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Axonal neuropathy

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https://www.readbyqxmd.com/read/28914883/cidp-and-other-inflammatory-neuropathies-in-diabetes-diagnosis-and-management
#1
REVIEW
Yusuf A Rajabally, Mark Stettner, Bernd C Kieseier, Hans-Peter Hartung, Rayaz A Malik
Distal symmetric polyneuropathy (DSPN) is the most common neuropathy to occur in diabetes mellitus. However, patients with diabetes can also develop inflammatory neuropathies, the most common and most treatable of which is chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Whether diabetes is a risk factor for CIDP remains under debate. Early studies suggested that patients with diabetes were at increased risk of CIDP, but epidemiological studies failed to confirm the association, and subsequent data have re-opened the debate...
September 15, 2017: Nature Reviews. Neurology
https://www.readbyqxmd.com/read/28912677/reversible-axonal-dystrophy-by-calcium-modulation-in-frataxin-deficient-sensory-neurons-of-yg8r-mice
#2
Belén Mollá, Diana C Muñoz-Lasso, Fátima Riveiro, Arantxa Bolinches-Amorós, Federico V Pallardó, Angel Fernandez-Vilata, María de la Iglesia-Vaya, Francesc Palau, Pilar Gonzalez-Cabo
Friedreich's ataxia (FRDA) is a peripheral neuropathy involving a loss of proprioceptive sensory neurons. Studies of biopsies from patients suggest that axonal dysfunction precedes the death of proprioceptive neurons in a dying-back process. We observed that the deficiency of frataxin in sensory neurons of dorsal root ganglia (DRG) of the YG8R mouse model causes the formation of axonal spheroids which retain dysfunctional mitochondria, shows alterations in the cytoskeleton and it produces impairment of axonal transport and autophagic flux...
2017: Frontiers in Molecular Neuroscience
https://www.readbyqxmd.com/read/28912674/a-mechanistic-understanding-of-axon-degeneration-in-chemotherapy-induced-peripheral-neuropathy
#3
REVIEW
Yusuke Fukuda, Yihang Li, Rosalind A Segal
Chemotherapeutic agents cause many short and long term toxic side effects to peripheral nervous system (PNS) that drastically alter quality of life. Chemotherapy-induced peripheral neuropathy (CIPN) is a common and enduring disorder caused by several anti-neoplastic agents. CIPN typically presents with neuropathic pain, numbness of distal extremities, and/or oversensitivity to thermal or mechanical stimuli. This adverse side effect often requires a reduction in chemotherapy dosage or even discontinuation of treatment...
2017: Frontiers in Neuroscience
https://www.readbyqxmd.com/read/28912156/neutrophils-are-critical-for-myelin-removal-in-a-peripheral-nerve-injury-model-of-wallerian-degeneration
#4
Jane A Lindborg, Matthias Mack, Richard E Zigmond
Wallerian degeneration (WD) is considered an essential preparatory stage to the process of axonal regeneration. In the peripheral nervous system, infiltrating monocyte-derived macrophages that utilize the chemokine receptor CCR2 to gain entry to injured tissues from the bloodstream, are purportedly necessary for efficient WD. However, our lab has previously reported that myelin clearance in the injured sciatic nerve proceeds unhindered in the Ccr2(-/-) mouse model. Here, we extensively characterize WD in male Ccr2(-/-) mice and identify a compensatory mechanism of WD that is facilitated primarily by neutrophils...
September 14, 2017: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/28904467/post-bariatric-surgery-acute-axonal-polyneuropathy-doing-your-best-is-not-always-enough
#5
Zakia Mohammad Yasawy, Ali Hassan
Neurological complications are frequently recognized with weight reduction surgeries for morbid obesity. The spectrum of peripheral neuropathies complicating the weight loss surgery is wide, and among them, the acute axonal peripheral neuropathy resembling Guillain-Barre syndrome is rare and only less than a dozen cases are reported. We present three cases, which after bariatric surgery developed acute polyneuropathy that rapidly progressed over 4 weeks from the onset. All patients responded to aggressive parenteral Vitamin B1 and B12 replacement therapy...
July 2017: Annals of Indian Academy of Neurology
https://www.readbyqxmd.com/read/28904462/clinical-spectrum-therapeutic-outcomes-and-prognostic-predictors-in-sjogren-s-syndrome-associated-neuropathy
#6
Ajith Sivadasan, Karthik Muthusamy, Bimal Patel, Rohit Ninan Benjamin, A T Prabhakar, Vivek Mathew, Sanjith Aaron, Mathew Alexander
OBJECTIVES: There are limited data regarding long-term follow-up and therapeutic outcomes in Sjogren's syndrome (SS)-associated peripheral neuropathy. In this study, we aim to study the clinical, electrophysiological spectrum and therapeutic responses among the different subtypes of SS-associated neuropathy. The predictors of suboptimal treatment response will be identified. METHODS: The study included a retrospective cohort of patients with SS-associated neuropathy between January 2012 and November 2015...
