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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.

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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 lysosome
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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.

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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
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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.

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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
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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.

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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 neurodege
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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.

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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, a
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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.

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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
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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.

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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 m
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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.

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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
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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.

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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 dise
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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.

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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
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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.

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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 n
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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.

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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
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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.

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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 kno
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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.

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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.

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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
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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.

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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,
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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.

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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 varie
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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.

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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 physiologic
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19

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.

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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 s
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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.

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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 mye
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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.

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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 c
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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.

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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 prev
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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.

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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. Histopatho
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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.

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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 mod
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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.

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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 t
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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.

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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 rem
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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.

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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 column
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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.

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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
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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.

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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.
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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.

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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 di
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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.

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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. R
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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.

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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 r
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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.

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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
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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.

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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
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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.

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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 pediat
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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.

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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 m
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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.

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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 oligoden
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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.

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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.
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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.

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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 ri
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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.

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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
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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.

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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 l
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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.

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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
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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.

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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 i
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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.

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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 achievi
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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.

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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 loc
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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.

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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, a
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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.

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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 physiolo
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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.

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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 disord
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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.

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<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 com
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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.

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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. I
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