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1
Maegawa, Gustavo H. B. "Lysosomal Leukodystrophies Lysosomal Storage Diseases Associated With White Matter Abnormalities." Journal of Child Neurology 34, no. 6 (February 13, 2019): 339–58. http://dx.doi.org/10.1177/0883073819828587.
Анотація:
The leukodystrophies are a group of genetic metabolic diseases characterized by an abnormal development or progressive degeneration of the myelin sheath. The myelin is a complex sheath composed of several macromolecules covering axons as an insulator. Each of the leukodystrophies is caused by mutations in genes encoding enzymes that are involved in myelin production and maintenance. The lysosomal storage diseases are inborn disorders of compartmentalized cellular organelles with broad clinical manifestations secondary to the progressive accumulation of undegraded macromolecules within lysosomes and related organelles. The more than 60 different lysosomal storage diseases are rare diseases; however, collectively, the incidence of lysosomal storage diseases ranges just over 1 in 2500 live births. The majority of lysosomal storage diseases are associated with neurologic manifestations including developmental delay, seizures, acroparesthesia, motor weakness, and extrapyramidal signs. These inborn organelle disorders show wide clinical variability affecting individuals from all age groups. In addition, several of neurologic, also known as neuronopathic, lysosomal storage diseases are associated with some level of white matter disease, which often triggers the diagnostic investigation. Most lysosomal storage diseases are autosomal recessively inherited and few are X-linked, with females being at risk of presenting with mild, but clinically relevant neurologic manifestations. Biochemical assays are the basis of the diagnosis and are usually confirmed by molecular genetic testing. Novel therapies have emerged. However, most affected patients with lysosomal storage diseases have only supportive management to rely on. A better understanding of the mechanisms resulting in the leukodystrophy will certainly result in innovative and efficacious disease-modifying therapies.
2
Velichko, Ivan A., and Marina A. Barabanova. "GUILLAIN — BARRÉ SYNDROME AS A RELEVANT ISSUE OF NEUROLOGY (A LITERATURE REVIEW)." Kuban Scientific Medical Bulletin 26, no. 2 (May 17, 2019): 150–61. http://dx.doi.org/10.25207/1608-6228-2019-26-2-150-161.
Анотація:
Acute infl ammatory polyneuropathy is an important research problem of modern neurology. Guillain — Barré syndrome is a severe form of acute polyneuropathy, which is based on autoimmune infl ammation of the myelin sheath of roots and peripheral nerves. Guillain — Barré syndrome is an example of one of the most severe diseases of the nervous system, in which timely diagnosis, proper therapy and qualifi ed care facilitate the achievement of the full recovery of lost functions in most patients. Following an extensive review of Russian and foreign literature, this article discusses modern concepts of Guillain — Barré syndrome, in particular questions related to its epidemiology, etiopathogenesis, classifi cation, clinical features, diagnosis, treatment and prognosis.
3
Popovich, Sofia G., Lyudmila M. Kuzenkova, Olga B. Kondakova, Alexey I. Firumyants, Tatyana V. Podkletnova, and Eugeniya V. Uvakina. "A clinical case of POL3A-associated hypomyelinating leukodystrophy with spinal cord lesion with a debut in early childhood." L.O. Badalyan Neurological Journal 3, no. 3 (September 30, 2022): 122–26. http://dx.doi.org/10.46563/2686-8997-2022-3-3-122-126.
Анотація:
Leukodystrophies are a group of hereditary progressive diseases of the central nervous system characterized by selective lesions in white matter with specific involvement of glial cells. There are hypomyelinating (absence of myelin deposition), demyelinating (loss of previously deposited myelin), dysmyelinating (deposition of structurally or biochemically abnormal myelin), and myelinolytic leukodystrophies (myelin vacuolization). Hypomyelinating leukodystrophies (HL), like most leukodystrophies, debut in childhood or adolescence and are characterized by a progressive course of the disease. HL occurs as a result of impaired synthesis of proteins responsible for the development, structure, and integrity of the myelin sheath, involved in the processes of transcription and translation. In the latter group, the main role is assigned to HL associated with biallelic mutations in the genes of the RNA polymerase III transcription complex, POLR3: POLR3A, POLR3B, POLR1C, and POLR3K. The diagnosis can be confirmed by magnetic resonance imaging of the brain. POLR3A-associated HL is manifested by hypomyelination, hypodontia, and hypogonadotropic hypogonadism. The magnetic resonance features of POLR3-associated HL include diffuse hypomyelination with relative preservation of the dentate nuclei, anterolateral nuclei of the thalamus, globus pallidus, pyramidal tracts at the level of the posterior part of the internal capsules, and the corona radiata. In some cases, thinning of the corpus callosum and atrophy of the cerebellum were also noted. The article presents a clinical case of a patient with POL3A-associated HL with spinal cord injury with the debut in early childhood.
4
Zhang, Juan, Zhu Chen, Hui Chen, Yan Deng, Song Li, and Lian Jin. "Recent Advances in the Roles of MicroRNA and MicroRNA-Based Diagnosis in Neurodegenerative Diseases." Biosensors 12, no. 12 (November 24, 2022): 1074. http://dx.doi.org/10.3390/bios12121074.
Анотація:
Neurodegenerative diseases manifest as progressive loss of neuronal structures and their myelin sheaths and lead to substantial morbidity and mortality, especially in the elderly. Despite extensive research, there are few effective treatment options for the diseases. MicroRNAs have been shown to be involved in the developmental processes of the central nervous system. Mounting evidence suggest they play an important role in the development of neurodegenerative diseases such as Alzheimer’s disease and Parkinson’s disease. However, there are few reviews regarding the roles of miRNAs in neurodegenerative diseases. This review summarizes the recent developments in the roles of microRNAs in neurodegenerative diseases and presents the application of microRNA-based methods in the early diagnosis of these diseases.
5
Livak, P. E., O. S. Korchuk, and N. P. Kozhukh. "Physical rehabilitation and recovery in neurological diseases." Shidnoevropejskij zurnal vnutrisnoi ta simejnoi medicini 2022, no. 2 (2022): 77–80. http://dx.doi.org/10.15407/internalmed2022.02.077.
Анотація:
The author proved that the problem of neurological diseases is relevant despite the high development of medicine, because in conditions of stress, constant fatigue and poor ecology, we see the growth of patients with nervous disorders. Every year, more and more people of all ages turn to doctors with symptoms that indicate complex neurological diseases. Neurology includes a large number of diseases that can occur after viral diseases or due to improper lifestyle. The most common neurological diseases that cause a large number of deaths in Ukraine are Alzheimer’s disease, Parkinson’s disease, and multiple sclerosis. Some neurological diseases are more treatable and easier to tolerate, the progression and development of which can be stopped, while others are severe, which official medicine defines as incurable and lead to disability. It is important to seek the help of a specialist in time to correctly diagnose the disease and avoid unforeseen complications. For each patient it is necessary to select an individual treatment program that depends on the severity of the disease and diagnosis. Today, such a complex and currently incurable neurological disease as multiple sclerosis is becoming more common. This is a chronic autoimmune disease in which the myelin sheath of the nerve fibers of the brain and spinal cord is damaged and thus the functioning of the nervous system is disrupted. Today, this disease is one of the leading causes of disability in young patients. Occurs for unknown reasons and is constantly progressing, leading to serious physical changes and disability. The author argues that physical rehabilitation is a natural and extremely effective method of treating neurological diseases, including multiple sclerosis.
6
Pourakbari, Hakimeh, Yashar Sarbaz, Jalal Parvin, and Mohammad Hossein Vojudi. "Proper Features Extraction from the Multiple Sclerosis Disease Postural Disorders for Decision Support System Definition." Applied Mechanics and Materials 666 (October 2014): 230–34. http://dx.doi.org/10.4028/www.scientific.net/amm.666.230.
Анотація:
Multiple Sclerosis (MS) is one of the most common neurological diseases that it is often progressive and disabling. Its main cause is destruction of myelin sheaths by the immune system. Myelin damage seriously affects people’s physical activities, such as postural impairments. Early detection of the disease is very important in disease management. Unfortunately, currently there is no definite test for MS diagnosis. Of course, there are some tests that help to confirm the diagnosis in advanced stages of the disease butnone of them can independently confirm the disease and have some restrictions and errors. It seems that quantitative analysis of movement disorders especially postural disorders can be helpful in diagnosis of MS even in its early stages. In this study, posturalimpairment was studied. First postural disorders were extracted, then obtained signals were processed quantitatively sovariance and proper frequency features were extracted. At the end, using statistical tests it was shown that these features were significantly different. Therefore based on the results it is possible to design a classifier that can be a firm basis for presenting Decision Support System (DSS) for multiple sclerosis diagnosis.
7
Stamate, Iulia Georgiana, Daniel Alexa, Bogdan Ignat, and Cristian Dinu Popescu. "Ankylosing spondylitis and multiple sclerosis: a surprising parallel." Romanian Journal of Neurology 13, no. 3 (September 30, 2014): 93–102. http://dx.doi.org/10.37897/rjn.2014.3.1.
