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https://www.readbyqxmd.com/read/28332470/adult-onset-demyelinating-neuropathy-associated-with-fbln5-gene-mutation
#1
Si Cheng, He Lv, Wei Zhang, Zhaoxia Wang, Xin Shi, Wei Liang, Yun Yuan
Rare forms of autosomal-dominant Charcot-Marie-Tooth disease (AD-CMT) may be associated with mutations in Fibulin-5 (FBLN5) as AD-CMT is genetically heterogeneous. Here, we report the first pathological study of an Asian family. The proband was a 46-year-old man with slowly progressive distal numbness and weakness for 12 years. He had a history of diabetes mellitus for 12 years. His mother was 81 years old and had mild polyneuropathy. His 16-year-old daughter was asymptomatic. The nerve conduction velocities (NCVs) and compound muscular action potential (CMAP) amplitudes were moderately to severely reduced in the proband, and moderately reduced in his daughter and mother...
March 23, 2017: Clinical Neuropathology
https://www.readbyqxmd.com/read/28325700/the-relationship-between-preoperative-needle-electromyography-findings-and-muscle-power-restoration-after-surgery-in-severe-carpal-tunnel-syndrome-patients
#2
Yuki Hara, Yasumasa Nishiura, Naoyuki Ochiai, Shinji Murai, Masashi Yamazaki
PURPOSE: Needle electromyography provides essential information about the functional aspects of the muscle. But little attention has been given in the literature to needle electromyography examinations in carpal tunnel syndrome. We examined the relationship between preoperative needle electromyography findings and functional recovery of the abductor pollicis brevis (APB) muscle in severe carpal tunnel syndrome patients. METHODS: The subjects of this study were 49 patients, 58 hands, who fit the following 5 criteria: (1) idiopathic carpal tunnel syndrome; (2) pre-op MMT grade of the APB muscle was M0 or M1; (3) APB-CMAP (compound muscle action potential) was not evoked in a median nerve conduction study; (4) needle electromyography of the APB muscle had been done; (5) underwent carpal tunnel release only...
March 18, 2017: Journal of Orthopaedic Science: Official Journal of the Japanese Orthopaedic Association
https://www.readbyqxmd.com/read/28319875/electrophysiologic-features-of-ulnar-neuropathy-in-childhood-and-adolescence
#3
Ioannis Karakis, Wendy Liew, Heather Szelag Fournier, H Royden Jones, Basil T Darras, Peter B Kang
OBJECTIVE: To analyze patterns of nerve injury in pediatric ulnar neuropathy (PUN). METHODS: Retrospective analysis of 49 children with PUN. RESULTS: Sensory loss in digit V was the prevailing complaint (89%). Predominant localization was at the elbow (55%). Diminished ulnar SNAP was the most common abnormality (71%) with median axon loss estimate (MAXE) of 62%. Dorsal ulnar cutaneous (DUC) sensory nerve action potential (SNAP) was reduced in 55% with MAXE of 43%...
February 20, 2017: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
https://www.readbyqxmd.com/read/28287078/the-design-of-and-chronic-tissue-response-to-a-composite-nerve-electrode-with-patterned-stiffness
#4
Max Freeberg, Matthew Stone, Ronald Triolo, Dustin Tyler
As neural interfaces demonstrate success in chronic applications, a novel class of reshaping electrodes with patterned regions of stiffness will enable application to a widening range of anatomical locations. Patterning stiff regions and flexible regions of the electrode enables nerve reshaping while accommodating anatomical constraints of various implant locations ranging from peripheral nerves to spinal and autonomic plexi. Introduced is a new composite electrode enabling patterning of regions of various electrode mechanical properties...
March 13, 2017: Journal of Neural Engineering
https://www.readbyqxmd.com/read/28256512/transcriptome-based-repurposing-of-apigenin-as-a-potential-anti-fibrotic-agent-targeting-hepatic-stellate-cells
#5
Daniel F Hicks, Nicolas Goossens, Ana Blas-García, Takuma Tsuchida, Benjamin Wooden, Michael C Wallace, Natalia Nieto, Abigale Lade, Benjamin Redhead, Arthur I Cederbaum, Joel T Dudley, Bryan C Fuchs, Youngmin A Lee, Yujin Hoshida, Scott L Friedman
We have used a computational approach to identify anti-fibrotic therapies by querying a transcriptome. A transcriptome signature of activated hepatic stellate cells (HSCs), the primary collagen-secreting cell in liver, and queried against a transcriptomic database that quantifies changes in gene expression in response to 1,309 FDA-approved drugs and bioactives (CMap). The flavonoid apigenin was among 9 top-ranked compounds predicted to have anti-fibrotic activity; indeed, apigenin dose-dependently reduced collagen I in the human HSC line, TWNT-4...
