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Motor Neuropathy

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https://www.readbyqxmd.com/read/28442382/relationship-between-sensorimotor-peripheral-nerve-function-and-indicators-of-cardiovascular-autonomic-function-in-older-adults-from-the-health-aging-and-body-composition-study
#1
Brittney S Lange-Maia, Anne B Newman, John M Jakicic, Jane A Cauley, Robert M Boudreau, Ann V Schwartz, Eleanor M Simonsick, Suzanne Satterfield, Aaron I Vinik, Sasa Zivkovic, Tamara B Harris, Elsa S Strotmeyer
BACKGROUND: Age-related peripheral nervous system (PNS) impairments are highly prevalent in older adults. Although sensorimotor and cardiovascular autonomic function have been shown to be related in persons with diabetes, the nature of the relationship in general community-dwelling older adult populations is unknown. METHODS: Health, Aging and Body Composition participants (n=2399, age=76.5±2.9years, 52% women, 38% black) underwent peripheral nerve testing at the 2000/01 clinic visit...
April 22, 2017: Experimental Gerontology
https://www.readbyqxmd.com/read/28440863/fewdon-mnd-syndrome-finger-extension-weakness-and-downbeat-nystagmus-a-novel-motor-neuron-disorder
#2
Aline Delva, Nimish Thakore, Erik Pioro, Koen Poesen, Rachel Saunders-Pullman, Inge A Meijer, Janet C Rucker, John T Kissel, Philip Van Damme
Introduction Disturbances of eye movements are infrequently encountered in motor neuron diseases or motor neuropathies, and there is no known syndrome that combines progressive muscle weakness with downbeat nystagmus. Methods To describe the core clinical features of a syndrome of motor neuron disease associated with downbeat nystagmus, clinical features were collected from 6 patients. Results All patients had slowly progressive muscle weakness and wasting, in combination with downbeat nystagmus, which was clinically most obvious in downward and lateral gaze...
April 25, 2017: Muscle & Nerve
https://www.readbyqxmd.com/read/28434966/diagnostic-biopsy-of-a-motor-branch-of-the-superficial-peroneal-nerve-to-the-peroneus-longus-a-convenient-alternative-for-motor-nerve-biopsy
#3
Randy S D'Amico, Christopher J Winfree
INTRODUCTION: Motor nerve biopsy is performed to supplement clinical, serological, and imaging data in the work-up of neuropathies of unknown origin, especially when motor neuron disease is suspected. METHODS: We describe a surgical technique for biopsy of a motor branch of the superficial peroneal nerve innervating the peroneus longus muscle. RESULTS: Three patients presented with weakness concerning for motor neuropathy and underwent biopsy of a motor branch of the superficial peroneal nerve innervating the peroneus longus muscle...
April 18, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28434507/motor-neuropathies-and-lower-motor-neuron-syndromes
#4
REVIEW
A Verschueren
Motor or motor-predominant neuropathies may arise from disease processes affecting the motor axon and/or its surrounding myelin. Lower motor neuron syndrome (LMNS) arises from a disease process affecting the spinal motor neuron itself. The term LMNS is more generally used, rather than motor neuronopathy, although both entities are clinically similar. Common features are muscle weakness (distal or proximal) with atrophy and hyporeflexia, but no sensory involvement. They can be acquired or hereditary. Immune-mediated neuropathies (multifocal motor neuropathy, motor-predominant chronic inflammatory demyelinating polyneuropathy) are important to identify, as effective treatments are available...
April 20, 2017: Revue Neurologique
https://www.readbyqxmd.com/read/28429469/high-body-mass-and-kidney-dysfunction-relate-to-worse-nerve-function-even-in-adults-without-neuropathy
#5
Rens Hanewinckel, M Arfan Ikram, Oscar H Franco, Albert Hofman, Judith Drenthen, Pieter A van Doorn
Polyneuropathy is a prevalent and disabling disorder. Despite extensive evaluation, the cause often remains unknown. Factors that predispose for the development of polyneuropathy need to be identified. We investigated the effect of anthropometric and metabolic factors on peripheral nerve function in 908 participants of the population-based Rotterdam Study without any symptoms or signs of polyneuropathy. Participants underwent nerve conduction studies of the sural and peroneal nerve. Data on age, height, weight, waist circumference, diabetes, lipid levels, hypertension and kidney function were collected...