Анотація:
Ankylosing Spondylitis (AS) and Multiple Sclerosis (MS) are two different chronic autoimmune and inflammatory diseases, one rheumatic and the other one neurological, apparently without any connection between them. Yet a few case reports proved that AS may be associated with MS and this situation should be considered in some epidemiological environment. Although the geographic map of incidence is approximately superposed for both entities there are only a few cases of association. Recent studies revealed that most loci associated with MS are located very close to or even within genes encoding molecules involved in immune system function, confirming the hypothesis that MS is a disease with immunologic mechanism. In addition, some of these loci are also associated at least with another autoimmune disease, thus reinforcing the idea that autoimmune diseases may have a common origin. If MS is more common in patients with AS than in the general population, we can speculate the existence of common factors involved in these diseases. Because of the limited number of cases that associated these two diseases, the correct diagnosis of both entities considered separately or together becomes mandatory especially reflecting on the anti-TNFα agents used to treat AS which may exacerbate or induce a latent MS or demyelination processes in the central nervous system. In terms of pathogenic mechanisms, they are poorly known in both diseases, but each is characterized by an initial immune and inflammatory attack, in MS the target is the myelin sheath and in AS the enthesis tissue. A case with association between the two diseases raises important issues in choosing an appropriate treatment.
8
Fedorova, V. S., A. G. Smochilin, A. I. Kulyakhtin, A. A. Yakovlev, M. S. Pushkaryov, A. V. Gavrichenko, E. A. Gavrilova, and R. A. Gapeshin. "Charcot - Marie - Toots disease: description of 2 clinical cases of the disease in members of the same family (father and daughter)." Scientific Notes of the Pavlov University 27, no. 2 (September 25, 2020): 63–71. http://dx.doi.org/10.24884/1607-4181-2020-27-2-63-71.
Анотація:
Charcot — Marie — Tooth disease belongs to a genetically heterogeneous group of monogenic diseases with a predominant lesion of the peripheral nervous system associated with predominant degeneration of the myelin sheath and/or axon of the motor and sensory nerves and spinal roots, accompanied by motor disorders and specific pain syndrome. The article presents a description of 2 clinical cases of patients (father and daughter), whose disease was manifested by chronically progressive weakness and atrophy of the distal limb muscles, reflexes reduction, foot and hand deformation, gait changes and sensory disorders. Genetic tests were performed to detect duplication/deletion of the PMP22 gene and the expansion of GAA repeats in the FXN gene, which yielded negative results. Based on complaints, neurological examination, family medical history and data of instrumental research (EMNG), we made a clinical diagnosis of the CMT with an autosomal dominant type of inheritance and a high degree of penetrance. At the department, patients received the infusion therapy (neurometabolic therapy, vitamin therapy), physical therapy courses according to an individual plan, physiotherapy and reflexotherapy with a moderate positive effect in the form of a subjective increase in muscle strength in the lower extremities, increasing walking stability and confidence. The article also highlights the peculiarities of the pathogenesis of different genetic variants of CMT and prospect for pathogenetic treatment of this disease.
9
Victor, Praznikov. "Diagnosis and Treatment of Alzheimers Disease and Parkinsons Disease with Resonance Medicine." Journal of Biomedical Research & Environmental Sciences 3, no. 9 (October 2022): 1000–1006. http://dx.doi.org/10.37871/jbres1544.
Анотація:
Parkinson Disease (PD) is a degenerative disease of the extrapyramidal motor system. The formations of the midbrain are affected and are expressed in 1. Tremor, 2. Hypokinesia, 3. Muscle rigidity, 4. Postural instability. Alzheimer's Disease (AD) is a progressive form of senile dementia, leading to a complete loss of cognitive abilities, developing mainly after 60-65 years of age. With these diseases, a large number of brain structures degenerate, including demyelinating brain processes. Both diseases are considered incurable. This article presents materials for the effective treatment of diseases by the method of resonance medicine - mainly the resonance of creation. With the help of the resonance of creation, the degenerated structures of the brain are restored. Until now, no methods have been known to restore dead brain cells. This review aims to present materials on the effective treatment (cure) of these diseases. For the treatment, the resonance therapy method was used, which has been known for at least 25 years and which has been modified in our work since 2016. The essence of our modification was that the author began to use 1. Not only low (previously known), but also high potencies of resonant drugs. 2. We have created a direction in resonance therapy, which is called "resonance of creation." So far, only the direction known as “resonance of destruction” is known, with the help of which oncological tissues, infectious processes, cysts, stones, etc. are treated - destroyed. With the help of the resonance of creation, various biological structures that have undergone degeneration and death are restored - the myelin sheath of the nerves in multiple sclerosis, nerve cells in Parkinson's disease and Alzheimer's disease, etc. With the help of the resonance of creation, the beta cells of the tail of the pancreas are restored in type 1 diabetes, and restoration of the lymphoid system in autoimmune diseases. For the treatment of PD and AD, appropriate preparations are prepared from the tested organopreparations. They are recorded on sugar grains in the potency that is necessary for treatment and resonant treatment of patients is carried out. Treatment of all tested brain structures showed that the patient responds to this treatment quite adequately. In the process of treatment, the “insular cortex” was the first to stop testing, then the “basal nucleus”. As the testability of these brain structures decreased, the patient reported that his condition became significantly better, not only in terms of short-term memory recovery, but also in other indicators. In patients with moderate dementia, in the first weeks of treatment, organ preparations, the "insular cortex", "basal nucleus", "temporal lobe", "isocortex" and those that are characteristic of Parkinson's disease - "lenticular nucleus", "paranigra dopamine nuclei of the middle brain", "roof of the midbrain". Subsequently, the hippocampus, the piriform lobe, the insular field, and the hippocampal pre-basement were no longer tested. At the same time, the reports of the patients' relatives changed: “Our patient began to cry less, be less offended by us and began to recognize loved ones more often - children, grandchildren. Another very important thing is that it has become easier to get up from a chair. Before starting treatment, getting up from a chair was a difficult and lengthy process for her. She became less touchy, became less likely to incontinence. Treatment is carried out until the complete loss of AD symptoms. It has been established that in Alzheimer's disease, the restoration of degenerated formations - the hippocampus, neocortex, amygdala, basal nucleus, isocortex, the pre-foundation of the hippocampus, the insular cortex, the brain septum, the temporal lobe, the parietal lobe, the paralimbic cortex, the piriform lobe led to the restoration these degenerated masses and effective clinical improvement in patients with Alzheimer's disease. The above indicates the effective treatment of PD and AD by the method of resonance of creation.
10
Рушкевич, Ю. Н., С. А. Лихачев, Л. В. Костоправова, Д. В. Науменко, Т. Г. Гвищ, and С. Г. Клюнчик. "Clinical Observation of a Combination of Neurofibromatosis Type I and Multiple Sclerosis." Неврология и нейрохирургия. Восточная Европа, no. 1 (April 29, 2020): 127–38. http://dx.doi.org/10.34883/pi.2020.10.1.051.
Анотація:
В статье изложено описание клинического наблюдения пациента с сочетанием нейрофиброматоза I типа и рассеянного склероза. Наличие этих двух заболеваний у одного и того же пациента встречается крайне редко. Ни одно из клинических проявлений нейрофиброматоза I типа, являющегося генетически детерминированным заболеванием, не связано с демиелинизацией, в то время как центральным звеном в патогенезе рассеянного склероза является разрушение миелиновой оболочки аксонов центральной нервной системы. Проводился детальный анализ течения болезни пациентки с указанием развившихся осложнений. Для визуальной оценки прилагаются иллюстрации магнитно-резонансных и компьютерных томограмм, рентгенограмм. Выполнен обзор литературных данных, согласно которым описан ряд клинических наблюдений пациентов с сочетанием нейрофиброматоза I типа и рассеянного склероза. Приведены сведения о генетической природе наследственного заболевания: ген нейрофиброматоза 1 кодирует белок нейрофибромин, который обладает проонкогенным действием. Рассмотрены возможные патогенетические механизмы сочетанного возникновения этих заболеваний у одного и того же пациента. В качестве основных диагностических критериев изложены клинические симптомы, рекомендованные Международным комитетом экспертов по нейрофиброматозу, также подробно описаны дерматологические, офтальмологические и другие проявления болезни, часто выявляемые осложнения. Представлены современные аспекты диагностики и лечения нейрофиброматоза 1. ThearticledescribestheclinicalobservationofapatientwithacombinationoftypeIneurofibromatosis and multiple sclerosis. The presence of these two diseases in the same patient is extremely rare. None of the clinical manifestations of type I neurofibromatosis, which is a genetically determined disease, is associated with demyelination, while the central link in the pathogenesis of multiple sclerosis is the destruction of the myelin sheath of the axons of the central nervous system. A detailed analysis of the course of the patients disease was carried out, indicating the complications that developed. For visual assessment, illustrations of magnetic resonance and computed tomograms, radiographs are attached. A review of the literature data has been performed, according to which a number of clinical observations of patients with a combination of type I neurofibromatosis and multiple sclerosis are described. Information is given on the genetic nature of a hereditary disease: the neurofibromatosis I gene encodes a protein neurofibromin, which has a pro-oncogenic effect. Possible pathogenetic mechanisms of the combined occurrence of these diseases in the same patient are considered. The main diagnostic criteria are clinical symptoms recommended by the International Committee of Experts on Neurofibromatosis, and dermatological, ophthalmic and other manifestations of the disease, often identified complications, are also described in detail. The modern aspects of the diagnosis and treatment of neurofibromatosis I are presented.
