68,051 results match your criteria Neuropathy of Friedreich Ataxia


Idiopathic toe walking-A follow-up survey of gait analysis assessment.

Gait Posture 2018 Dec 6;68:300-304. Epub 2018 Dec 6.

School of Physiotherapy, Royal College of Surgeons in Ireland, 123 St Stephen's Green, Dublin 2, Ireland.

Background: Toe-walking is a normal variant in children up to 3 years of age but beyond this a diagnosis of idiopathic toe-walking (ITW) must be considered. ITW is an umbrella term that covers all cases of toe-walking without any diagnosed underlying medical condition and before assigning these diagnosis potential differential diagnoses such as cerebral palsy, peripheral neuropathy, spinal dysraphism and myopathy must be ruled out. Gait laboratory assessment (GLA) is thought to be useful in the evaluation of ITW, and kinematic, kinetic and electromyography features associated with ITW have been described. Read More

View Article
December 2018

Critical role of Ca3.2 T-type calcium channels in the peripheral neuropathy induced by bortezomib, a proteasome-inhibiting chemotherapeutic agent, in mice.

Toxicology 2018 Dec 12. Epub 2018 Dec 12.

Laboratory of Pharmacology and Pathophysiology, Faculty of Pharmacy, Kindai University (formerly known as Kinki University), Higashi-Osaka, 577-8802, Japan. Electronic address:

Bortezomib, a first-line agent for treatment of multiple myeloma, exhibits anticancer activity through proteasome inhibition. However, bortezomib-induced peripheral neuropathy (BIPN) is one of the most serious side effects. Since decreased proteasomal degradation of Ca3. Read More

View Article
December 2018

What Is Behind Cerebellar Vertigo and Dizziness?

Cerebellum 2018 Dec 14. Epub 2018 Dec 14.

Department of Neurology, Ludwig-Maximilians University, Marchioninistrasse 15, 81377, Munich, Germany.

The differential diagnosis of vertigo or dizziness as a result of cerebellar disorders can be difficult as many patients with a cerebellar pathology do not present with the full spectrum of cerebellar signs. The main goal of this study was to describe the typical clinical features of these patients with vertigo or dizziness of a cerebellar origin. We reviewed the medical records of 5400 patients with vertigo and dizziness from our tertiary outpatient clinic for vertigo and balance disorders. Read More

View Article
December 2018

Serum and Urinary Selenium Status in Patients with the Pre-diabetes and Diabetes in Northeast China.

Biol Trace Elem Res 2018 Dec 14. Epub 2018 Dec 14.

Department of Laboratory Medicine, First Hospital of Jilin University, Changchun, 130021, China.

Homeostasis imbalance of selenium (Se) in diabetes has received great attention. This study investigated serum and urinary Se levels in patients with impaired fasting glucose (IFG), impaired glucose tolerance (IGT), type 1 diabetes (T1D), and type 2 diabetes (T2D) in Northeast Chinese populations. From January 2010 to October 2011, patients with IFG (n = 12), IGT (n = 15), T1D (n = 25), T2D (n = 137), and healthy controls (n = 50) were enrolled in the First Hospital of Jilin University. Read More

View Article
December 2018

Patient-Reported Toxicities During Chemotherapy Regimens in Current Clinical Practice for Early Breast Cancer.

Oncologist 2018 Dec 14. Epub 2018 Dec 14.

School of Medicine, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina, USA.

Background: This study explores the incidence of patient-reported major toxicity-symptoms rated "moderate," "severe," or "very severe"-for chemotherapy regimens commonly used in early breast cancer.

Patients And Methods: Female patients aged 21 years or older completed a validated Patient-Reported Symptom Monitoring instrument and rated 17 symptoms throughout adjuvant or neoadjuvant chemotherapy. Fisher's exact tests compared differences in percentages in symptom ratings, and general linear regression was used to model the incidence of patient-reported major toxicity. Read More

View Article
December 2018

FDA Approval Summary: Pertuzumab for adjuvant treatment of HER2-positive early breast cancer.

Clin Cancer Res 2018 Dec 14. Epub 2018 Dec 14.

Office of Hematology and Oncology, FDA.

On December 20, 2017, the Food and Drug Administration granted regular approval to pertuzumab in combination with trastuzumab and chemotherapy for the adjuvant treatment of patients with HER2-positive early breast cancer (EBC) at high risk of recurrence. Approval was based on data from the APHINITY trial, which randomized patients to receive pertuzumab or placebo in combination with trastuzumab and chemotherapy. After 45. Read More

View Article
December 2018

Diagnostic sensitivity of electrophysiology and ultrasonography in ulnar neuropathies of different severity.

Clin Neurophysiol 2018 Dec 7. Epub 2018 Dec 7.

Department of Neurology and Clinical Neurophysiology, Auckland District Health Board, Auckland Hospital, Auckland, New Zealand.

