6,936 results match your criteria Autonomic Neuropathy


Inotersen (transthyretin-specific antisense oligonucleotide) for treatment of transthyretin amyloidosis.

Neurodegener Dis Manag 2018 Dec 18. Epub 2018 Dec 18.

Ionis Pharmaceuticals, Carlsbad, CA, USA.

Hereditary  transthyretin amyloidosis (ATTR) is a fatal systemic disease that results from deposition of the misfolded protein transthyretin (TTR) in tissues. Common clinical manifestations of ATTR include peripheral neuropathy, cardiomyopathy, autonomic dysfunction, diarrhea and constipation. Historically there have not been effective therapies for this devastating disease. Read More

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http://dx.doi.org/10.2217/nmt-2018-0037DOI Listing
December 2018

Non-invasive evaluation of sudomotor function in patients with myasthenia gravis.

Neurophysiol Clin 2018 Dec 13. Epub 2018 Dec 13.

Department of Neurosciences and Mental Health, Hospital de Santa Maria-CHLN, avenue Professor-Egas-Moniz, 1648-028 Lisbon, Portugal; IMM and Institute of Physiology, Faculdade de Medicina, Universidade de Lisboa, Lisboa, Portugal.

Objectives: Myasthenia gravis (MG) is an autoimmune disease associated with antibodies against the nicotinic muscle acetylcholine receptor (AChR) at the neuromuscular junction. Dysautonomia has been previously described in MG. Electrochemical skin conductance (ESC), assessed by Sudoscan, is a non-invasive method that allows evaluation of sudomotor function. Read More

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http://dx.doi.org/10.1016/j.neucli.2018.12.002DOI Listing
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

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https://onlinelibrary.wiley.com/doi/abs/10.1111/jdi.12988
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http://dx.doi.org/10.1111/jdi.12988DOI Listing
December 2018
1 Read

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

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http://biomed.papers.upol.cz/doi/10.5507/bp.2018.073.html
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http://dx.doi.org/10.5507/bp.2018.073DOI Listing
December 2018
1 Read

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

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http://dx.doi.org/10.3389/fnagi.2018.00373DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6262302PMC
November 2018

Implications of the Intestinal Microbiota in Diagnosing the Progression of Diabetes and the Presence of Cardiovascular Complications.

J Diabetes Res 2018 12;2018:5205126. Epub 2018 Nov 12.

Department of Internal Medicine, "Grigore T. Popa" University of Medicine and Pharmacy, Iasi, Romania.

The prevalence of diabetes is steadily rising, and once it occurs, it can cause multiple complications with a negative impact on the whole organism. Complications of diabetes may be macrovascular: such as stroke and ischemic heart disease as well as peripheral vascular and microvascular diseases-retinopathy, nephropathy, and neuropathy. Key factors that cause cardiovascular disease in people with diabetes include hyperglycemia, dyslipidemia, obesity, insulin resistance, inflammation, hypertension, autonomic dysfunction, and decreased vascular response capacity. Read More

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http://dx.doi.org/10.1155/2018/5205126DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6260408PMC
November 2018
1 Read

Pulmonary Vein Ganglia Are Remodeled in the Diabetic Heart.

J Am Heart Assoc 2018 Dec;7(23):e008919

2 Department of Molecular Pharmacology and Physiology Morsani College of Medicine University of South Florida Tampa FL.

Background Cardiac autonomic neuropathy is thought to cause adverse cardiovascular effects in diabetes mellitus. Pulmonary vein ganglia ( PVG ), which have been implicated in normal and abnormal heart rhythm regulation, have not been fully investigated in type 1 diabetes mellitus (T1D). We examined the functional and anatomical effects of T1D on PVG and studied the details of T1D-induced remodeling on the PVG structure and function. Read More

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http://dx.doi.org/10.1161/JAHA.118.008919DOI Listing
December 2018
2 Reads

Neurosurgical issues of bariatric surgery: A systematic review of the literature and principles of diagnosis and treatment.

Clin Neurol Neurosurg 2018 Nov 13;176:34-40. Epub 2018 Nov 13.

Neurosurgery Unit, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy; Department of Clinical-Surgical, Diagnostic and Pediatric Sciences, University of Pavia, Italy.

Bariatric surgery is gaining popularity as the treatment of choice of morbid obesity since this condition is constantly increasing over the last decades. Several complications have emerged as the number of surgeries and follow-up data increase. No systematic review of the neurosurgery-related potential complications has been performed to date. Read More

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http://dx.doi.org/10.1016/j.clineuro.2018.11.009DOI Listing
November 2018
2 Reads

A novel nonsense mutation in WNK1/HSN2 associated with sensory neuropathy and limb destruction in four siblings of a large Iranian pedigree.

BMC Neurol 2018 Nov 29;18(1):195. Epub 2018 Nov 29.

Neuroscience Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran.

Background: Hereditary sensory and autonomic neuropathy type 2 (HSAN2) is an autosomal recessive disorder with predominant sensory dysfunction and severe complications such as limb destruction. There are different subtypes of HSAN2, including HSAN2A, which is caused by mutations in WNK1/HSN2 gene.

Methods: An Iranian family with four siblings and autosomal recessive inheritance pattern whom initially diagnosed with HSAN2 underwent whole exome sequencing (WES) followed by segregation analysis. Read More

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http://dx.doi.org/10.1186/s12883-018-1201-6DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6262971PMC
November 2018
1 Read

Diabetic Neuropathic Cachexia in a Young Woman.