11
Liu, Bin, Wang Xin, Jian-Rong Tan, Rui-Ping Zhu, Ting Li, Dan Wang, Sha-Sha Kan, et al. "Myelin sheath structure and regeneration in peripheral nerve injury repair." Proceedings of the National Academy of Sciences 116, no. 44 (October 14, 2019): 22347–52. http://dx.doi.org/10.1073/pnas.1910292116.
Анотація:
Observing the structure and regeneration of the myelin sheath in peripheral nerves following injury and during repair would help in understanding the pathogenesis and treatment of neurological diseases caused by an abnormal myelin sheath. In the present study, transmission electron microscopy, immunofluorescence staining, and transcriptome analyses were used to investigate the structure and regeneration of the myelin sheath after end-to-end anastomosis, autologous nerve transplantation, and nerve tube transplantation in a rat model of sciatic nerve injury, with normal optic nerve, oculomotor nerve, sciatic nerve, and Schwann cells used as controls. The results suggested that the double-bilayer was the structural unit that constituted the myelin sheath. The major feature during regeneration was the compaction of the myelin sheath, wherein the distance between the 2 layers of cell membrane in the double-bilayer became shorter and the adjacent double-bilayers tightly closed together and formed the major dense line. The expression level of myelin basic protein was positively correlated with the formation of the major dense line, and the compacted myelin sheath could not be formed without the anchoring of the lipophilin particles to the myelin sheath.
12
Demyanova, I. M., D. A. Vshivkov, T. E. Taranushenko, T. V. Kustova, N. A. Konkov, and M. F. Sharoglazov. "HEREDITARY MOTOSENSORY NEUROPATHY TYPE I (CHARCOT–MARIE–TOOTH DISEASE, TYPE I A): A CLINICAL CASE OF AN EARLY ONSET OF THE DISEASE." Pediatria. Journal named after G.N. Speransky 101, no. 2 (April 15, 2022): 176–81. http://dx.doi.org/10.24110/0031-403x-2022-101-2-176-181.
Анотація:
Hereditary motor sensory neuropathies (HMSNs) are the most common degenerative disorders of the peripheral nervous system in children. In this pathology, degenerative lesions of the myelin sheaths and/or axons lead predominantly to distal paralytic amyotrophy, affecting mainly the lower extremities, and are accompanied by areflexia. A clinical case of HMSN type I (Charcot–Marie–Tooth disease, type I A) with an early (up to 1 year) onset of the disease is described. The clinical features of this observation are a very early onset of the disease with a debut before the age of 1 year, the absence of pronounced amyotrophies and characteristic deformities of the feet («hollow foot»). To diagnose this genetic disease, a molecular genetic study was carried out. As a result of DNA sequencing (panel «Neuromuscular diseases»), data were obtained on the presence of a duplication of a segment of chromosome 17 with approximate boundaries of 14005424 – 15162520 bp, covering sections of several genes, including the PMP22 gene. Chromosomal micromatrix targeted analysis revealed a microduplication of the short arm (p) region of chromosome 17 from position 14076430 to position 15441811, covering the 17p12 region. The duplication zone also includes the PMP22 gene, the duplication of which is the cause of Charcot–Marie–Tooth disease, type 1A. At this time, there are no methods of pathogenetic therapy for HMSN, therefore, early diagnosis is of great practical importance, which makes it possible to start rehabilitation measures and prevent the disease in burdened families in a timely manner, based on medical genetic counseling and prenatal DNA diagnostics.
13
Knoll, W., F. Natali, J. Peters, R. Nanekar, C. Wang, and P. Kursula. "Dynamic properties of a reconstituted myelin sheath." Spectroscopy 24, no. 6 (2010): 585–92. http://dx.doi.org/10.1155/2010/317525.
Анотація:
Myelin is a multilamellar membrane which, wrapping the nerve axons, increases the efficiency of nervous signal transmission. Indeed, the molecular components of the myelin sheath interact tightly with each other and molecules on the axonal surface to drive myelination, to keep both myelin and the axon intact, and to transduce signals from myelin to the axon and vice versa. Myelin is strongly affected in human demyelinating diseases in both the central and peripheral nervous system (CNS and PNS, respectively). Despite the presence of a well-defined set of myelin-specific proteins, little is known about the structure and the dynamics of these proteins, their interactions with the membrane and their influence on myelin stability. We present here the first neutron scattering results on the dynamics of the myelin sheath in PNS and of the interaction between its constituents. Specifically, the human P2 protein is shown to stabilize the lipid membrane upon binding to it.
14
Ravera, Silvia, and Isabella Panfoli. "Role of myelin sheath energy metabolism in neurodegenerative diseases." Neural Regeneration Research 10, no. 10 (2015): 1570. http://dx.doi.org/10.4103/1673-5374.167749.
15
KRAWCZYK-MARĆ, IZABELA, AGATA WAWRZYNIAK, IWONA ŁUSZCZEWSKA-SIERAKOWSKA, MAREK BABICZ, and STANISŁAW ORKISZ. "Oligodendrocytes: Morphology, functions and involvement in neurodegenerative diseases." Medycyna Weterynaryjna 75, no. 05 (2019): 6258–2019. http://dx.doi.org/10.21521/mw.6258.
Анотація:
Oligodendrocytes (OLs) are myelinating cells of the central nervous system (CNS). They are a highly specialized type of glial cell in the CNS of vertebrates, which guarantee the transmission of action potentials over long distances by producing a myelin sheath that wraps adjacent axons. Although they are often credited merely with participation in myelination, recent research has led to a radical change in the understanding the role of these glial cells. OLs are currently understood to be plastic and adaptive cells, capable of responding quickly to changes taking place in the spatial neuronal network in the CNS. Due to their complex differentiation process and their physiology, OLs are among the most sensitive cells in the CNS. Finding answers about their interactions with other types of glial cells may result in benefits in the form of neuroprotection and axon plasticity. Damage to OLs and the myelin sheath is one of processes contributing to the development of crippling neurological diseases, although the role of these cells in neurodegeneration remains controversial. This article not only presents OLs as cells whose ultimate goal is to produce myelin sheaths, but also discusses their involvement in neurodegenerative diseases.
16
Alanazi, Asma. "Epstein–Barr Virus (EBV) and Multiple Sclerosis Disease: A Biomedical Diagnosis." Computational Intelligence and Neuroscience 2022 (August 18, 2022): 1–4. http://dx.doi.org/10.1155/2022/3762892.
Анотація:
Multiple sclerosis (MS) is a degenerative disease that affects 2.8 million people worldwide. It is a central nervous system disease (CNS), in which the myelin sheath covering the brain and spinal cord neurons is attacked. If the myelin sheath is damaged, a person can suffer permanent damage to the nerves. There are a number of factors that can increase a person’s risk of developing MS, such as obesity, smoking, vitamin D deficiency, certain tissue types (HLADRB1 ∗ 15 : 01) and infection with the Epstein–Barr virus (EBV). The latter virus can cause infectious mononucleosis, which can, in turn, result in lifelong infection in the host. To establish the relationship between MS and EBV, the author conducted a study on 1176 MS patients admitted to Saudi Arabia King Abdulaziz City centers. The researcher determined that MS occurred twice as much in females as it did in males, and also that EBV was much more widespread in MS female patients than MS male patients (27 : 1). Age was not a factor in the occurrence of EBV. There were limitations on data completeness and availability. Other trials using larger cohorts of patients are needed.
17
Barjas Qaswal, Abdallah. "The Myelin Sheath Maintains the Spatiotemporal Fidelity of Action Potentials by Eliminating the Effect of Quantum Tunneling of Potassium Ions through the Closed Channels of the Neuronal Membrane." Quantum Reports 1, no. 2 (December 6, 2019): 287–94. http://dx.doi.org/10.3390/quantum1020026.
Анотація:
The myelin sheath facilitates action potential conduction along the axons, however, the mechanism by which myelin maintains the spatiotemporal fidelity and limits the hyperexcitability among myelinated neurons requires further investigation. Therefore, in this study, the model of quantum tunneling of potassium ions through the closed channels is used to explore this function of myelin. According to the present calculations, when an unmyelinated neuron fires, there is a probability of 9.15 × 10 − 4 that it will induce an action potential in other unmyelinated neurons, and this probability varies according to the type of channels involved, the channels density in the axonal membrane, and the surface area available for tunneling. The myelin sheath forms a thick barrier that covers the potassium channels and prevents ions from tunneling through them to induce action potential. Hence, it confines the action potentials spatiotemporally and limits the hyperexcitability. On the other hand, lack of myelin, as in unmyelinated neurons or demyelinating diseases, exposes potassium channels to tunneling by potassium ions and induces the action potential. This approach gives different perspectives to look at the interaction between neurons and explains how quantum physics might play a role in the actions occurring in the nervous system.
18
Dziedzic, Angela, Elzbieta Miller, Joanna Saluk-Bijak, and Michal Bijak. "The GPR17 Receptor—A Promising Goal for Therapy and a Potential Marker of the Neurodegenerative Process in Multiple Sclerosis." International Journal of Molecular Sciences 21, no. 5 (March 8, 2020): 1852. http://dx.doi.org/10.3390/ijms21051852.