March 3, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28249248/neuroprotective-effects-of-octreotide-on-diabetic-neuropathy-in-rats
#6
Volkan Solmaz, Bilge Piri Çınar, Gürkan Yiğittürk, Hatice Köse Özlece, Hüseyin Avni Eroglu, Aslan Tekatas, Oytun Erbaş, Dilek Taşkıran
The purpose of the present study is to investigate the possible healing effects of octreotide (OCT) on motor performance, electrophysiological and histopathological findings of diabetic neuropathy in a rat model of diabetes mellitus (DM). To induce diabetes, rats were administered a single dose (60mg/kg) of streptozotocin (STZ). Diabetic rats were treated either with saline (1ml/kg/day, n=7) or OCT (0.1mg/kg/day, n=7) for four weeks. Seven rats served as control group and received no treatment. At the end of the study, electromyography (EMG), gross motor function (inclined plate test), general histology and the perineural thickness of sciatic nerve were evaluated...
February 26, 2017: Biomedicine & Pharmacotherapy, Biomédecine & Pharmacothérapie
https://www.readbyqxmd.com/read/28224637/compound-motor-action-potential-duration-and-latency-are-markers-of-recurrent-laryngeal-nerve-injury
#7
Neel K Bhatt, Andrea M Park, Mohammad T Al-Lozi, Derrick C Gale, Randal C Paniello
OBJECTIVE: Compound motor action potential (CMAP) can quantitatively evaluate innervation following injury to the recurrent laryngeal nerve (RLN) in canines. CMAP duration (the total time of CMAP) and latency (the time between the nerve impulse and the onset of action potentials) have not been assessed following RLN injury. STUDY DESIGN: Animal study. METHODS: Twelve canine hemilaryngeal preparations were investigated. Baseline CMAP duration and latency were derived...
February 22, 2017: Laryngoscope
https://www.readbyqxmd.com/read/28161089/are-electrophysiological-features-related-to-disability-in-patients-with-anti-mag-neuropathy
#8
Aude Gesquière-Dando, Emilien Delmont, Mael Launay, Jose Boucraut, Sharham Attarian
OBJECTIVES: To explore clinical-neurophysiological correlations in anti-myelin-associated glycoprotein (anti-MAG) neuropathy. METHODS: Clinical and electrophysiological data of 42 patients with anti-MAG neuropathy were retrospectively analysed. Disability was evaluated using the Overall Neuropathy Limitation Scale (ONLS), motor impairment through MRC sum score and sensory deficiency through INCAT sensory score. Compound motor action potential (CMAP) sum score was calculated adding the distal CMAP amplitude of the median, ulnar, tibial and fibular nerves of both sides...
February 2017: Neurophysiologie Clinique, Clinical Neurophysiology
https://www.readbyqxmd.com/read/28153715/neuromuscular-excitability-changes-produced-by-sustained-voluntary-contraction-and-response-to-mexiletine-in-myotonia-congenita
#9
Federica Ginanneschi, Andrea Mignarri, Sabrina Lucchiari, Gianna Ulzi, Giacomo P Comi, Alessandro Rossi, Maria Teresa Dotti
OBJECTIVE: To investigate the cause of transient weakness in myotonia congenita (MC) and the mechanism of action of mexiletine in reducing weakness. METHODS: The changes in neuromuscular excitability produced by 1min of maximal voluntary contractions (MVC) were measured on the amplitude of compound muscle action potentials (CMAP) in two patients with either recessive or dominant MC, compared to control values obtained in 20 healthy subjects. Measurements were performed again in MC patients after mexiletine therapy...
January 30, 2017: Neurophysiologie Clinique, Clinical Neurophysiology
https://www.readbyqxmd.com/read/28150521/drug-repurposing-and-therapeutic-anti-microrna-predictions-for-inhibition-of-oxidized-low-density-lipoprotein-induced-vascular-smooth-muscle-cell-associated-diseases
#10
Shun-Tsung Chen, Chien-Hung Huang, Victor C Kok, Chi-Ying F Huang, Jin-Shuei Ciou, Jeffrey J P Tsai, Nilubon Kurubanjerdjit, Ka-Lok Ng
Drug repurposing is a new method for disease treatments, which accelerates the identification of new uses for existing drugs with minimal side effects for patients. MicroRNA-based therapeutics are a class of drugs that have been used in gene therapy following the FDA's approval of the first anti-sense therapy. This study examines the effects of oxLDL on vascular smooth muscle cells (VSMCs) and identifies potential drugs and antimiRs for treating VSMC-associated diseases. The Connectivity Map (cMap) database is utilized to identify potential new uses of existing drugs...
