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Proximal neuropathy

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https://www.readbyqxmd.com/read/28447661/the-nonsystemic-vasculitic-neuropathies
#1
REVIEW
Michael P Collins, Robert D Hadden
Nonsystemic vasculitic neuropathy (NSVN) is an under-recognized single-organ vasculitis of peripheral nerves that can only be diagnosed with a nerve biopsy. A Peripheral Nerve Society guideline group published consensus recommendations on the classification, diagnosis and treatment of NSVN in 2010, and new diagnostic criteria for vasculitic neuropathy were developed by the Brighton Collaboration in 2015. In this Review, we provide an update on the classification, diagnosis and treatment of NSVN. NSVN subtypes include Wartenberg migratory sensory neuropathy and postsurgical inflammatory neuropathy...
April 27, 2017: Nature Reviews. Neurology
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/28434507/motor-neuropathies-and-lower-motor-neuron-syndromes
#3
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/28432188/magnetic-resonance-neurography-visualizes-abnormalities-in-sciatic-and-tibial-nerves-in-patients-with-type-1-diabetes-and-neuropathy
#4
Michael Vaeggemose, Mirko Pham, Steffen Ringgaard, Hatice Tankisi, Niels Ejskjaer, Sabine Heiland, Per L Poulsen, Henning Andersen
This study evaluates whether diffusion-tensor-imaging MR-Neurography (DTI-MRN), T2-relaxation-time and proton-spin-density can detect and grade neuropathic abnormalities in patients with type 1 diabetes.Forty-nine patients with type 1 diabetes (11 with severe polyneuropathy (sDPN), 13 with mild polyneuropathy (mDPN) and 25 without polyneuropathy (nDPN)) and 30 healthy controls (HC) were included. Clinical examinations, nerve-conduction-studies and vibratory-perception-thresholds determined the presence and severity of DPN...
April 21, 2017: Diabetes
https://www.readbyqxmd.com/read/28428919/tibial-bone-grafting-for-wrist-reconstruction
#5
Schneider K Rancy, Stephanie D Malliaris, Scott W Wolfe
Background Iliac crest bone graft harvesting is routinely performed for upper extremity orthopedic procedures despite high complication rates associated with sensory nerve injury, hematoma, chronic pain, and fracture. Description of Technique Cancellous bone graft is harvested from the proximal tibia using a medial approach, minimizing donor site morbidity. Patients and Methods A retrospective review was undertaken of 14 patients who underwent cancellous proximal tibia bone graft harvesting to augment healing of wrist reconstruction, with 2 months minimum follow-up...
May 2017: Journal of Wrist Surgery
https://www.readbyqxmd.com/read/28397860/whole-exome-sequencing-of-congenital-glaucoma-patients-reveals-hypermorphic-variants-in-gpatch3-a-new-gene-involved-in-ocular-and-craniofacial-development
#6
Jesús-José Ferre-Fernández, José-Daniel Aroca-Aguilar, Cristina Medina-Trillo, Juan-Manuel Bonet-Fernández, Carmen-Dora Méndez-Hernández, Laura Morales-Fernández, Marta Corton, María-José Cabañero-Valera, Marta Gut, Raul Tonda, Carmen Ayuso, Miguel Coca-Prados, Julián García-Feijoo, Julio Escribano
Congenital glaucoma (CG) is a heterogeneous, inherited and severe optical neuropathy that originates from maldevelopment of the anterior segment of the eye. To identify new disease genes, we performed whole-exome sequencing of 26 unrelated CG patients. In one patient we identified two rare, recessive and hypermorphic coding variants in GPATCH3, a gene of unidentified function, and 5% of a second group of 170 unrelated CG patients carried rare variants in this gene. The recombinant GPATCH3 protein activated in vitro the proximal promoter of CXCR4, a gene involved in embryo neural crest cell migration...
April 11, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28389774/takayasu-arteritis-presenting-as-isolated-anterior-ischemic-optic-neuropathy
#7
Guohong Tian, Qian Chen, Wenji Wang
Takayasu arteritis (TA) is a systemic vasculitis of unknown etiology that affects the aorta and its primary branches or large arteries in the proximal upper or lower extremities. Ocular manifestations of TA include microaneurysm formation, small-vessel dilation, arteriovenous anastomosis, retinal ischemia, and neovascular glaucoma. We herein report a case involving a 23-year-old Asian woman who presented with isolated acute anterior ischemic optic neuropathy and was initially misdiagnosed with optic neuritis...
April 7, 2017: International Ophthalmology
https://www.readbyqxmd.com/read/28389398/-numb-leg-in-a-crossfit-athlete-a-case-presentation
#8
Stephan Esser, Mckennan Thurston, Krishna Nalluri, Aurelio Muzaurieta
Participation in CrossFit athletics and Olympic-style lifting by the general populace has rapidly increased in the last 10 years. Such athletic engagement poses unique, inadequately defined risks to the participant. We describe the case of a 36-year-old man who presented to an outpatient sports medicine clinic with 6 weeks of numbness and tingling in the lateral right proximal thigh. After thorough examination and electromyographic testing, he was found to have a lateral femoral cutaneous neuropathy caused by performing supine gluteal bridges with a weighted barbell resting across his anterior thighs...
