Read by QxMD icon Read

Autonomic Neuropathy

Christoph Niemietz, Christoph Röcken, Matthias Schilling, Jörg Stypman, Constantin E Uhlig, Hartmut H-J Schmidt
Transthyretin-related Familial Amyloid Polyneuropathy (ATTR Amyloidosis, former FAP, here called TTR-FAP) is a rare, progressive autosomal dominant inherited amyloid disease ending fatal within 5 - 15 years after final diagnosis. TTR-FAP is caused by mutations of transthyretin (TTR), which forms amyloid fibrils affecting peripheral and autonomic nerves, the heart and other organs. Due to the phenotypic heterogeneity and partly not specific enough clinical symptoms, diagnosis of TTR-FAP can be complicated...
March 2018: Deutsche Medizinische Wochenschrift
Syed Rizwan A Bokhari, Faisal Akhtar, Qurrat-Ul-Ain Abid, Uzma Jahanzaib, Maria R Bokhari, Sana Hasan, Khurshid Khan
Postural hypotension, as a manifestation of autonomic neuropathy is a very sinister long-term debilitating complication of diabetes, is usually irreversible and tough to manage with medications. The treatment of this condition following the standard treatment protocols can be contraindicated in the patients with underlying heart conditions. We report the case of a patient at our hospital who presented with full-blown symptomatic dysautonomia secondary to long-standing diabetes, with bedside testing positive for autonomic dysfunction...
January 8, 2018: Curēus
Amy C Arnold, Jessica Ng, Satish R Raj
Postural tachycardia syndrome (POTS) is a heterogeneous clinical syndrome that has gained increasing interest over the past few decades due to its increasing prevalence and clinical impact on health-related quality of life. POTS is clinically characterized by sustained excessive tachycardia upon standing that occurs in the absence of significant orthostatic hypotension and other medical conditions and or medications, and with chronic symptoms of orthostatic intolerance. POTS represents one of the most common presentations of syncope and presyncope secondary to autonomic dysfunction in emergency rooms and in cardiology, neurology, and primary care clinics...
February 28, 2018: Autonomic Neuroscience: Basic & Clinical
Stuart J Grice, James N Sleigh, M Zameel Cader
Dominant mutations in GARS , encoding the ubiquitous enzyme glycyl-tRNA synthetase (GlyRS), cause peripheral nerve degeneration and Charcot-Marie-Tooth disease type 2D (CMT2D). This genetic disorder exemplifies a recurring paradigm in neurodegeneration, in which mutations in essential genes cause selective degeneration of the nervous system. Recent evidence suggests that the mechanism underlying CMT2D involves extracellular neomorphic binding of mutant GlyRS to neuronally-expressed proteins. Consistent with this, our previous studies indicate a non-cell autonomous mechanism, whereby mutant GlyRS is secreted and interacts with the neuromuscular junction (NMJ)...
2018: Frontiers in Molecular Neuroscience
Bo Hu, Megan Mccollum, Vignesh Ravi, Sezgi Arpag, Daniel Moiseev, Ryan Castoro, Bret C Mobley, Bryan W Burnette, Carly Siskind, John W Day, Robin Yawn, Shawna Feely, Yuebing Li, Qing Yan, Michael E Shy, Jun Li
OBJECTIVE: Charcot-Marie-Tooth type 4J (CMT4J) is a rare autosomal recessive neuropathy caused by mutations in FIG4 that result in loss of FIG4 protein. This study investigates the natural history and mechanisms of segmental demyelination in CMT4J. METHODS: Over the past 9 years, we have enrolled and studied a cohort of 12 CMT4J patients, including 6 novel FIG4 mutations. We evaluated these patients and related mouse models using morphological, electrophysiological and biochemical approaches...
March 8, 2018: Annals of Neurology
Daisuke Matsutani, Masaya Sakamoto, Hiroyuki Iuchi, Souichirou Minato, Hirofumi Suzuki, Yosuke Kayama, Norihiko Takeda, Ryuzo Horiuchi, Kazunori Utsunomiya
BACKGROUND: It is presently unclear whether glycemic variability (GV) is associated with baroreflex sensitivity (BRS), which is an early indicator of cardiovascular autonomic neuropathy. The present study is the first to examine the relationships between BRS and GV measured using continuous glucose monitoring (CGM). METHODS: This was a multicenter, prospective, open-label clinical trial. A total of 102 patients with type 2 diabetes were consecutively recruited for this study...
March 7, 2018: Cardiovascular Diabetology
Alejandra Gonzalez-Duarte
PURPOSE: Hereditary transthyretin amyloidosis (hATTR amyloidosis) is a progressive disease primarily characterized by adult-onset sensory, motor, and autonomic neuropathy. In this article, we discuss the pathophysiology and principal findings of autonomic neuropathy in hATTR amyloidosis, the most common methods of assessment and progression, and its relation as a predictive risk factor or a measure of progression in the natural history of the disease. METHODS: A literature search was performed using the terms "autonomic neuropathy," "dysautonomia," and "autonomic symptoms" in patients with hereditary transthyretin amyloidosis and familial amyloid polyneuropathy...