Анотація:
One of the most important goals in the treatment of demyelinating diseases such as multiple sclerosis (MS) is, in addition to immunomodulation, reconstruction of the lost myelin sheath. The modulator of the central nervous system myelination is the metabotropic receptor coupled to the G-protein: GPR17. GPR17 receptors are considered to be sensors of local damage to the myelin sheath, and play a role in the reconstruction and repair of demyelinating plaques caused by ongoing inflammatory processes. GPR17 receptors are present on nerve cells and precursor oligodendrocyte cells. Under physiological conditions, they are responsible for the differentiation and subsequent maturation of oligodendrocytes, while under pathological conditions (during damage to nerve cells), their expression increases to become mediators in the demyelinating processes. Moreover, they are essential not only in both the processes of inducing damage and the death of neurons, but also in the local repair of the damaged myelin sheath. Therefore, GPR17 receptors may be recognized as the potential goal in creating innovative therapies for the treatment of the neurodegenerative process in MS, based on the acceleration of the remyelination processes. This review examines the role of GRP17 in pathomechanisms of MS development.
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Rabelo Gonzalez Veldman, Danielle, Fernando Henrique Batista da Mota, Laís Ribeiro Vieira, Igor Diego Carrijo dos Santo, Anna Carolina Araújo Marques, Alessandra Roma Rodrigues, and Pieter Monteiro da Silva Veldman. "Malignant peripheral nerve sheath tumor - a case report and review." Health Residencies Journal - HRJ 1, no. 3 (May 16, 2020): 16–27. http://dx.doi.org/10.51723/hrj.v1i3.1.
Анотація:
This case report aims to contribute to the understanding and to present a new look on the treatment of a rare, aggressive and poorly understood pathology, known as malignant tumor of the peripheral nerve myelin sheath. The objective of this research is to help building a better understanding of this pathology and to evaluate what is new in diagnosis and treatment. We used PubMed’s articles with the descriptors: malignant peripheral nerve sheath tumor, Ki67, and malignant tumor immunohistochemistry of the peripheral nerve sheath. 17 articles were selected. We also used the descriptor “sarcoma staging” for the visualization of a book chapter and a journal. Malignant neoplasm of the peripheral nerve myelin sheath is a disease that mainly affects neurofibromatosis-1 or patients with prior radiotherapy, but may occur randomly, as with the patient in question. Due to illness multiple incidence locations, the symptoms may be late and staging is difficult. The staging is performed taking into account the size of the primary tumor, its location, lymph node involvement, presence of distant metastases and degree of cell differentiation. The diagnosis is made after resection of the piece with anatomopathological and immunohistochemical analysis, which may delay the treatment. Therefore, it has been seen that the most common treatment continues to be complete surgery with free margins, but there are promising studies in the genetics field for the treatment and better understanding of this pathology
20
Ye, Hui, and Jeffrey Ng. "Shielding effects of myelin sheath on axolemma depolarization under transverse electric field stimulation." PeerJ 6 (December 3, 2018): e6020. http://dx.doi.org/10.7717/peerj.6020.
Анотація:
Axonal stimulation with electric currents is an effective method for controlling neural activity. An electric field parallel to the axon is widely accepted as the predominant component in the activation of an axon. However, recent studies indicate that the transverse component to the axolemma is also effective in depolarizing the axon. To quantitatively investigate the amount of axolemma polarization induced by a transverse electric field, we computed the transmembrane potential (Vm) for a conductive body that represents an unmyelinated axon (or the bare axon between the myelin sheath in a myelinated axon). We also computed the transmembrane potential of the sheath-covered axonal segment in a myelinated axon. We then systematically analyzed the biophysical factors that affect axonal polarization under transverse electric stimulation for both the bare and sheath-covered axons. Geometrical patterns of polarization of both axon types were dependent on field properties (magnitude and field orientation to the axon). Polarization of both axons was also dependent on their axolemma radii and electrical conductivities. The myelin provided a significant “shielding effect” against the transverse electric fields, preventing excessive axolemma depolarization. Demyelination could allow for prominent axolemma depolarization in the transverse electric field, via a significant increase in myelin conductivity. This shifts the voltage drop of the myelin sheath to the axolemma. Pathological changes at a cellular level should be considered when electric fields are used for the treatment of demyelination diseases. The calculated term for membrane polarization (Vm) could be used to modify the current cable equation that describes axon excitation by an external electric field to account for the activating effects of both parallel and transverse fields surrounding the target axon.
21
Wu, WenYu, Xiangkai Zhen, and Ning Shi. "DNA-binding domain of myelin-gene regulatory factor: purification, crystallization and X-ray analysis." Acta Crystallographica Section F Structural Biology Communications 73, no. 7 (June 17, 2017): 393–97. http://dx.doi.org/10.1107/s2053230x17007828.
Анотація:
The myelin sheath, which envelops axons in the vertebrate central nervous system, is crucial for the rapid conduction of action potentials. Myelin-gene regulatory factor (MRF) is a recently identified transcription factor that is required for myelin-sheath formation. Loss of MRF leads to demyelinating diseases and motor learning deficiency. MRF is a membrane-bound transcription factor that undergoes autocleavage from the endoplasmic reticulum membrane. The N-terminus of MRF contains a DNA-binding domain (DBD) that functions as a homotrimer. In this study, the MRF DBD was cloned, purified and crystallized in order to understand the molecular mechanism that regulates the transcription of myelin genes. Selenomethionine was subsequently introduced into the crystals to obtain the phases for the MRF DBD structure. The native and selenomethionine-labelled crystals exhibited diffraction to 2.50 and 2.51 Å resolution, respectively. The crystals belonged to space groupP321 and the selenomethionine-labelled crystals had unit-cell parametersa= 104.0,b= 104.0,c= 46.7 Å, α = 90, β = 90, γ = 120°. The calculated Matthews coefficient was 3.04 Å3Da−1and the solvent content was 59.5%, indicating the presence of one MRF DBD molecule in the asymmetric unit.
22
Shah, Sangam, Rajeev Ojha, Sanjeeta Sitaula, Dosti Regmi, Ragesh Karn, Bikram Prasad Gajurel, Reema Rajbhandari, Niraj Gautam, Sunanda Paudel, and Aashish Shrestha. "Recurrent Bilateral Optic Neuritis Associated with Myelin Oligodendrocyte Glycoprotein Antibody: A Case Report from Nepal." Case Reports in Neurological Medicine 2021 (August 26, 2021): 1–3. http://dx.doi.org/10.1155/2021/8100423.
Анотація:
Neuromyelitis optica spectrum disorder (NMOSD) is an immune-mediated inflammatory condition involving spinal cord and optic nerves. Diagnosis of NMOSD is done by aquaporin-4 antibody (AQP4) in patients with optic neuritis. Myelin oligodendrocyte glycoprotein (MOG) expressed on the oligodendrocyte cell surface and on the outermost cell surface of the myelin sheath may also be present in patients with NMOSD bilateral optic neuritis. Here, we describe a case of a thirty-nine-year-old-female with recurrent bilateral optic neuritis with positive anti-MOG antibody, and anti-MOG syndrome has not previously been reported from Nepal.
23
Joshi, Anisha, and Deeptara Pathak Thapa. "Asymptomatic nodule on face: Dermoscopic and histopathological clue for diagnosis." Nepal Journal of Neuroscience 17, no. 1 (April 7, 2020): 63–65. http://dx.doi.org/10.3126/njn.v17i1.28369.
Анотація:
Schwannomas/ neurilemmomas are benign tumors of nerve sheath arising from Schwann cells that form myelin sheath around peripheral nerves. They are usually solitary, slow growing and encapsulated lesions. Head and neck are the common sites.
We report a case of a 38 years old Nepalese female who had presented with a solitary asymptomatic, slow growing nodule on the left side of the chin for the last three years. Dermoscopy of the lesion revealed arborizing vessels with brownish pigmentation overlying a whitish to pinkish background. Complete excision of the lesion was performed. Histopathological evaluation of the lesion revealed schwannoma.
Though schwannomas are a rare diagnosis, they should be considered as a differential diagnosis of any unilateral, asymptomatic, slow growing nodule in the head and neck region. Dermoscopy is a useful tool which helps to differentiate schwannoma from other lesions. Histopathology is the gold standard for diagnosis and the treatment of choice is surgical excision.
24
Mo, Yun Jeong, Yu-Seon Kim, Minseok S. Kim, and Yun-Il Lee. "Advantages of Adult Mouse Dorsal Root Ganglia Explant Culture in Investigating Myelination in an Inherited Neuropathic Mice Model." Methods and Protocols 5, no. 4 (July 22, 2022): 66. http://dx.doi.org/10.3390/mps5040066.
Анотація:
A co-culture of neurons and Schwann cells has frequently been used to investigate myelin sheath formation. However, this approach is restricted to myelin-related diseases of the peripheral nervous system. This study introduces and compares an ex vivo model of adult-mouse-derived dorsal root ganglia (DRG) explant, with an in vitro co-culture of dissociated neurons from mouse embryo DRG and Schwann cells from a mouse sciatic nerve. The 2D co-culture has disadvantages of different mouse isolation for neurons and Schwann cells, animal number, culture duration, and the identification of disease model. However, 3D DRG explant neurons and myelination cells in Matrigel-coated culture are obtained from the same mouse, the culture period is shorter than that of 2D co-culture, and fewer animals are needed. In addition, it has simpler and shorter experimental steps than 2D co-culture. This culture system may prove advantageous in studies of biological functions and pathophysiological mechanisms of disease models, since it can reflect disease characteristics as traditional co-culture does. Therefore, it is suggested that a DRG explant culture is a scientifically, ethically, and economically more practical option than a co-culture system for studying myelin dynamics, myelin sheath formation, and demyelinating disease.