Korean J Fam Med 2018 Nov 30. Epub 2018 Nov 30.

School of Medical Science, Universiti Sains Malaysia Health Campus, Kubang Kerian, Malaysia.

Profound weight loss with painful symmetrical peripheral neuropathy in diabetic patients was first described as diabetic neuropathic cachexia more than 4 decades ago. It is a distinct type of diabetic peripheral neuropathy that occurs in the absence of other microvascular and autonomic complications of diabetes. The mechanism and precipitating cause are unknown. Read More

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http://dx.doi.org/10.4082/kjfm.17.0127DOI Listing
November 2018
2 Reads

7,8-Dihydroxyflavone Protects High Glucose-Damaged Neuronal Cells against Oxidative Stress.

Biomol Ther (Seoul) 2018 Nov 27. Epub 2018 Nov 27.

Jeju National University School of Medicine and Jeju Research Center for Natural Medicine, Jeju 63243, Republic of Korea.

Oxidative stress is considered a major contributor in the pathogenesis of diabetic neuropathy and in diabetes complications, such as nephropathy and cardiovascular diseases. Diabetic neuropathy, which is the most frequent complications of diabetes, affect sensory, motor, and autonomic nerves. This study aimed to investigate whether 7,8-dihydroxyflavone (7,8-DHF) protects SH-SY5Y neuronal cells against high glucose-induced toxicity. Read More

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http://dx.doi.org/10.4062/biomolther.2018.202DOI Listing
November 2018
1 Read

Patisiran, an RNAi therapeutic for the treatment of hereditary transthyretin-mediated amyloidosis.

Neurodegener Dis Manag 2018 Nov 27. Epub 2018 Nov 27.

Amyloidosis Research & Treatment Centre, Fondazione IRCCS Policlinico San Matteo, Pavia, 27100, Italy.

Hereditary transthyretin-mediated amyloidosis is a rapidly progressive, heterogeneous disease caused by the accumulation of misfolded transthyretin protein as amyloid fibrils at multiple sites, and is characterized by peripheral sensorimotor neuropathy, autonomic neuropathy and/or cardiomyopathy. Current treatment options have limited efficacy and often do not prevent disease progression. Patisiran is a novel RNA interference therapeutic that specifically reduces production of both wild-type and mutant transthyretin protein. Read More

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http://dx.doi.org/10.2217/nmt-2018-0033DOI Listing
November 2018
1 Read

Autonomic dysfunction and HPV immunization: an overview.

Immunol Res 2018 Nov 27. Epub 2018 Nov 27.

Rheumatology Department, National Institute of Cardiology, Mexico City, Mexico.

This article reviews the case series reported from several countries describing patients with suspected severe side effects to the HPV vaccines. The described symptom clusters are remarkably similar and include disabling fatigue, headache, widespread pain, fainting, gastrointestinal dysmotility, limb weakness, memory impairment episodes of altered awareness, and abnormal movements. This constellation of symptoms and signs has been labeled with different diagnoses such as complex regional pain syndrome (CRPS), postural orthostatic tachycardia syndrome (POTS), small fiber neuropathy (SFN), myalgic encephalomyelitis/chronic fatigue syndrome (ME/CFS), or fibromyalgia. Read More

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http://dx.doi.org/10.1007/s12026-018-9036-1DOI Listing
November 2018
1 Read

The prevalence of cardiovascular autonomic neuropathy and its influence on post induction hemodynamic variables in patients with and without diabetes; A prospective cohort study.

PLoS One 2018 26;13(11):e0207384. Epub 2018 Nov 26.

Department of Anesthesiology, Amsterdam UMC, University of Amsterdam, Amsterdam, the Netherlands.

Background: Cardiovascular autonomic neuropathy (CAN) is a known complication of diabetes, but is also diagnosed in patients without diabetes. CAN may be related to perioperative hemodynamic instability. Our objective was to investigate if patients with diabetes would have a higher prevalence of CAN compared to patients without diabetes undergoing surgery. Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0207384PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6261040PMC
November 2018
1 Read

Rituximab treatment in seronegative autoimmune autonomic neuropathy and autoimmune autonomic ganglionopathy: Case-report and literature review.

J Neuroimmunol 2019 Jan 14;326:28-32. Epub 2018 Nov 14.

Department of Internal Medicine, Infectious Diseases, and Clinical Immunology, Reims Teaching Hospitals, Reims, France; University of Reims Champagne-Ardenne, EA-4684 / SFR CAP-SANTE, Reims F-51095, France. Electronic address:

Background And Purpose: Autoimmune autonomic ganglionopathy (AAG) is a rare disease with no well-established treatment. Until recently, AAG could be seropositive (50 to 60% of patients) or seronegative for ganglionic (α3-type) nicotinic acetylcholine receptor (Gα3NAChR) antibodies. In early 2018, the two forms of the disease were distinguished, separating seropositive from seronegative ones, designating this latter form "seronegative autoimmune autonomic neuropathy" (SAAN). Read More

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http://dx.doi.org/10.1016/j.jneuroim.2018.11.009DOI Listing
January 2019
8 Reads

Prevalence of Diabetic Neuropathy in Young Adults with Type 1 Diabetes and the Association with Insulin Pump Therapy.

