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Proximal neuropathy

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https://www.readbyqxmd.com/read/28859662/chronic-pain-in-gaucher-disease-skeletal-or-neuropathic-origin
#1
Grazia Devigili, Michele De Filippo, Giovanni Ciana, Andrea Dardis, Christian Lettieri, Sara Rinaldo, Daniela Macor, Alessandro Moro, Roberto Eleopra, Bruno Bembi
BACKGOUND: Pain is one of the most disabling symptoms of Gaucher disease. It is referred by the majority of Gaucher patients and often persists despite long-term enzyme replacement treatment. It has been mainly considered as nociceptive pain secondary to skeletal involvement but it is described even in the absence of bone disease without a clear explanation. In the last years an increasing number of reports have described the presence of neurological manifestation in Gaucher type 1 patients, including subclinical large fibre neuropathy...
August 31, 2017: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/28856021/wrist-ultrasound-examination-scanning-technique-and-ultrasound-anatomy-part-2-ventral-wrist
#2
REVIEW
Cyprian Olchowy, Daniel Soliński, Mateusz Łasecki, Paweł Dąbrowski, Szymon Urban, Urszula Zaleska-Dorobisz
Ultrasound imaging of the musculoskeletal system is an important element of the diagnostic and therapeutic protocol. Clinical decisions, including those regarding surgical procedures, are often based solely on ultrasound imaging. However, detailed knowledge on the anatomy and a correct scanning technique are crucial for an accurate diagnosis. Modern ultrasonographic equipment allows obtaining detailed anatomical images of muscle tendons, ligaments, nerves and vessels of the carpal area. Ventral wrist ultrasound is one of the most common diagnostic procedures in patients with suspected carpal tunnel syndrome...
June 2017: Journal of Ultrasonography
https://www.readbyqxmd.com/read/28838115/endoscopic-endonasal-approach-to-the-ventral-jugular-foramen-anatomical-basis-technical-considerations-and-clinical-series
#3
Francisco Vaz-Guimaraes, Ana Carolina I Nakassa, Paul A Gardner, Eric W Wang, Carl H Snyderman, Juan C Fernandez-Miranda
BACKGROUND: Surgical exposure of the jugular foramen (JF) is challenging given its complex regional anatomy and proximity to critical neurovascular structures. OBJECTIVE: To describe the anatomical basis, surgical technique, and outcomes of a group of patients who underwent the endoscopic endonasal approach to the JF. METHODS: Five silicon-injected anatomical specimens were prepared for dissection. Additionally, a chart review was conducted through our patient database, searching for endonasal exposure of the JF...
August 1, 2017: Operative Neurosurgery (Hagerstown, Md.)
https://www.readbyqxmd.com/read/28835396/human-dorsal-root-ganglion-in-vivo-morphometry-and-perfusion-in-fabry-painful-neuropathy
#4
Tim Godel, Philipp Bäumer, Mirko Pham, Anja Köhn, Nicole Muschol, Moritz Kronlage, Jennifer Kollmer, Sabine Heiland, Martin Bendszus, Victor-Felix Mautner
OBJECTIVE: To evaluate functional and morphometric magnetic resonance neurography of the dorsal root ganglion and peripheral nerve segments in patients with Fabry painful neuropathy. METHODS: In this prospective study, the lumbosacral dorsal root ganglia and proximal peripheral nerve segments of the lower extremity were examined in 11 male patients with Fabry disease by a standardized 3T magnetic resonance neurography protocol. Volumes of L3 to S2 dorsal root ganglia, perfusion parameters of L5-S1 dorsal root ganglia and the spinal nerve L5, and the cross-sectional area of the proximal sciatic nerve were compared to healthy controls...
August 23, 2017: Neurology
https://www.readbyqxmd.com/read/28819736/pain-and-compression-neuropathy-in-primary-inguinal-hernia
#5
R Wright, D E Born, N D'Souza, L Hurd, R Gill, D Wright
PURPOSE: Enlargement of the ilioinguinal nerve at the external inguinal ring is observed in 34% of patients undergoing primary open inguinal herniorrhaphy; in 88% of patients it occurs at the fascial edge where the hernia mushrooms with abdominal pressure. Compression neuropathy occurs near many anatomical nerve constriction sites and is associated with enlargement of the peripheral nerve accompanied by sensory changes. METHODS: In this prospective study, Carolina Comfort Scale (CCS) questionnaire data was collected for 35 primary hernia repairs...