July 2017: Annals of Indian Academy of Neurology
https://www.readbyqxmd.com/read/28899886/brainstem-encephalitis-and-acute-polyneuropathy-associated-with-hepatitis-e-infection
#7
Omar Jabbar Salim, Amy Davidson, Kathy Li, John Paul Leach, Craig Heath
A 59-year-old man presented with feverish illness. His Glasgow Coma Scale was 15, had reduced visual acuity in the left eye with partial left ptosis and mild left hemiparesis with an extensor left plantar. Over 48 hours, he accrued multiple cranial nerves palsies and progressed to a flaccid paralysis necessitating admission to an intensive care unit.Cerebrospinal fluid (CSF) study showed 20 lymphocytes and raised protein. Viral and bacterial PCRs were negative. Samples for Lyme, blood-borne viruses, syphilis and autoantibodies were also negative...
September 11, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28899883/microrna-146a-mimics-reduce-the-peripheral-neuropathy-in-type-ii-diabetic-mice
#8
Xian Shuang Liu, Baoyan Fan, Alexandra Szalad, Longfei Jia, Lei Wang, Xinli Wang, Wanlong Pan, Li Zhang, Ruilan Zhang, Jiani Hu, Xiao Ming Zhang, Michael Chopp, Zheng Gang Zhang
MicroRNA-146a (miR-146a) regulates multiple immune diseases. However, the role of miR-146a in diabetic peripheral neuropathy (DPN) has not been investigated. We found that mice (db/db) with type II diabetes exhibited substantial down-regulation of miR-146a in sciatic nerve tissue. Systemic administration of miR-146a mimics to diabetic mice elevated miR-146a levels in plasma and sciatic nerve tissue and substantially increased motor and sensory nerve conduction velocities by 29% and 11%, respectively, and regional blood flow by 50% in sciatic nerve tissue...
September 12, 2017: Diabetes
https://www.readbyqxmd.com/read/28899595/peripheral-nerve-pathology-at-fixed-stage-in-spinal-muscular-atrophy-with-respiratory-distress-type-1
#9
Azusa Ikeda, Sumimasa Yamashita, Yu Tsuyusaki, Mio Tanaka, Yukichi Tanaka, Akihiro Hashiguchi, Hiroshi Takashima, Tomohide Goto
Spinal muscular atrophy with respiratory distress type 1 (SMARD1) is characterized by severe respiratory failure due to diaphragmatic paralysis and distal muscular weakness in early infancy. After an initial decline in respiratory state and motor function until 1-2years of age, residual capabilities reach a plateau. We report the peripheral neuropathological findings of a patient with SMARD1 at 1year and 1month of age, when his muscle strength and respiratory symptoms had deteriorated and then stabilized for several months...
September 9, 2017: Brain & Development
https://www.readbyqxmd.com/read/28890682/impaired-mitophagy-plays-a-role-in-denervation-of-neuromuscular-junctions-in-als-mice
#10
Robert S Rogers, Sudheer Tungtur, Tomohiro Tanaka, Lisa L Nadeau, Yomna Badawi, Hua Wang, Hong-Min Ni, Wen-Xing Ding, Hiroshi Nishimune
Motor neurons in amyotrophic lateral sclerosis (ALS) patients and animal models show degeneration from the nerve terminal, known as dying-back neuropathy. To investigate the mechanism underlying this neuropathy, we analyzed the neuromuscular junctions (NMJs) and motor neuron cell bodies in SOD1(G93A) mice using electron microscopy. NMJs of SOD1(G93A) mice exhibited significantly higher numbers of autophagosomes and degenerated mitochondria compared to wild-type controls. Mitophagosomes were identified in the NMJ presynaptic terminals of wild-type mice and SOD1(G93A) mice...
2017: Frontiers in Neuroscience
https://www.readbyqxmd.com/read/28888467/early-electrophysiological-findings-in-fisher-bickerstaff-syndrome
#11
M A Alberti, M Povedano, J Montero, C Casasnovas
INTRODUCTION: The term Fisher-Bickerstaff syndrome (FBS) has been proposed to describe the clinical spectrum encompassing Miller-Fisher syndrome (MFS) and Bickerstaff brainstem encephalitis. The pathophysiology of FBS and the nature of the underlying neuropathy (demyelinating or axonal) are still subject to debate. This study describes the main findings of an early neurophysiological study on 12 patients diagnosed with FBS. PATIENTS AND METHODS: Retrospective evaluation of clinical characteristics and electrophysiological findings of 12 patients with FBS seen in our neurology department within 10 days of disease onset...