April 20, 2017: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/28407266/hereditary-neuropathy-with-liability-to-pressure-palsy-in-patients-under-thirty-neurophysiological-data-and-proposed-electrodiagnostic-criteria
#6
Florence Robert-Varvat, Guillemette Jousserand, Françoise Bouhour, Christophe Vial, Pascal Cintas, Andoni Echaniz-Laguna, Emilien Delmont, Pierre Clavelou, Guy Chauplannaz, Laurent Jomir, Yann Péreon, Sarah Léonard-Louis, Véronique Manel, Jean-Christophe Antoine, Arnaud Lacour, Jean-Philippe Camdessanché
INTRODUCTION: In young patients with mononeuropathy who lack family history and precipitating factors, hereditary neuropathy with liability to pressure palsy (HNPP) may be a possibility. Our objective is to propose neurophysiological criteria for HNPP in patients aged <30 years. METHODS: We conducted a national multicenter retrospective clinical and neurophysiological study in patients under 30 with genetically confirmed HNPP. RESULTS: All of the 51 included patients had at least 1 demyelinating pattern in 2 asymptomatic nerves, and 3 abnormalities were found in almost 90%, including slowed motor nerve conduction velocity across the elbow in at least 1 ulnar nerve (97...
April 13, 2017: Muscle & Nerve
https://www.readbyqxmd.com/read/28400666/limited-incision-carpal-tunnel-release
#7
Sunil Gaba, Sandeep Bhogesha, Onkar Singh
BACKGROUND: Carpal tunnel syndrome (CTS) is the most common peripheral entrapment neuropathy. Limited incision techniques for carpal tunnel release are gaining popularity. The main advantages of these techniques are less scar load, less pillar pain, shorter recovery, and return-to-work time. However, the completeness of release, and risk of neurovascular injury are always a concern. We devised a method of limited incision release with two mini-incisions and use of nasal speculum and a probe...
March 2017: Indian Journal of Orthopaedics
https://www.readbyqxmd.com/read/28399651/facial-pain-overlapping-syndromes
#8
Stefan Evers
Premise This review summarises the pain syndromes that overlap between headache and facial pain and overlap between pain and cranial nerve lesion. Problem These syndromes share two features in common. First, they show both cranial nerve impairment (e.g. palsy, autonomic dysfunction) and pain; second, they have inflammatory (and/or small vessel) processes as the underlying mechanism. A typical representative of these syndromes is recurrent painful ophthalmoplegic neuropathy, which was previously called ophthalmoplegic migraine and was regarded as a migraine subtype...
January 1, 2017: Cephalalgia: An International Journal of Headache
https://www.readbyqxmd.com/read/28399101/-clinical-and-epidemiological-characteristics-of-hereditary-motor-sensory-neuropathy-1x-caused-by-the-mutation-c-259c-g-p-p87a-in-the-gjb1-gene-of-patients-from-the-republic-of-bashkortostan
#9
E V Saifullina, R V Magzhanov, I M Khidiyatova, E K Khusnutdinova
BACKGROUND: Hereditary motor-sensory neuropathy 1X (НМСН 1X) is the second frequent form of hereditary motor-sensory neuropathies caused by mutations in the GJB1 gene (gap junction B1 type). The authors have established earlier that the с.259C>G (р.P87A) mutation is the most frequent cause of НМСН 1Х (92%) in patients from the Republic of Bashkortostan. AIM: To study in details the territorial ethnic distribution and clinical manifestations of the с...
2017: Zhurnal Nevrologii i Psikhiatrii Imeni S.S. Korsakova
https://www.readbyqxmd.com/read/28397393/early-discrimination-of-sensorimotor-guillain-barr%C3%A3-syndrome-into-demyelinating-or-axonal-subtype-by-automated-nerve-excitability-testing
#10
So Young Pyun, Mi-Ri Kang, Joo Young Lee, Kim Jong Kuk, Seong-Il Oh, Jong Seok Bae
In the early stage of disease, differentiating acute inflammatory demyelinating polyneuropathy (AIDP) and acute motor sensory axonal neuropathy (AMSAN) using only a conventional nerve conduction studies (NCS) may be difficult. We evaluated the differences in the motor axonal excitability properties of 16 cases of sensorimotor Guillain-Barré syndrome by nerve excitability testing (NET). The antiganglioside antibody assay and follow-up NCS resulted in 12 patients diagnosed as AIDP and 4 patients as AMSAN. Clinical and excitability parameters in each group were compared with those in 30 normal controls...
