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Dysautonomia

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https://www.readbyqxmd.com/read/27908630/non-linear-dose-response-of-aluminium-hydroxide-adjuvant-particles-selective-low-dose-neurotoxicity
#1
Guillemette Crépeaux, Housam Eidi, Marie-Odile David, Yasmine Baba-Amer, Eleni Tzavara, Bruno Giros, François-Jérôme Authier, Christopher Exley, Christopher A Shaw, Josette Cadusseau, Romain K Gherardi
Aluminium (Al) oxyhydroxide (Alhydrogel(®)), the main adjuvant licensed for human and animal vaccines, consists of primary nanoparticles that spontaneously agglomerate. Concerns about its safety emerged following recognition of its unexpectedly long-lasting biopersistence within immune cells in some individuals, and reports of chronic fatigue syndrome, cognitive dysfunction, myalgia, dysautonomia and autoimmune/inflammatory features temporally linked to multiple Al-containing vaccine administrations. Mouse experiments have documented its capture and slow transportation by monocyte-lineage cells from the injected muscle to lymphoid organs and eventually the brain...
November 28, 2016: Toxicology
https://www.readbyqxmd.com/read/27907966/getting-rid-of-weakness-in-the-icu-an-updated-approach-to-the-acute-management-of-myasthenia-gravis-and-guillain-barr%C3%A3-syndrome
#2
Alexis A Lizarraga, Karlo J Lizarraga, Michael Benatar
After prompt diagnosis, severe myasthenia gravis and Guillain-Barré syndrome (GBS) usually require management in the intensive care unit. In the myasthenic patient, recognition of precipitating factors is paramount, and frequent monitoring of bulbar, upper airway, and/or respiratory muscle strength is needed to identify impending myasthenic crisis. Noninvasive ventilation can be attempted prior to intubation and mechanical ventilation in the setting of respiratory failure. Cholinesterase inhibitors should be discontinued, but resumed prior to extubation, and steroid dosage could be increased once the airway is secured...
December 2016: Seminars in Neurology
https://www.readbyqxmd.com/read/27899087/paradoxical-physiological-responses-to-propranolol-in-a-rett-syndrome-patient-a-case-report
#3
P J Santosh, L Bell, K Lievesley, J Singh, F Fiori
BACKGROUND: Rett Syndrome (RTT), caused by a loss-of-function in the epigenetic modulator: X-linked methyl-CpG binding protein 2 (MeCP2), is a pervasive neurological disorder characterized by compromised brain functions, anxiety, severe mental retardation, language and learning disabilities, repetitive stereotyped hand movements and developmental regression. An imbalance in the sympathetic and the parasympathetic nervous system (dysautonomia) and the resulting autonomic storms is a frequent occurrence in patients with RTT...
November 29, 2016: BMC Pediatrics
https://www.readbyqxmd.com/read/27885125/immature-rumen-fluke-cause-deaths-of-ewes
#4
(no author information available yet)
Ewe deaths due to immature rumen flukeAmyloidosis in a Suffolk-cross lambHaemonchosis in Valais blacknose ewesDeaths and diarrhoea due to Salmonella Reading in 26-week-old giltsFeline dysautonomia in a kittenThese are among matters discussed in the disease surveillance report for August 2016 from SAC Consulting: Veterinary Services (SAC C VS).
November 26, 2016: Veterinary Record
https://www.readbyqxmd.com/read/27861223/gastrointestinal-and-urologic-sphincter-dysfunction-in-stiff-person-syndrome
#5
Oana M Dumitrascu, Evgeny I Tsimerinov, Richard A Lewis
OBJECTIVES: Stiff person syndrome is a neurologic disorder characterized by axial rigidity leading to progressive disability, with broad clinical spectrum. METHODS: We report 2 cases with unique clinical presentation. RESULTS: Two young men suffered progressive urinary retention requiring bladder catheterization, anorectal spasms and constipation, complicated subsequently with lower extremity trigger-induced spasms, and gait instability. Associated symptoms revealed brainstem involvement (vertigo, diplopia, and cranial neuropathies) and dysautonomia (abnormal sweating and orthostatic hypotension)...