August 17, 2017: Hernia: the Journal of Hernias and Abdominal Wall Surgery
https://www.readbyqxmd.com/read/28815909/proximal-neuropathy-and-associated-skeletal-muscle-changes-resembling-denervation-atrophy-in-hind-limbs-of-chronic-hypoglycaemic-rats
#6
Vivi F H Jensen, Anne-Marie Molck, Henrik Soeborg, Jette Nowak, Melissa Chapman, Jens Lykkesfeldt, Ingrid B Bogh
Peripheral neuropathy is one of the most common complications of diabetic hyperglycaemia. Insulin-induced hypoglycaemia (IIH) might potentially exacerbate or contribute to neuropathy as hypoglycaemia also causes peripheral neuropathy. In rats, IIH induces neuropathy associated with skeletal muscle changes. Aims of the present study were to investigate the progression and sequence of histopathologic changes caused by chronic IIH in rat peripheral nerves and skeletal muscle, and whether such changes were reversible...
August 16, 2017: Basic & Clinical Pharmacology & Toxicology
https://www.readbyqxmd.com/read/28795009/evaluation-and-management-of-shoulder-pain-in-skeletally-immature-athletes
#7
REVIEW
Dilip R Patel, Stephen Breisach
Shoulder pain in young athletes generally is a result of poor sports technique and overuse. A number intrinsic causes of shoulder pain have been identified. Pain may also be referred to shoulder area from cervical spine, neck and chest pathology. Overuse stress injury of the proximal humeral physis is important to recognize early in order to prevent later complications. Shoulder impingement syndrome is a general term used to describe multiple underlying lesions and relatively uncommon in young athletes. In adolescent athletes, glenohumeral instability is an important underlying pathomechanical basis for shoulder pain...
July 2017: Translational pediatrics
https://www.readbyqxmd.com/read/28753118/delayed-compression-of-the-common-peroneal-nerve-following-rotational-lateral-gastrocnemius-flap-case-report
#8
Benjamin T Himes, Thomas J Wilson, Andres A Maldonado, Naveen S Murthy, Robert J Spinner
The authors present a case of delayed peroneal neuropathy following a lateral gastrocnemius rotational flap reconstruction. The patient presented 1.5 years after surgery with a new partial foot drop, which progressed over 3 years. At operation, a fascial band on the deep side of the gastrocnemius flap was compressing the common peroneal nerve proximal to the fibular head, correlating with preoperative imaging. Release of this fascial band and selective muscle resection led to immediate improvement in symptoms postoperatively...
July 28, 2017: Journal of Neurosurgery
https://www.readbyqxmd.com/read/28746752/immunotherapy-for-diabetic-amyotrophy
#9
REVIEW
Yee Cheun Chan, Yew Long Lo, Edwin Sy Chan
BACKGROUND: People with diabetes mellitus (DM) sometimes present with acute or subacute, progressive, asymmetrical pain and weakness of the proximal lower limb muscles. The various names for the condition include diabetic amyotrophy, diabetic lumbosacral radiculoplexus neuropathies, diabetic femoral neuropathy or Bruns-Garland syndrome. Some studies suggest that diabetic amyotrophy may be an immune-mediated inflammatory microvasculitis causing ischaemic damage of the nerves. Immunotherapies would therefore be expected to be beneficial...
July 26, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28720009/the-effect-of-isolated-finger-stiffness-on-adjacent-digit-function
#10
Rasha Baaqeel, Kitty Wu, Shrikant J Chinchalkar, Douglas C Ross
BACKGROUND: Isolated stiffness in a single finger can affect the function of adjacent digits and decrease overall hand function due to the quadriga phenomenon. This study objectively quantifies the dysfunctional impact of each individual stiff finger upon the remaining digits. METHODS: Twenty-five individuals (10 men and 15 women) with a mean age of 31 years (range, 18-58 years) without any upper limb pathology, neuropathy, or systemic illness were recruited. Volar-based finger splints were used to hold individual digits of the dominant hand (24 right and 1 left) sequentially in full extension at the metacarpophalangeal (MCP), proximal interphalangeal (PIP), and distal interphalangeal (DIP) joints...