Objective: To assess the diagnostic performance of electrophysiology and nerve ultrasound in ulnar neuropathies of varying clinical severity in 135 consecutive patients.

Methods: Clinical severity of ulnar neuropathy was graded on a 4 point scale from very mild (symptoms only) to severe (marked atrophy of intrinsic hand muscles). Sensitivity and localization ability of electrophysiology and nerve ultrasound were assessed for each point of the scale. Read More

View Article
December 2018

Benzoxazinone-thiosemicarbazones as antidiabetic leads via aldose reductase inhibition: Synthesis, biological screening and molecular docking study.

Bioorg Chem 2018 Dec 11. Epub 2018 Dec 11.

Centre for Advanced Drug Research, COMSATS University Islamabad, Abbottabad Campus, Abbottabad 22060, Pakistan. Electronic address:

Aldose reductase is an important enzyme in the polyol pathway, where glucose is converted to fructose, and sorbitol is released. Aldose reductase activity increases in diabetes as the glucose levels increase, resulting in increased sorbitol production. Sorbitol, being less cell permeable tends to accumulate in tissues such as eye lenses, peripheral nerves and glomerulus that are not insulin sensitive. Read More

View Article
December 2018

A non-invasive, multi-target approach to treat diabetic retinopathy.

Biomed Pharmacother 2019 Jan 5;109:708-715. Epub 2018 Nov 5.

Department of Biotechnology, School of Bioengineering, Faculty of Engineering and Technology, SRM Institute of Science and Technology, Chennai 603 203, India. Electronic address:

Hyperglycemia invoke number of pathways resulting in development of diabetic retinopathy (DR), including protein kinase C activation, increased expression of VEGF, advanced glycation end product (AGEs) formation and activation of polyol pathway, among which the pathophysiology of aldose reductase (ALR2) of the polyol pathway is evident by more than a decade of research. Subtle involvement of ALR2 in invoking various pathways of diabetic complications has caused an increase in attention towards the identification of novel aldose reductase inhibitors (ARIs). Numerous ARIs of different classes were employed in the treatment of diabetic complications initially, but few came into light as drugs. Read More

View Article
January 2019
1 Read

Survival and outcome in MELAS not only depends on onset and disease duration.

J Neurol Sci 2018 Dec 6;397:9-10. Epub 2018 Dec 6.

University of Tunis El Manar and Genomics Platform, Pasteur Institute of Tunis, Tunisia.

View Article
December 2018

Biotinidase deficiency should be considered in individuals thought to have multiple sclerosis and related disorders.

Authors:
Barry Wolf

Mult Scler Relat Disord 2018 Nov 28;28:26-30. Epub 2018 Nov 28.

Division of Genetics, Birth Defects and Metabolism, Department of Pediatrics, Ann and Robert H. Lurie Children's Hospital of Chicago and Northwestern Feinberg School of Medicine Chicago, IL 60611, USA. Electronic address:

Background: Multiple sclerosis is a disorder of the central and peripheral nervous system of young and old adults that is characterized by muscle, coordination and vision abnormalities. Multiple sclerosis is likely due to numerous causes.

Methods: Recently, adolescents and adults with ophthalmological and or neurological findings have been diagnosed with biotinidase deficiency. Read More

View Article
November 2018
1 Read

Kynurenic acid and zaprinast diminished CXCL17-evoked pain-related behaviour and enhanced morphine analgesia in a mouse neuropathic pain model.

Pharmacol Rep 2018 Oct 6;71(1):139-148. Epub 2018 Oct 6.

Department of Pain Pharmacology, Institute of Pharmacology, Polish Academy of Sciences, Kraków, Poland. Electronic address:

Background: The G protein-coupled receptor 35 (GPR35), is considered important for nociceptive transmission, as suggested by accumulating evidence. This receptor was discovered in 1998; however, a lack of pharmacological tools prevented a complete understanding of its function and how to exploit it therapeutically. We studied the influence of CXCL17, kynurenic acid and zaprinast on nociceptive transmission in naïve and neuropathic mice. Read More

View Article
October 2018

Extended Duration of Hyperglycemia Result in Human-Like Corneal Nerve Lesions in Mice With Alloxan- and Streptozotocin-Induced Type 1 Diabetes.

Invest Ophthalmol Vis Sci 2018 Dec;59(15):5868-5875

Laboratory of Pathology, Faculty of Pharmaceutical Science, Setsunan University, Osaka, Japan.