December 2016: Journal of Clinical Neuromuscular Disease
https://www.readbyqxmd.com/read/27855745/sleep-apnea-in-familial-dysautonomia-a-reflection-of-apnea-pathogenesis
#6
Jeremy E Orr, Naomi Deacon, John Ravits
No abstract text is available yet for this article.
November 14, 2016: Journal of Clinical Sleep Medicine: JCSM: Official Publication of the American Academy of Sleep Medicine
https://www.readbyqxmd.com/read/27847465/the-many-faces-of-elongator-in-neurodevelopment-and-disease
#7
REVIEW
Marija Kojic, Brandon Wainwright
Development of the nervous system requires a variety of cellular activities, such as proliferation, migration, axonal outgrowth and guidance and synapse formation during the differentiation of neural precursors into mature neurons. Malfunction of these highly regulated and coordinated events results in various neurological diseases. The Elongator complex is a multi-subunit complex highly conserved in eukaryotes whose function has been implicated in the majority of cellular activities underlying neurodevelopment...
2016: Frontiers in Molecular Neuroscience
https://www.readbyqxmd.com/read/27843848/impact-of-disease-control-and-co-existing-risk-factors-on-heart-rate-variability-in-gujarati-type-2-diabetics-an-observational-study
#8
Jayesh Dalpatbhai Solanki, Sanket D Basida, Hemant B Mehta, Sunil J Panjwani, Bhakti P Gadhavi, Pathik Patel
BACKGROUND: Type 2 diabetes mellitus (T2DM) is a proven threat of cardiac dysautonomia with paucity of studies from India. Poor disease control makes it further worse with co-existence of hypertension in majority. Heart rate variability (HRV) is a validated noninvasive tool to assess cardiac autonomic status. AIM: We studied HRV parameters of type 2 diabetics looking for effects of disease control and other co-existing risk factors. MATERIALS AND METHODS: Ninety-eight hypertensive and forty normotensive under-treatment, Gujarati type 2 diabetics were evaluated for disease control and risk stratification...
April 2016: Journal of Family Medicine and Primary Care
https://www.readbyqxmd.com/read/27835060/impaired-camp-cgmp-cross-talk-during-cardiac-sympathetic-dysautonomia
#9
Emma N Bardsley, Hege E Larsen, David J Paterson
No abstract text is available yet for this article.
November 11, 2016: Channels
https://www.readbyqxmd.com/read/27822381/clinical-pearls-how-my-patients-taught-me-the-fainting-lark-symptom
#10
A Kuiper, M E van Egmond, M P M Harms, M D Oosterhoff, B van Harten, D A Sival, T J de Koning, M A J Tijssen
BACKGROUND: Compulsive movements, complex tics and stereotypies are frequent, especially among patients with autism or psychomotor retardation. These movements can be difficult to characterize and can mimic other conditions like epileptic seizures or paroxysmal dystonia, particularly when abnormal breathing and cerebral hypoxia are induced. CASE PRESENTATION: We describe an 18-year-old patient with Asperger syndrome who presented with attacks of tonic posturing of the trunk and neck...
2016: Journal of Clinical Movement Disorders
https://www.readbyqxmd.com/read/27821467/a-painful-neuropathy-associated-nav1-7-mutant-leads-to-time-dependent-degeneration-of-small-diameter-axons-associated-with-intracellular-ca2-dysregulation-and-decrease-in-atp-levels
#11
Harshvardhan Rolyan, Shujun Liu, Janneke Gj Hoeijmakers, Catharina G Faber, Ingemar Sj Merkies, Giuseppe Lauria, Joel A Black, Stephen G Waxman
Small fiber neuropathy is a painful sensory nervous system disorder characterized by damage to unmyelinated C- and thinly myelinated Aδ- nerve fibers, clinically manifested by burning pain in the distal extremities and dysautonomia. The clinical onset in adulthood suggests a time-dependent process. The mechanisms that underlie nerve fiber injury in small fiber neuropathy are incompletely understood, although roles for energetic stress have been suggested. In the present study, we report time-dependent degeneration of neurites from dorsal root ganglia neurons in culture expressing small fiber neuropathy-associated G856D mutant Nav1...