March 6, 2018: Clinical Autonomic Research: Official Journal of the Clinical Autonomic Research Society
Berthold Pemp, Stefan Palkovits, Kinga Howorka, Jiri Pumprla, Stefan Sacu, Gerhard Garhöfer, Michaela Bayerle-Eder, Leopold Schmetterer, Ursula Schmidt-Erfurth
PURPOSE: To evaluate the relationship of neuroretinal layer thickness with sensitive measures of cardiovascular autonomic neuropathy in diabetic patients with non-proliferative diabetic retinopathy (NPDR). METHODS: Twenty-seven eyes of 27 patients with type 1 diabetes presenting with mild-to-moderate NPDR were compared to 27 healthy control (HC) eyes matched for age and gender. The total macular volume (TMV) and the volumes of individual neurosensory layers in the macula were analysed from spectral domain optical coherence tomography using automated layer segmentation...
March 5, 2018: Acta Ophthalmologica
Kotb Abbass Metwalley, Sherifa Ahmed Hamed, Hekma Saad Farghaly
Cardiovascular autonomic neuropathy (CAN) is a major complication of type 1 diabetes (T1D). This study aimed to evaluate cardiac autonomic nervous system (ANS) function in children with T1D and its relation to different demographic, clinical and laboratory variable. This cross-sectional study included 60 children with T1D (mean age = 15.1 ± 3.3 years; duration of diabetes = 7.95 ± 3.83 years). The following 8 non-invasive autonomic testing were used for evaluation: heart rate at rest and in response to active standing (30:15 ratio), deep breathing and Valsalva maneuver (indicating parasympathetic function); blood pressure response to standing (orthostatic hypotension or OH), sustained handgrip and cold; and heart rate response to standing or positional orthostatic tachycardia syndrome or POTs (indicating sympathetic function)...
March 2, 2018: European Journal of Pediatrics
Atsushi Sato, Hiroshi Shibuya
The with no lysine (WNK) protein kinase family is conserved among many species. Some mutations in human WNK gene are associated with pseudohypoaldosteronism type II, a form of hypertension, and hereditary sensory and autonomic neuropathy type 2A. In kidney, WNK regulates the activity of STE20/SPS1-related, proline alanine-rich kinase and/or oxidative-stress responsive 1, which in turn regulate ion co-transporters. The misregulation of this pathway is involved in the pathogenesis of pseudohypoaldosteronism type II...
2018: PloS One
Cameron Green, Tess Baker, Ashwin Subramaniam
OBJECTIVE: To systematically review the literature regarding the ability of clinical features to predict respiratory failure in patients with Guillain-Barré syndrome (GBS). DATA SOURCES: We searched the PubMed and Ovid MEDLINE databases with the search terms "guillain barre syndrome" OR "acute inflammatory demyelinating polyneuropathy" OR "acute motor axonal neuropathy" OR "acute motor sensory axonal neuropathy" AND "respiratory failure" OR "mechanical ventilation"...
March 5, 2018: Medical Journal of Australia
Elias Tsiompanidis, Spyros I Siakavellas, Anastasios Tentolouris, Ioanna Eleftheriadou, Stamatia Chorepsima, Anastasios Manolakis, Konstantinos Oikonomou, Nikolaos Tentolouris
AIM: To examine the impact of liver cirrhosis on QT interval and cardiac autonomic neuropathy (CAN). METHODS: A total of 51 patients with cirrhosis and 51 controls were examined. Standard 12-lead electrocardiogram recordings were obtained and QT as well as corrected QT interval (QTc) and their dispersions (dQT, dQTc) were measured and calculated using a computer-based program. The diagnosis of CAN was based upon the battery of the tests proposed by Ewing and Clarke and the consensus statements of the American Diabetes Association...
February 15, 2018: World Journal of Gastrointestinal Pathophysiology
Kijung Sung, Luiz F Ferrari, Wanlin Yang, ChiHye Chung, Xiaobei Zhao, Yingli Gu, Suzhen Lin, Kai Zhang, Bianxiao Cui, Matthew L Pearn, Michael T Maloney, William C Mobley, Jon D Levine, Chengbiao Wu
Nerve growth factor (NGF) exerts multiple functions on target neurons throughout development. The recent discovery of a point mutation leading to a change from arginine to tryptophan at residue 100 in the mature NGFβ sequence (NGFR100W ) in patients with hereditary sensory and autonomic neuropathy, type V (HSAN V), made it possible to distinguish the signaling mechanisms that lead to two functionally different outcomes of NGF: trophic versus nociceptive. We performed extensive biochemical, cellular and live imaging experiments to examine the binding and signaling properties of NGFR100W Our results show that, similar to the wildtype NGF (wtNGF), the naturally occurring NGFR100W mutant was capable of binding to and activating the TrkA receptor and its downstream signaling pathways to support neuronal survival and differentiation...
