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Pathology of acute and chronic ischaemic neuropathy in atherosclerotic peripheral vascular disease.

The peripheral nerve pathology in ischaemic limbs with atherosclerotic peripheral vascular diseases (PVD) is difficult to ascertain because … the fascicles of individual nerves and between the nerves of individual subjects: demyelination and remyelination, endoneurial oedema particularly at the subperineurial region, swollen endothelial cells, various but infrequent axonal changes, and relative preservation of UMFs were also seen. All pathological changes found in acute and chronic PVD nerves, except …

Oligodendroglia in Neuromyelitis Optica Spectrum Disorder.

Neuromyelitis optica spectrum disorder (NMOSD) is an inflammatory autoimmune disease of the central nervous system, in which aquaporin-4 imm … y bystander effect, glutamate toxicity, connexin dysregulation, and blood-brain barrier disruption. Remyelination levels in acute NMOSD lesions are low.

Glia cell-derived extracellular vesicles as modulators in spinal cord injury repair.

STUDY DESIGN: Narrative review. OBJECTIVES: The aim of this review was to summarize and critically evaluate current evidence on glial cell- … atory EVs promote recovery. Oligodendrocyte-derived EVs (ODEVs) contribute to metabolic support and remyelination but may also carry inhibitory molecules that limit axonal regeneration. Schwann cell-derived EVs (SCEVs) reduce scar formation and enhance axonal growth, in some models outperforming Schwann cell transplantation. Olfactory ensheathing cell-derived E …

Comparative analysis of Antigliotic Guiding Regenerative Gel (AGRG - LDP-916) for peripheral nerve reconstruction: Evidence from prospective randomized controlled studies.

INTRODUCTION: Peripheral nerve injuries (PNIs) with segmental defects remain a challenge. Autologous nerve grafts (ANG) are the gold standar … ative Gel (AGRG), also known as LDP-916, a bioactive hydrogel designed to promote axonal growth and remyelination. It examines whether AGRG can achieve outcomes comparable to or exceeding ANG while avoiding donor-site morbidity. METHODS: Data were synthesized from two published prospective randomized controlled preclinical studies: (1) acute rat sciatic nerve …

Early-Age Cuprizone Exposure Induces Region-Specific Demyelination and Neuroinflammation in Mice.

Myelin, essential for rapid nerve conduction and axonal integrity in the central and peripheral nervous systems, is compromised in demyelinating diseases, leading to neurological deficits and progressive neurodegeneration. Although remyelination can occur, regeneration in adults is often limited, resulting in incomplete repair and impaired nerve function. In multiple sclerosis (MS), an immune-mediated demyelinating disease with diverse clinical phenotypes, progression and disability correlate with demyelination and failed remyelination, influenced by genetic and environmental factors. A well-e…

Myelin and Oligodendrocyte Dysfunction in Demyelinating and Neurodegenerative Disorders: Signaling Pathways and Therapeutic Targets.

INTRODUCTION: Oligodendrocytes (OLs) synthesize myelin, a substance that plays a significant role in ensuring proper functioning of the Cent … ing Scopus, Google Scholar, Web of Science, and PubMed, focusing on papers relating to myelination, remyelination, and OLs, particularly those addressing signaling pathways and treatment strategies. RESULTS: MS is an autoimmune disease characterized by inflammation that causes demyelination and OL dysfunction. Oxidative stress and mitochondrial dysfunction pla …

Идентификация генов, связанных с Ферроптозом и Гипоксией, в олигодендроцитных клетках, созданных на основе iPSC, от множественных склерозных пациентов.

Множественный склероз (MS) — хроническое провокационное расстройство, характеризующееся обезвоживанием, с неудачным восстановлением, приводящим к постепенной потере аксона в хронической фазе. Клетки-прекурсоры олигодендроциты (ОПК) имеют решающее значение для ремиелиции. Недавние исследования показывают, что как гипоксия, так и ферроптоз играют важнейшую роль в дисфункциональной дифференциации ОПС. Цель этого исследования заключается в выявлении ключевых генов, связанных с гипоксией и ферропотозом и иммунной инфильтрацией в ОПС, которые получены из индуцированных плюрипотентных стволовых клето

Совпадение в аннотации оригинала
Multiple sclerosis (MS) is a chronic inflammatory disorder characterized by demyelination, with failed remyelination leading to progressive axon loss in chronic stages. Oligodendrocyte precursor cells (OPCs) are critical for remyelination. Recent studies suggest that both hypoxia and ferroptosis play crucial roles in the dysfunctional differentiation of OPCs…

Uncompacted myelin in hereditary neuropathy with liability to pressure palsies with the 17 p11.2 deletion.