Diabetes Technol Ther 2018 Nov 21. Epub 2018 Nov 21.

1 Department of Clinical Epidemiology, Steno Diabetes Center Copenhagen , Gentofte, Denmark .

Aims: The aim was to investigate the prevalence of diabetic sensorimotor polyneuropathy (DSPN) and cardiovascular autonomic neuropathy (CAN) in a Danish population of young adults with type 1 diabetes (T1D) using both established and novel measuring modalities. Furthermore, to investigate the association between continuous subcutaneous insulin infusion (CSII) treatment and these complications.

Materials And Methods: CAN was assessed by cardiovascular autonomic reflex tests. Read More

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http://dx.doi.org/10.1089/dia.2018.0249DOI Listing
November 2018
1 Read

Novel peptides derived from neuropeptide Y prevent chemotherapy-induced bone marrow damage by regulating hematopoietic stem cell microenvironment.

Anim Cells Syst (Seoul) 2018 5;22(5):281-288. Epub 2018 Sep 5.

Stem Cell Neuroplasticity Research Group, Kyungpook National University, Daegu, South Korea.

Chemotherapy-induced bone marrow damage is accompanied by acute nerve injury in the bone marrow (BM), resulting in sensory and autonomic neuropathy. Cisplatin, a popular chemotherapy drugs, induces the impairment of hematopoietic stem cells (HSCs) and bone marrow regeneration, leading to chronic bone marrow abnormalities. Previously, we reported the protective roles of neuropeptide Y (NPY) against cisplatin-induced bone marrow impairment. Read More

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http://dx.doi.org/10.1080/19768354.2018.1517826DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6171453PMC
September 2018
6 Reads

Thermoregulation in neuropathies.

Authors:
Robert D Fealey

Handb Clin Neurol 2018 ;157:777-787

Department of Neurology, Mayo Clinic, Rochester, MN, United States. Electronic address:

Peripheral neuropathy affecting autonomic and small sensory fibers can cause abnormalities of both autonomic and behavioral thermoregulation. Quantitative autonomic and sensory neurophysiologic tests and quantification of the linear density of intraepidermal nerve fibers potentially can stratify those at risk of impaired thermoregulation during cold and heat challenges. New data relating to thermoregulatory sweating impairment in neuropathy are presented in this chapter. Read More

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http://dx.doi.org/10.1016/B978-0-444-64074-1.00048-3DOI Listing
January 2018
8 Reads

Thermoregulation in Parkinson disease.

Handb Clin Neurol 2018 ;157:715-725

Department of Neurology, Mayo Clinic, Rochester, MN, United States.

Autonomic dysfunction in Parkinson disease encompasses thermoregulatory symptoms and was first noted by Gowers in the late 19th century when he described abnormal temperature sensation and sweating in Parkinson disease patients. These thermoregulatory symptoms became more recognized in the postlevodopa era when Parkinson disease patients were more readily tested with objective autonomic function tests. Objective thermoregulatory testing in Parkinson disease reveals deficits of sweating and vasomotor tone which often correlate with the severity of other autonomic deficits. Read More

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http://dx.doi.org/10.1016/B978-0-444-64074-1.00043-4DOI Listing
January 2018
3 Reads

Cochlear dysfunction and microvascular complications in patients with type 1 diabetes mellitus.

Diabetol Metab Syndr 2018 9;10:81. Epub 2018 Nov 9.

Endocrinology Division - Programa de Pós-Graduação em Oncologia e Ciências Médicas, University Hospital João de Barros Barreto, Federal University of Pará, Mundurucus Street, 4487, Guamá, Belém, PA Postal Code: 66073-000 Brazil.

Sensorineural hearing impairment has been associated with DM, and it is probably linked to the same pathophysiological mechanisms as well-established in microvascular diabetes complications. The study of otoacoustic emissions (OAEs) is useful to identify subclinical cochlear dysfunction. Therefore, the aim of this study was to evaluate the association between abnormal OAEs responses, diabetic kidney disease (DKD) and diabetic cardiac autonomic neuropathy (CAN). Read More

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https://dmsjournal.biomedcentral.com/articles/10.1186/s13098
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http://dx.doi.org/10.1186/s13098-018-0380-zDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6230237PMC
November 2018
12 Reads

Effects of Severe Hypoglycemia on Cardiovascular Outcomes and Death in the Veterans Affairs Diabetes Trial.

Diabetes Care 2018 Nov 19. Epub 2018 Nov 19.

U.S. Department of Veterans Affairs, Washington, DC.

Objective: To determine the risk factors for severe hypoglycemia and the association between severe hypoglycemia and serious cardiovascular adverse events and cardiovascular and all-cause mortality in the Veterans Affairs Diabetes Trial (VADT).

Research Design And Methods: This post hoc analysis of data from the VADT included 1,791 military veterans (age 60.5 ± 9. Read More

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http://dx.doi.org/10.2337/dc18-1144DOI Listing
November 2018
2 Reads

[Emphysematous cystitis in a patient with type-1diabetes mellitus - diagnostic difficulties].

Wiad Lek 2018 ;71(7):1424-1428

Katedra I Zakład Medycyny Rodzinnej, Warszawski Uniwersytet Medyczny, Warszawa, Polska.