February 24, 2018: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
Rasmus Bo Jansen, Tomas Møller Christensen, Jens Bülow, Lene Rørdam, Per E Holstein, Ole Lander Svendsen
OBJECTIVE: Charcot foot is a severe complication to diabetes mellitus, associated with diabetic neuropathy. Any long-term effects of a Charcot foot on the progress of neuropathy are still largely unexplored. The objective was to investigate whether a previous Charcot foot had any long-term effects on the progress of neuropathy. RESULTS: An 8.5-year follow-up case-control study of 49 individuals with diabetes mellitus, 24 of whom also had Charcot foot at baseline visit in 2005-2007...
February 20, 2018: BMC Research Notes
Jin Ook Chung, Seon-Young Park, Ji Hyun Han, Dong Hyeok Cho, Dong Jin Chung, Min Young Chung
OBJECTIVE: To evaluate the relationship between levels of serum apolipoproteins and the prevalence of cardiovascular autonomic neuropathy (CAN) in type 2 diabetes. METHODS: In total, 3199 individuals with type 2 diabetes were investigated in a cross-sectional study. The diagnosis of CAN was made based on the results of a cardiovascular reflex test. Serum apolipoprotein A-I (apoA-I) and apolipoprotein B (apoB) levels were measured. RESULTS: Serum apoA-1 levels were significantly low in individuals with CAN, but there was no significant association between serum apoB levels and CAN...
January 31, 2018: Journal of Diabetes and its Complications
Guanghong Jia, Michael A Hill, James R Sowers
Heart failure and related morbidity and mortality are increasing at an alarming rate, in large part, because of increases in aging, obesity, and diabetes mellitus. The clinical outcomes associated with heart failure are considerably worse for patients with diabetes mellitus than for those without diabetes mellitus. In people with diabetes mellitus, the presence of myocardial dysfunction in the absence of overt clinical coronary artery disease, valvular disease, and other conventional cardiovascular risk factors, such as hypertension and dyslipidemia, has led to the descriptive terminology, diabetic cardiomyopathy...
February 16, 2018: Circulation Research
Mario Estévez-Báez, Calixto Machado, Julio Montes-Brown, Javier Jas-García, Gerry Leisman, Adam Schiavi, Andrés Machado-García, Claudia Carricarte-Naranjo, Eli Carmeli
Literature reports on the very high frequency (VHF) range of 0.4-0.9 Hz in heart rate variability (HRV) are scanty. The VHF presence in cardiac transplant patients and other conditions associated with reduced vagal influence on the heart encouraged us to explore this spectral band in healthy subjects and in patients diagnosed with cardiac autonomic neuropathy (CAN), and to assess the potential clinical value of some VHF indices. The study included 80 healthy controls and 48 patients with spinocerebellar ataxia type 2 (SCA2) with CAN...
February 11, 2018: Advances in Experimental Medicine and Biology
Brian C Callaghan, Rong Xia, Evan Reynolds, Mousumi Banerjee, Charles Burant, Amy Rothberg, Rodica Pop-Busui, Emily Villegas-Umana, Eva L Feldman
OBJECTIVE: To determine the comparative diagnostic characteristics of neuropathy measures in an obese population. METHODS: We recruited obese participants from the University of Michigan's Weight Management Program. Receiver operative characteristic analysis determined the area under the curve (AUC) of neuropathy measures for distal symmetric polyneuropathy (DSP), small fiber neuropathy (SFN), and cardiovascular autonomic neuropathy (CAN). The best test combinations were determined using stepwise and Score subset selection models...
March 2018: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
Francisco-J Renero-C
Background: One of the complications of the diabetes mellitus is the amputation of the lower limbs. This complication may be developed after an insidious ulcer, that may be raised by the peripheral neuropathy or the ischaemic limb, and that the ulcer get infected. That is, to develop an ulcer, in the diabetic patient, three factors should be taken into the account, the autonomic nervous system, the blood supply and the inmune system. Methods: In this work, the thermogram is used to identify regions on the plantar skin with blood supply deficiencies and the behaviour of the thermoregulation process...
2018: Diabetic Foot & Ankle
Christopher T Doughty, Reza Sadjadi
Peripheral neuropathy is commonly encountered in the primary care setting and is associated with significant morbidity, including neuropathic pain, falls, and disability. The clinical presentation of neuropathy is diverse, with possible symptoms including weakness, sensory abnormalities, and autonomic dysfunction. Accordingly, the primary care clinician must be comfortable using the neurologic examination-including assessment of motor function, multiple sensory modalities, and the deep tendon reflexes-to recognize and characterize neuropathy...
February 1, 2018: American Journal of Medicine
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"