A 16-year-old girl with a typical features of hereditary neuropathy with liability to pressure palsies (HNPP) and deletion on chromosome 17p11.2 was described. In the mother who was asymptomatic the same genetic defect was found. In a sural nerve biopsy obtained from the girl myelin thickenings characteristic for this disease and de- and remyelination in nerve fibers were found. Special attention was paid to the occurrence of uncompacted myelin, which was present in diffuse and focal forms. It is concluded that high amount of uncompacted myelin is characteristic for HNPP and it is probably rel…

Astrocyte Activation Persists after Recovery of Myelin and Motor Deficits in the Cuprizone Model: a Longitudinal PET and CNS Tissue Analysis Study.

PURPOSE: The cuprizone (CPZ) mouse model for multiple sclerosis enables researchers to investigate the underlying mechanisms of demyelination and remyelination, as well as the effect of therapeutic interventions thereon. Over the last five decades, research revealed detailed analyses of brain tissue derived from CPZ-fed animals at different times of de- and re-myelination. Yet, longitudinal analysis of the effects of a CPZ diet on locomotor performance, myelination and neuroinflammation over a two-month recovery period are still unexplored. PROCEDURES: In this study, we comprehensively examin…

The dual role of mTOR in multiple sclerosis pathophysiology: a systematic review.

Multiple sclerosis (MS) is a chronic autoimmune disease characterized by demyelination, neuroinflammation, and progressive neurodegeneration … aling and may enhance autophagy, offering neuroprotection. In contrast, activation of mTOR promotes remyelination by enhancing oligodendrocyte differentiation and maturation. These remyelinating effects may be masked in inflammatory environments, because activation of mTOR supports immune cell expansion and glial reactivity, inducing inflammation and oxidative …

Multiple sclerosis lesions exhibit sex-specific steroid changes.

Sex differences in disease susceptibility and progression in multiple sclerosis (MS) indicate hormonal influences. We previously found sex differences in steroidogenic enzyme expression in mixed active/inactive (mixed) and inactive lesions, suggesting increased synthesis and signalling of progesterone in females and of estradiol (E2) in males. Here we measured steroid levels in mixed and inactive MS lesions (14 males, 16 females) and control donor white matter (10 males, 10 females) by gas chromatography-mass spectrometry (GC/MS). In mixed and inactive lesions, we found a female-specific incre

Astrocytic phagocytosis of myelin debris and reactive characteristics in vivo and in vitro.

BACKGROUND INFORMATION: Persistent myelin debris can inhibit axonal regeneration, thereby hindering remyelination. Effective removal of myelin debris is essential to eliminate the interference of myelin debris in oligodendrocyte progenitor cell (OPC) activation, recruitment to demyelinating sites and/or differentiation into mature oligodendrocytes (OLs). In addition to microglia, it has been reported that astrocytic phagocytosis of myelin debris is a feature of early demyelination. RESULTS: In the present study, astrocytes effectively phagocytized myelin debris in vitro and in vivo. On the 5t…

Exploring the Nerve Regenerative Capacity of Compounds with Differing Affinity for PPARγ In Vitro and In Vivo.

Damage to peripheral nerves can cause debilitating consequences for patients such as lifelong pain and disability. At present, no drug treatments are routinely given in the clinic following a peripheral nerve injury (PNI) to improve regeneration and remyelination of damaged nerves. Appropriately targeted therapeutic agents have the potential to be used at different stages following nerve damage, e.g., to maintain Schwann cell viability, induce and sustain a repair phenotype to support axonal growth, or promote remyelination. The development of therapies to promote nerve regeneration is current…

[Protective effect of transplantation of human oligodendrocyte precursor cells in a rat model of white matter injury].

