Demylination

Altered expression of peripheral myelin protein 22 (PMP22) results in demyelinating peripheral neuropathy. PMP22 exhibits a highly restricted tissue distribution with marked expression in the myelinating Schwann cells of peripheral nerves. Auditory and vestibular Schwann cells and the afferent neurons also express PMP22, suggesting a unique role in hearing and balancing. Indeed, neuropathic patients diagnosed with PMP22-linked hereditary neuropathies often present with auditory and balance deficits, an understudied clinical complication...

Huperzine A (HupA) is a natural acetylcholinesterase inhibitor (AChEI) with the advantages of high efficiency, selectivity as well as reversibility and can exhibit significant therapeutic effects against certain neurodegenerative diseases. It is also beneficial in reducing the neurological impairment and neuroinflammation of experimental autoimmune encephalomyelitis (EAE), a classic model for multiple sclerosis (MS). However, whether HupA can directly regulate oligodendrocyte differentiation and maturation and promote remyelination has not been investigated previously...

Acute flare of multiple sclerosis usually presents with sensorimotor deficits in limbs or one side of the face, optic neuritis, internuclear ophthalmoplegia, and/or cerebellar signs and symptoms. Isolated aphasia is observed only in a handful of cases. Herein, we present a case of a patient who presented with isolated transcortical motor aphasia. Initial thought was that the patient was having a cerebrovascular accident as he had a history of uncontrolled hypertension. It was only later found on magnetic resonance imaging (MRI) of the brain that the patient had demyelinating lesions compatible with his new symptoms...

IM: Here we used a demyelination model using an injection of Lysophosphatidylcholine )LPC( in the corpus collosum to examine the myelination activity of differentiated oligodendrocytes derived from Human dental pulp stem cells )hDPDSCs( according to a two step induction protocol. METHODS AND MATERIALS: The cells were cultured in DMEM-F12 medium containing 1M Retinoic acid and were treated with 5ng/ml platelet-derived growth factor, 10 ng/ml basic fibroblast growth factor for 8-10 days...

High mobility group box 1 protein (HMGB1) is known to be a trigger of inflammation in experimental autoimmune encephalomyelitis (EAE), an animal model of multiple sclerosis (MS). However, it may play a different role in some way. Here we investigated the effect of HMGB1 on promoting sonic hedgehog (shh) release from astrocytes as well as the possible signal pathway involved in it. Firstly, shh increased in astrocytes after administration of recombinant HMGB1 or decreased after HMGB1 was blocked when stimulated by homogenate of the onset stage of EAE...

Evidence on the adverse effects of agricultural pesticide use by farmers under the actual field conditions on their peripheral nerve conduction in China is limited. This study was to investigate the association of agricultural pesticide use with the abnormalities of farmers' peripheral nerve conduction based on two rounds of conventional nerve conduction studies. The level of pesticide exposure was assessed by measuring total amount of pesticides used by farmers in 2012. The logistic and negative binomial regression analyses were performed on a cohort study of 218 farmers...

Cognitive impairment and memory deficit are common features in multiple Sclerosis patients. The mechanism of memory impairment in MS is unknown, but neuroimaging studies suggest that hippocampal demyelination is involved. Here, we investigate the role of GABA A receptor on spatial memory in the local model of hippocampal demyelination. Demyelination was induced in male Wistar rats by bilaterally injection of lysophosphatidylcholine (LPC) 1% into the CA1 region of the hippocampus. The treatment groups were received daily intraventricular injection of bicuculline (0...

BACKGROUND: Multiple sclerosis is a common demylinating and inflamatory disease of the CNS with a presumed autoimmune etiology. Interferon β have been used for relapsing remitting multiple sclerosis through its regulatory properties on T-cell activation and cytokines production. OBJECTIVES: During the war Benghazi-Libya (17 February 2011 revolution) multiple sclerosis patients have got stopped β interferon because unavailability of drugs which allow to evaluate the efficacy of β interferon on relapsing remitting multiple sclerosis regarding frequency and severity of relapses during months of stopping it...

Mast cells (MCs) derive from a distinct precursor in the bone marrow and are predominantly found in tissues at the interface between the host and the external environment where they can secrete mediators without overt degranulation. Mast cells mature under local tissue microenvironmental factors and are necessary for the development of allergic reactions, through crosslinking of their surface receptors for IgE (FcεRI), leading to degranulation and the release of vasoactive, pro-inflammatory and nociceptive mediators that include histamine, pro-inflammatory and anti-inflammatory cytokines and proteolytic enzymes...

