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Axonal neuropathy

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https://www.readbyqxmd.com/read/29775143/testing-the-effectiveness-and-the-contribution-of-experimental-supercharge-reversed-end-to-side-nerve-transfer
#1
Mustafa Nadi, Sudheesh Ramachandran, Abir Islam, Joanne Forden, Gui Fang Guo, Rajiv Midha
OBJECTIVE Supercharge end-to-side (SETS) transfer, also referred to as reverse end-to-side transfer, distal to severe nerve compression neuropathy or in-continuity nerve injury is gaining clinical popularity despite questions about its effectiveness. Here, the authors examined SETS distal to experimental neuroma in-continuity (NIC) injuries for efficacy in enhancing neuronal regeneration and functional outcome, and, for the first time, they definitively evaluated the degree of contribution of the native and donor motor neuron pools...
May 18, 2018: Journal of Neurosurgery
https://www.readbyqxmd.com/read/29770116/peripheral-glial-cells-in-the-development-of-diabetic-neuropathy
#2
REVIEW
Nádia Pereira Gonçalves, Christian Bjerggaard Vægter, Lone Tjener Pallesen
The global prevalence of diabetes is rapidly increasing, affecting more than half a billion individuals within the next few years. As diabetes negatively affects several physiological systems, this dramatic increase represents not only impaired quality of life on the individual level but also a huge socioeconomic challenge. One of the physiological consequences affecting up to half of diabetic patients is the progressive deterioration of the peripheral nervous system, resulting in spontaneous pain and eventually loss of sensory function, motor weakness, and organ dysfunctions...
2018: Frontiers in Neurology
https://www.readbyqxmd.com/read/29769041/cortical-atrophy-and-hypofibrinogenemia-due-to-fgg-and-tbcd-mutations-in-a-single-family-a-case-report
#3
Joshi Stephen, Sheela Nampoothiri, K P Vinayan, Dhanya Yesodharan, Preetha Remesh, William A Gahl, May Christine V Malicdan
BACKGROUND: Blended phenotypes or co-occurrence of independent phenotypically distinct conditions are extremely rare and are due to coincidence of multiple pathogenic mutations, especially due to consanguinity. Hereditary fibrinogen deficiencies result from mutations in the genes FGA, FGB, and FGG, encoding the three different polypeptide chains that comprise fibrinogen. Neurodevelopmental abnormalities have not been associated with fibrinogen deficiencies. In this study, we report an unusual patient with a combination of two independently inherited genetic conditions; fibrinogen deficiency and early onset cortical atrophy...
May 16, 2018: BMC Medical Genetics
https://www.readbyqxmd.com/read/29766026/development-of-intrathecal-aav9-gene-therapy-for-giant-axonal-neuropathy
#4
Rachel M Bailey, Diane Armao, Sahana Nagabhushan Kalburgi, Steven J Gray
An NIH-sponsored phase I clinical trial is underway to test a potential treatment for giant axonal neuropathy (GAN) using viral-mediated GAN gene replacement (https://clinicaltrials.gov/ct2/show/NCT02362438). This trial marks the first instance of intrathecal (IT) adeno-associated viral (AAV) gene transfer in humans. GAN is a rare pediatric neurodegenerative disorder caused by autosomal recessive loss-of-function mutations in the GAN gene, which encodes the gigaxonin protein. Gigaxonin is involved in the regulation, turnover, and degradation of intermediate filaments (IFs)...
June 15, 2018: Molecular Therapy. Methods & Clinical Development
https://www.readbyqxmd.com/read/29763602/a-ketogenic-diet-reduces-metabolic-syndrome-induced-allodynia-and-promotes-peripheral-nerve-growth-in-mice
#5
Michael A Cooper, Blaise W Menta, Consuelo Perez-Sanchez, Megan M Jack, Zair W Khan, Janelle M Ryals, Michelle Winter, Douglas E Wright
Current experiments investigated whether a ketogenic diet impacts neuropathy associated with obesity and prediabetes. Mice challenged with a ketogenic diet were compared to mice fed a high-fat diet or a high-fat diet plus exercise. Additionally, an intervention switching to a ketogenic diet following 8 weeks of high-fat diet was performed to compare how a control diet, exercise, or a ketogenic diet affects metabolic syndrome-induced neural complications. When challenged with a ketogenic diet, mice had reduced bodyweight and fat mass compared to high-fat-fed mice, and were similar to exercised, high-fat-fed mice...