April 11, 2017: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/28397326/diagnosis-and-management-of-neuropathies-associated-with-plasma-cell-dyscrasias
#11
REVIEW
Evan Rosenbaum, Douglas Marks, Shahzad Raza
Neuropathies associated with plasma cell dyscrasias are a major cause of morbidity for patients managed by medical oncologists. Because of similarities in clinical presentation and on nerve conduction studies, identifying the underlying disease leading to a paraproteinemic neuropathy can often be difficult. In addition, the degree of neurologic deficit does not strictly correlate with the extent of abnormalities on common clinical laboratory testing. Fortunately, with increasing understanding into the biologic mechanisms of underlying hematologic diseases, additional biomarkers have recently been developed, thus improving our diagnostic capacity...
April 10, 2017: Hematological Oncology
https://www.readbyqxmd.com/read/28393703/mir-106a-is-associated-with-diabetic-peripheral-neuropathy-through-the-regulation-of-12-15-lox-mediated-oxidative-nitrative-stress
#12
Yuanbo Wu, Dandan Xu, Xiang Zhu, Guangwei Yang, Mingshan Ren
BACKGROUND: Diabetic peripheral neuropathy (DPN) is a common complication of diabetes that with a complex pathogenesis. Study has reported that oxidative stress and nitrative stress were all involved in the DPN, however, the mechanism between them was still unclear. In the present study, we investigated whether miR-106a signaling regulated 12/15-Lipoxygenase (12/15-LOX) on oxidative/nitrative stress in DPN. METHODS: Dorsal root ganglion (DRG) was isolated from 10 healthy mice and 10 DPN mice...
April 4, 2017: Current Neurovascular Research
https://www.readbyqxmd.com/read/28389474/perineurial-glial-plasticity-and-the-role-of-tgf-%C3%AE-in-the-development-of-the-blood-nerve-barrier
#13
Angela D Morris, Gwendolyn M Lewis, Sarah Kucenas
Precisely orchestrated interactions between spinal motor axons and their ensheathing glia are vital for forming and maintaining functional spinal motor nerves. Following perturbations to peripheral myelinating glial cells, centrally-derived oligodendrocyte progenitor cells (OPCs) ectopically exit the spinal cord and myelinate peripheral nerves in myelin with central nervous system (CNS) characteristics. However, whether remaining peripheral ensheathing glia, such as perineurial glia, properly encase the motor nerve despite this change in glial cell and myelin composition, remains unknown...
April 7, 2017: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/28382108/two-years-long-term-follow-up-in-chronic-inflammatory-demyelinating-polyradiculoneuropathy-efficacy-of-intravenous-immunoglobulin-treatment
#14
Gisa Ellrichmann, Ralf Gold, Ilya Ayzenberg, Min-Suk Yoon, Christiane Schneider-Gold
BACKGROUND: Administration of intravenous immunoglobulins (IVIgs) is established for long-term treatment of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Prevention of secondary axonal loss going along with permanent clinical disability and muscular atrophy is a major aim in CIDP therapy. To assess long-term clinical efficacy of IVIg treatment despite heterogenous disease course and variable complaints reported by the patients, long-term electrophysiological monitoring was performed for systematic evaluation of therapeutic efficacy of IVIg...
February 2017: Therapeutic Advances in Neurological Disorders
https://www.readbyqxmd.com/read/28376643/effect-of-tempol-on-peripheral-neuropathy-in-diet-induced-obese-and-high-fat-fed-low-dose-streptozotocin-treated-c57bl6-j-mice
#15
Alexander Obrosov, Hanna Shevalye, Lawrence J Coppey, Mark A Yorek
In this study, we sought to determine the efficacy of tempol on multiple neuropathic endpoints in a diet-induced obese mouse, a model of pre-diabetes, and a high-fat fed low-dose streptozotocin treated mouse, a model of type 2 diabetes. Tempol (4-hydroxy-2,2,6,6-tetramethylpiperdine -1-oxyl) is a low molecular weight, water soluble, membrane permeable, and metal-independent superoxide dismutase mimetic that has been widely used in cellular studies for the removal of intracellular and extracellular superoxide...
April 19, 2017: Free Radical Research
https://www.readbyqxmd.com/read/28375940/electrophysiological-studies-to-detect-peripheral-neuropathy-in-children-treated-with-vincristine
#16
Marko Kavcic, Blaz Koritnik, Matevz Krzan, Orjana Velikonja, Tomaz Prelog, Milica Stefanovic, Maruša Debeljak, Janez Jazbec
Patients treated with vincristine predictably develop peripheral neuropathy. The aim of our study was to investigate the pattern of vincristine-induced neuropathy in children by nerve conduction studies and somatosensory-evoked potentials (SSEPs). We included data from 39 children who received vincristine for various pediatric malignancies, and we performed initial and follow-up (after a minimum of 4 doses of vincristine 1.5 mg/m) conduction studies in 27 patients and SSEPs studies in 34 patients. On follow-up the most prevalent symptoms were paresthesias (44%) and constipation (22%), and the most common neurological sign was impaired myotatic reflexes (89%)...