Purpose: Previous experimental studies assessing corneal nerves as a measure of the severity of diabetic peripheral neuropathy have yielded discordant results; this may have been due to the effect of the short duration of the induced diabetes. We investigated whether increases in the duration of hyperglycemia result in the development of corneal lesions in a mouse model of alloxan (AL)- or streptozotocin (STZ)-induced type 1 diabetes. We further determined whether corneal nerve fiber density, intraepidermal nerve fiber density (IENFD), and sural nerve morphology can be used as morphologic markers of diabetic peripheral neuropathy in rodent models. Read More

View Article
December 2018

Bony Orbital Volume Expansion in Thyroid Eye Disease.

Ophthalmic Plast Reconstr Surg 2018 Dec 7. Epub 2018 Dec 7.

Doheny Eye Institute, Division of Orbital and Ophthalmic Plastic Surgery, University of California-Los Angeles, Los Angeles, California, U.S.A.

Purpose: To quantify changes to the bony orbital volume (BOV) in patients with thyroid eye disease (TED) relative to normal controls.

Methods: In this case-control study, all patients affected with TED seen over a 2-year period were screened for study entry. Eligible participants were adults with clinical evidence of TED and CT scans of their orbits obtained during the course of their routine care. Read More

View Article
December 2018
1 Read

[Research progress on auditory neuropathy spectrum disorder and cochlear implantation in infants and young children].

Authors:
J Y Chen Y X Li

Lin Chung Er Bi Yan Hou Tou Jing Wai Ke Za Zhi 2018 Jul;32(14):1120-1124

Auditory neuropathy is a disorder characterized by absent or severely abnormal auditory brainstem response (ABR) with intact outer hair cell function, as evidenced by the presence of evoked otoacoustic emissions and/or cochlear microphonics. Unlike patients with sensory hearing loss who show clinical evidence of impaired outer hair cell function. For ANSD patients, clinical rehabilitation is mainly limited to hearing aid wearing and cochlear implantation. Read More

View Article

[Application of image navigation assisted nasal endoscopic surgery in optic nerve decompression].

Authors:
L L Shi H T Zhen

Lin Chung Er Bi Yan Hou Tou Jing Wai Ke Za Zhi 2018 Dec;32(24):1893-1896

Department of Otolaryngology Head and Neck Surgery, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430030, China.

To explore the application and to evaluate the advantage of image navigation assisted nasal endoscopic surgery in optic nerve decompression. Sixty patients accepted the image navigation assisted nasal endoscopic surgery therapy in optic nerve decompression were included in this retrospective study and followed up for about six months to four years. The visual acuity was improved in 16 cases with visual acuity above light. Read More

View Article
December 2018

[Application of image-guided system in endoscopic sinus and skull base surgery].

Lin Chung Er Bi Yan Hou Tou Jing Wai Ke Za Zhi 2018 Dec;32(24):1856-1859

Department of Otorhinolaryngology Head and Neck Surgery, Huadong Hospital, Fudan University, Shanghai, 200040,China.

To evaluate the applicative value of image-guided system in endoscopic sinus and skull base surgeries. A total of 103 endoscopic surgical procedures were performed.All these procedures were conducted with the utilization of image-guided system, among which there were 92 cases of sinonasal-skull base surgery(including nasal sinuses resection of benign and malignant tumors involving skull base lesions, the cumulative orbital lesion resection of nasal sinus lesions, etc. Read More

View Article
December 2018

BDNF Val66Met is Associated with Pre-existing but not with Paclitaxel-induced Peripheral Neuropathy in an Israeli Cohort of Breast Cancer Patients.

Isr Med Assoc J 2018 Dec;20(12):746-748

Department of Hematology, Galilee Medical Center, Nahariya, Israel, affiliated with Faculty of Medicine in the Galilee, Bar-Ilan University, Safed, Israel.

Background: Brain-derived neurotrophic factor (BDNF) is a neuronal growth factor that is important for the development, maintenance, and repair of the peripheral nervous system. The BDNF gene commonly carries a single nucleotide polymorphism (Val66Met-SNP), which affects the cellular distribution and activity-dependent secretion of BDNF in neuronal cells.

Objectives: To check the association between BDNF Val66Met-SNP as a predisposition that enhances the development of chemotherapy-induced peripheral neuropathy in an Israeli cohort of patients with breast cancer who were treated with paclitaxel. Read More

View Article
December 2018

[Morphological and dimensional changes of the magnetic resonance imaging in the carpal tunnel (pre- and postoperatively)].

Acta Ortop Mex 2018 Jul-Aug;32(4):209-213

Unidad Médica de Alta Especialidad de Traumatología y Ortopedia «Lomas Verdes» del Instituto Mexicano del Seguro Social (IMSS). México.

Introduction: Carpal tunnel syndrome is the most common compression neuropathy of the upper limb, and the release of the median nerve is the surgical treatment that is carried out. There are several surgical techniques used to treat this condition; However, this virtual cavity, which we call the carpal tunnel, undergoes several morphological and dimensional changes, prior to surgery and after surgery using the various techniques that the surgeon has a predilection for.