May 12, 2018: Experimental Neurology
https://www.readbyqxmd.com/read/29760923/finger-drop-sign-characteristic-pattern-of-distal-weakness-in-guillain-barr%C3%A3-syndrome-a-case-report-and-review-of-the-literature
#6
Yong Chuan Chee, Beng Hooi Ong
Guillain-Barré Syndrome is an acquired acute autoimmune polyradiculoneuropathy that commonly presents with limb weakness and occasional cranial nerve, respiratory and autonomic involvement. Although the classic description of Guillain-Barré Syndrome is that of a demyelinating neuropathy with ascending weakness, predominant bilateral finger drop as presenting feature has rarely been reported. A characteristic pattern of weakness involving the extensor components of the fingers known as "finger drop sign" has been first described to be specific in acute motor axonal neuropathy form of Guillain-Barré Syndrome in the literature...
2018: SAGE Open Medical Case Reports
https://www.readbyqxmd.com/read/29760184/structural-and-functional-rescue-of-chronic-metabolically-stressed-optic-nerves-through-respiration
#7
Mohammad Harun-Or-Rashid, Nate Pappenhagen, Peter G Palmer, Matthew A Smith, Victoria Gevorgyan, Gina N Wilson, Samuel D Crish, Denise M Inman
Axon degeneration can arise from metabolic stress, potentially a result of mitochondrial dysfunction or lack of appropriate substrate input. In this study, we investigated whether the metabolic vulnerability observed during optic neuropathy in the DBA/2J (D2) model of glaucoma is due to dysfunctional mitochondria or impaired substrate delivery to axons, the latter based on our observation of significantly decreased glucose and monocarboxylate transporters in D2 optic nerve (ON), human ON, and mice subjected to acute glaucoma injury...
May 14, 2018: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/29750001/effect-of-combined-dyslipidemia-and-hyperglycemia-on-diabetic-peripheral-neuropathy-in-alloxan-induced-diabetic-wbn-kob-rats
#8
Kiyokazu Ozaki, Yui Terayama, Tetsuro Matsuura, Isao Narama
Clinical and experimental research have suggested that dyslipidemia aggravates diabetic peripheral neuropathy (DPN). However, whether dyslipidemia is a risk factor for DPN remains unclear. To investigate the effect of dyslipidemia on DPN, morphological features of peripheral nerves were analyzed in diabetic rats treated with a high-fat diet (HFD). Male rats were divided into four groups: nondiabetic rats (N), alloxan-induced diabetic rats (AL), diabetic rats treated with an HFD (AH), and nondiabetic rats treated with an HFD (HF)...
April 2018: Journal of Toxicologic Pathology
https://www.readbyqxmd.com/read/29749662/high-resolution-nerve-ultrasound-and-electrophysiological-findings-in-restless-legs-syndrome
#9
K Pitarokoili, M Fels, A Kerasnoudis, L Toenges, R Gold, M-S Yoon
BACKGROUND AND PURPOSE: Restless legs syndrome (RLS) is a multifactorial network disorder of a sensorimotor system extending from dopaminergic and glutamatergic cerebral structures to the spinal neurons and peripheral nerves. The role of peripheral nerve damage in the causality and severity progression for RLS patients remains unclear. METHODS: We performed a clinical and epidemiological study on a cohort of 34 RLS patients focusing on RLS risk factors and disease severity...
May 11, 2018: Journal of Neuroimaging: Official Journal of the American Society of Neuroimaging
https://www.readbyqxmd.com/read/29747701/astrocyte-remodeling-without-gliosis-precedes-optic-nerve-axonopathy
#10
Melissa L Cooper, John W Collyer, David J Calkins
Astroyctes serve myriad functions but are especially critical in white matter tracts, where energy-demanding axons propagate action potentials great distances between neurons. Axonal dependence on astrocytes for even normal function accentuates the critical role astrocytes serve during disease. In glaucoma, the most common optic neuropathy, sensitivity to intraocular pressure (IOP) challenges RGC axons early, including degradation of anterograde transport to the superior colliculus (SC). Astrocyte remodeling presages overt axon degeneration in glaucoma and thus may present a therapeutic opportunity...
May 10, 2018: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/29746890/use-of-a-capillary-alginate-gel-capgel%C3%A2-to-study-the-three-dimensional-development-of-sensory-nerves-reveals-the-formation-of-a-rudimentary-perineurium
#11
Wesley A Anderson, Alicia R Willenberg, Alexander J Bosak, Bradley J Willenberg, Stephen Lambert
BACKGROUND: Peripheral neuropathies affect approximately 20 million people in the United States and often stem from other chronic conditions, such as diabetes. In vitro methodologies to facilitate the understanding and treatment of these disorders often lack the cellular and functional complexity required to accurately model peripheral neuropathies. In particular, they are often 2D and fail to faithfully reproduce the 3D in vivo microenvironment. NEW METHOD: Embryonic dorsal root ganglion (DRG) explants were inserted into laminin derivatized capillary alginate gel (Capgel™), a bioabsorbable, self-assembling biomaterial, possessing parallel microchannel architecture, and cultured to mimic normal nerve development, including Schwann cell myelination...