April 5, 2017: PM & R: the Journal of Injury, Function, and Rehabilitation
https://www.readbyqxmd.com/read/28388973/why-do-inguinal-hernia-patients-have-pain-histology-points-to-compression-neuropathy
#9
Robert Wright, Donald E Born, Natasha D'Souza, Larissa Hurd, Rachel Gill, Daniel Wright
PURPOSE: The purpose of this study is to describe the known soft tissue neuro-histology factors associated with compression neuropathy in relation to the incidence of preoperative pain in primary inguinal hernia. Enlargement of the ilioinguinal nerve occurs in 63% of patients with primary inguinal hernia; compression neuropathy has similar gross features. METHODS: Patients completed pain questionnaires pertaining to preoperative pain and the quality of pain experienced...
March 22, 2017: American Journal of Surgery
https://www.readbyqxmd.com/read/28364294/intermediate-charcot-marie-tooth-disease-an-electrophysiological-reappraisal-and-systematic-review
#10
REVIEW
José Berciano, Antonio García, Elena Gallardo, Kristien Peeters, Ana L Pelayo-Negro, Silvia Álvarez-Paradelo, José Gazulla, Miriam Martínez-Tames, Jon Infante, Albena Jordanova
Charcot-Marie-Tooth disease (CMT) is the most frequent form of inherited neuropathy with great variety of phenotypes, inheritance patterns, and causative genes. According to median motor nerve conduction velocity (MNCV), CMT is divided into demyelinating (CMT1) with MNCV below 38 m/s, axonal (CMT2) with MNCV above 38 m/s, and intermediate CMT with MNCV between 25 and 45 m/s. In each category, transmission may be autosomal dominant, autosomal recessive, or X-linked. The nosology of intermediate CMT is controversial because of concerns about electrophysiological delimitation...
March 31, 2017: Journal of Neurology
https://www.readbyqxmd.com/read/28345147/nerve-conduction-velocity-and-cross-sectional-area-in-ulnar-neuropathy-at-the-elbow
#11
Simon Podnar, Gregor Omejec, Marko Bodor
INTRODUCTION: In the precise localization of ulnar neuropathy at the elbow (UNE) we have noted discrepancies between electrodiagnostic (EDx) and ultrasonographic (US) findings. We aimed to explore the relationship between the 2 methods. METHODS: Four study-blind examiners took a history and performed neurologic, EDx, and US examinations of a group of prospectively recruited patients with UNE. They assessed the relationship between ulnar nerve cross-sectional area (CSA) and motor nerve conduction velocity (MNCV)...
March 27, 2017: Muscle & Nerve
https://www.readbyqxmd.com/read/28341088/the-latest-treatment-strategy-for-the-rheumatoid-hand-deformity
#12
Hajime Ishikawa
With a remarkable improvement in the pharmacotherapy of rheumatoid arthritis (RA), severely handicapped patients are very rare to see. Healing, repair and drug-free, and toward radical cure are coming to be possible. In the clinical practice, more than 50% of the patients are in remission. However, some patients are still difficult to reach remission due to comorbidities and economic burden. In the patient with clinical remission, smoldering synovitis so called "silent destructor" is often detected by ultrasonograpy or by synovial histology in the small joints of the hand...
March 22, 2017: Journal of Orthopaedic Science: Official Journal of the Japanese Orthopaedic Association
https://www.readbyqxmd.com/read/28328138/homozygous-mutation-in-ptrh2-gene-causes-progressive-sensorineural-deafness-and-peripheral-neuropathy
#13
Rajech Sharkia, Stavit A Shalev, Abdelnaser Zalan, Milit Marom-David, Nathan Watemberg, Jill E Urquhart, Sarah B Daly, Sanjeev S Bhaskar, Simon G Williams, William G Newman, Ronen Spiegel, Abdussalam Azem, Orly Elpeleg, Muhammad Mahajnah
PTRH2 is an evolutionarily highly conserved mitochondrial protein that belongs to a family of peptidyl-tRNA hydrolases. Recently, patients from two consanguineous families with mutations in the PTRH2 gene were reported. Global developmental delay associated with microcephaly, growth retardation, progressive ataxia, distal muscle weakness with ankle contractures, demyelinating sensorimotor neuropathy, and sensorineural hearing loss were present in all patients, while facial dysmorphism with widely spaced eyes, exotropia, thin upper lip, proximally placed thumbs, and deformities of the fingers and toes were present in some individuals...
April 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28319875/electrophysiologic-features-of-ulnar-neuropathy-in-childhood-and-adolescence
#14
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%...
