{"title":"ST-segment changes during tilt-table testing in postural tachycardia syndrome: are they relevant?","authors":"Frank I Marcus","doi":"10.1007/s10286-019-00654-4","DOIUrl":"https://doi.org/10.1007/s10286-019-00654-4","url":null,"abstract":"","PeriodicalId":354493,"journal":{"name":"Clinical autonomic research : official journal of the Clinical Autonomic Research Society","volume":" ","pages":"9-10"},"PeriodicalIF":5.8,"publicationDate":"2020-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1007/s10286-019-00654-4","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"37565372","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Autonomic dysfunction in Parkinson disease and animal models.","authors":"Jeanette M Metzger, Marina E Emborg","doi":"10.1007/s10286-018-00584-7","DOIUrl":"https://doi.org/10.1007/s10286-018-00584-7","url":null,"abstract":"<p><p>Parkinson disease has traditionally been classified as a movement disorder, despite patients' accounts of diverse symptoms stemming from impairments in numerous body systems. Today, Parkinson disease is increasingly recognized by clinicians and scientists as a complex neurodegenerative disorder featuring both motor and nonmotor manifestations concomitant with pathology throughout all major branches of the nervous system. Dysfunction of the autonomic nervous system, or dysautonomia, is a common feature of Parkinson disease. It produces signs and symptoms that severely affect patients' quality of life, such as blood pressure dysregulation, hyperhidrosis, and constipation. Treatment options for dysautonomia are limited to symptom alleviation because the cause of these symptoms and Parkinson disease overall are still unknown. Animal models provide a platform to interrogate mechanisms of Parkinson disease-related autonomic nervous system dysfunction and test novel treatment strategies. Several animal models of Parkinson disease are available, each with different effects on the autonomic nervous system. This review critically analyses key dysautonomia signs and symptoms and associated pathology in Parkinson disease patients and relevant findings in animal models. We focus on the cardiovascular system, adrenal medulla, skin/thermoregulation, bladder, pupils, and gastrointestinal tract, to assess the contribution of animal models to the understanding of Parkinson disease autonomic dysfunction.</p>","PeriodicalId":354493,"journal":{"name":"Clinical autonomic research : official journal of the Clinical Autonomic Research Society","volume":" ","pages":"397-414"},"PeriodicalIF":5.8,"publicationDate":"2019-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1007/s10286-018-00584-7","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"36872663","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Alessandra Fanciulli, Nicole Campese, Gregor K Wenning
{"title":"The Schellong test: detecting orthostatic blood pressure and heart rate changes in German-speaking countries.","authors":"Alessandra Fanciulli, Nicole Campese, Gregor K Wenning","doi":"10.1007/s10286-019-00619-7","DOIUrl":"https://doi.org/10.1007/s10286-019-00619-7","url":null,"abstract":"","PeriodicalId":354493,"journal":{"name":"Clinical autonomic research : official journal of the Clinical Autonomic Research Society","volume":" ","pages":"363-366"},"PeriodicalIF":5.8,"publicationDate":"2019-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1007/s10286-019-00619-7","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"37117884","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Induced pluripotent stem cells for disease modeling, cell therapy and drug discovery in genetic autonomic disorders: a review.","authors":"Kenyi Saito-Diaz, Nadja Zeltner","doi":"10.1007/s10286-018-00587-4","DOIUrl":"https://doi.org/10.1007/s10286-018-00587-4","url":null,"abstract":"<p><p>The autonomic nervous system (ANS) regulates all organs in the body independent of consciousness, and is thus essential for maintaining homeostasis of the entire organism. Diseases of the ANS can arise due to environmental insults such as injury, toxins/drugs and infections or due to genetic lesions. Human studies and animal models have been instrumental to understanding connectivity and regulation of the ANS and its disorders. However, research into cellular pathologies and molecular mechanisms of ANS disorders has been hampered by the difficulties in accessing human patient-derived ANS cells in large numbers to conduct meaningful research, mainly because patient neurons cannot be easily biopsied and primary human neuronal cultures cannot be expanded.Human-induced pluripotent stem cell (hiPSC) technology can elegantly bridge these issues, allowing unlimited access of patient-derived ANS cell types for cellular, molecular and biochemical analysis, facilitating the discovery of novel therapeutic targets, and eventually leading to drug discovery. Additionally, such cells may provide a source for cell replacement therapy to replenish lost or injured ANS tissue in patients.Here, we first review the anatomy and embryonic development of the ANS, as this knowledge is crucial for understanding disease modeling approaches. We then review the current advances in human stem cell technology for modeling diseases of the ANS, recent strides toward cell replacement therapy and drug discovery initiatives.</p>","PeriodicalId":354493,"journal":{"name":"Clinical autonomic research : official journal of the Clinical Autonomic Research Society","volume":" ","pages":"367-384"},"PeriodicalIF":5.8,"publicationDate":"2019-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1007/s10286-018-00587-4","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"36854167","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Eugene Won, Jose-Alberto Palma, Horacio Kaufmann, Sarah S Milla, Benjamin Cohen, Lucy Norcliffe-Kaufmann, James S Babb, Yvonne W Lui
