyelination. Our findings support a two-hit process of cuprizone-induced demyelinat...
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PMID: 20154684
PDF is available here.
PDF is available here.
Acta Neurologica Belgica (110)1 2010
Abstract
We report the case of a 58-year-old patient who developed distal paraparesis. Serum electrophoresis demonstrated monoclonal IgM paraproteinemia having an anti-GM1 but no anti-MAG activity. Conduction velocities showed demyelinating pattern. Work-up excluded a lymphoproliferative disorder After IVIg...
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PMID: 20514936
PDF is available here.
PDF is available here.
Abstract
We hypothesized that recombinant human erythropoietin (rhEPO) would ameliorate brain damage in anovine model of fetal inflammation. At 107 +/- 1 day of gestational age (DGA), chronically catheterized fetal sheep received on 3 consecutive days 1) an intravenous bolus dose of lipopolysaccharide ([LPS]...
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PMID: 20142760
PDF is available here.
PDF is available here.
Nature Neuroscience (13)2 2010
HDAC1 nuclear export induced by pathological conditions is essential for the onset of axonal damage.
Abstract
We detected cytosolic HDAC1 in damaged axons in brains of humans with multiple sclerosis and of mice with cuprizone-induced demyelination, in ex vivo models of demyelination and in cultured neurons exposed to glutamate and tumor necrosis factor-alpha. Nuclear export of HDAC1 was mediated by the inte...
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PMID: 20037577
PDF is available here.
PDF is available here.
Abstract
Abstract
We report extremely rare combination of central and peripheral demyelination with immune polymyositis in a patient with Bardet-Biedl syndrome. The inciting factor postulated was mycoplasma pneumonia infection.
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PMID: 20653154
PDF is available here.
PDF is available here.
Journal of Virology (84)3 2010
Abstract
We previously generated a mutant virus from our infectious DA full-length clone that has a change of the L* AUG codon to ACG (with no change in the polyprotein's amino acid sequence). Studies of this mutant virus showed that L* was key to the TO subgroup phenotype because the mutant had a decreased...
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PMID: 19923182
PDF is available here.
PDF is available here.
Neuroimmunomodulation (17)4 2010
Abstract
Treatment with high-dose R(+)WIN55,212-2 (10 and 20 mg/kg) significantly improved the clinical performance of the animals during relapse. Interestingly, treatment at any dosage did not affect the brain levels of TNF-alpha, IL-12 and IFN-gamma (T(H)1 response), whereas high-dose cannabinoid treatment...
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PMID: 20203531
PDF is available here.
PDF is available here.
Current Medicinal Chemistry (17)30 2010
Abstract
As the central energy source, the mitochondria are of great importance in the maintenance of the glia cells of the brain. It is presumed that mitochondrial energy production is affected not only by well-characterized genetic mutations of the mitochondria, which are associated with severe malfunction...
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PMID: 20738244
PDF is available here.
PDF is available here.
Abstract
We found that lysophosphatidic acid receptor (LPA1) signaling initiates sciatic nerve injury-induced neuropathic pain and demyelination.
In the present study, we have demonstrated that sciatic nerve injury induces marked demyelination accompanied by myelin-associated glycoprotein (MAG) down-regulati...
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PMID: 21062487
PDF is available here.
PDF is available here.
Gaceta medica de Mexico (146)1 2010
Abstract
These results showed that the taiep rat, a myelin mutant, is a good model for the electrophysiological and pharmacological study of absence epilepsy, particularly considering its resistance to pilocarpine-induced seizures....
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PMID: 20422929
PDF is available here.
PDF is available here.
Acta medica portuguesa (23)4 2010
Abstract
INTRODUCTION: Myelinolysis is defined as acute demyelinating disorder, associated with flaccid quadriplegia, speech and swallowing impairment. The pathogenesis is usually related to hydroelectrolytic imbalance, particularly with severe hyponatremia and its rapid correction. The imagiologic diagnosti...
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PMID: 20688002
PDF is available here.
PDF is available here.
Pediatric Dentistry (32)5 2010
Abstract
4H syndrome/ADDH, a disease of the cerebral white matter, seems to be associated with delayed tooth eruption and other dental abnormalities, which so far could not be assessed conclusively-mainly because patients were too young. The aim of this study was to characterize these abnormalities in a samp...
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PMID: 21070704
PDF is available here.
PDF is available here.
Neurology India (58)3 2010
Abstract
We describe our experience with inflammatory demyelinating disorders of CNS in six children, classified as per the proposed criteria by the Pediatric Multiple Sclersois Study Group. We emphasize the importance of appropriate diagnosis and follow-up to distinguish transient inflammatory demyelinating...
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PMID: 20644279
PDF is available here.
PDF is available here.