May 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28374019/cntnap1-mutations-cause-cns-hypomyelination-and-neuropathy-with-or-without-arthrogryposis
#17
Holger Hengel, Alex Magee, Muhammad Mahanjah, Jean-Michel Vallat, Robert Ouvrier, Mohammad Abu-Rashid, Jamal Mahamid, Rebecca Schüle, Martin Schulze, Ingeborg Krägeloh-Mann, Peter Bauer, Stephan Züchner, Rajech Sharkia, Ludger Schöls
OBJECTIVE: To explore the phenotypic spectrum and pathophysiology of human disease deriving from mutations in the CNTNAP1 gene. METHODS: In a field study on consanguineous Palestinian families, we identified 3 patients carrying homozygous mutations in the CNTNAP1 gene using whole-exome sequencing. An unrelated Irish family was detected by screening the GENESIS database for further CNTNAP1 mutations. Neurophysiology, MRI, and nerve biopsy including electron microscopy were performed for deep phenotyping...
April 2017: Neurology. Genetics
https://www.readbyqxmd.com/read/28370538/shoulder-position-sense-in-volleyball-players-with-infraspinatus-atrophy-secondary-to-suprascapular-nerve-neuropathy
#18
Samuele Contemori, Andrea Biscarini
Isolated infraspinatus atrophy (IIA) is a common condition among overhead-activity athletes, which affects the hitting shoulder and is caused by suprascapular nerve injury. Since the suprascapular nerve is a mixed nerve, such damage could lead to reduced afferent proprioceptive information and impaired shoulder sensorimotor control. This study aims to evaluate the proprioception of the shoulder with IIA, through the assessment of shoulder position sense. The shoulder position sense was assessed in 24 professional volleyball players (12 players with IIA, and 12 healthy players) with a blind dynamic shoulder repositioning test (all participants were blindfolded during the test)...
March 29, 2017: Scandinavian Journal of Medicine & Science in Sports
https://www.readbyqxmd.com/read/28369282/recessive-dystrophic-epidermolysis-bullosa-results-in-painful-small-fibre-neuropathy
#19
Sofia von Bischhoffshausen, Dinka Ivulic, Paola Alvarez, Victor C Schuffeneger, Juan Idiaquez, Constanza Fuentes, Pilar Morande, Ignacia Fuentes, Francis Palisson, David L H Bennett, Margarita Calvo
Small fibres in the skin are vulnerable to damage in metabolic or toxic conditions such as diabetes mellitus or chemotherapy resulting in small fibre neuropathy and associated neuropathic pain. Whether injury to the most distal portion of sensory small fibres due to a primary dermatological disorder can cause neuropathic pain is still unclear. Recessive dystrophic epidermolysis bullosa (RDEB) is a rare condition in which mutations of proteins of the dermo-epidermal junction lead to cycles of blistering followed by regeneration of the skin...
March 28, 2017: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/28369220/a-recurrent-wars-mutation-is-a-novel-cause-of-autosomal-dominant-distal-hereditary-motor-neuropathy
#20
Pei-Chien Tsai, Bing-Wen Soong, Inès Mademan, Yen-Hua Huang, Chia-Rung Liu, Cheng-Tsung Hsiao, Hung-Ta Wu, Tze-Tze Liu, Yo-Tsen Liu, Yen-Ting Tseng, Kon-Ping Lin, Ueng-Cheng Yang, Ki Wha Chung, Byung-Ok Choi, Garth A Nicholson, Marina L Kennerson, Chih-Chiang Chan, Peter De Jonghe, Tzu-Hao Cheng, Yi-Chu Liao, Stephan Züchner, Jonathan Baets, Yi-Chung Lee
Distal hereditary motor neuropathy is a heterogeneous group of inherited neuropathies characterized by distal limb muscle weakness and atrophy. Although at least 15 genes have been implicated in distal hereditary motor neuropathy, the genetic causes remain elusive in many families. To identify an additional causal gene for distal hereditary motor neuropathy, we performed exome sequencing for two affected individuals and two unaffected members in a Taiwanese family with an autosomal dominant distal hereditary motor neuropathy in which mutations in common distal hereditary motor neuropathy-implicated genes had been excluded...
March 22, 2017: Brain: a Journal of Neurology
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