Material And Methods: Observational, prospective and longitudinal study conducted in the period from March to November 2009. Read More

View Article
December 2018

Patient Reported Symptom-Mapping in Carpal Tunnel Syndrome.

Muscle Nerve 2018 Dec 14. Epub 2018 Dec 14.

Clinical Professor of Orthopaedic Surgery, Wayne State University School of Medicine, 25700 Southwood Dr., Southfield, MI, USA, 48075, 2487526328.

Introduction: To date, no method has been described or utilized to study the distribution of symptoms in carpal tunnel syndrome. We describe a technique of symptom-mapping that yields a population-based "anatomic profile" of carpal tunnel syndrome.

Methods: Symptoms were mapped on visual questionnaires depicting the volar hand, wrist and forearm. Read More

View Article
December 2018

Validation of diagnostic methods for traumatic sensory neuropathy and neuropathic pain.

Muscle Nerve 2018 Dec 14. Epub 2018 Dec 14.

Departments of Clinical Neurophysiology, University of Turku and Turku University Hospital, Postal Box 52, 20521, Turku, Finland.

Introduction: We evaluated diagnostic value of sensory tests during recovery from iatrogenic sensory neuropathy using intraoperatively verified nerve injury with subjective symptoms as gold standard.

Methods: Inferior alveolar nerves were monitored neurophysiologically throughout mandibular osteotomy in 19 patients. Sensory disturbance was registered and sensation tested using clinical and quantitative sensory (QST) and neurophysiologic tests postoperatively at 1, 3, 6, 12 months. Read More

View Article
December 2018

Genetic variants in DNMT1 and the risk of cardiac autonomic neuropathy in females with Type 1 diabetes.

J Diabetes Investig 2018 Dec 11. Epub 2018 Dec 11.

Laboratório de Carboidratos e Radioimunoensaio LIM-18, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo, Sao Paulo, SP, BR. Av. Dr. Arnaldo, 455São Paulo, SP, BR.

Aims/introduction: Epigenetics participate in the pathogenesis of metabolic memory, a situation in which hyperglycemia exerts prolonged deleterious effects even after its normalization. We tested the hypothesis that genetic variants in an epigenetic gene could predispose to diabetes complications.

Material And Methods: We assessed the frequency of 5 single nucleotide polymorphisms (SNPs) in the gene encoding DNA methytransferase 1 (DNMT1; rs8112895, rs7254567, rs11085721, rs17291414, and rs10854076) and their associations with diabetic kidney disease, retinopathy, distal polyneuropathy and autonomic cardiovascular neuropathy (CAN) in 359 long-term Type 1 diabetes individuals. Read More

View Article
December 2018

Optical coherence tomography in the investigation of systemic neurologic disease.

Clin Exp Optom 2018 Dec 11. Epub 2018 Dec 11.

Institute of Health and Biomedical Innovation, Queensland University of Technology, Brisbane, Queensland, Australia.

Optical coherence tomography (OCT) is a well-established technique for the clinical examination, diagnosis, severity staging and monitoring of ophthalmic disorders. The application of this technology has more recently been extended beyond ophthalmic disease, whereby it has been demonstrated that OCT can serve as an ophthalmic marker for a range of systemic neurodegenerative disorders, such as Parkinson's disease, multiple sclerosis, Alzheimer's disease and diabetic peripheral neuropathy. This review will focus on the clinical utility of OCT-derived retinal measures for the investigation of these conditions. Read More

View Article
December 2018

A Life-Saving Palsy: Hereditary Neuropathy with Liability to Pressure Palsies (HNPP) Presenting As Hand Weakness during Cardiopulmonary Resuscitation (CPR) Training

Ir Med J 2018 09 10;111(8):808. Epub 2018 Sep 10.

Department of Clinical Neurophysiology, St. Vincent’s University Hospital, Elm Park, Dublin 4, Ireland.

Aim To describe a case of acute and transient hand weakness that developed during cardiopulmonary resuscitation (CPR) training. Hereditary neuropathy with liability to pressure palsies (HNPP) should be considered in patients with recurrent, painless motor or sensory neuropathies at sites of peripheral nerve compression. Methods Nerve conduction studies confirmed neuropraxia of the distal ulnar nerve with a mild background demyelinating peripheral neuropathy. Read More

View Article
September 2018

Interferon-α-induced retinopathy in chronic hepatitis C treatment: summary, considerations, and recommendations.

Graefes Arch Clin Exp Ophthalmol 2018 Dec 13. Epub 2018 Dec 13.

Department of Ophthalmology and Vision Sciences, Princess Margaret Hospital/University Health Network, University of Toronto, 610 University Avenue, Toronto, ON, M5G 2M9, Canada.