March 1, 2017: Hand: Official Journal of the American Association for Hand Surgery
https://www.readbyqxmd.com/read/28709447/cryptic-amyloidogenic-elements-in-mutant-nefh-causing-charcot-marie-tooth-2-trigger-aggresome-formation-and-neuronal-death
#11
Arnaud Jacquier, Cécile Delorme, Edwige Belotti, Raoul Juntas-Morales, Guilhem Solé, Odile Dubourg, Marianne Giroux, Claude-Alain Maurage, Valérie Castellani, Adriana Rebelo, Alexander Abrams, Stephan Züchner, Tanya Stojkovic, Laurent Schaeffer, Philippe Latour
Neurofilament heavy chain (NEFH) gene was recently identified to cause autosomal dominant axonal Charcot-Marie-Tooth disease (CMT2cc). However, the clinical spectrum of this condition and the physio-pathological pathway remain to be delineated. We report 12 patients from two French families with axonal dominantly inherited form of CMT caused by two new mutations in the NEFH gene. A remarkable feature was the early involvement of proximal muscles of the lower limbs associated with pyramidal signs in some patients...
July 14, 2017: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/28688623/neuropsychiatric-debut-as-a-presentation-of-guillain-barr%C3%A3-syndrome-an-atypical-clinical-case-and-literature-review
#12
Dinesh Sangroula, Richard Durrance, Shirshak Bhattarai, Thambirajah Nandakumar
INTRODUCTION: Guillain Barré Syndrome (GBS) is an acute immune-mediated polyneuropathy most frequently presenting two to four weeks after an acute mild-moderately severe infection as progressive muscular weakness of the lower limbs extending proximally with dysreflexia and autonomic dysfunction. While GBS is typically believed to be isolated to the Peripheral Nervous System, Central Nervous System (CNS) and psychiatric manifestations as a sequela of the disease have been described in different imaging and clinical studies...
October 2017: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/28685718/clinical-characteristics-electrophysiology-and-skin-biopsy-of-38-peripheral-neuropathy-cases-with-small-fiber-involvement-of-various-etiologies
#13
Bo Sun, Li-Zhi Liu, Yi-Fan Li, Zhao-Hui Chen, Li Ling, Fei Yang, Fang Cui, Xu-Sheng Huang
BACKGROUND: In small fiber neuropathy (SFN), thinly myelinated Aδ and unmyelinated C fibers are primarily affected, resulting in sensory and/or autonomic symptoms. Various etiologies have been shown to be associated with SFN. This study was aimed to analyze a variety of features in peripheral neuropathy (PN) with small fiber involvement, and to compare disease severity among patients with idiopathic PN, PN associated with impaired glucose tolerance (IGT), and metabolic syndrome (MS) PN...
July 20, 2017: Chinese Medical Journal
https://www.readbyqxmd.com/read/28667178/a-new-potential-biomarker-for-dementia-with-lewy-bodies-skin-nerve-%C3%AE-synuclein-deposits
#14
Vincenzo Donadio, Alex Incensi, Giovanni Rizzo, Sabina Capellari, Roberta Pantieri, Michelangelo Stanzani Maserati, Grazia Devigili, Roberto Eleopra, Giovanni Defazio, Federico Montini, Agostino Baruzzi, Rocco Liguori
OBJECTIVE: To investigate whether (1) phosphorylated α-synuclein (p-syn) deposits in skin nerves could be useful in differentiating dementia with Lewy bodies (DLB) from different forms of dementia and (2) small fiber neuropathy (SFN) is associated with DLB. METHODS: We studied 18 well-characterized patients with DLB (11 with autonomic dysfunction), 23 patients with nonsynucleinopathy dementia (NSD; 13 with young-onset Alzheimer disease dementia, 6 frontotemporal dementia, and 4 vascular dementia), and 25 healthy controls...
July 25, 2017: Neurology
https://www.readbyqxmd.com/read/28646510/compound-muscle-action-potential-duration-in-critical-illness-neuromyopathy
#15
Christopher L Kramer, Andrea J Boon, C Michel Harper, Brent P Goodman
INTRODUCTION: We sought to determine the specificity of compound muscle action potential (CMAP) durations and amplitudes in a large critical illness neuromyopathy (CINM) cohort relative to controls with other neuromuscular conditions. METHODS: Fifty-eight patients with CINM who had been seen over a 17-year period were retrospectively studied. Electrodiagnostic findings of the CINM cohort were compared with patients with axonal peripheral neuropathy and myopathy due to other causes...
