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https://www.readbyqxmd.com/read/28222533/type-1-diabetes-mellitus-and-cognitive-impairments-a-systematic-review
#1
Wei Li, Edgar Huang, Sujuan Gao
Type 1 diabetes mellitus (T1DM) is a major subtype of diabetes and is usually diagnosed at a young age with insulin deficiency. The life expectancy of T1DM patients has increased substantially in comparison with that three decades ago due to the availability of exogenous insulin, though it is still shorter than that of healthy people. However, the relation remains unclear between T1DM and dementia as an aging-related disease. We conducted a systematic review of existing literature on T1DM and cognition impairments by carrying out searches in electronic databases Medline, EMBASE, and Google Scholar...
February 10, 2017: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/28221309/a-case-of-idiopathic-femoral-neuropathy-with-subsequent-quadriceps-atrophy
#2
Elle Rathbun
No abstract text is available yet for this article.
March 2017: Journal of Clinical Neuromuscular Disease
https://www.readbyqxmd.com/read/28221308/intravenous-immunoglobulin-in-treatment-of-hereditary-neuropathy-with-liability-to-pressure-palsy
#3
Zhong Hong Liew, Yew Long Lo
No abstract text is available yet for this article.
March 2017: Journal of Clinical Neuromuscular Disease
https://www.readbyqxmd.com/read/28221306/whole-exome-sequencing-identifies-atypical-welander-distal-myopathy-in-patient
#4
Jennifer Gass, Patrick Blackburn, Jessica Jackson, Kimberly Harris, Duygu Selcen, Elliot Dimberg, Paldeep Atwal
Welander distal myopathy is a rare autosomal dominant disorder characterized by muscle weakness in the hands and feet. Exome sequencing of affected families discovered a segregating p.Glu384Lys pathogenic variant in TIA-1 as the main genetic cause of Welander distal myopathy. TIA-1 encodes an RNA-binding protein which serves as a key component of stress granules. This protein also regulates splicing and translation of mRNA. Our patient developed progressive weakness in his hands and feet during his late 40s that was misdiagnosed as a neuropathy that caused muscle atrophy...
March 2017: Journal of Clinical Neuromuscular Disease
https://www.readbyqxmd.com/read/28221302/diagnostic-criteria-for-small-fiber-neuropathy
#5
Derrick Blackmore, Zaeem A Siddiqi
OBJECTIVES: Despite its relative common occurrence, definitive diagnosis of small fiber neuropathy (SFN) remains problematic. In practice, patients with pain, numbness, and/or paresthesias in their lower limbs are diagnosed with SFN if found to have dissociated sensory loss in their feet, that is, impaired pinprick perception (PP) but relatively preserved vibration. We sought to assess the sensitivity and specificity of clinical examination and various diagnostic tools available for screening SFN...
March 2017: Journal of Clinical Neuromuscular Disease
https://www.readbyqxmd.com/read/28221301/electrodiagnostic-characterization-of-hereditary-neuropathy-with-liability-to-pressure-palsies
#6
Sachiko Takahashi, Marvin Chum, Kurt Kimpinski
OBJECTIVES: The study objective was electrodiagnostic characterization of a large cohort of patients with genetically confirmed hereditary neuropathy with liability to pressure palsies (HNPP). METHODS: A retrospective review was conducted on all patients with HNPP seen at the neuromuscular clinic (London, Canada) from 1977 to 2015. Clinical data obtained included patient characteristics, examination findings, and nerve conduction study results. RESULTS: A total of 46 patients were analyzed...
March 2017: Journal of Clinical Neuromuscular Disease
https://www.readbyqxmd.com/read/28220906/experiences-with-preventing-carpal-tunnel-syndrome-in-an-automotive-plant
#7
Věra Žídková, Marie Nakládalová, Jana Zapletalová, Zdeněk Nakládal, Helena Kollárová
OBJECTIVES: Carpal tunnel syndrome (CTS) is a common occupational disease. The aim was to assess the effect of preventive measures in automotive assembly workers. MATERIAL AND METHODS: The analysis summarizes data from annual crosssectional studies. The 7-year analysis of data was based on medical records obtained from an occupational physician and inspections carried out at the workplace where targeted preventive measures were introduced, including better ergonomic arrangement of the workplace, technical adjustments facilitating the work, preventive nerve conduction studies (NCS) testing of the median nerve once a year, switching of workers and their targeted rotation within the workplace...