Interferons are cytokines that regulate the host's response to viral infection, particularly in the setting of the immunologic response to the hepatitis C virus (HCV). While the virus has the ability to evade the host's innate and specific immunity, exogenous interferon-α with combined ribavirin, treatments have been found to achieve a significant sustained viral response in subgroups of patients with chronic HCV. One of the major side effects of interferon-α is an ocular retinopathy characterized by flame-shaped hemorrhages and cotton wool spots visualized on funduscopic examination. Read More

View Article
December 2018

Late adult-onset adrenomyeloneuropathy evolving with atypical severe frontal lobe syndrome: Importance of neuroimaging.

Radiol Case Rep 2019 Mar 5;14(3):309-314. Epub 2018 Dec 5.

Department of Medical, Surgical, Neurological, Metabolic Sciences, and Aging, 2nd Division of Neurology, Center for Rare Diseases and InterUniversity Center for Research in Neurosciences, University of Campania "Luigi Vanvitelli", Via Sergio Pansini, 80131 Naples, Italy.

X-linked adrenoleukodystrophy (X-ALD) is a rare inherited metabolic disease affecting the nervous system and the adrenal glands. It is caused by a mutation of the gene, resulting in the impaired degradation of very long-chain fatty acids and their subsequent accumulation in several organs and tissues. X-ALD is notable for its high phenotypical variability, that includes isolated adrenocortical insufficiency, slowly progressive myelopathy with paraparesis, ataxia, and peripheral neuropathy to severe childhood cerebral forms. Read More

View Article

Hypotension as a symptom of autonomic neuropathy in patients with advanced malignancies.

Biomed Pap Med Fac Univ Palacky Olomouc Czech Repub 2018 Dec 11. Epub 2018 Dec 11.

Department of Oncology, Faculty Hospital Trencin, Legionarska 28, Trencin, Slovak Republic.

Aims: Hypotension can be a symptom of paraneoplastic autonomic neuropathy (PAN). Onconeural antibodies (OA) provide strong evidence for the paraneoplastic origin of neurological syndromes. Our goal was to assess the frequency of PAN among patients with advanced malignancies and hypotension using OA. Read More

View Article
December 2018
1 Read

Insulin-like growth factor-1 activates AMPK to augment mitochondrial function and correct neuronal metabolism in sensory neurons in type 1 diabetes.

Mol Metab 2018 Nov 28. Epub 2018 Nov 28.

Division of Neurodegenerative Disorders, St Boniface Hospital Albrechtsen Research Centre, Winnipeg, MB, Canada; Department of Pharmacology and Therapeutics, University of Manitoba, Winnipeg, MB, Canada. Electronic address:

Objective: Diabetic sensorimotor polyneuropathy (DSPN) affects approximately half of diabetic patients leading to significant morbidity. There is impaired neurotrophic growth factor signaling, AMP-activated protein kinase (AMPK) activity and mitochondrial function in dorsal root ganglia (DRG) of animal models of type 1 and type 2 diabetes. We hypothesized that sub-optimal insulin-like growth factor 1 (IGF-1) signaling in diabetes drives loss of AMPK activity and mitochondrial function, both contributing to development of DSPN. Read More

View Article
November 2018
1 Read

Compressive radial neuropathy by a synovial cyst during pregnancy: A clinical case report.

Medicine (Baltimore) 2018 Dec;97(49):e13455

Department of Physical Medicine and Rehabilitation, Tung's Taichung Metroharbor Hospital, Taichung, Taiwan.

Rationale: Compressive radial neuropathy by a synovial cyst in the radial tunnel during pregnancy is a rare occurrence. The management of radial nerve compression caused by a synovial cyst in a pregnant patient is a surgical dilemma owing to the fetal and maternal risks of treatment.

Patient Concerns And Diagnosis: A 37-year-old pregnant woman presented with progressive forearm pain at the gestational age of 12 weeks. Read More

View Article
December 2018
1 Read

Volume-staged Gamma Knife radiosurgery for orbital venous malformations.

Authors:

J Neurosurg 2018 Dec;129(Suppl1):26-30

OBJECTIVEThis article is a preliminary evaluation of the efficacy of volume-staged Gamma Knife radiosurgery (GKRS) in the treatment of patients with orbital venous malformations (OVMs).METHODSTwenty patients with moderate to large OVMs were treated with volume-staged GKRS between March 2005 and October 2015. The series included 8 male and 12 female patients with an average age of 22. Read More

View Article
December 2018

Hepatic arterial therapy with oxaliplatin and systemic capecitabine for patients with liver metastases from breast cancer.

Breast 2018 Dec 5;43:113-119. Epub 2018 Dec 5.

Department of Oncology, Herlev & Gentofte Hospital, University of Copenhagen, Herlev Ringvej 75, DK-2730 Herlev, Denmark.

Objectives: Hepatic arterial treatment (HAT) for liver metastases in patients with metastatic breast cancer (MBC) has only been investigated in few studies.