25
Fiala, Clare, Dalia Rotstein, and Maria D. Pasic. "Pathobiology, Diagnosis, and Current Biomarkers in Neuromyelitis Optica Spectrum Disorders." Journal of Applied Laboratory Medicine 7, no. 1 (January 1, 2022): 305–10. http://dx.doi.org/10.1093/jalm/jfab150.
Анотація:
Abstract Background Neuromyelitis optica spectrum disorder (NMOSD) is characterized by chronic inflammation of the central nervous system (CNS), particularly the optic nerves and spinal cord. Although it displays some clinical features similar to multiple sclerosis (MS), the etiology and treatment are distinct, and therefore accurate diagnosis is essential. Autoantibodies targeting the water channel protein aquaporin-4 (AQP4) and the myelin sheath protein myelin oligodendrocyte glycoprotein are the major antigen-specific serological biomarkers known to date, with destruction of astrocytes as the primary mode of CNS damage in AQP4-positive disease. Content This mini-review summarizes the pathobiology, clinical features, and current methods of serological testing used to assess NMOSD and differentiate this disorder from MS. A brief summary of emerging therapies is also presented. Summary NMOSD can be distinguished from MS through a combination of clinical findings, imaging investigations, and serological analysis. Seronegative cases are particularly difficult to diagnose and can pose a challenge to clinicians. As knowledge deepens, new therapies and biomarkers are expected to improve treatment of this rare debilitating disease.
26
Ai, Ruo-Song, Kun Xing, Xin Deng, Juan-Juan Han, Dong-Xia Hao, Wen-Hui Qi, Bing Han, Ya-Na Yang, Xing Li та Yuan Zhang. "Baicalin Promotes CNS Remyelination via PPARγ Signal Pathway". Neurology - Neuroimmunology Neuroinflammation 9, № 2 (1 лютого 2022): e1142. http://dx.doi.org/10.1212/nxi.0000000000001142.
Анотація:
Background and ObjectivesDemyelinating diseases in the CNS are characterized by myelin sheath destruction or formation disorder that leads to severe neurologic dysfunction. Remission of such diseases is largely dependent on the differentiation of oligodendrocytes precursor cells (OPCs) into mature myelin-forming OLGs at the demyelinated lesions, which is defined as remyelination. We discover that baicalin (BA), a natural flavonoid, in addition to its well-known antiinflammatory effects, directly stimulates OLG maturation and CNS myelin repair.MethodsTo investigate the function of BA on CNS remyelination, we develop the complementary in vivo and in vitro models, including physiologic neonatal mouse CNS myelinogenesis model, pathologic cuprizone-induced (CPZ-induced) toxic demyelination model, and postnatal OLG maturation assay. Furthermore, molecular docking, pharmacologic regulation, and transgenic heterozygous mice were used to clarify the target and action of the mechanism of BA on myelin repair promotion.ResultsAdministration of BA was not only merely effectively enhanced CNS myelinogenesis during postnatal development but also promoted remyelination and reversed the coordination movement disorder in the CPZ-induced toxic demyelination model. Of note, myelin-promoting effects of BA on myelination or regeneration is peroxisome proliferator-activated receptor γ (PPARγ) signaling-dependent.DiscussionOur work demonstrated that BA promotes myelin production and regeneration by activating the PPARγ signal pathway and also confirmed that BA is an effective natural product for the treatment of demyelinating diseases.
27
Baranova, Svetlana V., Pavel S. Dmitrienok, Valentina N. Buneva, and Georgy A. Nevinsky. "HIV-Infected Patients: Cross Site-Specific Hydrolysis of H2a and H2b Histones and Myelin Basic Protein with Antibodies against These Three Proteins." Biomolecules 10, no. 11 (October 30, 2020): 1501. http://dx.doi.org/10.3390/biom10111501.
Анотація:
Anti-DNA antibodies are usually produced against histone-DNA complexes appearing during cell apoptosis, while histones are known as damage-associated molecules. A myelin sheath of axons contains myelin basic protein (MBP) playing an important role in the pathogenesis of autoimmune diseases. Antibodies with enzymatic activities (abzymes) are distinctive features of some autoimmune and viral diseases. Abzymes against different proteins can usually only hydrolyze these specific proteins. Using sequential chromatographies of homogeneous IgG preparations from sera of HIV-infected patients on columns with immobilized MBP, H2a, and H2b histones, the anti-MBP, anti-H2a, and anti-H2b antibodies were obtained. It was first shown that IgGs against H2a and H2b effectively hydrolyze these histones and MBP, while anti-MBP split MBP, H2a, and H2b, but no other control proteins. Using the MALDI mass spectrometry, the cleavage sites of H2a, H2b, and MBP by abzymes against these three proteins were found. Among 14 sites of hydrolysis of H2a by IgGs against H2a and 10 sites by anti-MBP IgGs, only one site of hydrolysis was the same for these abzymes. Eleven cleavage sites of H2b with IgGs against H2b and 10 sites of its hydrolysis with antibodies against MBP were different. Anti-H2a, anti-H2b, and anti-MBP abzymes are unpredictable examples of IgGs possessing not only cross-complexation but also catalytic cross-reactivity, which may be a common phenomenon for such abzymes in patients with different autoimmune diseases. The existence of cross-reactivity of abzymes against H2a and H2b histones and MBP represent a great danger to humans since, in contrast with MBP, histones due to cell apoptosis constantly occur in human blood. Anti-H2a, anti-H2b, and anti-MBP can attack and hydrolyze myelin basic protein of the myelin sheath of axons and plays a negative role in the pathogenesis of several pathologies.
28
Breton, Jocelyn M., Kimberly L. P. Long, Matthew K. Barraza, Olga S. Perloff, and Daniela Kaufer. "Hormonal Regulation of Oligodendrogenesis II: Implications for Myelin Repair." Biomolecules 11, no. 2 (February 16, 2021): 290. http://dx.doi.org/10.3390/biom11020290.
Анотація:
Alterations in myelin, the protective and insulating sheath surrounding axons, affect brain function, as is evident in demyelinating diseases where the loss of myelin leads to cognitive and motor dysfunction. Recent evidence suggests that changes in myelination, including both hyper- and hypo-myelination, may also play a role in numerous neurological and psychiatric diseases. Protecting myelin and promoting remyelination is thus crucial for a wide range of disorders. Oligodendrocytes (OLs) are the cells that generate myelin, and oligodendrogenesis, the creation of new OLs, continues throughout life and is necessary for myelin plasticity and remyelination. Understanding the regulation of oligodendrogenesis and myelin plasticity within disease contexts is, therefore, critical for the development of novel therapeutic targets. In our companion manuscript, we review literature demonstrating that multiple hormone classes are involved in the regulation of oligodendrogenesis under physiological conditions. The majority of hormones enhance oligodendrogenesis, increasing oligodendrocyte precursor cell differentiation and inducing maturation and myelin production in OLs. Thus, hormonal treatments present a promising route to promote remyelination. Here, we review the literature on hormonal regulation of oligodendrogenesis within the context of disorders. We focus on steroid hormones, including glucocorticoids and sex hormones, peptide hormones such as insulin-like growth factor 1, and thyroid hormones. For each hormone, we describe whether they aid in OL survival, differentiation, or remyelination, and we discuss their mechanisms of action, if known. Several of these hormones have yielded promising results in both animal models and in human conditions; however, a better understanding of hormonal effects, interactions, and their mechanisms will ultimately lead to more targeted therapeutics for myelin repair.
29
Mazhar, Faizan, and Nafis Haider. "Some Unique Considerations in Treatment of Multiple Sclerosis." Asian Journal of Pharmaceutical Research and Health Care 8, no. 3 (June 23, 2016): 72. http://dx.doi.org/10.18311/ajprhc/2016/3909.
Анотація:
Multiple sclerosis is one of several diseases described as demyelinating because it causes damage to the myelin sheath. The presence of additional medical condition like pregnancy, osteoporosis and infections is common with multiple sclerosis that adversely affects the health outcomes. The treatment of MS becomes more complex when compounded by these existing additional medical condition. This review highlight important pharmacotherapeutic considerations in treatment of MS in these special patient population.
30
Das Sarma, Jayasri. "A Mechanism of Virus-Induced Demyelination." Interdisciplinary Perspectives on Infectious Diseases 2010 (2010): 1–28. http://dx.doi.org/10.1155/2010/109239.
Анотація:
Myelin forms an insulating sheath surrounding axons in the central and peripheral nervous systems and is essential for rapid propagation of neuronal action potentials. Demyelination is an acquired disorder in which normally formed myelin degenerates, exposing axons to the extracellular environment. The result is dysfunction of normal neuron-to-neuron communication and in many cases, varying degrees of axonal degeneration. Numerous central nervous system demyelinating disorders exist, including multiple sclerosis. Although demyelination is the major manifestation of most of the demyelinating diseases, recent studies have clearly documented concomitant axonal loss to varying degrees resulting in long-term disability. Axonal injury may occur secondary to myelin damage (outside-in model) or myelin damage may occur secondary to axonal injury (inside-out model). Viral induced demyelination models, has provided unique imminent into the cellular mechanisms of myelin destruction. They illustrate mechanisms of viral persistence, including latent infections, virus reactivation and viral-induced tissue damage. These studies have also provided excellent paradigms to study the interactions between the immune system and the central nervous system (CNS). In this review we will discuss potential cellular and molecular mechanism of central nervous system axonal loss and demyelination in a viral induced mouse model of multiple sclerosis.
