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Axonal neuropathy

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https://www.readbyqxmd.com/read/27911358/the-rodent-model-of-nonarteritic-anterior-ischemic-optic-neuropathy-rnaion
#1
Yan Guo, Zara Mehrabian, Steven L Bernstein
Nonarteritic anterior ischemic optic neuropathy (NAION) is a focal ischemic lesion of the optic nerve that affects 1/700 individuals throughout their lifetime. NAION results in optic nerve edema, selective loss of the retinal ganglion cell neurons (RGCs) and atrophy of the optic nerve. A rodent model of NAION that expresses most NAION features and sequelae has been developed, which is applicable to both rats and mice. This model utilizes a focal laser application of 532 nm wavelength to illuminate a photoactive dye, Rose Bengal (RB), to cause capillary damage and leakage at the targeted anterior optic nerve (the laminar region)...
November 20, 2016: Journal of Visualized Experiments: JoVE
https://www.readbyqxmd.com/read/27907123/mice-hemizygous-for-a-pathogenic-mitofusin-2-allele-exhibit-hind-limb-foot-gait-deficits-and-phenotypic-perturbations-in-nerve-and-muscle
#2
Peter Bannerman, Travis Burns, Jie Xu, Laird Miers, David Pleasure
Charcot-Marie-Tooth disease type 2A (CMT2A), the most common axonal form of hereditary sensory motor neuropathy, is caused by mutations of mitofusin-2 (MFN2). Mitofusin-2 is a GTPase required for fusion of mitochondrial outer membranes, repair of damaged mitochondria, efficient mitochondrial energetics, regulation of mitochondrial-endoplasmic reticulum calcium coupling and axonal transport of mitochondria. We knocked T105M MFN2 preceded by a loxP-flanked STOP sequence into the mouse Rosa26 locus to permit cell type-specific expression of this pathogenic allele...
2016: PloS One
https://www.readbyqxmd.com/read/27904477/axon-injury-induced-endoplasmic-reticulum-stress-and-neurodegeneration
#3
REVIEW
Yang Hu
Injury to central nervous system axons is a common early characteristic of neurodegenerative diseases. Depending on its location and the type of neuron, axon injury often leads to axon degeneration, retrograde neuronal cell death and progressive permanent loss of vital neuronal functions. Although these sequential events are clearly connected, ample evidence indicates that neuronal soma and axon degenerations are active autonomous processes with distinct molecular mechanisms. By exploiting the anatomical and technical advantages of the retinal ganglion cell (RGC)/optic nerve (ON) system, we demonstrated that inhibition of the PERK-eIF2α-CHOP pathway and activation of the X-box binding protein 1 pathway synergistically protect RGC soma and axon, and preserve visual function, in both acute ON traumatic injury and chronic glaucomatous neuropathy...
October 2016: Neural Regeneration Research
https://www.readbyqxmd.com/read/27896434/towards-a-functional-pathology-of-hereditary-neuropathies
#4
REVIEW
Joachim Weis, Kristl G Claeys, Andreas Roos, Hamid Azzedine, Istvan Katona, J Michael Schröder, Jan Senderek
A growing number of hereditary neuropathies have been assigned to causative gene defects in recent years. The study of human nerve biopsy samples has contributed substantially to the discovery of many of these neuropathy genes. Genotype-phenotype correlations based on peripheral nerve pathology have provided a comprehensive picture of the consequences of these mutations. Intriguingly, several gene defects lead to distinguishable lesion patterns that can be studied in nerve biopsies. These characteristic features include the loss of certain nerve fiber populations and a large spectrum of distinct structural changes of axons, Schwann cells and other components of peripheral nerves...
November 28, 2016: Acta Neuropathologica
https://www.readbyqxmd.com/read/27894788/neurotoxic-mechanisms-of-paclitaxel-are-local-to-the-distal-axon-and-independent-of-transport-defects
#5
Erica L Gornstein, Thomas L Schwarz
Chemotherapy-induced peripheral neuropathy (CIPN) is a dose-limiting side effect of paclitaxel and other chemotherapeutic agents. Paclitaxel binds and stabilizes microtubules, but the cellular mechanisms that underlie paclitaxel's neurotoxic effects are not well understood. We therefore used primary cultures of adult murine dorsal root ganglion neurons, the cell type affected in patients, to examine leading hypotheses to explain paclitaxel neurotoxicity. We address the role of microtubule hyperstabilization and its downstream effects...
