-
Texas Heart Institute Journal Feb 2020
Review
Topics: Blood Pressure; Cardiovascular Agents; Diagnosis, Differential; Humans; Postural Orthostatic Tachycardia Syndrome; Posture; Predictive Value of Tests; Prognosis; Tilt-Table Test
PubMed: 32148459
DOI: 10.14503/THIJ-19-7060 -
Journal of Diabetes Investigation Nov 2020Impaired awareness of hypoglycemia (IAH) is a reduction in the ability to recognize low blood glucose levels that would otherwise prompt an appropriate corrective... (Review)
Review
Impaired awareness of hypoglycemia (IAH) is a reduction in the ability to recognize low blood glucose levels that would otherwise prompt an appropriate corrective therapy. Identified in approximately 25% of patients with type 1 diabetes, IAH has complex pathophysiology, and might lead to serious and potentially lethal consequences in patients with diabetes, particularly in those with more advanced disease and comorbidities. Continuous glucose monitoring systems can provide real-time glucose information and generate timely alerts on rapidly falling or low blood glucose levels. Given their improvements in accuracy, affordability and integration with insulin pump technology, continuous glucose monitoring systems are emerging as critical tools to help prevent serious hypoglycemia and mitigate its consequences in patients with diabetes. This review discusses the current knowledge on IAH and effective diagnostic methods, the relationship between hypoglycemia and cardiovascular autonomic neuropathy, a practical approach to evaluating cardiovascular autonomic neuropathy for clinicians, and recent evidence from clinical trials assessing the effects of the use of CGM technologies in patients with type 1 diabetes with IAH.
Topics: Autonomic Nervous System Diseases; Blood Glucose Self-Monitoring; Diabetes Complications; Diabetes Mellitus; Diabetic Neuropathies; Health Knowledge, Attitudes, Practice; Humans; Hypoglycemia; Prognosis; Risk Factors
PubMed: 32403204
DOI: 10.1111/jdi.13290 -
Neurological Sciences : Official... Jan 2022The α-synucleinopathies comprise a group of adult-onset neurodegenerative disorders including Parkinson's disease (PD), multiple system atrophy (MSA), dementia with... (Review)
Review
The α-synucleinopathies comprise a group of adult-onset neurodegenerative disorders including Parkinson's disease (PD), multiple system atrophy (MSA), dementia with Lewy bodies (DLB,) and - as a restricted non-motor form - pure autonomic failure (PAF). Neuropathologically, the α-synucleinopathies are characterized by aggregates of misfolded α-synuclein in the central and peripheral nervous system. Cardiovascular autonomic failure is a common non-motor symptom in people with PD, a key diagnostic criterion in MSA, a supportive feature for the diagnosis of DLB and disease-defining in PAF. The site of autonomic nervous system lesion differs between the α-synucleinopathies, with a predominantly central lesion pattern in MSA versus a peripheral one in PD, DLB, and PAF. In clinical practice, overlapping autonomic features often challenge the differential diagnosis among the α-synucleinopathies, but also distinguish them from related disorders, such as the tauopathies or other neurodegenerative ataxias. In this review, we discuss the differential diagnostic yield of cardiovascular autonomic failure in individuals presenting with isolated autonomic failure, parkinsonism, cognitive impairment, or cerebellar ataxia.
Topics: Diagnosis, Differential; Humans; Lewy Body Disease; Multiple System Atrophy; Parkinson Disease; Pure Autonomic Failure; Synucleinopathies; alpha-Synuclein
PubMed: 34817726
DOI: 10.1007/s10072-021-05746-6 -
Journal of Nuclear Cardiology :... Apr 2022Huntington's disease (HD) patients often present with abnormal modulation of blood pressure and heart rate. We investigated whether cardiac autonomic innervation...
BACKGROUND
Huntington's disease (HD) patients often present with abnormal modulation of blood pressure and heart rate. We investigated whether cardiac autonomic innervation assessed by 123I-metaiodobenzylguanidine (MIBG) imaging is impaired in HD patients, in comparison with controls (Ctrl).
METHODS
Fifteen patients (6 F and 9 M) were assessed by the motor section of the Unified HD Rating Scale, the Total Function Capacity, and the scale for outcomes in Parkinson's disease-autonomic (SCOPA-AUT) questionnaire. All patients and 10 Ctrl (5 F and 5 M) underwent 123I-MIBG imaging. From planar images, the early and late heart-to-mediastinum (H/M) ratios and myocardial washout rates (WR) were calculated.
RESULTS
We did not find significant differences in early and late H/M ratios and WR between the two groups. At individual level, three patients showed reduced early and/or late H/M ratios. The most common autonomic complaints were gastrointestinal and genitourinary disorders. SCOPA-AUT questionnaire score results positively correlated with the disease duration and WR.