A case report of 28 year old female with medical history of bed controlled type 1 diabetes mellitus complicated by autonomic neuropathy in the form of gastroparesis, suffered by emphysematous cystitis caused by Escherichia coli was described. Emphysematous cystitis is a rare urinary tract infection connected with the presence of gas in the bladder lumen or/and within the bladder wall, which occurs mainly in women, in older age, suffering from type 2 diabetes, complicated by microangiopathy, neuropathy, with urinary tract obstruction and weakness of immunity system. Diagnostic difficulties and the delay in correct diagnosis in described case were caused by the dominated complaint of the upper gastrointestinal tract and difficulties in interpretation of imaging methods, such as abdominal X-ray and ultrasound scan. Read More

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January 2018
5 Reads

The association of diabetic microvascular and macrovascular disease with cutaneous circulation in patients with type 2 diabetes mellitus.

J Diabetes Complications 2018 Oct 12. Epub 2018 Oct 12.

Diabetes Center, First Department of Propaedeutic Internal Medicine, Medical School, National and Kapodistrian University of Athens, Laiko General Hospital, Athens, Greece. Electronic address:

Aims: To study the impact of diabetic neuropathy, both peripheral sensorimotor (DPN) and cardiac autonomic neuropathy (CAN), on transcutaneous oxygen tension (TcPO) in patients with type 2 diabetes mellitus (T2DM).

Methods: A total of 163 participants were recruited; 100 with T2DM and 63 healthy individuals. Peripheral arterial disease (PAD) was defined as ankle-brachial index (ABI) values ≤0. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S10568727183039
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http://dx.doi.org/10.1016/j.jdiacomp.2018.10.008DOI Listing
October 2018
7 Reads

Decreased β-Cell Function is Associated with Cardiovascular Autonomic Neuropathy in Chinese Patients Newly Diagnosed with Type 2 Diabetes.

Neurosci Bull 2018 Nov 15. Epub 2018 Nov 15.

Department of Endocrinology and Metabolism, Guangdong Provincial Key Laboratory of Diabetology, Third Affiliated Hospital of Sun Yat-sen University, Guangzhou, 510630, China.

The influence of β-cell function on cardiovascular autonomic neuropathy (CAN), an important diabetes-related complication, is still unclear. In this study, we aimed to investigate the association between residual β-cell function and CAN in patients newly diagnosed with type 2 diabetes. We enrolled 90 newly-diagnosed type 2 diabetic patients and 37 participants with normal glucose tolerance as controls. Read More

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http://link.springer.com/10.1007/s12264-018-0304-9
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http://dx.doi.org/10.1007/s12264-018-0304-9DOI Listing
November 2018
3 Reads

Temperature as a Causative Factor in Diabetic Foot Ulceration: A Call to Revisit Ulcer Pathomechanics.

J Am Podiatr Med Assoc 2018 Nov 14. Epub 2018 Nov 14.

University of North Texas Health Science Center.

Background: Diabetic foot ulcers are a major burden to patients and to the healthcare systems of many countries. To prevent and/or treat ulcers more effectively, predictive biomarkers are needed. We examined temperature as a biomarker and as a causative factor in ulcer development. Read More

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http://www.japmaonline.org/doi/10.7547/17-131
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http://dx.doi.org/10.7547/17-131DOI Listing
November 2018
7 Reads

V144D Mutation of Can Present with Both Painful and Painless Phenotypes in Hereditary Sensory and Autonomic Neuropathies Type I.

Case Rep Genet 2018 18;2018:1898151. Epub 2018 Oct 18.

Department of Neurology, University of Florida, USA.

Hereditary sensory and autonomic neuropathy type I (HSAN I) is an autosomal dominant disease characterized by distal sensory loss, pain insensitivity, and autonomic disturbances. The major underlying causes of HSAN I are point mutations in the SPTLC1 gene. Patients with mutations in the SPTLC1 genes typically exhibit dense sensory loss and incidence of lancinating pain. Read More

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https://www.hindawi.com/journals/crig/2018/1898151/
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http://dx.doi.org/10.1155/2018/1898151DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6211148PMC
October 2018
3 Reads

Integrated treatment for autonomic paraneoplastic syndrome improves performance status in a patient with small lung cell carcinoma: a case report.

BMC Neurol 2018 Nov 10;18(1):189. Epub 2018 Nov 10.

Department of Neurology, Aomori Prefectural Central Hospital, 2-1-1 Higashi-Tsukurimichi, Aomori, 030-8551, Japan.

Background: Paraneoplastic neurological syndromes (PNS) are rare disorders associated with cancer and are believed to be immune mediated. Patients with autonomic PNS suffer from variable combinations of parasympathetic and sympathetic failure. Autonomic PNS are usually associated with other PNS, such as encephalomyelitis and sensory neuropathy; however, autonomic symptoms may rarely manifest as PNS symptoms. Read More

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https://bmcneurol.biomedcentral.com/articles/10.1186/s12883-
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http://dx.doi.org/10.1186/s12883-018-1192-3DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6230283PMC
November 2018
6 Reads

Cardiovascular Autonomic Neuropathy and its Association with Cardiovascular and All-cause Mortality in Patients with End-stage Renal Disease.

Cureus 2018 Aug 31;10(8):e3243. Epub 2018 Aug 31.