31
Del Giovane, Alice, and Antonella Ragnini-Wilson. "Targeting Smoothened as a New Frontier in the Functional Recovery of Central Nervous System Demyelinating Pathologies." International Journal of Molecular Sciences 19, no. 11 (November 20, 2018): 3677. http://dx.doi.org/10.3390/ijms19113677.
Анотація:
Myelin sheaths on vertebrate axons provide protection, vital support and increase the speed of neuronal signals. Myelin degeneration can be caused by viral, autoimmune or genetic diseases. Remyelination is a natural process that restores the myelin sheath and, consequently, neuronal function after a demyelination event, preventing neurodegeneration and thereby neuron functional loss. Pharmacological approaches to remyelination represent a promising new frontier in the therapy of human demyelination pathologies and might provide novel tools to improve adaptive myelination in aged individuals. Recent phenotypical screens have identified agonists of the atypical G protein-coupled receptor Smoothened and inhibitors of the glioma-associated oncogene 1 as being amongst the most potent stimulators of oligodendrocyte precursor cell (OPC) differentiation in vitro and remyelination in the central nervous system (CNS) of mice. Here, we discuss the current state-of-the-art of studies on the role of Sonic Hedgehog reactivation during remyelination, referring readers to other reviews for the role of Hedgehog signaling in cancer and stem cell maintenance.
32
Pi, Keng-Shuo, Yurou Sang, and Suzana K. Straus. "Viral Proteins with PxxP and PY Motifs May Play a Role in Multiple Sclerosis." Viruses 14, no. 2 (January 28, 2022): 281. http://dx.doi.org/10.3390/v14020281.
Анотація:
Multiple sclerosis (MS) is a debilitating disease that arises from immune system attacks to the protective myelin sheath that covers nerve fibers and ensures optimal communication between brain and body. Although the cause of MS is unknown, a number of factors, which include viruses, have been identified as increasing the risk of displaying MS symptoms. Specifically, the ubiquitous and highly prevalent Epstein–Barr virus, human herpesvirus 6, cytomegalovirus, varicella–zoster virus, and other viruses have been identified as potential triggering agents. In this review, we examine the specific role of proline-rich proteins encoded by these viruses and their potential role in MS at a molecular level.
33
Intisar, Aseer, Woon-Hae Kim, Hyun Young Shin, Min Young Kim, Yu Seon Kim, Heejin Lim, Hyun Gyu Kang, et al. "An electroceutical approach enhances myelination via upregulation of lipid biosynthesis in the dorsal root ganglion." Biofabrication 14, no. 1 (January 1, 2022): 015017. http://dx.doi.org/10.1088/1758-5090/ac457c.
Анотація:
Abstract As the myelin sheath is crucial for neuronal saltatory conduction, loss of myelin in the peripheral nervous system (PNS) leads to demyelinating neuropathies causing muscular atrophy, numbness, foot deformities and paralysis. Unfortunately, few interventions are available for such neuropathies, because previous pharmaceuticals have shown severe side effects and failed in clinical trials. Therefore, exploring new strategies to enhance PNS myelination is critical to provide solution for such intractable diseases. This study aimed to investigate the effectiveness of electrical stimulation (ES) to enhance myelination in the mouse dorsal root ganglion (DRG)—an ex vivo model of the PNS. Mouse embryonic DRGs were extracted at E13 and seeded onto Matrigel-coated surfaces. After sufficient growth and differentiation, screening was carried out by applying ES in the 1–100 Hz range at the beginning of the myelination process. DRG myelination was evaluated via immunostaining at the intermediate (19 days in vitro (DIV)) and mature (30 DIV) stages. Further biochemical analyses were carried out by utilizing ribonucleic acid sequencing, quantitative polymerase chain reaction and biochemical assays at both intermediate and mature myelination stages. Imaging of DRG myelin lipids was carried out via time-of-flight secondary ion mass spectrometry (ToF-SIMS). With screening ES conditions, optimal condition was identified at 20 Hz, which enhanced the percentage of myelinated neurons and average myelin length not only at intermediate (129% and 61%) but also at mature (72% and 17%) myelination stages. Further biochemical analyses elucidated that ES promoted lipid biosynthesis in the DRG. ToF-SIMS imaging showed higher abundance of the structural lipids, cholesterol and sphingomyelin, in the myelin membrane. Therefore, promotion of lipid biosynthesis and higher abundance of myelin lipids led to ES-mediated myelination enhancement. Given that myelin lipid deficiency is culpable for most demyelinating PNS neuropathies, the results might pave a new way to treat such diseases via electroceuticals.
34
Kravchuk, N. O. "Clinical case: criteria for differential diagnosis and treatment of chronic inflammatory demyelinating polyneuropathy." East European Journal of Neurology, no. 4(4) (September 20, 2015): 48–52. http://dx.doi.org/10.33444/2411-5797.2015.4(4).48-52.
Анотація:
Chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare neurological disease that is characterized by inflammation of nerve roots and peripheral nerves, nerve destruction of the myelin sheath, the appearance of slowly-progressive symmetric symptoms, motor and sensory disorders. CIDPsometimes considered a chronic form of acute inflammatory demyelinating polyneuropathy (АIDP) - Guillain Barré syndrome (GBS). In contrast to GBS, most patients do not mark HDPNP previous viral or infectious disease. CIDP is subacute-progressive disease that clinically takes 3-4 weeks, then usually comes plateau phase, which changes the phase of gradual regression of symptoms. CIDP can affect any age group. CIDP registered twice as often in men. The average age of onset of the disease is 50 years. CIDP prevalence is estimated at about 5-7 cases per 100 thousand people.
35
Meiling, James Bryan, and Priya Kaji Bui. "Recurring Migraines as the Presenting Symptom of Pediatric Multiple Sclerosis in a Teenage Girl: A Case Report." Journal of Pediatric Neurology 18, no. 05 (July 19, 2019): 263–66. http://dx.doi.org/10.1055/s-0039-1693482.
Анотація:
AbstractMultiple sclerosis (MS) is a chronic disease of the central nervous system that leads to a progressive breakdown of the myelin sheath by self-harming autoantibodies. Both MS and migraines have a predilection for women as opposed to men. In addition, both can come across as acute attacks on the body that negatively affect the ability of an individual to function. Are they associative concurrent afflictions or is one the primary causality of the other? This case report represents a teenage girl who presented to her pediatrician with recurrent migraines, which led to a diagnosis of pediatric MS.
36
Gomes, Ana Beatriz Ayroza Galvão Ribeiro, and Tarso Adoni. "Differential diagnosis of demyelinating diseases: what's new?" Arquivos de Neuro-Psiquiatria 80, no. 5 suppl 1 (May 2022): 137–42. http://dx.doi.org/10.1590/0004-282x-anp-2022-s109.
Анотація:
ABSTRACT Background: Acquired demyelinating disorders lead to overlapping visual, pyramidal, sensory, autonomic, and cerebellar deficits and may lead to severe disability. Early diagnosis and start of treatment are fundamental towards preventing further attacks and halting disability. Objective: In this paper we provide an updated overview of the differential diagnoses of acquired demyelinating disorders. Methods: We performed a critical targeted review of the diagnoses of the most prevalent demyelinating disorders: multiple sclerosis (MS), neuromyelitis optica spectrum disorders (NMOSD) and myelin oligodendrocyte glycoprotein antibody disease (MOGAD). Results: We discuss the workup, diagnostic criteria and new biomarkers currently being used for the diagnosis of these disease entities taking into account the particularities of the Brazilian population and healthcare system. Conclusion: A comprehensive analysis of medical history, physical examination, biomedical and imaging data should be performed to obtain differential diagnosis. Diagnostic criteria should be mindfully employed considering ethnic and environmental particularities of each patient.
37
Wu, Minghui, Qi Kang, Yuezhi Kang, Yanping Tong, Tao Yang, and Yongping Fan. "Catalpol Regulates Oligodendrocyte Regeneration and Remyelination by Activating the GEF-Cdc42/Rac1 Signaling Pathway in EAE Mice." Evidence-Based Complementary and Alternative Medicine 2022 (November 25, 2022): 1–18. http://dx.doi.org/10.1155/2022/7074157.