February 21, 2017: International Journal of Occupational Medicine and Environmental Health
https://www.readbyqxmd.com/read/28219164/-a-combination-use-of-endoscope-and-microscope-in-cerebral-pontine-angle-surgery
#8
Z Y Wang, H Jia, J Yang, H Y Tan, H Wu
Objective: To evaluate the application of combination use of microscope and endoscope in cerebral pontine angle (CPA) surgery. Methods: A total of 72 patients undergone lateral skull base surgeries via endoscope under microscopic control from January 2006 to January 2015 was reviewed respectively. The patients including 35 males and 37 females were composed of 22 cases of vestibular swannnomas, 45 cases of cranial neuropathy and 5 cases of CPA chelesteatoma. Twenty cases of vestibular swannnomas, 15 cases of cranial neuropathy and 2 cases of CPA chelesteatoma undergone the surgery via retrosigmoid approach, while other cases undergone the surgery via retrolabyrinthine approach...
February 7, 2017: Zhonghua Er Bi Yan Hou Tou Jing Wai Ke za Zhi, Chinese Journal of Otorhinolaryngology Head and Neck Surgery
https://www.readbyqxmd.com/read/28217942/hypertrophic-pachymeningitis-is-a-characteristic-manifestation-of-granulomatosis-with-polyangiitis-a-retrospective-study-of-anti-neutrophil-cytoplasmic-antibody-associated-vasculitis
#9
Yasuhiro Shimojima, Dai Kishida, Akiyo Hineno, Masahide Yazaki, Yoshiki Sekijima, Shu-Ichi Ikeda
AIM: To elucidate the characteristics of patients with hypertrophic pachymeningitis (HP) in a population with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). METHODS: We retrospectively investigated the clinical records of 39 patients who were diagnosed with AAV. To determine the characteristics of HP in AAV, the epidemiological and clinical data from patients with HP were statistically compared with those from patients without HP. RESULTS: Of 39 patients with AAV, seven (17...
February 20, 2017: International Journal of Rheumatic Diseases
https://www.readbyqxmd.com/read/28217821/physicians-experiences-as-patients-with-statin-side-effects-a-case-series
#10
Hayley J Koslik, Athena Hathaway Meskimen, Beatrice Alexandra Golomb
Physicians are among those prescribed statins and therefore, subject to potential statin adverse effects (AEs). There is little information on the impact of statin AEs on physicians affected by them. We sought to assess the character and impact of statin AEs occurring in physicians and retired physicians, and to ascertain whether/how personal experience of AEs moderated physicians' attitude toward statin use. Seven active or retired physicians from the United States communicated with the Statin Effects Study group regarding their personal experience of statin AEs...
December 2017: Drug Safety—Case Reports
https://www.readbyqxmd.com/read/28217248/iatrogenic-amyloid-polyneuropathy-after-domino-liver-transplantation
#11
REVIEW
Diana Mnatsakanova, Saša A Živković
Liver transplantation has been used in treatment of transthyretin amyloidosis, and some patients undergo domino liver transplantation (DLT) with explanted liver being transplanted to another patient with liver failure as the liver is otherwise usually functionally normal. Until end of 2015, there were 1154 DLT performed worldwide. DLT for transthyretin amyloidosis is associated with the risk of developing de novo systemic amyloidosis and amyloid neuropathy, and the risk may be greater with some non-Val30Met mutations...