September 6, 2017: Neurología: Publicación Oficial de la Sociedad Española de Neurología
https://www.readbyqxmd.com/read/28886934/transglutaminase-6-antibodies-in-gluten-neuropathy
#12
Panagiotis Zis, Dasappaiah Ganesh Rao, Ptolemaios Georgios Sarrigiannis, Pascale Aeschlimann, Daniel P Aeschlimann, David Sanders, Richard A Grünewald, Marios Hadjivassiliou
BACKGROUND: TG6 antibodies have been shown to be a marker of gluten ataxia but their presence in the context of other neurological manifestations of gluten sensitivity has not been explored. We investigated the presence of TG6 antibodies in gluten neuropathy (GN), defined as as an otherwise idiopathic peripheral neuropathy associated with serological markers of gluten sensitivity (one or more of antigliadin IgG and/or IgA, endomysial and transglutaminase-2 antibodies). METHODS: This was a cross-sectional study conducted at the Sheffield Institute of Gluten Related Diseases, Royal Hallamshire Hospital, Sheffield, UK...
August 10, 2017: Digestive and Liver Disease
https://www.readbyqxmd.com/read/28884982/comparison-of-somatic-and-sudomotor-nerve-fibers-in-type-2-diabetes-mellitus
#13
Eun Hee Sohn, Kyu Sang Song, Ju Yeon Lee, Ae Young Lee
BACKGROUND AND PURPOSE: The objective of this study was to find a sensitive method for the early detection of diabetic polyneuropathy (DPN) and determine the relationship between the functions of somatic and autonomic small nerve fibers in DPN. METHODS: Patients with type 2 diabetes mellitus and DPN based on clinical symptoms, signs, intraepidermal nerve fiber density (IENFD), and findings in the quantitative sudomotor axon reflex test (QSART) were enrolled retrospectively...
September 4, 2017: Journal of Clinical Neurology
https://www.readbyqxmd.com/read/28879885/anti-ganglioside-antibodies-profile-in-guillain-barr%C3%A3-syndrome-correlation-with-clinical-features-electrophysiological-pattern-and-outcome
#14
G Sivaram Naik, A Kanikannan Meena, B Ashok Kumar Reddy, Rukmini K Mridula, Shaik A Jabeen, Rupam Borgohain
BACKGROUND: Guillain-Barré syndrome (GBS) and its subtypes are associated with distinct anti-ganglioside antibodies. Hence, we aimed to determine the frequency of anti-ganglioside antibodies and its correlation with clinical features, electrophysiological patterns, and outcome in patients with GBS. MATERIAL AND METHODS: The data regarding clinical features, electrophysiological patterns, and outcome at 6 months were collected and analyzed from the case records of patients diagnosed with GBS during 2008-2013 at a tertiary care hospital in south India...
September 2017: Neurology India
https://www.readbyqxmd.com/read/28873125/neurological-complications-associated-with-anti-programmed-death-1-pd-1-antibodies
#15
Justin C Kao, Bing Liao, Svetomir N Markovic, Christopher J Klein, Elie Naddaf, Nathan P Staff, Teerin Liewluck, Julie E Hammack, Paola Sandroni, Heidi Finnes, Michelle L Mauermann
Importance: Neurological complications are an increasingly recognized consequence of the use of anti-programmed death 1 (PD-1) antibodies in the treatment of solid-organ tumors, with an estimated frequency of 4.2%. To date, the clinical spectrum and optimum treatment approach are not established. Objective: To investigate the frequency, clinical spectrum, and optimum treatment approach to neurological complications associated with anti-PD-1 therapy. Design, Setting, and Participants: This single-center, retrospective cohort study was conducted from either September or December 2014 (the approval dates of the study drugs by the US Food and Drug Administration) to May 19, 2016...
September 5, 2017: JAMA Neurology
https://www.readbyqxmd.com/read/28872488/acute-motor-and-sensory-axonal-neuropathy-related-to-treatment-with-mek-inhibitors-in-a-patient-with-advanced-melanoma
#16
Tarek Taha, Tzahala Tzuk-Shina, Itay Forschner, Gil Bar-Sela
Approximately one-half of advanced cutaneous melanomas have a V600 mutation in the BRAF gene that activates the mitogen-activated protein kinase pathway. The combination of BRAF plus MEK inhibitors is one of the most effective treatments for these patients. Severe neurological toxicities have been reported in the literature. However, these toxicities are very rare. Here, we present one patient with acute motor and sensory axonal neuropathy, which is a subtype of Guillain-Barre syndrome, secondary to treatment with MEK inhibitors...