Materials And Methods: Two phase II trials were initiated simultaneously to evaluate capecitabine in combination with oxaliplatin in patients with MBC and liver metastases. These two trials are reported together. Read More

View Article
December 2018

Mesenchymal Stem Cells Therapy Enhances the Efficacy of Pregabalin and Prevents Its Motor Impairment in Paclitaxel-Induced Neuropathy in Rats: Role of Notch1 Receptor and JAK/STAT Signaling Pathway.

Behav Brain Res 2018 Dec 10. Epub 2018 Dec 10.

Department of Pharmacology and Toxicology, Faculty of Pharmacy, Cairo University, Cairo, Egypt. Electronic address:

Peripheral neuropathy is a common adverse effect observed during use of paclitaxel (PTX) as chemotherapy. The present investigation was directed to estimate the modulatory effect of bone marrow derived mesenchymal stem cells (BM-MSCs) on pregabalin (PGB) treatment in PTX-induced peripheral neuropathy. Neuropathic pain was induced in rats by injecting PTX (2 mg/kg, i. Read More

View Article
December 2018

Treatment of an acute motor and sensory axonal neuropathy with propionate in a 33-year-old male.

Ther Adv Neurol Disord 2018 3;11:1756286418809580. Epub 2018 Nov 3.

Department of Neurology, Ruhr University Bochum, St. Josef-Hospital, Gudrunstrasse 56, Bochum 44791, Germany.

Background: The aim of this work was to report a case of an acute motor and sensory axonal neuropathy (AMSAN) treated with propionate to evaluate its therapeutic potential in AMSAN.

Materials And Methods: The patient was investigated by clinical examination, electroneurography, high-resolution nerve ultrasound and confocal corneal microscopy at baseline and the 2 month follow up. We compared the outcome with those of five other patients with acute motor axonal neuropathy (AMAN) and AMSAN of who were referred to our neurology department in the past 5 years. Read More

View Article
November 2018

Strategies for Targeted Delivery to the Peripheral Nerve.

Front Neurosci 2018 27;12:887. Epub 2018 Nov 27.

Audie L. Murphy VA Hospital, San Antonio, TX, United States.

Delivery of compounds to the peripheral nervous system has the potential to be used as a treatment for a broad range of conditions and applications, including neuropathic pain, regional anesthesia, traumatic nerve injury, and inherited and inflammatory neuropathies. However, efficient delivery of therapeutic doses can be difficult to achieve due to peripheral neuroanatomy and the restrictiveness of the blood-nerve barrier. Depending on the underlying integrity of the blood-nerve barrier in the application at hand, several strategies can be employed to navigate the peripheral nerve architecture and facilitate targeted delivery to the peripheral nerve. Read More

View Article
November 2018

Comment on: A British Ophthalmic Surveillance Unit (BOSU) study into dysthyroid optic neuropathy in the United Kingdom.

Eye (Lond) 2018 Dec 12. Epub 2018 Dec 12.

Thyroid Eye Disease Service, Department of Ophthalmology, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK.

View Article
December 2018

Simultaneous bilateral posterior ischemic optic neuropathy secondary to giant cell arteritis: a case presentation and review of the literature.

BMC Ophthalmol 2018 Dec 12;18(1):317. Epub 2018 Dec 12.

Neurology division, Department of Medicine, College of Medicine, King Saud University, Riyadh, Saudi Arabia.

Background: This report highlights a rare case of simultaneous bilateral blindness due to posterior ischemic optic neuropathy. Typically, ophthalmic involvement in giant cell arteritis is monocular or sequential ischemia of the anterior portion of the optic nerve, and less frequently simultaneous.

Case Presentation: An 80-year-old Saudi male came with a history of simultaneous bilateral vision loss 5 days prior to presentation. Read More

View Article
December 2018

Impact of impaired glucose regulation on the functions of large and small fibers of peripheral nerves

Turk J Med Sci 2018 12 12;48(6):1207-1213. Epub 2018 Dec 12.

Department of Neurology, Third Central Hospital of Tianjin, Tianjin, P.R. China

Background/aim: This article analyzes the incidence and characteristics of peripheral neuropathy in patients with impaired glucose regulation (IGR).

Materials And Methods: A total of 120 IGR patients and 60 healthy controls were enrolled. All subjects underwent nerve conduction study (NCS) of large fibers and skin sympathetic response (SSR) and contact heat pain evoked potential (CHEP) testing of small fibers with a Medtronic Keypoint machine (Medoc Ltd. Read More

View Article
December 2018

Diagnostic value of ultrasonography in peroneal neuropathy

Turk J Med Sci 2018 12 12;48(6):1115-1120. Epub 2018 Dec 12.