2016: Molecular Pain
https://www.readbyqxmd.com/read/27804276/impact-of-disease-duration-and-cardiovascular-dysautonomia-on-hypertension-in-parkinson-s-disease
#12
Davide L Vetrano, Maria S Pisciotta, Vincenzo Brandi, Maria R Lo Monaco, Alice Laudisio, Graziano Onder, Domenico Fusco, Paolo D L Angiocola, Anna R Bentivoglio, Roberto Bernabei, Giuseppe Zuccalà
The authors evaluated the association of Parkinson's disease (PD) duration with hypertension, assessed by office measurements and 24-hour (ambulatory) monitoring, in 167 patients. Hypertension was evaluated through both office and ambulatory blood pressure (BP) measurements. Among participants (mean age 73.4±7.6 years; 35% women), the prevalence of hypertension was 60% and 69% according to office and ambulatory BP measurements, respectively (Cohen's k=0.61; P<.001). PD duration was inversely associated with hypertension as diagnosed by office measurements (odds ratio [OR], 0...
November 2, 2016: Journal of Clinical Hypertension
https://www.readbyqxmd.com/read/27800280/in-regard-to-autism-in-the-son-of-a-woman-with-mitochondrial-myopathy-and-dysautonomia-a-case-report
#13
Keith Fluegge
No abstract text is available yet for this article.
May 2016: Innovations in Clinical Neuroscience
https://www.readbyqxmd.com/read/27793437/familial-amyloidosis-with-polyneuropathy-type-1-caused-by-transthyretin-mutation-val50met-val30met-4-cases-in-a-non-endemic-area
#14
N Andrés, J J Poza, J F Martí Massó
INTRODUCTION: Transthyretin-related familial amyloid polyneuropathy (TTR-FAP) typically arises as an autonomic neuropathy primarily affecting small fibres and it occurs in adult patients in their second or third decades of life. It progresses rapidly and can lead to death in approximately 10 years. Other phenotypes have been described in non-endemic areas. OBJECTIVES AND METHODS: We described 4 cases from the Spanish province of Guipuzcoa, a non-endemic area, to highlight the clinical variability of this disease...
October 26, 2016: Neurología: Publicación Oficial de la Sociedad Española de Neurología
https://www.readbyqxmd.com/read/27793235/infrared-image-monitoring-of-local-anesthetic-poisoning-in-rats
#15
Angelo Manoel G Carstens, Elizabeth Milla Tambara, Daniel Colman, Márcio G Carstens, Jorge Eduardo Fouto Matias
BACKGROUND AND OBJECTIVES: To evaluate the thermographic predictive value of local anesthetic poisoning in rats that indicates the early recognition of thermal signs of intoxication and enable the immediate start of advanced life support. METHODS: Wistar rats underwent intraperitoneal injection of saline and ropivacaine; they were allocated into pairs, and experiments performed at baseline and experimental times. For thermography, central and peripheral compartment were analyzed, checking the maximum and average differences of temperatures between groups...
November 2016: Brazilian Journal of Anesthesiology
https://www.readbyqxmd.com/read/27788421/profile-of-autonomic-dysfunctions-in-patients-with-primary-brain-tumor-and-possible-autoimmunity
#16
Magdalena Koszewicz, Slawomir Michalak, Malgorzata Bilinska, Slawomir Budrewicz, Mikolaj Zaborowski, Krzysztof Slotwinski, Ryszard Podemski, Maria Ejma
OBJECTIVE: Cerebral lesion due to different neurological conditions could be complicated by autonomic dysfunction, reported in the literature as a sympathetic hyperactivity. The mechanisms of dysautonomia still remains partial. The aim of the study was to assess the profile of autonomic dysfunction in patient with primary brain tumors, with attempt to estimate the additional factors in pathogenesis of dysautonomia. MATERIAL AND METHODS: Neurological examinations, the Low's autonomic disorder questionnaire, electrophysiological autonomic tests (Heart Rate Variability test at rest and during deep breathing, spectral analysis of R-R intervals, sympathetic skin response test), studies of peripheral nerves, blood sampling collection for antibodies were done in 33 patients with recognized primary brain tumors...