January 28, 2017: World Journal of Hepatology
https://www.readbyqxmd.com/read/28217183/affection-of-the-respiratory-muscles-in-combined-complex-i-and-iv-deficiency
#12
Josef Finsterer, Helmut Rauschka, Liane Segal, Gabor G Kovacs, Boris Rolinski
OBJECTIVES: Combined complex I+IV deficiency has rarely been reported to manifest with the involvement of the respiratory muscles. CASE REPORT: A 45y male was admitted for hypercapnia due to muscular respiratory insufficiency. He required intubation and mechanical ventilation. He had a previous history of ophthalmoparesis since age 6y, ptosis since age 23y, and anterocollis since at least age 40y. Muscle biopsy from the right deltoid muscle at age 41y was indicative of mitochondrial myopathy...
2017: Open Neurology Journal
https://www.readbyqxmd.com/read/28217155/acute-bulbar-palsy-plus-syndrome-a-rare-variant-of-guillain-barre-syndrome
#13
Sanghamitra Ray, Prakash Chand Jain
Guillain-Barre syndrome (GBS) is the most common cause of acute flaccid paralysis worldwide both in adult and pediatric population. Although flaccid paralysis is the hallmark of this disease, there are some rare variants which may be easily missed unless suspected. Here, we present a very rare variant of GBS - acute bulbar palsy plus syndrome in a pediatric patient. A 13-year-old female child presented with right-sided lower motor neuron type of facial palsy and palsy of bilateral glossopharyngeal and vagus nerve of 2 weeks duration...
October 2016: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/28216809/healing-ulcers-and-preventing-their-recurrences-in-the-diabetic-foot
#14
S Raja Sabapathy, Madhu Periasamy
Fifteen percent of people with diabetes develop an ulcer in the course of their lifetime. Eighty-five percent of the major amputations in diabetes mellitus are preceded by an ulcer. Management of ulcers and preventing their recurrence is important for the quality of life of the individual and reducing the cost of care of treatment. The main causative factors of ulceration are neuropathy, vasculopathy and limited joint mobility. Altered bio-mechanics due to the deformities secondary to neuropathy and limited joint mobility leads to focal points of increased pressure, which compromises circulation leading to ulcers...
September 2016: Indian Journal of Plastic Surgery: Official Publication of the Association of Plastic Surgeons of India
https://www.readbyqxmd.com/read/28216074/autoimmunity-in-guillain-barr%C3%A3-syndrome-associated-with-zika-virus-infection-and-beyond
#15
REVIEW
Carlos A Pinto-Díaz, Yhojan Rodríguez, Diana M Monsalve, Yeny Acosta-Ampudia, Nicolás Molano-González, Juan-Manuel Anaya, Carolina Ramírez-Santana
Autoimmune diseases share common immunopathogenic mechanisms (i.e., the autoimmune tautology), which explain the clinical similarities among them as well as their familial clustering. Guillain-Barré syndrome (GBS), an autoimmune peripheral neuropathy, has been recently associated with Zika virus (ZIKV) infection. Based on a series of cases, this review article provides a comparative analysis of GBS associated with ZIKV infection, contrasted with the general characteristics of GBS in light of the autoimmune tautology, including gender differences in prevalence, subphenotypes, polyautoimmunity, familial autoimmunity, age at onset, pathophysiology, ecology, genetics, ancestry, and treatment...
February 13, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28216073/autoimmune-neuropathies-associated-to-rheumatic-diseases
#16
REVIEW
Alberto R M Martinez, Ingrid Faber, Anamarli Nucci, Simone Appenzeller, Marcondes C França
Systemic manifestations are frequent in autoimmune rheumatic diseases and include peripheral nervous system damage. Neuron cell body, axons and myelin sheath may all affected in this context. This involvement results in severe and sometimes disabling symptoms. Sensory, motor and autonomic features may be present in different patterns that emerge as peculiar clinical pictures. Prompt recognition of these neuropathies is pivotal to guide treatment and reduce the risks of long term disability. In this review, we aim to describe the main immune-mediated neuropathies associated to rheumatic diseases: sensory neuronopathies, multiple mononeuropathies and chronic inflammatory demyelinating polyradiculoneuropathy, with an emphasis on clinical features and therapeutic options...