Department of Neurology, Faculty of Medicine, Ondokuz Mayıs University, Samsun, Turkey

Background/aim: Peroneal neuropathy at the fibular head (PNFH) is one of the most common entrapment neuropathies. Our aim in this study was to analyze the efficiency of ultrasonography in the diagnosis of PNFH.

Materials And Methods: The study included 15 peroneal nerves of 12 patients with PNFH and 24 peroneal nerves of 12 healthy controls. Read More

View Article
December 2018

Stroke recovery in rats after 24h-delayed, intramuscular neurotrophin-3 infusion.

Ann Neurol 2018 Dec 7. Epub 2018 Dec 7.

Neurorestoration Group, Wolfson Centre for Age-Related Diseases, King's College London, 16-18 Newcomen Street, London SE1 1UL, UK.

Objectives: Neurotrophin-3 (NT3) plays a key role in the development and function of locomotor circuits including descending serotonergic and corticospinal tract (CST) axons and afferents from muscle and skin. We have previously shown that gene therapy delivery of human NT3 into affected forelimb muscles improves sensorimotor recovery after stroke in adult and elderly rats. Here, to move towards the clinic, we tested the hypothesis that intramuscular infusion of NT3 protein could improve sensorimotor recovery after stroke. Read More

View Article
December 2018

Molecular Modulation of Osteoblasts and Osteoclasts in Type 2 Diabetes.

J Diabetes Res 2018 4;2018:6354787. Epub 2018 Nov 4.

Department of Health and Biomedical Sciences, College of Health Affairs, University of Texas Rio Grande Valley, 1201, W University Dr, Edinburg, TX 78539, USA.

Diabetes is a common disease affecting majority of populations worldwide. Since 1980, there has been an increase in the number of people diagnosed as prediabetic and diabetic. Diabetes is characterized by high levels of circulating glucose and leads to most microvascular and macrovascular complications such as retinopathy, nephropathy, neuropathy, stroke, and myocardial infarction. Read More

View Article
November 2018

Corneal Subbasal Nerve Plexus Changes in Severe Diabetic Charcot Foot Deformity: A Pilot Study in Search for a DNOAP Biomarker.

J Diabetes Res 2018 4;2018:5910639. Epub 2018 Nov 4.

Department of Ophthalmology, Rostock University Medical Center, Doberaner Straße 140, 18057 Rostock, Germany.

Introduction: Diabetic neuroosteoarthropathy (DNOAP) early symptoms are unspecific, mimicking general infectious symptoms and rendering a diagnosis challenging. Consequently, unfavourable outcomes occur frequently, with recurrent foot ulceration, infectious complications, and eventually amputation. Corneal confocal microscopy (CCM) of the subbasal nerve plexus (SNP) is used to detect early peripheral neuropathy in diabetic patients without diabetic retinopathy. Read More

View Article
November 2018

Optic Nerve Head Drusen: An Update.

Neuroophthalmology 2018 Dec 25;42(6):367-384. Epub 2018 Apr 25.

Surgery & Anaesthesia, University of Otago Wellington, Wellington, New Zealand.

Optic nerve head drusen are benign acellular calcium concretions that usually form early in life, just anterior to the lamina cribrosa. Improving imaging using optical coherence tomography suggests they are common and may be present in many clinically normal discs. These drusen may change in appearance in early life, but are generally stable in adulthood, and may be associated with visual field defects, anterior ischaemic optic neuropathy, or rarer complications. Read More

View Article
December 2018

The Pulfrich Phenomenon: Practical Implications of the Assessment of Cases and Effectiveness of Treatment.

Neuroophthalmology 2018 Dec 25;42(6):349-355. Epub 2018 Apr 25.

Department of Neuro-ophthalmology, Moorfields Eye Hospital, London, United Kingdom.

The Pulfrich phenomenon, originally described in normal observers, is a treatable disorder of the perception of movement in depth in cases of unilateral or asymmetric optic neuropathy. Treatment is highly bespoke and factors influencing treatment response and failure remain unclear. We assessed 25 adults with suspected Pulfrich phenomenon due to a range of conditions in two tertiary referral centres. Read More

View Article
December 2018

Targeted Mutation (R100W) of the Gene Encoding NGF Leads to Deficits in the Peripheral Sensory Nervous System.

Front Aging Neurosci 2018 13;10:373. Epub 2018 Nov 13.

Department of Neurosciences, University of California, San Diego, San Diego, CA, United States.

Nerve growth factor (NGF) exerts multifaceted functions through different stages of life. A missense mutation (R100W) in the beta-NGF gene was found in hereditary sensory autonomic neuropathy V (HSAN V) patients with severe loss of pain perception but without overt cognitive impairment. To better understand the pathogenesis of HSAN V, we generated the first NGF knock in mouse model for HSAN V. Read More

View Article
November 2018

The Role of Endogenous Neuroprotective Mechanisms in the Prevention of Retinal Ganglion Cells Degeneration.