OBJECTIVE: To study the effect of human oligodendrocyte precursor cell (hOPC) transplantation in the treatment of white matter injury (WMI). … WMI at the age of 90 days. The electron microscope images showed that hOPC transplantation promoted remyelination in the brain of WMI rats. Compared with the sham-operation group, the model group had a significant increase in the g-ratio (total axon diameter/total fiber diameter). Compared with the model group, the transplantation group had a significant reduct …

Optic nerve involvement in experimental autoimmune encephalomyelitis to homologous spinal cord homogenate immunization in the dark agouti rat.

Dark-Agouti rats were immunized with spinal cord homogenate to develop Experimental Autoimmune Encephalomyelitis, a model of multiple sclerosis. We assessed motor signs and recorded VEPs for five or eight weeks with epidural or epidermal electrodes, respectively, with final histopathology of optic nerves (ONs). Injected rats exhibited motor deficits a week after immunization. VEP delays arose from the 2nd to the 5th week, when a recovery occurred in epidermal-recorded rats. ON damage appeared in epidural-, but not in epidermal-recorded rats, probably due to a remyelination process. VEP could b…

Molecular triggers of neuroinflammation in mouse models of demyelinating diseases.

Myelinating cells wrap axons with multi-layered myelin sheaths for rapid impulse propagation. Dysfunctions of oligodendrocytes or Schwann cells are often associated with neuroinflammation, as observed in animal models of leukodystrophies and peripheral neuropathies, respectively. The neuroinflammatory response modulates the pathological changes, including demyelination and axonal injury, but also remyelination and repair. Here we discuss different immune mechanisms as well as factors released or exposed by myelinating glia in disease conditions. The spectrum of inflammatory mediators varies wi…

The clinical and pathological features of peripheral neuropathy accompanied with HTLV-I associated myelopathy.

We describe the clinical and pathological studies in HTLV-I associated myelopathy (HAM)/tropical spastic paraparesis (TSP) patients with peripheral neuropathy as proven by sural nerve biopsy. Sural nerve pathology in HAM/TSP patients revealed that the most common type of pathologic change is a combination of both demyelination and remyelination and axonal degeneration and regeneration, and this change is modified by the complications. The pathologic changes were correlated with neither the duration of disease nor human T lymphotropic virus type I (HTLV-I) proviral load. This study suggests tha…

[Mitochondrial encephalomyoneuropathy. Case report].

Mitochondrial myopathies and encephalomyopathies constitute a group of degenerative disorders characterized by a striking degree of clinical, biochemical and genetic heterogenity. We present a case of a 42-year old woman with clinical, electrophysiological and laboratory features of a mitochondrial encephalomyoneuropathy. A muscle biopsy specimen showed ragged-red fibres. Signs of demyelination and remyelination of a few fibers were observed in a sural nerve biopsy specimen. MR imaging revealed symmetrical multifocal white matter lesions predominantly in the parieto-occipital region. We stress…

Congenital demyelinating motor and sensory neuropathy with focally folded myelin sheaths.

Six patients (5 index cases and 1 sib) with a congenital motor and sensory neuropathy are described. The clinical, genetic and electrophysiological features resembled Dejerine-Sottas disease or hereditary motor and sensory neuropathy (HMSN) type III. Sural nerve biopsy of 5 patients revealed segmental demyelination and remyelination with hypertrophic changes, although onion bulbs were not as ubiquitous as in classical HMSN type III. A striking discriminating feature from HMSN type III was an abundance of focal myelin thickenings (tomacula) present in nearly all teased fibres. Possible pathogen…

AAEE case report #17: Peripheral neuropathy in monoclonal gammopathy of undetermined significance.

Monoclonal gammopathy of undetermined significance (MGUS) is the most common paraproteinemia associated with polyneuropathy. Although the clinical and electrodiagnostic manifestations most resemble those of chronic inflammatory demyelinating polyneuropathy, some patients manifest a pure sensory neuropathy or neuronopathy. The M protein is usually IgM, and its concentration in serum is low. Nerve pathology from patients with demyelinating disease shows a reduction of large myelinated fibers and segmental demyelination with remyelination. In some cases, the M protein possesses antibody activity …