October 22, 2016: Clinical Neurology and Neurosurgery
https://www.readbyqxmd.com/read/27784877/an-autopsy-case-of-leptomeningeal-amyloidosis-associated-with-transthyretin-gly47arg-mutation
#17
Takuya Uehara, Keita Kakuda, Hisae Sumi-Akamaru, Amane Yamauchi, Hideki Mochizuki, Takashi Naka
We report the case of a 47-year-old woman with a 4-year history of progressive numbness in the distal portions of both her lower limbs, diarrhea alternating with periods of constipation, and orthostatic syncope. She demonstrated sensory dominant neuropathy and dysautonomia including orthostatic hypotension, paralytic ileus, and urinary retention. A systemic mutation analysis revealed a G47R mutation in transthyretin (TTR). Her general condition was so poor that we could not perform active treatment. Her consciousness had been impaired for a few months...
November 29, 2016: Rinshō Shinkeigaku, Clinical Neurology
https://www.readbyqxmd.com/read/27772753/antioxidant-and-inflammatory-biomarkers-for-the-identification-of-prodromal-parkinson-s-disease
#18
Jonica Campolo, Renata De Maria, Lorena Cozzi, Marina Parolini, Stefano Bernardi, Paola Proserpio, Lino Nobili, Giorgio Gelosa, Immacolata Piccolo, Elio C Agostoni, Maria G Trivella, Paolo Marraccini
OBJECTIVES: We explored the role of oxidative stress and inflammatory molecules as potential Parkinson (PD) biomarkers and correlated biological with non-motor abnormalities (olfactory impairment and dysautonomia), in patients with idiopathic REM behavior disorder (iRBD) (prodromal PD) and established PD. METHODS: We recruited 11 iRBD and 15 patients with idiopathic PD (Hohen&Yahr 1-3, on L-DOPA and dopamine agonists combination therapy) and 12 age- and sex-matched controls (CTRL)...
November 15, 2016: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/27752785/dexmedetomidine-for-refractory-adrenergic-crisis-in-familial-dysautonomia
#19
Ryan C Dillon, Jose-Alberto Palma, Christy L Spalink, Diana Altshuler, Lucy Norcliffe-Kaufmann, David Fridman, John Papadopoulos, Horacio Kaufmann
OBJECTIVE: Adrenergic crises are a cardinal feature of familial dysautonomia (FD). Traditionally, adrenergic crises have been treated with the sympatholytic agent clonidine or with benzodiazepines, which can cause excessive sedation and respiratory depression. Dexmedetomidine is a centrally-acting α 2-adrenergic agonist with greater selectivity and shorter half-life than clonidine. We evaluated the preliminary effectiveness and safety of intravenous dexmedetomidine in the treatment of refractory adrenergic crisis in patients with FD...
October 17, 2016: Clinical Autonomic Research: Official Journal of the Clinical Autonomic Research Society
https://www.readbyqxmd.com/read/27749843/elevated-basal-serum-tryptase-identifies-a-multisystem-disorder-associated-with-increased-tpsab1-copy-number
#20
Jonathan J Lyons, Xiaomin Yu, Jason D Hughes, Quang T Le, Ali Jamil, Yun Bai, Nancy Ho, Ming Zhao, Yihui Liu, Michael P O'Connell, Neil N Trivedi, Celeste Nelson, Thomas DiMaggio, Nina Jones, Helen Matthews, Katie L Lewis, Andrew J Oler, Ryan J Carlson, Peter D Arkwright, Celine Hong, Sherene Agama, Todd M Wilson, Sofie Tucker, Yu Zhang, Joshua J McElwee, Maryland Pao, Sarah C Glover, Marc E Rothenberg, Robert J Hohman, Kelly D Stone, George H Caughey, Theo Heller, Dean D Metcalfe, Leslie G Biesecker, Lawrence B Schwartz, Joshua D Milner
Elevated basal serum tryptase levels are present in 4-6% of the general population, but the cause and relevance of such increases are unknown. Previously, we described subjects with dominantly inherited elevated basal serum tryptase levels associated with multisystem complaints including cutaneous flushing and pruritus, dysautonomia, functional gastrointestinal symptoms, chronic pain, and connective tissue abnormalities, including joint hypermobility. Here we report the identification of germline duplications and triplications in the TPSAB1 gene encoding α-tryptase that segregate with inherited increases in basal serum tryptase levels in 35 families presenting with associated multisystem complaints...
October 17, 2016: Nature Genetics
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