February 2017: Journal of Bioinformatics and Computational Biology
https://www.readbyqxmd.com/read/28139721/therapeutic-properties-of-scutellaria-baicalensis-in-db-db-mice-evaluated-using-connectivity-map-and-network-pharmacology
#11
Bu-Yeo Kim, Kwang Hoon Song, Chi-Yeon Lim, Su-In Cho
We have reported that an extract of Scutellaria baicalensis (ESB) has effects against obesity and hypertriglyceridemia in type 2 diabetic animal model (db/db mouse). In the present study, we tried to explain the pharmacological effects of ESB by integrating gene expression information from db/db mouse liver with that of ESB-treated HepG2 hepatocellular carcinoma cells. Using Connectivity Map (cmap) analysis, we found an inverse relationship in the pharmaceutical profiles based on gene expression between db/db mouse liver and ESB-treated HepG2 cells...
January 31, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28139516/effects-of-head-down-tilt-on-nerve-conduction-in-rhesus-monkeys
#12
Bo Sun, Xiao-Yun Zhang, Li-Zhi Liu, Zhao-Hui Chen, Zhong-Quan Dai, Xu-Sheng Huang
BACKGROUND: Few studies have focused on peripheral nerve conduction during exposure to microgravity. The -6° head-down tilt (HDT) comprises an experimental model used to simulate the space flight environment. This study investigated nerve conduction characteristics of rhesus monkeys before and after prolonged exposure to HDT. METHODS: Six rhesus monkeys (3-4 years old) were tilted backward 6° from the horizontal. Nerve conduction studies (NCSs) were performed on the median, ulnar, tibial, and fibular motor nerves...
February 5, 2017: Chinese Medical Journal
https://www.readbyqxmd.com/read/28132610/upper-limb-recovery-in-spinal-cord-injury
#13
Jens A Petersen, Martina Spiess, Armin Curt, Norbert Weidner, Rüdiger Rupp, Rainer Abel, Martin Schubert
BACKGROUND AND OBJECTIVE: The course of central and peripheral motor recovery after cervical spinal cord injury (SCI) may be investigated by electrophysiological measures. The goal of this study was to compare the 2 over the first year after injury in relation to motor gains. METHODS: Compound motor action potentials (CMAPs), motor-evoked potentials (MEPs), and F-waves were recorded from the abductor digiti minimi and CMAP and F-waves from abductor hallucis muscles in 305 patients at about 15 days, 1 month, 3 months, 6 months, and 12 months following an acute traumatic SCI...
January 1, 2017: Neurorehabilitation and Neural Repair
https://www.readbyqxmd.com/read/28069634/a-review-of-connectivity-map-and-computational-approaches-in-pharmacogenomics
#14
Aliyu Musa, Laleh Soltan Ghoraie, Shu-Dong Zhang, Galina Galzko, Olli Yli-Harja, Matthias Dehmer, Benjamin Haibe-Kains, Frank Emmert-Streib
Large-scale perturbation databases, such as Connectivity Map (CMap) or Library of Integrated Network-based Cellular Signatures (LINCS), provide enormous opportunities for computational pharmacogenomics and drug design. A reason for this is that in contrast to classical pharmacology focusing at one target at a time, the transcriptomics profiles provided by CMap and LINCS open the door for systems biology approaches on the pathway and network level. In this article, we provide a review of recent developments in computational pharmacogenomics with respect to CMap and LINCS and related applications...
January 9, 2017: Briefings in Bioinformatics
https://www.readbyqxmd.com/read/28068522/motor-evoked-potential-gain-is-a-helpful-test-for-the-detection-of-corticospinal-tract-dysfunction-in-amyotrophic-lateral-sclerosis
#15
Y Duclos, A M Grapperon, E Jouve, R Truillet, C Zemmour, A Verschueren, J Pouget, S Attarian
OBJECTIVE: The detection of upper motor neuron (UMN) dysfunction is necessary for the diagnosis of amyotrophic lateral sclerosis (ALS). However, signs of UMN dysfunction may be difficult to establish. This study aimed to determine whether motor-evoked potential (MEP) gain (MEP area/background electromyographic activity) represents an efficient alternative to assess UMN dysfunction. METHODS: MEP area, MEP/compound muscle action potential (CMAP) area ratio, and MEP gain were tested at different force levels in healthy control subjects and ALS patients...