{"title":"Quantitative magnetic resonance evaluation of the trigeminal nerve in familial dysautonomia.","authors":"Eugene Won, Jose-Alberto Palma, Horacio Kaufmann, Sarah S Milla, Benjamin Cohen, Lucy Norcliffe-Kaufmann, James S Babb, Yvonne W Lui","doi":"10.1007/s10286-019-00593-0","DOIUrl":"https://doi.org/10.1007/s10286-019-00593-0","url":null,"abstract":"<p><strong>Purpose: </strong>Familial dysautonomia (FD) is a rare autosomal recessive disease that affects the development of sensory and autonomic neurons, including those in the cranial nerves. We aimed to determine whether conventional brain magnetic resonance imaging (MRI) could detect morphologic changes in the trigeminal nerves of these patients.</p><p><strong>Methods: </strong>Cross-sectional analysis of brain MRI of patients with genetically confirmed FD and age- and sex-matched controls. High-resolution 3D gradient-echo T1-weighted sequences were used to obtain measurements of the cisternal segment of the trigeminal nerves. Measurements were obtained using a two-reader consensus.</p><p><strong>Results: </strong>Twenty pairs of trigeminal nerves were assessed in ten patients with FD and ten matched controls. The median (interquartile range) cross-sectional area of the trigeminal nerves in patients with FD was 3.5 (2.1) mm<sup>2</sup>, compared to 5.9 (2.0) mm<sup>2</sup> in controls (P < 0.001). No association between trigeminal nerve area and age was found in patients or controls.</p><p><strong>Conclusions: </strong>Using conventional MRI, the caliber of the trigeminal nerves was significantly reduced bilaterally in patients with FD compared to controls, a finding that appears to be highly characteristic of this disorder. The lack of correlation between age and trigeminal nerve size supports arrested neuronal development rather than progressive atrophy.</p>","PeriodicalId":354493,"journal":{"name":"Clinical autonomic research : official journal of the Clinical Autonomic Research Society","volume":" ","pages":"469-473"},"PeriodicalIF":5.8,"publicationDate":"2019-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1007/s10286-019-00593-0","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"36982205","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Lone F Knudsen, Astrid J Terkelsen, Peter D Drummond, Frank Birklein
{"title":"Complex regional pain syndrome: a focus on the autonomic nervous system.","authors":"Lone F Knudsen, Astrid J Terkelsen, Peter D Drummond, Frank Birklein","doi":"10.1007/s10286-019-00612-0","DOIUrl":"https://doi.org/10.1007/s10286-019-00612-0","url":null,"abstract":"<p><strong>Purpose: </strong>Although autonomic features are part of the diagnostic criteria for complex regional pain syndrome (CRPS), the role of the autonomic nervous system in CRPS pathophysiology has been downplayed in recent years. The purpose of this review is to redress this imbalance.</p><p><strong>Methods: </strong>We focus in this review on the contribution of the autonomic nervous system to CRPS pathophysiology. In particular, we discuss regional sympathetic and systemic autonomic disturbances in CRPS and the mechanisms which may underlie them, and consider links between these mechanisms, immune disturbances and pain.</p><p><strong>Results: </strong>The focused literature research revealed that immune reactions, alterations in receptor populations (e.g., upregulation of adrenoceptors and reduced cutaneous nerve fiber density) and central changes in autonomic drive seem to contribute to regional and systemic disturbances in sympathetic activity and to sympathetically maintained pain in CRPS.</p><p><strong>Conclusions: </strong>We conclude that alterations in the sympathetic nervous system contribute to CRPS pathology. Understanding these alterations may be an important step towards providing appropriate treatments for CRPS.</p>","PeriodicalId":354493,"journal":{"name":"Clinical autonomic research : official journal of the Clinical Autonomic Research Society","volume":" ","pages":"457-467"},"PeriodicalIF":5.8,"publicationDate":"2019-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1007/s10286-019-00612-0","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"37256179","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Concomitant cardiac and cerebral takotsubo syndrome requires specific management.","authors":"Josef Finsterer, Claudia Stöllberger","doi":"10.1007/s10286-018-0566-3","DOIUrl":"https://doi.org/10.1007/s10286-018-0566-3","url":null,"abstract":"<p><p></p>","PeriodicalId":354493,"journal":{"name":"Clinical autonomic research : official journal of the Clinical Autonomic Research Society","volume":" ","pages":"601-602"},"PeriodicalIF":5.8,"publicationDate":"2018-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1007/s10286-018-0566-3","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"36500099","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"The link between narcolepsy and autonomic cardiovascular dysfunction: a translational perspective.","authors":"Chiara Berteotti, Alessandro Silvani","doi":"10.1007/s10286-017-0473-z","DOIUrl":"https://doi.org/10.1007/s10286-017-0473-z","url":null,"abstract":"<p><p>Narcolepsy is a rare disease that entails excessive daytime sleepiness, often associated with sudden episodes of muscle weakness known as cataplexy. Narcolepsy with cataplexy (NC) is due to the loss of hypothalamic neurons that release the neuropeptides orexin A and B. Orexin neuron