Internal Medicine, Allama Iqbal Medical College/Jinnah Hospital, Lahore, PAK.

Background End-stage renal disease frequently leads to increased cardiovascular mortality. Cardiovascular autonomic neuropathy (CAN) may be predictive of cardiac arrhythmias and sudden cardiac death in patients with end-stage renal disease. Methods A total of 70 patients with end-stage renal disease were included in the study. Read More

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http://dx.doi.org/10.7759/cureus.3243DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6214649PMC
August 2018
1 Read

Co-occurrence of early diabetes-related complications in adolescents and young adults with type 1 diabetes: an observational cohort study.

Lancet Child Adolesc Health 2019 Jan 6;3(1):35-43. Epub 2018 Nov 6.

Department of Pediatrics, University of Colorado School of Medicine, Aurora, CO, USA; Department of Epidemiology, Colorado School of Public Health, Aurora, CO, USA.

Background: One in three adolescents and young adults with type 1 diabetes have at least one early diabetes-related complication or comorbidity. We aimed to examine the prevalence and pattern of co-occurring complications in this population, as well as the related risk factors.

Methods: This observational cohort study includes data from individuals diagnosed with type 1 diabetes before age 20 years who participated in the SEARCH for Diabetes in Youth Study across five sites in the USA. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S23524642183030
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http://dx.doi.org/10.1016/S2352-4642(18)30309-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6295346PMC
January 2019
10 Reads

Impaired nocturnal blood pressure dipping in patients with type 2 diabetes mellitus.

Hypertens Res 2019 Jan 6;42(1):59-66. Epub 2018 Nov 6.

Laboratory for Clinical Cardiovascular Physiology, Department of Medical Biology, Amsterdam Cardiovascular Sciences, Amsterdam University Medical Centers, location Academic Medical Center, Amsterdam, The Netherlands.

Hypertension is a common comorbidity of type 2 diabetes mellitus (T2DM). Both conditions are associated with an increased cardiovascular risk, which is reduced by tight blood pressure (BP) and glycemic control. However, nondipping BP status continues to be an enduring cardiovascular risk factor in T2DM. Read More

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http://www.nature.com/articles/s41440-018-0130-5
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http://dx.doi.org/10.1038/s41440-018-0130-5DOI Listing
January 2019
4 Reads

Cardiovascular Autonomic Neuropathy as a New Complication of Postsurgical Chronic Hypoparathyroidism.

J Bone Miner Res 2018 Nov 5. Epub 2018 Nov 5.

Unit of Endocrinology and Diabetes, University Campus Bio-Medico, Rome, Italy.

Postsurgical hypoparathyroidism (hypoPT) increases fatigue and seems to affect the risk of mortality. Cardiovascular autonomic neuropathy (CAN) is an impairment of the cardiovascular autonomic system, a cause of increased mortality, and associated with increased fatigability. The aim of this study is to evaluate CAN in hypoPT and its relationship with hypocalcemia, PTH levels, and hyperphosphatemia. Read More

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http://doi.wiley.com/10.1002/jbmr.3623
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http://dx.doi.org/10.1002/jbmr.3623DOI Listing
November 2018
3 Reads

Painless Nerve Growth Factor: A TrkA biased agonist mediating a broad neuroprotection via its actions on microglia cells.

Pharmacol Res 2018 Nov 1;139:17-25. Epub 2018 Nov 1.

Bio@SNS Laboratory of Biology, Scuola Normale Superiore, Pisa, Italy; Section of Human Physiology, Department of Biomedical and Specialty Surgical Sciences, University of Ferrara, Ferrara, Italy.

Nerve Growth Factor (NGF) is a therapeutic candidate for Alzheimer's disease, based on its well known actions on basal forebrain cholinergic neurons. However, because of its pro-nociceptive activity, in current clinical trials NGF has to be administered intraparenchymally into the brain by neurosurgery via cell or gene therapy approaches. To prevent the NGF pain-inducing collateral effects, thus avoiding the necessity for local brain injection, we developed painless NGF (hNGFp), based on the human genetic disease Hereditary Sensory and Autonomic Neuropathy type V (HSAN V). Read More

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https://linkinghub.elsevier.com/retrieve/pii/S10436618183128
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http://dx.doi.org/10.1016/j.phrs.2018.10.028DOI Listing
November 2018
4 Reads

IGHMBP2 mutation associated with organ-specific autonomic dysfunction.

Neuromuscul Disord 2018 Dec 29;28(12):1012-1015. Epub 2018 Aug 29.

MRC Centre for Neuromuscular Diseases, National Hospital for Neurology and Neurosurgery and UCL Institute of Neurology, Queen Square, London WC1N 3AR, UK. Electronic address:

Biallelic mutations in the IGHMBP2 have been associated with two distinct phenotypes: spinal muscular atrophy with respiratory distress type 1 (SMARD1) and CMT2S. We describe a patient who developed progressive muscle weakness and wasting in her upper and lower limbs from infancy. She developed respiratory involvement at age 9, eventually requiring 24-h non-invasive ventilation, and severe autonomic dysfunction restricted to the gastrointestinal tract. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S09608966173155
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http://dx.doi.org/10.1016/j.nmd.2018.08.010DOI Listing
December 2018
2 Reads

Generation of the human induced pluripotent stem cell line UKWNLi002-A from dermal fibroblasts of a woman with a heterozygous c.608 C>T (p.Thr203Met) mutation in exon 3 of the nerve growth factor gene potentially associated with hereditary sensory and autonomic neuropathy type 5.