Анотація:
The main obstacle to remyelination in demyelinating diseases, such as multiple sclerosis, is the inability of oligodendrocyte precursor cells (OPCs) to differentiate into mature oligodendrocytes (OLs) in the demyelinating region. Consequently, promoting OL differentiation and myelin remodeling is a key goal in the search for treatments. Rho GTPases play diverse and important roles throughout the development of neuronal axons and the formation of the myelin sheath. The current study aimed to investigate the direct protective effects of catalpol on demyelination damage induced by myelin oligodendrocyte glycoprotein (MOG) immunization and to explore whether the GEF-Cdc42/Rac1 signaling pathway contributes to the regeneration effect induced by catalpol. In the MOG-induced experimental autoimmune encephalomyelitis (EAE) mouse model of demyelination, we observed that catalpol significantly promoted OL development by enhancing the expression of glutathione S-transferase pi (GST-pi) in the affected brain. By Luxol fast blue staining and myelin basic protein (MBP) expression assessment, catalpol was found to increase MBP expression and promote myelin repair. Furthermore, catalpol promoted OL differentiation associated with the upregulation of Cdc42/Rac1 expression and activation in vivo. In addition, PAK1/MRCKα, proteins downstream of Cdc42/Rac1, was positively regulated by catalpol. We also found that catalpol alleviated clinical neurological dysfunction, inhibited inflammatory infiltration, increased the proportion of Treg cells, and suppressed demyelination. Overall, our study is the first to reveal that catalpol can promote OL generation and myelination and contributes to the crucial regulatory process of GEF-Cdc42/Rac1 signaling expression and activation. Therefore, catalpol is a promising drug candidate for the potential treatment of demyelinating diseases.
38
Sousa, Aurea D., and Manzoor A. Bhat. "Cytoskeletal transition at the paranodes: the Achilles' heel of myelinated axons." Neuron Glia Biology 3, no. 2 (March 15, 2007): 169–78. http://dx.doi.org/10.1017/s1740925x07000415.
Анотація:
AbstractMyelination organizes axons into distinct domains that allow nerve impulses to propagate in a saltatory manner. The edges of the myelin sheath are sealed at the paranodes by axon–glial junctions that have a crucial role in organizing the axonal cytoskeleton. Here we propose a model in which the myelinated axons depend on the axon–glial junctions to stabilize the cytoskeletal transition at the paranodes. Thus paranodal regions are likely to be particularly susceptible to damage induced by demyelinating diseases such as multiple sclerosis.
39
Pathapati, Deepthi, Kiran Barla, Monal Dayal, Rajitha Gati, and Praveen Kumar Lakota. "Facial Nerve Schwannoma: The Rare/Great Mimicker of Vestibular Schwannoma/Neuroma." Indian Journal of Radiology and Imaging 31, no. 02 (April 2021): 510–13. http://dx.doi.org/10.1055/s-0041-1734356.
Анотація:
AbstractSchwannomas are benign tumors arising from Schwann cells which are a protective casing of nerves, composing myelin sheath and can develop in any nerve where Schwann cells are present. Most common are vestibulocochlear nerve schwannomas. Facial nerve schwannomas (FNSs) are uncommon tumors involving seventh nerve of which geniculate ganglion involvement is most common. Clinical presentations and the imaging appearances of FNSs are influenced by the topographical anatomy of the facial nerve and vary according to the segments involved. We report a case of 73-year-old man presenting with right side facial weakness of lower motor neuron type involvement. Computed tomography and magnetic resonance imaging are clinching the diagnosis. An early diagnosis is important in containing the disease facilitating early surgical intervention.
40
Libich, David S., Mumdooh A. M. Ahmed, Ligang Zhong, Vladimir V. Bamm, Vladimir Ladizhansky, and George Harauz. "Fuzzy complexes of myelin basic protein: NMR spectroscopic investigations of a polymorphic organizational linker of the central nervous systemThis paper is one of a selection of papers published in this special issue entitled “Canadian Society of Biochemistry, Molecular & Cellular Biology 52nd Annual Meeting — Protein Folding: Principles and Diseases” and has undergone the Journal's usual peer review process." Biochemistry and Cell Biology 88, no. 2 (April 2010): 143–55. http://dx.doi.org/10.1139/o09-123.
Анотація:
The classic 18.5 kDa isoform of myelin basic protein (MBP) is central to maintaining the structural homeostasis of the myelin sheath of the central nervous system. It is an intrinsically disordered, promiscuous, multifunctional, peripheral membrane protein, whose conformation adapts to its particular environment. Its study requires the selective and complementary application of diverse approaches, of which solution and solid-state NMR spectroscopy are the most powerful to elucidate site-specific features. We review here several recent solution and solid-state NMR spectroscopic studies of 18.5 kDa MBP, and the induced partial disorder-to-order transitions that it has been demonstrated to undergo when complexed with calmodulin, actin, and phospholipid membranes.
41
Träger, Jennica, Katharina Widder, Andreas Kerth, George Harauz, and Dariush Hinderberger. "Effect of Cholesterol and Myelin Basic Protein (MBP) Content on Lipid Monolayers Mimicking the Cytoplasmic Membrane of Myelin." Cells 9, no. 3 (February 25, 2020): 529. http://dx.doi.org/10.3390/cells9030529.
Анотація:
Myelin basic protein (MBP) is located in the insulating covers of nerve cells in the brain and spinal cord. By interacting with lipid membranes, it is responsible for compaction of the myelin sheath in the central nervous system, which is weakened in demyelinating diseases. The lipid composition of the myelin leaflet has a high impact on the interaction between the membrane and MBP. Cholesterol is present in the cytoplasmic leaflet with a rather high amount of 44% (mol%). In this study, the focus is on the effect of cholesterol, mainly by varying its content, on the interaction of MBP with a lipid monolayer. Therefore, Langmuir lipid monolayers mimicking the cytoplasmic membrane of myelin and monolayers with variations of cholesterol content between 0% and 100% were measured at the air/water interface with additional imaging by fluorescence microscopy. All experiments were performed with and without bovine MBP to study the dependence of the interaction of the protein with the monolayers on the cholesterol content. The native amount of 44% cholesterol in the monolayer combines optima in the order of the monolayer (presumably correlating to compaction and thermodynamic stability) and protein interaction and shows unique features in comparison to lower or higher cholesterol contents.
42
Cao, James, Yanping Hu, Mohammed Salman Shazeeb, Carlos E. Pedraza, Nilesh Pande, Daniel Weinstock, Gregory H. Polites, Wenfei Zhang, Karen J. Chandross, and Xiaoyou Ying. "In Vivo Optical Imaging of Myelination Events in a Myelin Basic Protein Promoter-Driven Luciferase Transgenic Mouse Model." ASN Neuro 10 (January 2018): 175909141877732. http://dx.doi.org/10.1177/1759091418777329.
Анотація:
The compact myelin sheath is important for axonal function, and its loss can lead to neuronal cell death and irreversible functional deficits. Myelin is vulnerable to a variety of metabolic, toxic, and autoimmune insults. In diseases like multiple sclerosis, there is currently no therapy to stop myelin loss, underscoring the need for neuroprotective and remyelinating therapies. Noninvasive, robust techniques are also needed to confirm the effect of such therapies in animal models. This article describes the generation, characterization, and potential uses for a myelin basic protein-luciferase (MBP-luci) transgenic mouse model, in which the firefly luciferase reporter gene is selectively controlled by the MBP promoter. In vivo bioluminescence imaging can be used to visualize and quantify demyelination and remyelination at the transcriptional level, noninvasively, and in real time. Transgenic mice were assessed in the cuprizone-induced model of demyelination, and luciferase activity highly correlated with demyelination and remyelination events as confirmed by both magnetic resonance imaging and postmortem histological analysis. Furthermore, MBP-luci mice demonstrated enhanced luciferase signal and remyelination in the cuprizone model after treatment with a peroxisome proliferator activated receptor-delta selective agonist and quetiapine. Imaging sensitivity was further enhanced by using CycLuc 1, a luciferase substrate, which has greater blood–brain barrier penetration. We demonstrated the utility of MBP-luci model in tracking myelin changes in real time and supporting target and therapeutic validation efforts.
43
Teuber-Hanselmann, Sarah, Karl Worm, Nicole Macha, and Andreas Junker. "MGMT-Methylation in Non-Neoplastic Diseases of the Central Nervous System." International Journal of Molecular Sciences 22, no. 8 (April 8, 2021): 3845. http://dx.doi.org/10.3390/ijms22083845.
Анотація:
Quantifying O6-methylguanine-DNA methyltransferase (MGMT) promoter methylation plays an essential role in assessing the potential efficacy of alkylating agents in the chemotherapy of malignant gliomas. MGMT promoter methylation is considered to be a characteristic of subgroups of certain malignancies but has also been described in various peripheral inflammatory diseases. However, MGMT promoter methylation levels have not yet been investigated in non-neoplastic brain diseases. This study demonstrates for the first time that one can indeed detect slightly enhanced MGMT promoter methylation in individual cases of inflammatory demyelinating CNS diseases such as multiple sclerosis and progressive multifocal leucencephalopathy (PML), as well as in other demyelinating diseases such as central pontine and exptrapontine myelinolysis, and diseases with myelin damage such as Wallerian degeneration. In this context, we identified a reduction in the expression of the demethylase TET1 as a possible cause for the enhanced MGMT promoter methylation. Hence, we show for the first time that MGMT hypermethylation occurs in chronic diseases that are not strictly associated to distinct pathogens, oncogenic viruses or neoplasms but that lead to damage of the myelin sheath in various ways. While this gives new insights into epigenetic and pathophysiological processes involved in de- and remyelination, which might offer new therapeutic opportunities for demyelinating diseases in the future, it also reduces the specificity of MGMT hypermethylation as a tumor biomarker.
44
N.I. Fister and M.M. Oros. "The use of nucleotides in diseases of the peripheral nervous system." INTERNATIONAL NEUROLOGICAL JOURNAL 16, no. 8 (March 10, 2021): 33–36. http://dx.doi.org/10.22141/2224-0713.16.8.2020.221958.