OBJECTIVE: To find an association between human leukocyte antigen (HLA) class II DRB1, DRB3, DRB4, and DRB5 alleles frequencies in a sample of Iraqi patients with Guillain-Barre syndrome (GBS) and compare with a healthy control group. METHODS: We performed a cross-sectional study consisting of 30 Iraqi Arab patients with GBS attending the Neurological Department in the Neuroscience Hospital, Baghdad, Iraq between September 2012 and June 2013. The control group comprised 42 apparently healthy volunteers...

Organophosphate pesticide phosphomedon was exposed to albino rat at a concentration of 35 ppm in the time interval of 30, 45 and 60 days. During the exposure period neurobehavioral symptoms such as reduce food intake, weight loss, increase water intake, low defecation frequency, increase locomotion frequency at high dose were observed. Locomotion frequency were less initially but higher in increasing dose concentration. The result was also different in both the sexes. A decrease social interaction and increase force swimming with increasing dose concentration, which was not significant as comparison to control...

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Translocator protein (18 kDa) (TSPO) is a marker of inflammation in the brain. Positron emission tomography (PET) scans with ligands for this receptor show increased expression of TSPO in many neuropathologic conditions. However, expression of TSPO in the periphery and its possible correlation to central nervous system (CNS) inflammation has been largely unstudied. In this paper PBR28, a recently synthesized ligand for TSPO that is shown to have 80-fold higher specific binding than its predecessor PK11195, is used to quantify peripheral TSPO...

Biotinidase deficiency is an autosomal recessively inherited disorder characterized by neurological and cutaneous abnormalities. We have developed a transgenic knock-out mouse with biotinidase deficiency to better understand aspects of pathophysiology and natural history of the disorder in humans. Neurological deficits observed in symptomatic mice with biotinidase deficiency are similar to those seen in symptomatic children with the disorder. Using a battery of functional neurological assessment tests, the symptomatic mice performed poorly compared to wild-type mice...

The spectrum of chronic dysimmune neuropathies has widened well beyond chronic demyelinating polyradiculoneuropathy (CIDP). Pure motor (multifocal motor neuropathy), sensorimotor with asymmetrical involvement (multifocal acquired demylinating sensory and motor neuropathy), exclusively distal sensory (distal acquired demyelinating sensory neuropathy) and very proximal sensory (chronic immune sensory polyradiculopathy) constitute the variants of CIDP. Correct diagnosis of these entities is of importance in terms of initiation of appropriate therapy as well as prognostication of these patients...

BACKGROUND: X-linked adrenoleukodystrophy (X-ALD) is a neurodegenerative genetic disease characterized by progressive demylination of the brain, adrenal insufficiency and elevated VLCFA level. ABCD1gene is the disease gene and more than 500 unique mutations in the ABCD1gene have been recorded in the database, approximately 60% of which are noncurrent ones. Although great progress has been made in the treatment of X-ALD, prenatal diagnosis is still badly needed by X-ALD-stricken families...

Acute manifestations of Organophosphate Compound (OPC) poison are due to effect cholinergic excess. Others are intermediate syndrome [IMS], organophosphate induced delayed neuropathy [OPIND] and chronic organophosphate induced neuropsychiatric disorder [COPIND]. All these manifestation have specific period of occurrence and duration. There are very sparse reports of toxic demylination due to OPC poisoning. We report a case of Guillain-Barre Syndrome (GBS) due to toxic demyelination following OPC poison.

Generation of homogeneous oligodendrocytes as donor cells is essential for human embryonic stem cell (hESC)-based cell therapy for demylinating diseases. Herein we present a novel method for efficiently obtaining mature oligodendrocytes from hESCs with high purity (79.7+/-6.9%), using hepatocyte growth factor (HGF) and G5 supplement (containing insulin, transferrin, selenite, biotin, hydrocortisone, basic fibroblast growth factor and epidermal growth factor) in a four-step method. We induced hESCs into neural progenitors (NP) with HGF (5ng/ml) and G5 (1x) supplemented medium in an adherent differentiation system...

Axonal damage, demylination and inflammation of the central nervous system are the major pathological features of the human multiple sclerosis (MS). MS is thought to be due to abnormal T cell mediated immune response. Oxidative stress plays an important role in the advancement of MS. The management of oxidative stress by outlining central role of reduced glutathione. In our experiment we used Experimental autoimmune encephalomyelitis (EAE) animal model that mimic human MS and tested the effect of Thymoquinone (TQ), an oil constituent of Nigella Sativa also known as black seed...