Stem Cell Res 2018 Dec 12;33:171-174. Epub 2018 Oct 12.

Department of Neurology, University of Würzburg, Würzburg, Germany. Electronic address:

In this study, we report the human induced pluripotent stem cell line (iPSC) HSAN5-T203 M-iPSC, generated from human dermal fibroblasts (HDF) of a woman carrying a heterozygous c.608C > T mutation in the nerve growth factor gene potentially associated with hereditary sensory and autonomic neuropathy type 5 (HSAN5). HDF were reprogrammed using transient expression of key transcription factors for pluripotency and immune evasion via transfection of synthetic mRNA and miRNA. Read More

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http://dx.doi.org/10.1016/j.scr.2018.10.017DOI Listing
December 2018

Cardiovascular autonomic neuropathy and falls in Parkinson disease: a prospective cohort study.

J Neurol 2018 Oct 31. Epub 2018 Oct 31.

Department of Neuroscience Rita Levi Montalcini, University of Turin, via Cherasco 15, 10124, Torino, Italy.

Background: Falls represent one of the main complications of Parkinson's disease (PD), significantly lowering quality of life. Cardiovascular autonomic neuropathy (cAN) is one of the key contributing factors to PD-associated falls. However, a direct quantification of its impact on the risk of falling in PD is still lacking. Read More

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http://link.springer.com/10.1007/s00415-018-9104-4
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http://dx.doi.org/10.1007/s00415-018-9104-4DOI Listing
October 2018
6 Reads

A clinical and electrophysiological study of peripheral neuropathies in peritoneal dialysis patients: Our experience from rural South India.

Saudi J Kidney Dis Transpl 2018 Sep-Oct;29(5):1139-1149

Department of Neurology, Sri Venkateswara Institute of Medical Sciences, Tirupati, Andhra Pradesh, India.

The objective was to study the prevalence, clinical features, electrophysiological features, and severity of peripheral neuropathy in chronic kidney disease (CKD) patients on peritoneal dialysis (PD) and effect of the presence of diabetes mellitus (DM). Between May 2015 and December 2016, 100 CKD patients on PD were assessed. The prevalence of peripheral neuropathy was 65% based on clinical symptoms and 92% based on electrophysiological parameters. Read More

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http://www.sjkdt.org/text.asp?2018/29/5/1139/243942
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http://dx.doi.org/10.4103/1319-2442.243942DOI Listing
November 2018
5 Reads

Editorial: Autonomic Neuropathy and Cardiovascular Disease in Aging.

Authors:
A Shams J E Morley

J Nutr Health Aging 2018 ;22(9):1028-1033

John E. Morley, MB,BCh, Division of Geriatric Medicine, Saint Louis University School of Medicine, 1402 S. Grand Blvd., M238, St. Louis, MO 63104, Email:  .

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http://dx.doi.org/10.1007/s12603-018-1097-2DOI Listing
January 2018

Imaging cardiac innervation in hereditary transthyretin (ATTRm) amyloidosis: A marker for neuropathy or cardiomyopathy in case of heart failure?

J Nucl Cardiol 2018 Oct 29. Epub 2018 Oct 29.

Department of Nuclear Medicine and Molecular Imaging, Medical Imaging Center, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands.

Background: Nuclear imaging modalities using Iodine-metaiodobenzylguanidine (I-MIBG) and bone seeking tracers identify early cardiac involvement in ATTRm amyloidosis patients. However, little is known whether results from I-MIBG scintigraphy actually correlate to markers for either cardiac autonomic neuropathy or cardiomyopathy.

Methods: All TTR mutation carriers and ATTRm patients who underwent both I-MIBG and Technetium-hydroxymethylene diphosphonate (Tc-HDP) scintigraphy were included. Read More

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http://link.springer.com/10.1007/s12350-018-01477-y
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http://dx.doi.org/10.1007/s12350-018-01477-yDOI Listing
October 2018
2 Reads

Molecular diagnosis of inherited peripheral neuropathies by targeted next-generation sequencing: molecular spectrum delineation.

BMJ Open 2018 Oct 28;8(10):e021632. Epub 2018 Oct 28.

Département de génétique médicale, Hôpital Timone enfants, Assistance Publique Hôpitaux de Marseille, Marseille, France.

Purpose: Inherited peripheral neuropathies (IPN) represent a large heterogenous group of hereditary diseases with more than 100 causative genes reported to date. In this context, targeted next-generation sequencing (NGS) offers the opportunity to screen all these genes with high efficiency in order to unravel the genetic basis of the disease. Here, we compare the diagnostic yield of targeted NGS with our previous gene by gene Sanger sequencing strategy. Read More

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http://bmjopen.bmj.com/lookup/doi/10.1136/bmjopen-2018-02163
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http://dx.doi.org/10.1136/bmjopen-2018-021632DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6224714PMC
October 2018
7 Reads

Catechin attenuates diabetic autonomic neuropathy in streptozotocin induced diabetic rats.

Biomed Pharmacother 2018 Dec 9;108:1517-1523. Epub 2018 Oct 9.

Shobhaben Pratapbhai Patel School of Pharmacy & Technology Management, SVKM's NMIMS, V. L. Mehta Road, Vile Parle (W), Mumbai, 400056, India.