projections prominently target brain structures involved in wake-sleep state switching and the central autonomic network. This review provides an updated summary of the links between NC and autonomic cardiovascular dysfunction from a translational perspective. The available evidence suggests that, compared with control subjects, the heart rate in patients and animal models with NC is variable during wakefulness and normal to high during sleep. Responses of the heart rate to internal stimuli (arousal from sleep, leg movements during sleep, defense response) are blunted. These alterations result from orexin deficiency and, at least during wakefulness before sleep, involve decreased parasympathetic modulation of the heart rate. On the other hand, NC in patients and animal models is associated with a blunted fall in arterial blood pressure from wakefulness to sleep, and particularly to the REM state, coupled to a variable decrease in arterial blood pressure during wakefulness. The former effect is caused, at least in part, by deranged control of the heart, whereas the latter may be due to decreased vasoconstrictor sympathetic activity. Systematic studies are warranted to help clarify whether and how the links between NC and autonomic dysfunction impact on the cardiovascular risk of patients with narcolepsy.</p>","PeriodicalId":354493,"journal":{"name":"Clinical autonomic research : official journal of the Clinical Autonomic Research Society","volume":" ","pages":"545-555"},"PeriodicalIF":5.8,"publicationDate":"2018-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1007/s10286-017-0473-z","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"35596479","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Ludovico Furlan, Monica Solbiati, Veronica Pacetti, Franca Dipaola, Martino Meda, Mattia Bonzi, Elisa Fiorelli, Giulia Cernuschi, Daniele Alberio, Giovanni Casazza, Nicola Montano, Raffaello Furlan, Giorgio Costantino
{"title":"Diagnostic accuracy of ICD-9 code 780.2 for the identification of patients with syncope in the emergency department.","authors":"Ludovico Furlan, Monica Solbiati, Veronica Pacetti, Franca Dipaola, Martino Meda, Mattia Bonzi, Elisa Fiorelli, Giulia Cernuschi, Daniele Alberio, Giovanni Casazza, Nicola Montano, Raffaello Furlan, Giorgio Costantino","doi":"10.1007/s10286-018-0509-z","DOIUrl":"https://doi.org/10.1007/s10286-018-0509-z","url":null,"abstract":"<p><strong>Purpose: </strong>Syncope is a common condition that affects individuals of all ages and is responsible for 1-3% of all emergency department (ED) visits. Prospective studies on syncope are often limited by the exiguous number of subjects enrolled. A possible alternative approach would be to use of hospital discharge diagnoses from administrative databases to identify syncope subjects in epidemiological observational studies. We assessed the accuracy of the International Classification of Diseases, Ninth Revision (ICD-9) code 780.2 \"syncope and collapse\" to identify patients with syncope.</p><p><strong>Methods: </strong>Patients in two teaching hospitals in Milan, Italy with a triage assessment for ED access that was possibly related to syncope were recruited in this study. We considered the index test to be the attribution of the ICD-9 code 780.2 at ED discharge and the reference standard to be the diagnosis of syncope by the ED physician.</p><p><strong>Results: </strong>The sensitivity, specificity, positive and negative predictive values of the ICD-9 code 780.2 to identify patients with syncope were 0.63 (95% confidence interval [CI] 0.58-0.67), 0.98 (95% CI 0.98-0.99), 0.83 (95% CI 0.79-0.87) and 0.95 (95% CI 0.94-0.95), respectively.</p><p><strong>Conclusions: </strong>The moderate sensitivity of ICD-9 code 780.2 should be considered when the code is used to identify patients with syncope through administrative databases.</p>","PeriodicalId":354493,"journal":{"name":"Clinical autonomic research : official journal of the Clinical Autonomic Research Society","volume":" ","pages":"577-582"},"PeriodicalIF":5.8,"publicationDate":"2018-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1007/s10286-018-0509-z","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"35825683","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Stefania Nannoni, Philippe Maeder, François Vingerhoets, Patrik Michel
{"title":"Horner syndrome in ipsilateral lenticulostriate stroke: a novel localization for a classic stroke syndrome.","authors":"Stefania Nannoni, Philippe Maeder, François Vingerhoets, Patrik Michel","doi":"10.1007/s10286-018-0544-9","DOIUrl":"https://doi.org/10.1007/s10286-018-0544-9","url":null,"abstract":"<p><p>Horner's syndrome has important localizing and etiological value in acute ischemic stroke. In a prospective series of consecutive acute ischemic stroke patients, we identified three patients with Horner's syndrome and contralateral hemiparesis due to ipsilateral small deep infarction in the middle cerebral artery territory (lenticulostriate arteries). Lacunar stroke was the most likely stroke mechanism in all three patients. This observation might suggest the existence of an uncrossed cortico-hypothalamic sympathetic pathway passing through the basal ganglia and modulating oculosympathetic function.</p>","PeriodicalId":354493,"journal":{"name":"Clinical autonomic research : official journal of the Clinical Autonomic Research Society","volume":" ","pages":"583-587"},"PeriodicalIF":5.8,"publicationDate":"2018-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1007/s10286-018-0544-9","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"36295619","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}