June 23, 2017: Muscle & Nerve
https://www.readbyqxmd.com/read/28634514/cystic-lesions-of-peripheral-nerves-are-we-missing-the-diagnosis-of-the-intraneural-ganglion-cyst
#16
Jyoti Panwar, Anil Mathew, Binu P Thomas
AIM: To highlight the salient magnetic resonance imaging (MRI) features of the intraneural ganglion cyst (INGC) of various peripheral nerves for their precise diagnosis and to differentiate them from other intra and extra-neural cystic lesions. METHODS: A retrospective analysis of the magnetic resonance (MR) images of a cohort of 245 patients presenting with nerve palsy involving different peripheral nerves was done. MR images were analyzed for the presence of a nerve lesion, and if found, it was further characterized as solid or cystic...
May 28, 2017: World Journal of Radiology
https://www.readbyqxmd.com/read/28620719/large-coverage-mr-neurography-in-cidp-diagnostic-accuracy-and-electrophysiological-correlation
#17
Moritz Kronlage, Philipp Bäumer, Kalliopi Pitarokoili, Daniel Schwarz, Véronique Schwehr, Tim Godel, Sabine Heiland, Ralf Gold, Martin Bendszus, Min-Suk Yoon
The objective of this study was to evaluate large coverage magnetic resonance neurography (MRN) in chronic inflammatory demyelinating polyneuropathy (CIDP). In this prospective study, 18 patients with CIDP and 18 healthy controls were examined by a standardized MRN protocol at 3 T. Lumbosacral plexus was imaged by a T2-weighted 3D sequence and peripheral nerves of the upper and lower extremity by axial T2-weighted turbo spin-echo sequences. Lesions were characterized by nerve cross-sectional area (CSA) and T2-weighted signal (nT2)...
July 2017: Journal of Neurology
https://www.readbyqxmd.com/read/28595831/-charcot-marie-tooth-disease-associated-with-hip-dysplasia-in-an-adolescent
#18
T Langlais, J-C Leonard, C Ursu, C Morin
Charcot-Marie-Tooth disease (CMT) is classified into hereditary motor and sensory neuropathy and can induce severe neuro-orthopaedics deformities, disabling at an early age. Hip dysplasia is present in 6% of CMT patients affecting preferentially CMT1 patients and can appear from the age of 8 years. The pathophysiological is paradoxical because we are confronted with proximal osteoarthritis deformations but genetics research brings use new trail. The main functional complaint is a hip joint pain during walking...
June 5, 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/28594495/ulnar-tunnel-syndrome-radial-tunnel-syndrome-anterior-interosseous-nerve-syndrome-and-pronator-syndrome
#19
Adam B Strohl, David S Zelouf
In addition to the more common carpal tunnel and cubital tunnel syndromes, orthopaedic surgeons must recognize and manage other potential sites of peripheral nerve compression. The distal ulnar nerve may become compressed as it travels through the wrist, which is known as ulnar tunnel or Guyon canal syndrome. The posterior interosseous nerve may become entrapped in the proximal forearm as it travels through the radial tunnel, which results in a pain syndrome without motor weakness. The median nerve may become entrapped in the proximal forearm, which can result in a variety of symptoms...
February 15, 2017: Instructional Course Lectures
https://www.readbyqxmd.com/read/28578139/altered-leverage-around-the-ankle-in-people-with-diabetes-a-natural-strategy-to-modify-the-muscular-contribution-during-walking
#20
Milos Petrovic, Kevin Deschamps, Sabine M Verschueren, Frank L Bowling, Constantinos N Maganaris, Andrew J M Boulton, Neil D Reeves
Diabetes patients display gait alterations compared to controls including a higher metabolic cost of walking. This study aimed to investigate whether differences in external moment arm (ExtMA) and effective mechanical advantage (EMA) at the ankle in diabetes patients could partly explain the increased cost of walking compared to controls. Thirty one non-diabetic controls (Ctrl); 22 diabetes patients without peripheral neuropathy (DM) and 14 patients with moderate/severe diabetic peripheral neuropathy (DPN) underwent gait analysis using a motion analysis system and force plates...
May 19, 2017: Gait & Posture
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