Diabetic autonomic neuropathy (DAN) is one of the least understood and attended complications which affects the quality of life. Increased oxidative stress, advanced glycation end products (AGEs), overactivation of protein kinase C (PKC) and reduced myoinositol content in autonomic nerves are major pathological conditions responsible for DAN. Limited treatment options appeal researchers to search for alternative treatments for DAN. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S07533322183525
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http://dx.doi.org/10.1016/j.biopha.2018.09.179DOI Listing
December 2018
5 Reads

Recessive mutations in the neuronal isoforms of DST, encoding dystonin, lead to abnormal actin cytoskeleton organization and HSAN type VI.

Hum Mutat 2019 Jan 18;40(1):106-114. Epub 2018 Nov 18.

Laboratory of Molecular and Cell Biology, Istituto Dermopatico dell'Immacolata, IDI-IRCCS, Rome, Italy.

Hereditary sensory and autonomic neuropathies (HSAN) are clinically and genetically heterogeneous disorders, characterized by a progressive sensory neuropathy often complicated by ulcers and amputations, with variable motor and autonomic involvement. Several pathways have been implicated in the pathogenesis of neuronal degeneration in HSAN, while recent observations point to an emerging role of cytoskeleton organization and function. Here, we report novel biallelic mutations in the DST gene encoding dystonin, a large cytolinker protein of the plakin family, in an adult form of HSAN type VI. Read More

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http://doi.wiley.com/10.1002/humu.23678
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http://dx.doi.org/10.1002/humu.23678DOI Listing
January 2019
5 Reads
5.144 Impact Factor

Surgical treatment for chronic Charcot neuroarthropathy.

Foot (Edinb) 2018 Sep 17;36:59-66. Epub 2018 Feb 17.

Università Cattolica del Sacro Cuore, Italy. Electronic address:

Charcot Neuro-arthropathy (CN) is a condition characterized by a progressive derangement of the joints, in individuals affected with sensitive and autonomic neuropathy. The pathogenesis of CN is multifactorial as neuropathy is a necessary, but insufficient condition for the onset of the disease. The most important indication for surgical treatment of Charcot foot is a severe deformity that compromises the functionality of the limb, causing a high risk for ulceration, infection and amputation. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S09582592173013
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http://dx.doi.org/10.1016/j.foot.2018.02.001DOI Listing
September 2018
5 Reads

Brazilian consensus for diagnosis, management and treatment of transthyretin familial amyloid polyneuropathy.

Arq Neuropsiquiatr 2018 Sep;76(9):609-621

Universidade Federal do Rio de Janeiro, Hospital Universitário Clementino Fraga Filho, Centro de Estudos em Paramiloidose Antônio Rodrigues de Mello, Rio de Janeiro RJ, Brasil.

Transthyretin familial amyloid polyneuropathy is an autosomal dominant inherited sensorimotor and autonomic polyneuropathy, which if untreated, leads to death in approximately 10 years. In Brazil, liver transplant and tafamidis are the only disease-modifying treatments available. This review consists of a consensus for the diagnosis, management and treatment for transthyretin familial amyloid polyneuropathy from the Peripheral Neuropathy Scientific Department of the Brazilian Academy of Neurology. Read More

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http://www.scielo.br/scielo.php?script=sci_arttext&pid=S
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http://dx.doi.org/10.1590/0004-282X20180094DOI Listing
September 2018
11 Reads

Parkinson's Disease and Current Treatments for Its Gastrointestinal Neurogastromotility Effects.

Curr Treat Options Gastroenterol 2018 Dec;16(4):489-510

Carolinas Healthcare-Charlotte, UNC School of Medicine, Charlotte Campus, 1025 Morehead Medical Dr. Suite 300, Charlotte, NC, 28204, USA.

Purpose Of Review: Gastrointestinal disturbances are seen in nearly all patients with Parkinson's disease and lead to impaired quality of life, affect drug pharmacodynamics, and potentially worsen patient's existing motor fluctuations, leading to further disability. Recent evidence links abnormal accumulations of α-synuclein aggregates in the periphery (gut) as seen in the cortex which causes dysfunctions impacting every level of the gastrointestinal tract from the esophagus, to the stomach, small bowel, colon, and rectum and can even predate the onset of the central neurologic disorder itself. Many treatments exist for the clinical phenotypes that result from the autonomic dysfunction and neuropathy involved in this neurodegenerative disorder. Read More

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http://dx.doi.org/10.1007/s11938-018-0201-3DOI Listing
December 2018
6 Reads

Blood Pressure Variability and Autonomic Dysfunction.

Curr Diab Rep 2018 Oct 25;18(12):137. Epub 2018 Oct 25.

Endocrinology, Department of Systems Medicine, University of Rome Tor Vergata, Via Montpellier, 1, 00133, Rome, Italy.

Purpose Of Review: This review considers the relationship between abnormal blood pressure (BP) variability and autonomic dysfunction through an attempt to answer questions about its clinical relevance and pertinence to diabetes and cardiovascular autonomic neuropathy (CAN) and which therapeutic measures can lessen its cardiovascular impact.