February 12, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28215760/two-novel-cases-of-compound-heterozygous-mutations-in-mitofusin2-finding-out-the-inheritance
#17
Alessandro Geroldi, Patrizia Lastella, Margherita Patruno, Fabio Gotta, Nicoletta Resta, Grazia Devigili, Carlo Sabbà, Rossella Gulli, Merit Lamp, Paola Origone, Paola Mandich, Emilia Bellone
MFN2 is the major gene involved in the axonal form of Charcot-Marie-Tooth disease. It usually has an autosomal dominant pattern of inheritance, but a few cases of homozygous or compound heterozygous mutations have been described. These patients usually present an earlier onset, more severe phenotype and their inheritance pattern can span from autosomal recessive to semidominant. Here we report two unrelated patients carrying two compound heterozygous MFN2 mutations. Both present a pure axonal neuropathy without any additional features...
January 17, 2017: Neuromuscular Disorders: NMD
https://www.readbyqxmd.com/read/28215457/synchronous-ipsilateral-cavernous-malformations-of-the-trochlear-nerve
#18
Christopher S Graffeo, William R Copeland, Perkins Mukunyadzi, Ali F Krisht
BACKGROUND: Cranial nerve cavernous malformations (CM) are rare benign congenital vascular anomalies, with approximately 44 preceding cases in the literature. We report the fifth case of trochlear CM, as well as the first instance of two discrete CM occurring simultaneously along the same cranial nerve. METHODS: Case report. RESULTS: A fifty-seven year-old man presented with several years of diplopia; physical examination identified a complete left trochlear nerve paralysis...
February 16, 2017: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/28214652/unilateral-oculomotor-palsy-in-charcot-marie-tooth-disease-1a-cmt-1a
#19
A Posa, A Emmer, M E Kornhuber
BACKGROUND: Charcot-Marie-Tooth disease (CMT) type 1A is the most common form of CMT 1 and one of the autosomal dominant demyelinating hereditary motor and sensory neuropathies (HMSN). Cranial nerves may be frequently subclinically affected in CMT disease. However manifest clinical signs of cranial nerve involvement are rare. METHODS: This case comprise neurological, ophthalmological, internal medicine and ear-nose-throat investigation, motor and sensory nerve conduction velocity, auditory evoked potentials and orbicularis-oculi reflex measurements, lumbar puncture and blood examination, inclusive molecular genetic testing, as well as electrocardiogram and cranial imaging such as computer tomography and magnetic resonance imaging RESULTS: The present case shows a Charcot-Marie-Tooth (CMT) 1A patient with complete unilateral oculomotor palsy in combination with predominant ipsilateral subclinical trigeminal demyelination...
February 13, 2017: Clinical Neurology and Neurosurgery
https://www.readbyqxmd.com/read/28214515/igg-degrading-enzyme-of-streptococcus-pyogenes-ides-prevents-disease-progression-and-facilitates-improvement-in-a-rabbit-model-of-guillain-barr%C3%A3-syndrome
#20
Yuzhong Wang, Qiguang Shi, Hui Lv, Ming Hu, Weifang Wang, Quanquan Wang, Baojun Qiao, Guorong Zhang, Zhanyun Lv, Christian Kjellman, Sofia Järnum, Lena Winstedt, Yong Zhang, Jiao Wen, Yanlei Hao, Nobuhiro Yuki
Autoantibodies binding to peripheral nerves followed by complement deposition and membrane attack complex formation results in nerve damage in Guillain-Barré syndrome (GBS). Strategies to remove the pathogenic autoantibodies or block the complement deposition benefit most patients with GBS. Immunoglobulin G-degrading enzyme of Streptococcus pyogenes (IdeS) is a cysteine protease which cleaves IgG antibodies into F(ab')2 and Fc fragments. In this study, using a rabbit model of axonal GBS, acute motor axonal neuropathy (AMAN), we demonstrated that IdeS treatment significantly reduced the disruption of Nav channels as well as activated C3 deposition at the anterior spinal root nodes of Ranvier in AMAN rabbits...
February 16, 2017: Experimental Neurology
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