CONCLUSIONS
Our study indicates that myocardial postganglionic sympathetic innervation is essentially preserved or only minimally involved in HD. These findings suggest that the cardiovascular dysfunction might be mainly due to the impairment of brain areas associated with the regulation and modulation of the heart function.
Topics: 3-Iodobenzylguanidine; Autonomic Nervous System Diseases; Heart; Humans; Huntington Disease; Iodine Radioisotopes; Myocardial Perfusion Imaging; Radiopharmaceuticals
PubMed: 32803674
DOI: 10.1007/s12350-020-02299-7 -
Autonomic Neuroscience : Basic &... Nov 2021As global numbers of COVID-19 grow, chronic neurological symptoms, including those of autonomic dysfunction, are being reported with increasing frequency. Mounting... (Review)
Review
As global numbers of COVID-19 grow, chronic neurological symptoms, including those of autonomic dysfunction, are being reported with increasing frequency. Mounting evidence suggests that many patients experience chronic and sometimes debilitating symptoms long after their acute infectious period, leading to the new diagnostic category of post-acute COVID syndrome. Many symptoms of post-acute COVID syndrome appear autonomic in nature, suggesting that autonomic impairment may play a central role in the underlying pathophysiology. In this review, we discuss the autonomic symptoms and manifestations of post-acute COVID syndrome, potential mechanisms involved, and future directions for a better understanding of this novel condition.
Topics: Autonomic Nervous System Diseases; COVID-19; Humans; Post-Acute COVID-19 Syndrome
PubMed: 34265539
DOI: 10.1016/j.autneu.2021.102841 -
European Journal of Neurology Dec 2022Disorders of the autonomic nervous system (ANS) are common conditions, but it is unclear whether access to ANS healthcare provision is homogeneous across European...
Clinical autonomic nervous system laboratories in Europe: A joint survey of the European Academy of Neurology and the European Federation of Autonomic Societies: A joint survey of the European Academy of Neurology and the European Federation of Autonomic Societies.
BACKGROUND AND PURPOSE
Disorders of the autonomic nervous system (ANS) are common conditions, but it is unclear whether access to ANS healthcare provision is homogeneous across European countries. The aim of this study was to identify neurology-driven or interdisciplinary clinical ANS laboratories in Europe, describe their characteristics and explore regional differences.
METHODS
We contacted the European national ANS and neurological societies, as well as members of our professional network, to identify clinical ANS laboratories in each country and invite them to answer a web-based survey.
RESULTS
We identified 84 laboratories in 22 countries and 46 (55%) answered the survey. All laboratories perform cardiovascular autonomic function tests, and 83% also perform sweat tests. Testing for catecholamines and autoantibodies are performed in 63% and 56% of laboratories, and epidermal nerve fiber density analysis in 63%. Each laboratory is staffed by a median of two consultants, one resident, one technician and one nurse. The median (interquartile range [IQR]) number of head-up tilt tests/laboratory/year is 105 (49-251). Reflex syncope and neurogenic orthostatic hypotension are the most frequently diagnosed cardiovascular ANS disorders. Thirty-five centers (76%) have an ANS outpatient clinic, with a median (IQR) of 200 (100-360) outpatient visits/year; 42 centers (91%) also offer inpatient care (median 20 [IQR 4-110] inpatient stays/year). Forty-one laboratories (89%) are involved in research activities. We observed a significant difference in the geographical distribution of ANS services among European regions: 11 out of 12 countries from North/West Europe have at least one ANS laboratory versus 11 out of 21 from South/East/Greater Europe (p = 0.021).
CONCLUSIONS
This survey highlights disparities in the availability of healthcare services for people with ANS disorders across European countries, stressing the need for improved access to specialized care in South, East and Greater Europe.
Topics: Humans; Laboratories; Autonomic Nervous System; Autonomic Nervous System Diseases; Surveys and Questionnaires; Neurology
PubMed: 36056590
DOI: 10.1111/ene.15538 -
International Journal of Molecular... Jun 2022Specific antibody responses to subfornical organs, including Na antibody, have been reported in patients with adipsic hypernatremia of unknown etiology who do not have... (Review)
Review
Specific antibody responses to subfornical organs, including Na antibody, have been reported in patients with adipsic hypernatremia of unknown etiology who do not have structural lesions in the hypothalamic-pituitary gland. The subfornical organ, also referred to as the window of the brain, is a sensing site that monitors sodium and osmotic pressure levels. On the other hand, ROHHAD syndrome is a rare disease for which the etiology of the hypothalamic disorder is unknown, and there have been some reports in recent years describing its association with autoimmune mechanisms. In addition, abnormal Na levels, including hypernatremia, are likely to occur in this syndrome. When comparing the clinical features of adipsic hypernatremia due to autoimmune mechanisms and ROHHAD syndrome, there are similar hypothalamic-pituitary dysfunction symptoms in addition to abnormal Na levels. Since clinical diagnoses of autoimmunological adipsic hypernatremia and ROHAD syndrome might overlap, we need to understand the essential etiology and carry out precise assessments to accurately diagnose patients and provide effective treatment. In this review, I review the literature on the autoimmune mechanism reported in recent years and describe the findings obtained so far and future directions.