Front Neurosci 2018 15;12:834. Epub 2018 Nov 15.

Chair and Department of Physiology, School of Medicine in Katowice, Medical University of Silesia, Katowice, Poland.

Retinal neurons are not able to undergo spontaneous regeneration in response to damage. A variety of stressors, i.e. Read More

View Article
November 2018

Increased IP-10 production by blood-nerve barrier in multifocal acquired demyelinating sensory and motor neuropathy and multifocal motor neuropathy.

J Neurol Neurosurg Psychiatry 2018 Dec 6. Epub 2018 Dec 6.

Department of Neurology and Clinical Neuroscience, Yamaguchi University Graduate School of Medicine, Ube, Japan

Objective: Dysfunction of the blood-nerve barrier (BNB) plays important roles in chronic inflammatory demyelinating polyneuropathy (CIDP) and multifocal motor neuropathy (MMN). The aim of the present study was to identify the candidate cytokines/chemokines that cause the breakdown of the BNB using sera from patients with CIDP and MMN.

Methods: We determined the levels of 27 cytokines and chemokines in human peripheral nerve microvascular endothelial cells (PnMECs) after exposure to sera obtained from patients with CIDP variants (typical CIDP and multifocal acquired demyelinating sensory and motor neuropathy [MADSAM]), MMN and amyotrophic lateral sclerosis (ALS), and healthy controls (HC), using a multiplexed fluorescent bead-based immunoassay system. Read More

View Article
December 2018

Retinopathy and RAAS Activation: Results From the Canadian Study of Longevity in Type 1 Diabetes.

Diabetes Care 2018 Dec 6. Epub 2018 Dec 6.

Division of Nephrology, Department of Medicine, University of Toronto, Toronto, Ontario, Canada.

Objective: The importance of renin-angiotensin-aldosterone system (RAAS) activation in retinopathy for long-standing diabetes is not well understood. We determined retinopathy stage and evaluated associations with other vascular complications before and after physiological RAAS activation in adults with long-standing (≥50 years duration) type 1 diabetes.

Research Design And Methods: Participants underwent retinal examination by digital funduscopic photography and optical coherence tomography and were classified as having nonproliferative diabetic retinopathy (NPDR), proliferative diabetic retinopathy (PDR), or no diabetic retinopathy (NDR) with or without diabetic macular edema (DME). Read More

View Article
December 2018
8.420 Impact Factor

General and Abdominal Obesity and Incident Distal Sensorimotor Polyneuropathy: Insights Into Inflammatory Biomarkers as Potential Mediators in the KORA F4/FF4 Cohort.

Diabetes Care 2018 Dec 6. Epub 2018 Dec 6.

German Center for Diabetes Research, München-Neuherberg, Germany.

Objective: To investigate the associations between different anthropometric measurements and development of distal sensorimotor polyneuropathy (DSPN) considering interaction effects with prediabetes/diabetes and to evaluate subclinical inflammation as a potential mediator.

Research Design And Methods: This study was conducted among 513 participants from the Cooperative Health Research in the Region of Augsburg (KORA) F4/FF4 cohort (aged 62-81 years). Anthropometry was measured at baseline. Read More

View Article
December 2018
7 Reads

Brentuximab vedotin with chemotherapy for CD30-positive peripheral T-cell lymphoma (ECHELON-2): a global, double-blind, randomised, phase 3 trial.

Lancet 2018 Dec 3. Epub 2018 Dec 3.

Universitätsmedizin Göttingen, Göttingen, Germany.

Background: Based on the encouraging activity and manageable safety profile observed in a phase 1 study, the ECHELON-2 trial was initiated to compare the efficacy and safety of brentuximab vedotin, cyclophosphamide, doxorubicin, and prednisone (A+CHP) versus cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) for the treatment of CD30-positive peripheral T-cell lymphomas.

Methods: ECHELON-2 is a double-blind, double-dummy, randomised, placebo-controlled, active-comparator phase 3 study. Eligible adults from 132 sites in 17 countries with previously untreated CD30-positive peripheral T-cell lymphomas (targeting 75% with systemic anaplastic large cell lymphoma) were randomly assigned 1:1 to receive either A+CHP or CHOP for six or eight 21-day cycles. Read More

View Article
December 2018

Femoral Nerve Palsy With Concomitant Patellar Dislocation in a Ballet Dancer.

Orthopedics 2018 Dec 11:1-3. Epub 2018 Dec 11.

Femoral nerve palsy with concomitant patellar dislocation is a rare clinical entity that has not previously been well documented. The authors present the case of a 16-year-old female ballet dancer who sustained a patellar dislocation with concomitant femoral nerve palsy. She experienced muscle weakness after the initial injury and developed neuropathic symptoms through the anterior left thigh. Read More

View Article
December 2018