May 7, 2018: Journal of Neuroscience Methods
https://www.readbyqxmd.com/read/29746557/optic-tract-injury-after-closed-head-traumatic-brain-injury-in-mice-a-model-of-indirect-traumatic-optic-neuropathy
#12
Nathan K Evanson, Fernanda Guilhaume-Correa, James P Herman, Michael D Goodman
Adult male C57BL/6J mice have previously been reported to have motor and memory deficits after experimental closed head traumatic brain injury (TBI), without associated gross pathologic damage or neuroimaging changes detectable by magnetic resonance imaging or diffusion tensor imaging protocols. The presence of neurologic deficits, however, suggests neural damage or dysfunction in these animals. Accordingly, we undertook a histologic analysis of mice after TBI. Gross pathology and histologic analysis using Nissl stain and NeuN immunohistochemistry demonstrated no obvious tissue damage or neuron loss...
2018: PloS One
https://www.readbyqxmd.com/read/29742495/progressive-degeneration-and-inhibition-of-peripheral-nerve-regeneration-in-the-sod1-g93a-mouse-model-of-amyotrophic-lateral-sclerosis
#13
Binbin Deng, Wenjing Lv, Weisong Duan, Yakun Liu, Zhongyao Li, Yanqin Ma, Guisen Zhang, Xueqin Song, Can Cui, Xiaoming Qi, Yuan Li, Chunyan Li
BACKGROUND: Myelination, degeneration and regeneration are implicated in crucial responses to injury in the peripheral nervous system. Considering the progression of amyotrophic lateral sclerosis (ALS), we used the superoxide dismutase 1 (SOD1)-G93A transgenic mouse model of ALS to investigate the effects of mutant SOD1 on the peripheral nerves. METHODS: Changes in peripheral nerve morphology were analyzed in SOD1 mutant mice at various stages of the disease by toluidine blue staining and electron microscopy (EM)...
May 4, 2018: Cellular Physiology and Biochemistry
https://www.readbyqxmd.com/read/29740279/activation-of-neuregulin-1-erbb-signaling-is-involved-in-the-development-of-tocp-induced-delayed-neuropathy
#14
Hai-Yang Xu, Pan Wang, Ying-Jian Sun, Ming-Yuan Xu, Li Zhu, Yi-Jun Wu
Organophosphate-induced delayed neuropathy (OPIDN) is characterized by progressive axonal degeneration and demyelination of the spinal cord and sciatic nerves. The neuregulin 1/epidermal growth factor receptor (ErbB) signaling pathway is crucial for axonal myelination. In this study, we investigated whether the neuregulin 1/ErbB signaling pathway mediated the progression of OPIDN. Adult hens were given tri- o -cresyl phosphate (TOCP), a typical neuropathic organophosphorus compound, to induce OPIDN. The ErbB inhibitor lapatinib was administered to hens 4 h prior to and 4 days after TOCP exposure...
2018: Frontiers in Molecular Neuroscience
https://www.readbyqxmd.com/read/29736880/comparison-of-peripheral-nerve-protection-between-insulin-based-glucose-control-and-alpha-lipoic-acid-ala-in-the-streptozotocin-stz-induced-diabetic-rat
#15
Kyung Ae Lee, Na Young Lee, Tae Sun Park, Heung Yong Jin
Strict glucose control is a well-proven therapeutic approach for peripheral neuropathies in patients with diabetes. Alpha lipoic acid (ALA) has also been accepted as a therapeutic agent for diabetic peripheral neuropathy (DPN) in the respect of pathogenesis. However, the potential of ALA as a treatment for DPN in comparison to that of glucose control is unclear. In this study, we compared the neuroprotective potential of glucose control and ALA. Animals were divided into 6 groups based on the intervention used, as follows: normal, diabetes (DM), DM+racemic form of ALA, DM+R form of ALA, DM+once daily insulin glargine, and DM+once daily insulin glargine with twice daily insulin glulisine...