Анотація:
Peripheral neuropathy (PN) is a common condition with a preva-lence of approximately 8 % in the elderly. Neuropathic pain is significant in the general population and affects more than half of all patients with PN. The pathophysiology of PN is characterized by lesions of myelin-producing Schwann cells in peripheral nerves. Regeneration/protection of the myelin sheath after nerve damage is the main element of recovery in PN. The problem of neuropathic pain is becoming increasingly important for clinicians of various profiles due to the significant spread of pathology and difficulties in achieving a persistent therapeutic effect. Despite the large number of pharmacological drugs recommended for the management of patients with neuropathic pain, the choice of the optimal treatment regimen often causes difficulties for clinicians. Over the last decade, a group of so-called nucleotides has been used quite actively in medical practice. Additional information about the effects of this group of drugs is accumulated, and there is a gradual transformation, including their composition. Thus, recently the attention of researchers has been devoted to studying the effectiveness of the combination “uridine monophosphate + vitamin B12 (cyanocobalamin) + folic acid”, which is characterized by a fairly high safety profile and good regenerative potential. The objective of this article is to highlight the mechanisms of action and results of clinical use of this combination.
45
Vassall, Kenrick A., Vladimir V. Bamm, and George Harauz. "MyelStones: the executive roles of myelin basic protein in myelin assembly and destabilization in multiple sclerosis." Biochemical Journal 472, no. 1 (October 30, 2015): 17–32. http://dx.doi.org/10.1042/bj20150710.
Анотація:
The classic isoforms of myelin basic protein (MBP, 14–21.5 kDa) are essential to formation of the multilamellar myelin sheath of the mammalian central nervous system (CNS). The predominant 18.5-kDa isoform links together the cytosolic surfaces of oligodendrocytes, but additionally participates in cytoskeletal turnover and membrane extension, Fyn-mediated signalling pathways, sequestration of phosphoinositides and maintenance of calcium homoeostasis. All MBP isoforms are intrinsically disordered proteins (IDPs) that interact via molecular recognition fragments (MoRFs), which thereby undergo local disorder-to-order transitions. Their conformations and associations are modulated by environment and by a dynamic barcode of post-translational modifications, particularly phosphorylation by mitogen-activated and other protein kinases and deimination [a hallmark of demyelination in multiple sclerosis (MS)]. The MBPs are thus to myelin what basic histones are to chromatin. Originally thought to be merely structural proteins forming an inert spool, histones are now known to be dynamic entities involved in epigenetic regulation and diseases such as cancer. Analogously, the MBPs are not mere adhesives of compact myelin, but active participants in oligodendrocyte proliferation and in membrane process extension and stabilization during myelinogenesis. A central segment of these proteins is pivotal in membrane-anchoring and SH3 domain (Src homology 3) interaction. We discuss in the present review advances in our understanding of conformational conversions of this classic basic protein upon membrane association, including new thermodynamic analyses of transitions into different structural ensembles and how a shift in the pattern of its post-translational modifications is associated with the pathogenesis and potentially onset of demyelination in MS.
46
Beydoun, Said R., Thomas H. Brannagan III, Peter Donofrio, Carol Lee Koski, and Eric Lancaster. "Chronic Inflammatory Demyelinating Polyradiculoneuropathy 101—Pitfalls and Pearls of Diagnosis and Treatment." US Neurology 13, no. 01 (2017): 18. http://dx.doi.org/10.17925/usn.2017.13.01.18.
Анотація:
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), which is caused by demyelination of the peripheral nerves, is characterized by progressive weakness and impaired sensory function in the arms and legs. CIDP is a treatable condition in which early diagnosis is crucial to limit chronic disability. CIDP can mimic other neuropathies and it is important to identify these in order to ensure prompt treatment. Patients with other causes of neuropathy should be suspected of having CIDP if there is rapid progress or proximal weakness. Intravenous immunoglobulin (IVIG), corticosteroids, and plasma exchange are first-line therapies. The IVIG CIDP Efficacy (ICE) trial, the largest trial reported of any CIDP treatment, demonstrated that IVIG therapy reduced disability and functional impairment, as well as improved quality of life. Autoantibodies against membrane proteins of the peripheral nerve axons or the myelin sheath have been reported recently, and an improved understanding of antibody responses in CIDP may enable the development of future targeted therapeutic interventions.
47
Marangon, Davide, Matteo Audano, Silvia Pedretti, Marta Fumagalli, Nico Mitro, Davide Lecca, Donatella Caruso, and Maria P. Abbracchio. "Rewiring of Glucose and Lipid Metabolism Induced by G Protein-Coupled Receptor 17 Silencing Enables the Transition of Oligodendrocyte Progenitors to Myelinating Cells." Cells 11, no. 15 (August 2, 2022): 2369. http://dx.doi.org/10.3390/cells11152369.
Анотація:
In the mature central nervous system (CNS), oligodendrocytes (OLs) provide support and insulation to axons thanks to the production of a myelin sheath. During their maturation to myelinating cells, OLs require energy and building blocks for lipids, which implies a great investment of energy fuels and molecular sources of carbon. The oligodendroglial G protein-coupled receptor 17 (GPR17) has emerged as a key player in OL maturation; it reaches maximal expression in pre-OLs, but then it has to be internalized to allow terminal maturation. In this study, we aim at elucidating the role of physiological GPR17 downregulation in OL metabolism by applying transcriptomics, metabolomics and lipidomics on differentiating OLs. After GPR17 silencing, we found a significant increase in mature OL markers and alteration of several genes involved in glucose metabolism and lipid biosynthesis. We also observed an increased release of lactate, which is partially responsible for the maturation boost induced by GPR17 downregulation. Concomitantly, GPR17 depletion also changed the kinetics of specific myelin lipid classes. Globally, this study unveils a functional link between GPR17 expression, lactate release and myelin composition, and suggests that innovative interventions targeting GPR17 may help to foster endogenous myelination in demyelinating diseases.
48
Andreula, C. F. "The Main Neuroimmune Diseases in Childhood: Multiple Sclerosis and Acute Disseminated Encephalomyelitis." Rivista di Neuroradiologia 18, no. 3 (June 2005): 315–28. http://dx.doi.org/10.1177/197140090501800307.
Анотація:
Demyelination is the hallmark of neuroimmune disease. It is caused by a breakdown of myelin already formed, relative sparing of myelin producing oligodendrocytes, neurons and astrocytes, and venous inflammation with perivenous infiltration. The neuroradiological investigation of choice in this setting is magnetic resonance (MR) due to its ability to monitor the neuropathological process. Acute disseminated encephalomyelitis (ADEM), also known as postinfectious or hyperergic encephalomyelitis, disseminated vasculoencephalomyelitis and perivenous encephalitis, is the commonest neuroimmune disorder of childhood. Neuroradiological investigation is highly sensitive for a diagnosis of demyelinating disease. MR is highly sensitive but not specific. Spectroscopy is useful for diagnosis: the reduction in axonal density leads to a decrease in N-acetylaspartate with a concomitant increase in choline and myoinositol due to glial proliferation. Spectroscopic findings positive for a decrease of NAA and an increase in choline during the first episode of demyelinating disease in adolescence are suggestive of an evolution to MS.
49
Parmar, Suchina, Jai Lal Davessar, and Gurbax Singh. "Facial nerve schwannoma: a rare case report." International Journal of Otorhinolaryngology and Head and Neck Surgery 3, no. 1 (December 28, 2016): 159. http://dx.doi.org/10.18203/issn.2454-5929.ijohns20164823.
Анотація:
<p class="abstract"><span lang="EN-IN">Schwannoma is a benign tumor arising from Schwann cells which is protective covering of nerves, called myelin sheath and can develop anywhere, where Schwann cells are present. Most common schwannomas are found with vestibulocochlear nerve. Facial nerve schwannoma are uncommon tumour involving 7th nerve out of which also most common site of involvement is geniculate ganglion. Facial nerve schwannoma is uncommon benign tumor. There are no typical patterns of presentation and can easily go untreated or misdiagnosed. Facial nerve palsy is most common mode of presentation. Here we present a case of 35 years male who presented with complaint of facial nerve paralysis. High degree of clinical suspicion and early imaging can lead to diagnosis. An early diagnosis is important as morbidity associated with this disease and as well as surgery leads to delay in diagnosis.</span></p>
50
Lotfy, Ahmed, Nourhan S. Ali, Mai Abdelgawad, and Mohamed Salama. "Mesenchymal stem cells as a treatment for multiple sclerosis: a focus on experimental animal studies." Reviews in the Neurosciences 31, no. 2 (January 28, 2020): 161–79. http://dx.doi.org/10.1515/revneuro-2019-0040.
Анотація:
AbstractMultiple sclerosis (MS) is a progressive and debilitating neurological condition in which the immune system abnormally attacks the myelin sheath insulating the nerves. Mesenchymal stem cells (MSCs) are found in most adult tissues and play a significant systemic role in self-repair. MSCs have promising therapeutic effects in many diseases, such as autoimmune diseases, including MS. MSCs have been tested in MS animal models, such as experimental autoimmune encephalomyelitis. Other studies have combined other agents with MSCs, genetically modified MSCs, or used culture medium from MSCs. In this review, we will summarize these studies and compare the main factors in each study, such as the source of MSCs, the type of animal model, the route of injection, the number of injected cells, and the mechanism of action.