September 1, 2017: Melanoma Research
https://www.readbyqxmd.com/read/28866056/mitochondrial-dynamics-transport-and-quality-control-a-bottleneck-for-retinal-ganglion-cell-viability-in-optic-neuropathies
#17
Yoko A Ito, Adriana Di Polo
Retinal ganglion cells, the neurons that selectively die in glaucoma and other optic neuropathies, are endowed with an exceedingly active metabolism and display a particular vulnerability to mitochondrial dysfunction. Mitochondria are exquisitely dynamic organelles that are continually responding to endogenous and environmental cues to readily meet the energy demand of neuronal networks. The highly orchestrated regulation of mitochondrial biogenesis, fusion, fission, transport and degradation is paramount for the maintenance of energy-expensive synapses at RGC dendrites and axon terminals geared for optimal neurotransmission...
September 1, 2017: Mitochondrion
https://www.readbyqxmd.com/read/28862523/l-and-t-type-calcium-channel-blockers-protect-against-the-inhibitory-effects-of-mipafox-on-neurite-outgrowth-and-plasticity-related-proteins-in-sh-sy5y-cells
#18
Laís Silva Fernandes, Neife Aparecida G Dos Santos, Guilherme Luz Emerick, Antonio Cardozo Dos Santos
Some organophosphorus compounds (OP), including the pesticide mipafox, produce late onset distal axonal degeneration, known as organophosphorus-induced delayed neuropathy (OPIDN). The underlying mechanism involves irreversible inhibition of neuropathy target esterase (NTE) activity, elevated intracellular calcium levels, increased activity of calcium-activated proteases and impaired neuritogenesis. Voltage-gated calcium channels (VGCC) appear to play a role in several neurologic disorders, including OPIDN. Therefore, this study aimed to examine and compare the neuroprotective effects of T-type (amiloride) and L-type (nimodipine) VGCC blockers induced by the inhibitory actions of mipafox on neurite outgrowth and axonal proteins of retinoic-acid-stimulated SH-SY5Y human neuroblastoma cells, a neuronal model widely employed to determine the neurotoxicity attributed to OP...
September 1, 2017: Journal of Toxicology and Environmental Health. Part A
https://www.readbyqxmd.com/read/28855885/laser-doppler-assessment-of-vasomotor-axon-reflex-responsiveness-to-evaluate-neurovascular-function
#19
REVIEW
Marie Luise Kubasch, Anne Sophie Kubasch, Juliana Torres Pacheco, Sylvia J Buchmann, Ben Min-Woo Illigens, Kristian Barlinn, Timo Siepmann
The vasomotor axon reflex can be evoked in peripheral epidermal nociceptive C-fibers to induce local vasodilation. This neurogenic flare response is a measure of C-fiber functional integrity and therefore shows impairment in patients with small fiber neuropathy. Laser Doppler flowmetry (LDF) and laser Doppler imaging (LDI) are both techniques to analyze vasomotor small fiber function by quantifying the integrity of the vasomotor-mediated axon reflex. While LDF assesses the flare response following acetylcholine iontophoresis with temporal resolution at a single defined skin point, LDI records flare responses with spatial and temporal resolution, generating a two-dimensional map of superficial blood flow...
2017: Frontiers in Neurology
https://www.readbyqxmd.com/read/28855494/charcot-marie-tooth-disease-type-2-caused-by-homozygous-mme-gene-mutation-superimposed-by-chronic-inflammatory-demyelinating-polyneuropathy
#20
Miwako Fujisawa, Yasuteru Sano, Masatoshi Omoto, Jyun-Ichi Ogasawara, Michiaki Koga, Hiroshi Takashima, Takashi Kanda
We report a 59-year-old Japanese male who developed gradually worsening weakness and numbness of distal four extremities since age 50. His parents were first cousins, and blood and cerebral spinal examinations were unremarkable. Homozygous mutation of MME gene was detected and thus he was diagnosed as autosomal-recessive Charcot-Marie-Tooth disease 2T (AR-CMT2T); however, electrophysiological examinations revealed scattered demyelinative changes including elongated terminal latency in several peripheral nerve trunks...
August 31, 2017: Rinshō Shinkeigaku, Clinical Neurology
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