November 25, 2016: Experimental Neurology
https://www.readbyqxmd.com/read/27891417/a-commonly-missed-well-known-entity-acute-intermittent-porphyria-a-case-report
#6
Smilu Mohanlal, Radha Gulati Ghildiyal, Alpana Kondekar, Poonam Wade, Richa Sinha
Acute Intermittent Porphyria (AIP) usually presents with abdominal pain, peripheral neuropathy and psychiatric manifestations. Incidence of AIP being 5 in 1,00,000. We present a case of an 11-year-old male child with multiple cranial nerve involvement, quadriparesis, focal convulsions, hypertension, hyponatremia with history of recurrent abdominal pain. His complete haemogram, ultrasonography (USG) abdomen, renal function tests were normal, he was also evaluated for tuberculosis which was negative. On further evaluation Electroencephalography (EEG) was suggestive of a generalised seizure disorder, MRI Brain suggestive of Posterior Reversible Encephalopathy Syndrome (PRES), Electromyography revealed a sensory motor axonal polyneuropathy and urine UV fluoresence test was positive for porphobilinogen which clinched the diagnosis of AIP...
October 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/27878436/peripheral-nerve-ultrasound-scoring-systems-benchmarking-and-comparative-analysis
#7
Alexander Grimm, Tim W Rattay, Natalie Winter, Hubertus Axer
Ultrasound of the nerves is an additive diagnostic tool to evaluate polyneuropathy. Recently, the need for standardized scoring systems has widely been discussed; different scores are described so far. Therefore, 327 patients with polyneuropathy were analyzed by ultrasound in our laboratory. Consequently, several ultrasound scoring tools were applied, i.e., the nerve pattern classification according to Padua et al. in all patients with CIDP and variants, the Bochum ultrasound score (BUS) and the neuritis ultrasound protocol in immune-mediated neuritis, the ultrasound pattern sum score, the homogeneity score, and the nerve enlargement distribution score in all neuropathies if possible...
November 22, 2016: Journal of Neurology
https://www.readbyqxmd.com/read/27875632/variable-phenotypic-expression-and-onset-in-myh14-distal-hmn-phenotype-in-a-large-multigenerational-north-american-family
#8
Stanley Iyadurai, W David Arnold, John T Kissel, Corey Ruhno, Vicki L McGovern, Pamela J Snyder, Thomas W Prior, Jennifer Roggenbuck, Arthur H Burghes, Stephen J Kolb
INTRODUCTION: Distal hereditary motor neuropathy (dHMN) causes distal-predominant weakness without prominent sensory loss. Myosin heavy chain disorders most commonly result in distal myopathy and cardiomyopathy with or without hearing loss, but a complex phenotype with dHMN, myopathy, hoarseness, and hearing loss was reported in a Korean family with a c.2822G>T mutation in MYH14. OBJECTIVE: To report phenotypic features in a North American family with the c.2822G>T in MYH14...
November 22, 2016: Muscle & Nerve
https://www.readbyqxmd.com/read/27872763/effects-of-microtubule-stabilization-by-epothilone-b-depend-on-the-type-and-age-of-neurons
#9
Eun-Hae Jang, Aeri Sim, Sun-Kyoung Im, Eun-Mi Hur
Several studies have demonstrated the therapeutic potential of applying microtubule- (MT-) stabilizing agents (MSAs) that cross the blood-brain barrier to promote axon regeneration and prevent axonal dystrophy in rodent models of spinal cord injury and neurodegenerative diseases. Paradoxically, administration of MSAs, which have been widely prescribed to treat malignancies, is well known to cause debilitating peripheral neuropathy and axon degeneration. Despite the growing interest of applying MSAs to treat the injured or degenerating central nervous system (CNS), consequences of MSA exposure to neurons in the central and peripheral nervous system (PNS) have not been thoroughly investigated...
2016: Neural Plasticity
https://www.readbyqxmd.com/read/27872270/hiv-glycoprotein-gp120-impairs-fast-axonal-transport-by-activating-tak1-signaling-pathways
#10
Sarah H Berth, Nichole Mesnard-Hoaglin, Bin Wang, Hajwa Kim, Yuyu Song, Maria Sapar, Gerardo Morfini, Scott T Brady
Sensory neuropathies are the most common neurological complication of HIV. Of these, distal sensory polyneuropathy (DSP) is directly caused by HIV infection and characterized by length-dependent axonal degeneration of dorsal root ganglion (DRG) neurons. Mechanisms for axonal degeneration in DSP remain unclear, but recent experiments revealed that the HIV glycoprotein gp120 is internalized and localized within axons of DRG neurons. Based on these findings, we investigated whether intra-axonal gp120 might impair fast axonal transport (FAT), a cellular process critical for appropriate maintenance of the axonal compartment...