Recent Findings: Office, ambulatory, and home BP monitoring identify posture-related, circadian, short-term, and long-term BP variabilities. Abnormal BP variability is a risk marker for organ damage, mortality, and cardiovascular events. Read More

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http://link.springer.com/10.1007/s11892-018-1108-z
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http://dx.doi.org/10.1007/s11892-018-1108-zDOI Listing
October 2018
7 Reads

Familial amyloid polyneuropathy with chronic paroxysmal dry cough in Mainland China: A Chinese family with a proven heterozygous missense mutation c.349G>T in the transthyretin gene.

J Clin Neurosci 2018 Oct 22. Epub 2018 Oct 22.

Department of Neurology, Xiangya Hospital, Central South University, Changsha, China; National Clinical Research Center for Geriatric Disorders, Central South University, Changsha, China; Key Laboratory of Hunan Province in Neurodegenerative Disorders, Central South University, Changsha, China; Key Laboratory of Organ Injury, Aging and Regenerative Medicine of Hunan Province, Changsha, China. Electronic address:

Familial amyloid polyneuropathy (FAP) is a rare autosomal dominant disorder characterized by amyloid accumulation in the peripheral nerves and other organs, including the heart, kidney, and eyes. So far, no case with FAP from Mainland China was reported with a heterozygous missense mutation c.349G>T in the Transthyretin (TTR) gene. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S09675868183142
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http://dx.doi.org/10.1016/j.jocn.2018.10.040DOI Listing
October 2018
7 Reads

Classical Hodgkin Lymphoma Presenting with Severe, Recurrent Hypothermic Episodes.

Case Rep Hematol 2018 25;2018:3726593. Epub 2018 Sep 25.

Department of Hematology, Aarhus University Hospital, Tage-Hansens Gade 2, DK-8000 Aarhus, Denmark.

We report a case presenting with recurrent episodes of severe hypothermia preceding the diagnosis of Hodgkin lymphoma. The episodes of hypothermia were accompanied by other symptoms of autonomic dysfunction, mainly hypotension, which could be caused by autonomic neuropathy as part of a paraneoplastic syndrome. In comparison with previous reports describing an association between the presence of hypothermia and an adverse outcome, the present patient has responded well to lymphoma-specific treatment and is currently in an ongoing complete remission. Read More

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https://www.hindawi.com/journals/crihem/2018/3726593/
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http://dx.doi.org/10.1155/2018/3726593DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6176302PMC
September 2018
12 Reads

Left ventricular remodelling and cardiac chamber sizes in long-term, normoalbuminuric type 1 diabetes patients with and without cardiovascular autonomic neuropathy.

J Diabetes Complications 2018 Oct 11. Epub 2018 Oct 11.

Department of Cardiology, Rigshospitalet, Copenhagen University Hospital, Denmark; Department of Radiology, Rigshospitalet, Copenhagen University Hospital, Denmark.

Aims: Type 1 diabetes is associated with increased cardiovascular (CV) morbidity and mortality, and cardiovascular autonomic neuropathy (CAN) is an important CV risk factor. The study aimed to explore associations between CAN and altered cardiac chamber sizes in persons with type 1 diabetes.

Methods: This was a cross-sectional study of 71 asymptomatic, normoalbuminuric participants with long-term type 1 diabetes (39 with CAN, determined by >1 abnormal autonomic function test) examined with cardiac multi detector computed tomography scans, which allowed measurements of left ventricular mass and all four cardiac chamber volumes. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S10568727183030
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http://dx.doi.org/10.1016/j.jdiacomp.2018.10.007DOI Listing
October 2018
5 Reads

Upper limb onset of hereditary transthyretin amyloidosis is common in non-endemic areas.

Eur J Neurol 2018 Oct 23. Epub 2018 Oct 23.

Centre National de Référence pour la Neuropathie Amyloïde Familiale, CHU Bicêtre, Assistance Publique Hôpitaux de Paris, Le Kremlin- Bicêtre, France.

Background And Purpose: The aim is to describe an uncommon phenotype of hereditary ATTR neuropathy with upper limb onset.

Methods: The French TTR Familial Amyloid Polyneuropathy database was used for a retrospective evaluation of 32 consecutive patients with upper limb onset of the neuropathy (study group) and they were compared to 31 Portuguese early-onset patients and 99 late-onset patients without upper limb onset.

Results: Initial upper limb symptoms were mostly sensory. Read More

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http://dx.doi.org/10.1111/ene.13845DOI Listing
October 2018
2 Reads

Exercise Training Attenuates the Development of Cardiac Autonomic Dysfunction in Diabetic Rats.

In Vivo 2018 Nov-Dec;32(6):1433-1441

Biomedical Research Foundation, Center of Clinical, Experimental Surgery, and Translational Research, Academy of Athens, Athens, Greece.

Background/aim: Exercise training usually complements pharmacological therapy of type 1 diabetes mellitus, however, little is known about its impact on cardiac autonomic neuropathy. Our aim was to evaluate the impact of exercise on electrocardiographic parameters and heart rate variability in diabetic rats.

Materials And Methods: Wistar rats were randomly assigned to four groups (n=12): Sedentary control (SC), sedentary diabetic (SD), exercise control (EC), and exercise diabetic (ED). Read More

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http://iv.iiarjournals.org/lookup/doi/10.21873/invivo.11396
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http://dx.doi.org/10.21873/invivo.11396DOI Listing
July 2018
6 Reads
1.150 Impact Factor