Topics: Autoimmunity; Autonomic Nervous System Diseases; Endocrine System Diseases; Humans; Hypernatremia; Hypothalamic Diseases; Rare Diseases; Respiration Disorders; Sodium; Syndrome
PubMed: 35805903
DOI: 10.3390/ijms23136899 -
Seminars in Neurology Oct 2020Investigations of the cellular and molecular mechanisms that mediate the development of the autonomic nervous system have identified critical genes and signaling... (Review)
Review
Investigations of the cellular and molecular mechanisms that mediate the development of the autonomic nervous system have identified critical genes and signaling pathways that, when disrupted, cause disorders of the autonomic nervous system. This review summarizes our current understanding of how the autonomic nervous system emerges from the organized spatial and temporal patterning of precursor cell migration, proliferation, communication, and differentiation, and discusses potential clinical implications for developmental disorders of the autonomic nervous system, including familial dysautonomia, Hirschsprung disease, Rett syndrome, and congenital central hypoventilation syndrome.
Topics: Autonomic Nervous System; Autonomic Nervous System Diseases; Dysautonomia, Familial; Hirschsprung Disease; Humans; Hypoventilation; Rett Syndrome; Sleep Apnea, Central
PubMed: 32927484
DOI: 10.1055/s-0040-1713926 -
Cleveland Clinic Journal of Medicine Jul 2023Postural orthostatic tachycardia syndrome (POTS)-sustained tachycardia upon standing without orthostatic hypotension-can be diagnosed clinically without an extensive... (Review)
Review
Postural orthostatic tachycardia syndrome (POTS)-sustained tachycardia upon standing without orthostatic hypotension-can be diagnosed clinically without an extensive diagnostic evaluation unless certain atypical features suggest an alternative diagnosis. A unifying pathophysiologic mechanism has not been identified, although several have been proposed. Similarities between POTS and various autoimmune disorders suggest an immune mechanism in a subset of patients. However, no causative antibody has been identified, and associated antibodies are rarely clinically relevant. Moreover, immunotherapies are not currently recommended for POTS, although clinical trials are underway to clarify their utility.
Topics: Humans; Postural Orthostatic Tachycardia Syndrome; Autoimmunity; Autoimmune Diseases; Heart Rate
PubMed: 37400156
DOI: 10.3949/ccjm.90a.22093 -
The Journal of Clinical Endocrinology... Sep 2023Hyperglycemia and autonomic dysfunction are bidirectionally related.
CONTEXT
Hyperglycemia and autonomic dysfunction are bidirectionally related.
OBJECTIVE
We investigated the association of longitudinal evolution of heart rate variability (HRV) with incident type 2 diabetes (T2D) among the general population.
METHODS
We included 7630 participants (mean age 63.7 years, 58% women) from the population-based Rotterdam Study who had no history of T2D and atrial fibrillation at baseline and had repeated HRV assessments at baseline and during follow-up. We used joint models to assess the association between longitudinal evolution of heart rate and different HRV metrics (including the heart rate-corrected SD of the normal-to-normal RR intervals [SDNNc], and root mean square of successive RR-interval differences [RMSSDc]) with incident T2D. Models were adjusted for cardiovascular risk factors. Bidirectional Mendelian randomization (MR) using summary-level data was also performed.
RESULTS
During a median follow-up of 8.6 years, 871 individuals developed incident T2D. One SD increase in heart rate (hazard ratio [HR] 1.20; 95% CI, 1.09-1.33), and log(RMSSDc) (HR 1.16; 95% CI, 1.01-1.33) were independently associated with incident T2D. The HRs were 1.54 (95% CI, 1.08-2.06) for participants younger than 62 years and 1.15 (95% CI, 1.01-1.31) for those older than 62 years for heart rate (P for interaction <.001). Results from bidirectional MR analyses suggested that HRV and T2D were not significantly related to each other.
CONCLUSION
Autonomic dysfunction precedes development of T2D, especially among younger individuals, while MR analysis suggests no causal relationship. More studies are needed to further validate our findings.
Topics: Humans; Female; Middle Aged; Male; Diabetes Mellitus, Type 2; Heart Rate; Atrial Fibrillation; Autonomic Nervous System Diseases; Risk Factors
PubMed: 37022971
DOI: 10.1210/clinem/dgad200