May 2017: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
https://www.readbyqxmd.com/read/28303912/new-technologies-for-the-assessment-of-neuropathies
#15
REVIEW
Roberto Gasparotti, Luca Padua, Chiara Briani, Giuseppe Lauria
Technical advances are rapidly changing the clinical and instrumental approach to peripheral nerve diseases. Magnetic resonance neurography, diffusion tensor imaging and nerve ultrasonography are increasingly entering the diagnostic workup of peripheral neuropathies as tools that complement neurophysiology and enable investigation of proximal structures, such as plexuses and roots. Progress in the design of magnetic resonance scanners and sequences, and the development of high-frequency ultrasound probes mean that high-resolution peripheral nerve imaging is possible, enabling detailed examination of nerve size, morphology and internal fascicular structure that can integrate nerve conduction studies into clinical practice...
March 17, 2017: Nature Reviews. Neurology
https://www.readbyqxmd.com/read/28252606/-diabetic-asymmetric-proximal-neuropathy
#16
S V Kotov, I G Rudakova, E V Isakova, Yu A Belova, T V Volchenkova, M Khirbek, G A Stashuk
AIM: To study the prevalence, clinical features and treatment of diabetic asymmetric proximal neuropathy (DAPN). MATERIAL AND METHODS: Four hundred and forty-five patients with diabetes mellitus (DM), 257 women and 188 men, mean age 47.6±0.5 years, including 163 patients with DM type I and 282 with DM type II, were examined. RESULTS AND CONCLUSION: Distal symmetric sensory motor polyneuropathy was found in 62% of the patients, autonomic neuropathy in 25...
2017: Zhurnal Nevrologii i Psikhiatrii Imeni S.S. Korsakova
https://www.readbyqxmd.com/read/28226286/peripheral-nerve-ultrasonography-in-patients-with-transthyretin-amyloidosis
#17
Simon Podnar, Stayko Sarafov, Ivailo Tournev, Gregor Omejec, Janez Zidar
OBJECTIVE: To systematically study peripheral nerve morphology in patients with transthyretin (TTR) amyloidosis and TTR gene mutation carriers using high-resolution ultrasonography (US). METHODS: In this prospective cross-sectional study we took a structured history, performed neurological examination, and measured peripheral nerve cross-sectional areas (CSAs) bilaterally at 28 standard locations using US. Demographic and US findings were compared to controls. RESULTS: Peripheral nerve CSAs were significantly larger in 33 patients with familial amyloid polyneuropathy (FAP) compared to 50 controls, most dramatically at the common entrapment sites (median nerve at the wrist, ulnar nerve at the elbow), and in the proximal nerve segments (median nerve in the upper arm, sciatic nerve in the thigh)...
April 2017: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
https://www.readbyqxmd.com/read/28214378/calcium-signaling-through-l-type-calcium-channels-role-in-pathophysiology-of-spinal-nociceptive-transmission
#18
REVIEW
Olivier Roca-Lapirot, Houda Radwani, Franck Aby, Frédéric Nagy, Marc Landry Pascal Fossat
L-type voltage-gated calcium channels (VGCCs) are ubiquitous channels in the central nervous system. L-type calcium channels (LTCs) are mostly post-synaptic channels regulating neuronal firing and gene expression. They play a role in important physio-pathological processes such as learning and memory, Parkinson's disease, autism and, as recognized more recently, in the pathophysiology of pain processes. Classically, the fundamental role of these channels in cardiovascular functions has limited the use of classical molecules to treat LTC-dependent disorders...
February 18, 2017: British Journal of Pharmacology
https://www.readbyqxmd.com/read/28213167/the-effect-of-peripheral-neuropathy-on-lower-limb-muscle-strength-in-diabetic-individuals
#19
Jean P Ferreira, Cristina D Sartor, Ângela M O Leal, Isabel C N Sacco, Tatiana O Sato, Ivana L Ribeiro, Alice S Soares, Jonathan E Cunha, Tania F Salvini
BACKGROUND: Skeletal muscle strength is poorly described and understood in diabetic participants with diabetic peripheral neuropathy. This study aimed to investigate the extensor and flexor torque of the knee and ankle during concentric, eccentric, and isometric contractions in men with diabetes mellitus type 2 with and without diabetic peripheral neuropathy. METHODS: Three groups of adult men (n=92), similar in age, body mass index, and testosterone levels, were analyzed: 33 non-diabetic controls, 31 with type 2 diabetes mellitus, and 28 with diabetic peripheral neuropathy...
February 9, 2017: Clinical Biomechanics
https://www.readbyqxmd.com/read/28198531/superficial-brachial-artery-a-possible-cause-for-idiopathic-median-nerve-entrapment-neuropathy
#20
Pilani Nkomozepi, Nkosi Xhakaza, Elaine Swanepoel
Nerve entrapment syndromes occur because of anatomic constraints at specific locations in both upper and lower limbs. Anatomical locations prone to nerve entrapment syndromes include sites where a nerve courses through fibro-osseous or fibromuscular tunnels or penetrates a muscle. The median nerve (MN) can be entrapped by the ligament of Struthers; thickened biceps aponeurosis; between the superficial and deep heads of the pronator teres muscle and by a thickened proximal edge of flexor digitorum superficialis muscle...
February 15, 2017: Folia Morphologica (Warsz)
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