December 2016: ASN Neuro
https://www.readbyqxmd.com/read/27871444/polyneuropathy-in-levodopa-treated-parkinson-s-patients
#11
Karol Szadejko, Krzysztof Dziewiatowski, Krzysztof Szabat, Piotr Robowski, Michał Schinwelski, Emilia Sitek, Jarosław Sławek
Recently published studies show that the prevalence of polyneuropathy (PNP) is higher in patients with Parkinson's disease (PD) than in age-matched controls. Its pathogenesis, however is a matter of controversy. The major hypothesis is the toxicity of high concentrations of homocysteine (Hcy) possibly related to levodopa (LD) therapy. The aim of the present study was to determine the prevalence of PNP, independent of other etiologies, and to determine the relationship to demographic and clinical factors in LD-treated Parkinson's patients...
December 15, 2016: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/27866733/review-of-the-literature-articles-published-in-the-last-five-years-that-have-changed-my-daily-practice
#12
REVIEW
E Delmont
Over the last five years, the management of peripheral neuropathies has become structured by the publication of recognized diagnostic criteria for inflammatory neuropathies and the elaboration of a function score, the R-ODS, used to evaluate the progression of these neuropathies. The concept of nodo-paranodopathy has enriched the concept of peripheral neuropathies, over-riding the classical mechanisms of axonal and demyelinating mechanisms. The structures of the nodes of Ranvier, gangliosides, contractin and neurofascin are preferential targets for auto-antibodies responsible for dysimmune neuropathies...
November 17, 2016: Revue Neurologique
https://www.readbyqxmd.com/read/27866730/hereditary-neuropathies-an-update
#13
REVIEW
T Stojkovic
Hereditary neuropathies are the most common inherited neuromuscular diseases. Charcot-Marie-Tooth (CMT) disease represents the most common form with an average prevalence ranging from 1/2500 to 1/1200, depending on the studies. To date and with the advances of the latest generation sequencing, more than 80 genes have been identified. Although the common clinical phenotype comprises a progressive distal muscle weakness and sensory loss, foot deformities and decreased or absent tendon reflexes, clinical and electrophysiological phenotypes exhibit great variability...
November 17, 2016: Revue Neurologique
https://www.readbyqxmd.com/read/27863451/cerebral-white-matter-abnormalities-in-patients-with-charcot-marie-tooth-disease
#14
Mina Lee, Chang-Hyun Park, Hwa-Kyung Chung, Hyeon Jin Kim, Yunseo Choi, Jeong Hyun Yoo, Young Chul Yoon, Young Bin Hong, Ki Wha Chung, Byung-Ok Choi, Hyang Woon Lee
Here, we report the structural evidence of cerebral white matter abnormalities in Charcot-Marie-Tooth (CMT) patients and the relationship between these abnormalities and clinical disability. Brain diffusion tensor imaging (DTI) was performed in CMT patients with demyelinating (CMT1A/CMT1E), axonal (CMT2A/CMT2E), or intermediate (CMTX1/DI-CMT) peripheral neuropathy. Although all patients had normal brain MRI, all genetic subgroups except CMT1A had abnormal DTI findings indicative of significant cerebral white matter abnormalities: decreased fractional anisotropy and axial diffusivity, and increased radial diffusivity...
November 18, 2016: Annals of Neurology
https://www.readbyqxmd.com/read/27859440/peripheral-nerve-ultrasound-in-cerebellar-ataxia-neuropathy-vestibular-areflexia-syndrome-canvas
#15
Luciana Pelosi, Ruth Leadbetter, Eoin Mulroy, Andrew M Chancellor, Stuart Mossman, Richard Roxburgh
INTRODUCTION: We report preliminary findings of nerve ultrasound in patients with cerebellar ataxia neuropathy vestibular areflexia syndrome (CANVAS) who have sensory impairment due to dorsal root ganglionopathy. METHODS: The ultrasound cross-sectional area (CSA) of median and ulnar nerves of 7 CANVAS patients was compared with 7 age- and gender-matched controls and with the mean CSA of our reference population. RESULTS: The nerve CSA of CANVAS patients was significantly smaller than that of controls at all sites (P < 0...
November 15, 2016: Muscle & Nerve
https://www.readbyqxmd.com/read/27857760/galanin-and-its-receptor-system-promote-the-repair-of-injured-sciatic-nerves-in-diabetic-rats
#16
Xiao-Feng Xu, Dan-Dan Zhang, Jin-Chi Liao, Li Xiao, Qing Wang, Wei Qiu
Various studies have reported that galanin can promote axonal regeneration of dorsal root ganglion neurons in vitro and inhibit neuropathic pain. However, little is known about its effects on diabetic peripheral neuropathy, and in vivo experimental data are lacking. We hypothesized that repeated applications of exogenous galanin over an extended time frame may also repair nerve damage in diabetic peripheral neuropathy, and relieve pain in vivo. We found that neuropathic pain occurred in streptozotocin-induced diabetic rats and was more severe after sciatic nerve pinch injury at 14 and 28 days than in diabetic sham-operated rats...