February 2017: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
https://www.readbyqxmd.com/read/28058586/key-genes-in-stomach-adenocarcinoma-identified-via-network-analysis-of-rna-seq-data
#16
Li Shen, Lizhi Zhao, Jiquan Tang, Zhiwei Wang, Weisong Bai, Feng Zhang, Shouli Wang, Weihua Li
RNA-seq data of stomach adenocarcinoma (STAD) were analyzed to identify critical genes in STAD. Meanwhile, relevant small molecule drugs, transcription factors (TFs) and microRNAs (miRNAs) were also investigated. Gene expression data of STAD were downloaded from The Cancer Genome Atlas (TCGA). Differential analysis was performed with package edgeR. Relationships with correlation coefficient > 0.6 were retained in the gene co-expression network. Functional enrichment analysis was performed for the genes in the network with DAVID and KOBASS 2...
January 5, 2017: Pathology Oncology Research: POR
https://www.readbyqxmd.com/read/28052373/best-time-window-for-the-use-of-calcium-modulating-agents-to-improve-functional-recovery-in-injured-peripheral-nerves-an-experiment-in-rats
#17
Yuhui Yan, Feng-Yi Shen, Michael Agresti, Lin-Ling Zhang, Hani S Matloub, John A LoGiudice, Robert Havlik, Jifeng Li, Yu-Dong Gu, Ji-Geng Yan
Peripheral nerve injury can have a devastating effect on daily life. Calcium concentrations in nerve fibers drastically increase after nerve injury, and this activates downstream processes leading to neuron death. Our previous studies showed that calcium-modulating agents decrease calcium accumulation, which aids in regeneration of injured peripheral nerves; however, the optimal therapeutic window for this application has not yet been identified. In this study, we show that calcium clearance after nerve injury is positively correlated with functional recovery in rats suffering from a crushed sciatic nerve injury...
January 4, 2017: Journal of Neuroscience Research
https://www.readbyqxmd.com/read/28043769/motor-unit-number-index-munix-detects-motor-neuron-loss-in-pre-symptomatic-muscles-in-amyotrophic-lateral-sclerosis
#18
Christoph Neuwirth, Paul E Barkhaus, Christian Burkhardt, José Castro, David Czell, Mamede de Carvalho, Sanjeev Nandedkar, Erik Stålberg, Markus Weber
OBJECTIVE: Motor Unit Number Index (MUNIX) is a quantitative neurophysiological measure that provides an index of the number of lower motor neurons supplying a muscle. It reflects the loss of motor neurons in patients with Amyotrophic Lateral Sclerosis (ALS). However, it is unclear whether MUNIX also detects motor unit loss in strong, non-wasted muscles. METHODS: Three centres measured MUNIX in 49 ALS patients every three months in six different muscles (abductor pollicis brevis, abductor digiti minimi, biceps brachii, tibialis anterior, extensor digitorum brevis, abductor hallucis) on the less affected side...
December 18, 2016: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
https://www.readbyqxmd.com/read/28024449/effects-of-different-force-fields-on-the-structural-character-of-%C3%AE-synuclein-%C3%AE-hairpin-peptide-35-56-in-aqueous-environment
#19
Sangeeta Kundu
The hallmark of Parkinson's disease (PD) is the intracellular protein aggregation forming Lewy Bodies (LB) and Lewy neuritis which comprise mostly of a protein, alpha synuclein (α-syn). Molecular dynamics (MD) simulation methods can augment experimental techniques to understand misfolding and aggregation pathways with atomistic resolution. The quality of MD simulations for proteins and peptides depends greatly on the accuracy of empirical force fields. The aim of this work is to investigate the effects of different force fields on the structural character of β hairpin fragment of α-syn (residues 35-56) peptide in aqueous solution...
January 23, 2017: Journal of Biomolecular Structure & Dynamics
https://www.readbyqxmd.com/read/28017534/direct-motor-evoked-potentials-and-cortical-mapping-using-the-nim%C3%A2-nerve-monitoring-system-a-technical-note
#20
Suparna Bharadwaj, Faizal Haji, Matthew Hebb, Jason Chui
Motor evoked potentials (MEPs) are commonly used to prevent neurological injury when operating in close proximity to the motor cortex or corticospinal pathway. We report a novel application of the NIM® nerve monitoring system (Medtronic@ NIM response 3.0) for intraoperative direct cortical (dc)-MEPs monitoring. A 69-year-old female patient presented with a 4month history of progressive left hemiparesis resulting from a large right sided posterior frontal meningioma that abutted and compressed the motor cortex...
April 2017: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
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