Abstract
We report two unusual cases of tumefactive demyelinating lesion. The first patient presented with recurrent right focal tonic-clonic seizures with secondary generalization of three-month duration. Her neurological examination was normal. Another patient presented with right homonymous hemianopia. In...
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PMID: 20622396
PDF is available here.
PDF is available here.
Abstract
Journal of Neurotrauma (26)12 2009
Abstract
We created a model that features continuous gas flow through the chamber during pressurization, and a negative feedback control system to rigorously control dissolved oxygen levels. Monitoring dissolved oxygen continuously during pressurization, we find that the ensuing response exhibits characteris...
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PMID: 19645529
PDF is available here.
PDF is available here.
Ideggyogyaszati szemle (62)11-12 2009
Abstract
We previously reported a male patient with an 18q21.3 deletion, hyperuricemia and typical symptoms of the Lesch-Nyhan syndrome who lacked hypoxanthine-guanine-phosphoribosyl-transferase (HGPRT) deficiency. The patient developed progressive peripheral neuropathy in additon to his profound mental reta...
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PMID: 20025132
PDF is available here.
PDF is available here.
Abstract
This study aims to observe the process of myelin loss and repair following the injection of the gliotoxic agent ethidium bromide (EB) in the sciatic nerve of rats previously induced to diabetes mellitus by streptozotocin. Injection of EB was also done in non-diabetic rats. The animals were euthanati...
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PMID: 20069221
PDF is available here.
PDF is available here.
Journal of Neuroscience (29)46 2009
Abstract
We have developed a myelin-imaging technique based on positron emission tomography (PET). PET is a quantitative imaging modality that has been widely used in clinical settings for direct assessment of biological processes at the molecular level. However, lack of myelin-imaging probes has hampered th...
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PMID: 19923299
PDF is available here.
PDF is available here.
Abstract
I discuss the estimated prevalence of demyelinating pathology, the currently proposed criteria for its identification, implications for therapeutic intervention, and predictors of disease progression....
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PMID: 19806149
PDF is available here.
PDF is available here.
Rinsho Shinkeigaku (49)11 2009
Abstract
Therapeutic options for patients with progressive multiple sclerosis are currently limited. This is in part due to lack of knowledge regarding the pathophysiology of the disease in this stage. This review summarizes recent findings, showing profound differences in the pathology between relapsing and...
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PMID: 20030193
PDF is available here.
PDF is available here.
Abstract
We report a 44-year-old man with the classical Gasperini syndrome due to pontine demyelination in multiple sclerosis. The clinical findings were correlated with changes on MRI. The present case shows that classical crossed brainstem syndromes are topological terms not invariably associated with brai...
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PMID: 19748860
PDF is available here.
PDF is available here.
Cerebellum (8)3 2009
Abstract
We used the cuprizone mouse model to investigate cerebellar demyelination in young adult male mice. The myelin status was analyzed by immunohistochemistry for proteolipoprotein and electron microscopy. The expression and presence of oligodendrocyte, astroglial, and microglia markers were supplementa...
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PMID: 19259754
PDF is available here.
PDF is available here.
Abstract
We report the case of a 22-year-old man who presented with headache and blurred vision which had started four days previous. A periventricular lesion was found in the magnetic resonance imaging. The patient was diagnosed with demyelinating neuritis and treated with intravenous methylprednisolone. DI...
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PMID: 19809927
PDF is available here.
PDF is available here.
Acta clinica Croatica (48)3 2009
Abstract
Demyelinating diseases of the central nervous system include a wide spectrum of different disorders that may resemble multiple sclerosis (MS). The diagnosis of MS is based on typical clinical and paraclinical criteria. The simplified McDonald's criteria, which combine clinical picture, NMR findings,...
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PMID: 20055261
PDF is available here.
PDF is available here.
Abstract
We established a perforin-deficient mouse model on the H-2 major histocompatibility complex background thereby removing confounding factors related to viral biology in this Theiler murine encephalomyelitis virus-susceptible strain. This permitted direct comparison of clinical and pathological parame...
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PMID: 19680139
PDF is available here.
PDF is available here.
Brain Research (1283) 2009
Abstract
We have previously shown that demyelination is also extensive in the cerebral cortex in the cuprizone model. In the current study, we have performed a detailed analysis of the dynamics of demyelination in the cortex in comparison to the corpus callosum. Prominent and almost complete demyelination in...
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PMID: 19524552
PDF is available here.
PDF is available here.
Journal of the Neurological Sciences (282)1-2 2009
Abstract
Gray matter (GM) pathology is an important component of the multiple sclerosis (MS) disease process. Accelerated gray matter atrophy has been observed in MS patients, but its relationship to neurological disability is not defined. This study was done to determine the relationship bet...
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PMID: 19100997
PDF is available here.
PDF is available here.