September 2016: Neural Regeneration Research
https://www.readbyqxmd.com/read/27854215/characterization-of-new-transgenic-mouse-models-for-two-charcot-marie-tooth-causing-hspb1-mutations-using-the-rosa26-locus
#17
Delphine Bouhy, Thomas Geuens, Vicky De Winter, Leonardo Almeida-Souza, Istvan Katona, Joachim Weis, Tino Hochepied, Steven Goossens, Jody J Haigh, Sophie Janssens, Vincent Timmerman
BACKGROUND: Charcot-Marie-Tooth (CMT) and associated neuropathies, the most common inherited diseases of the peripheral nervous system, remain so far incurable. Three existing murine models of Charcot-Marie-Tooth type 2F (CMT2F) and/or distal hereditary motor neuropathy type IIb (dHMNIIb), caused by mutations in the small heat shock protein B1 gene (HSPB1/HSP27), partially recapitulate the hallmarks of peripheral neuropathy. Because these models overexpress the HSPB1 mutant proteins they differ from the patients' situation...
May 27, 2016: Journal of Neuromuscular Diseases
https://www.readbyqxmd.com/read/27852232/novel-homozygous-missense-mutation-in-gan-associated-with-charcot-marie-tooth-disease-type-2-in-a-large-consanguineous-family-from-israel
#18
Sharon Aharoni, Katy E S Barwick, Rachel Straussberg, Gaurav V Harlalka, Yoram Nevo, Barry A Chioza, Meriel M McEntagart, Aviva Mimouni-Bloch, Michael Weedon, Andrew H Crosby
BACKGROUND: CMT-2 is a clinically and genetically heterogeneous group of peripheral axonal neuropathies characterized by slowly progressive weakness and atrophy of distal limb muscles resulting from length-dependent motor and sensory neurodegeneration. Classical giant axonal neuropathy (GAN) is an autosomal recessively inherited progressive neurodegenerative disorder of the peripheral and central nervous systems, typically diagnosed in early childhood and resulting in death by the end of the third decade...
November 16, 2016: BMC Medical Genetics
https://www.readbyqxmd.com/read/27845852/platelet-rich-plasma-a-source-of-autologous-growth-factors-and-biomimetic-scaffold-for-peripheral-nerve-regeneration
#19
Mikel Sánchez, Eduardo Anitua, Diego Delgado, Peio Sanchez, Roberto Prado, Gorka Orive, Sabino Padilla
In mammals, axons of injured peripheral nerves (PNI) can and do regenerate, but often the functional recovery is incomplete or suboptimal. In recent years, in vivo tissue engineering approaches through molecular intervention and scaffolding are offering promising outcomes. Evidence is accumulating in both preclinical and clinical settings indicating that platelet rich plasma (PRP) and fibrin scaffolds obtained from this technology hold an important adjuvant therapeutic potential. Areas covered: This review addresses current molecular and cellular data in intrinsic nerve repair processes and describes different strategies to harness and enhance these processes by using biochemical and biomechanical cues...
November 15, 2016: Expert Opinion on Biological Therapy
https://www.readbyqxmd.com/read/27823807/electrophysiological-assessment-of-guillain-barr%C3%A3-syndrome-with-both-gal-c-and-ganglioside-antibodies-tendency-for-demyelinating-type
#20
Makoto Samukawa, Motoi Kuwahara, Miyuki Morikawa, Rino Ueno, Yukihiro Hamada, Kazuo Takada, Makito Hirano, Yoshiyuki Mitsui, Masahiro Sonoo, Susumu Kusunoki
Whether patients who have GBS with antibodies to galactocerebroside (Gal-C) and gangliosides (Gal-C-GS-GBS) more often have demyelinating or axonal neuropathy remains controversial. We assessed the electrophysiological data from 16 patients with Gal-C-GS-GBS based on the two established criteria to clarify this issue. In this largest cohort of Gal-C-GS-GBS, eight patients had demyelinating neuropathy and none exhibited axonal neuropathy on either criterion. These data indicated that antibodies to Gal-C, a myelin antigen, might predominantly be associated with demyelinating neuropathy, even in the presence of concomitant antibodies to gangliosides...
October 24, 2016: Journal of Neuroimmunology
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