May 7, 2018: Endocrine
https://www.readbyqxmd.com/read/29729594/motor-unit-number-index-correlates-with-disability-in-charcot-marie-tooth-disease
#16
Joachim Bas, Emilien Delmont, Farzad Fatehi, Emmanuelle Salort-Campana, Annie Verschueren, Jean Pouget, Marie-Noëlle Lefebvre, Aude-Marie Grapperon, Shahram Attarian
OBJECTIVE: The aim of this study was to assess the usefulness of motor unit number index (MUNIX) technique in Charcot-Marie-Tooth disease and test the correlation between MUNIX and clinical impairment. METHODS: MUNIX technique was performed in the abductor pollicis brevis (APB), the abductor digiti minimi (ADM) and the tibialis anterior (TA) muscles in the nondominant side. A MUNIX sum score was calculated by adding the MUNIX of these 3 muscles. Muscle strength was measured using the MRC (medical research council) scale...
April 16, 2018: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
https://www.readbyqxmd.com/read/29723581/the-nitric-oxide-guanylate-cyclase-pathway-and-glaucoma
#17
REVIEW
Lauren K Wareham, Emmanuel S Buys, Rebecca M Sappington
Glaucoma is a prevalent optic neuropathy characterized by the progressive dysfunction and loss of retinal ganglion cells (RGCs) and their optic nerve axons leading to irreversible visual field loss. Multiple risk factors for the disease have been identified, but elevated intraocular pressure (IOP) remains the primary risk factor amenable to treatment. Reducing IOP however does not always prevent glaucomatous neurodegeneration, and many patients progress with the disease despite having IOP in the normal range...
April 30, 2018: Nitric Oxide: Biology and Chemistry
https://www.readbyqxmd.com/read/29722322/treatment-with-acetyl-l-carnitine-exerts-a-neuroprotective-effect-in-the-sciatic-nerve-following-loose-ligation-a-functional-and-microanatomical-study
#18
Daniele Tomassoni, Lorenzo Di Cesare Mannelli, Vincenzo Bramanti, Carla Ghelardini, Francesco Amenta, Alessandra Pacini
Peripheral neuropathies are chronic painful syndromes characterized by allodynia, hyperalgesia and altered nerve functionality. Nerve tissue degeneration represents the microanatomical correlate of peripheral neuropathies. Aimed to improve the therapeutic possibilities, this study investigated the hypersensitivity and the neuromorphological alterations related to the loose ligation of the sciatic nerve in rats. Effects elicited by treatment with acetyl-L-carnitine (ALCAR) in comparison to gabapentin were assessed...
April 2018: Neural Regeneration Research
https://www.readbyqxmd.com/read/29719281/expression-of-nrf2-promotes-schwann-cell-mediated-sciatic-nerve-recovery-in-diabetic-peripheral-neuropathy
#19
Wei Tang, Xiangfang Chen, Haoqi Liu, Qian Lv, Junjie Zou, Yongquan Shi, Zhimin Liu
BACKGROUND/AIMS: High glucose-induced oxidative stress and inflammatory responses play an important role in painful diabetic neuropathy by activating the TLR4/NFκB signal pathway. Schwann cells (SCs) are integral to peripheral nerve biology, contributing to saltatory conduction along axons, nerve and axon development, and axonal regeneration. SCs provide a microenvironment favoring vascular regeneration but their low survival ratio in hyperglycemic conditions suppress the function to promote nerve growth...
April 26, 2018: Cellular Physiology and Biochemistry
https://www.readbyqxmd.com/read/29718187/mutations-in-coa7-cause-spinocerebellar-ataxia-with-axonal-neuropathy
#20
Yujiro Higuchi, Ryuta Okunushi, Taichi Hara, Akihiro Hashiguchi, Junhui Yuan, Akiko Yoshimura, Kei Murayama, Akira Ohtake, Masahiro Ando, Yu Hiramatsu, Satoshi Ishihara, Hajime Tanabe, Yuji Okamoto, Eiji Matsuura, Takehiro Ueda, Tatsushi Toda, Sumimasa Yamashita, Kenichiro Yamada, Takashi Koide, Hiroaki Yaguchi, Jun Mitsui, Hiroyuki Ishiura, Jun Yoshimura, Koichiro Doi, Shinichi Morishita, Ken Sato, Masanori Nakagawa, Masamitsu Yamaguchi, Shoji Tsuji, Hiroshi Takashima
Several genes related to mitochondrial functions have been identified as causative genes of neuropathy or ataxia. Cytochrome c oxidase assembly factor 7 (COA7) may have a role in assembling mitochondrial respiratory chain complexes that function in oxidative phosphorylation. Here we identified four unrelated patients with recessive mutations in COA7 among a Japanese case series of 1396 patients with Charcot-Marie-Tooth disease (CMT) or other inherited peripheral neuropathies, including complex forms of CMT...
April 27, 2018: Brain: a Journal of Neurology
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