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Revue Neurologique 2024Pure autonomic failure (PAF) is a neurodegenerative disease affecting the sympathetic component of the autonomic nervous system and presenting as orthostatic hypotension... (Review)
Review
Pure autonomic failure (PAF) is a neurodegenerative disease affecting the sympathetic component of the autonomic nervous system and presenting as orthostatic hypotension (OH). It is a rare, sporadic disease of adults. Although OH is the primary symptom, the autonomic dysfunction may be more generalised, leading to genitourinary and intestinal dysfunction and sweating disorders. Autonomic symptoms in PAF may be similar to those observed in other autonomic neuropathies that need to be ruled out. PAF belongs to the group of α synucleinopathies and is characterised by predominant peripheral deposition of α-synuclein in autonomic ganglia and nerves. However, in a significant number of cases, PAF may convert into another synucleinopathy with central nervous system involvement with varying prognosis: Parkinson's disease (PD), multiple system atrophy (MSA), or dementia with Lewy bodies (DLB). The clinical features, the main differential diagnoses, the risk factors for "phenoconversion" to another synucleinopathy as well as an overview of treatment will be discussed.
Topics: Adult; Humans; Pure Autonomic Failure; Synucleinopathies; Parkinson Disease; Multiple System Atrophy; Lewy Body Disease; Autonomic Nervous System Diseases
PubMed: 38129276
DOI: 10.1016/j.neurol.2023.11.003 -
Heart (British Cardiac Society) Dec 2023
Topics: Humans; Syncope, Vasovagal; Bradycardia; Heart Rate; Tilt-Table Test
PubMed: 37591689
DOI: 10.1136/heartjnl-2023-323180 -
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 -
Journal of Neural Transmission (Vienna,... Oct 2023Cognitive impairment (CI), previously considered as a non-supporting feature of multiple system atrophy (MSA), according to the second consensus criteria, is not... (Review)
Review
Cognitive impairment (CI), previously considered as a non-supporting feature of multiple system atrophy (MSA), according to the second consensus criteria, is not uncommon in this neurodegenerative disorder that is clinically characterized by a variable combination of autonomic failure, levodopa-unresponsive parkinsonism, motor and cerebellar signs. Mild cognitive impairment (MCI), a risk factor for dementia, has been reported in up to 44% of MSA patients, with predominant impairment of executive functions/attention, visuospatial and verbal deficits, and a variety of non-cognitive and neuropsychiatric symptoms. Despite changing concept of CI in this synucleinopathy, the underlying pathophysiological mechanisms remain controversial. Recent neuroimaging studies revealed volume reduction in the left temporal gyrus, and in the dopaminergic nucleus accumbens, while other morphometric studies did not find any gray matter atrophy, in particular in the frontal cortex. Functional analyses detected decreased functional connectivity in the left parietal lobe, bilateral cuneus, left precuneus, limbic structures, and cerebello-cerebral circuit, suggesting that structural and functional changes in the subcortical limbic structures and disrupted cerebello-cerebral networks may be associated with early cognitive decline in MSA. Whereas moderate to severe CI in MSA in addition to prefrontal-striatal degeneration is frequently associated with cortical Alzheimer and Lewy co-pathologies, neuropathological studies of the MCI stage of MSA are unfortunately not available. In view of the limited structural and functional findings in MSA cases with MCI, further neuroimaging and neuropathological studies are warranted in order to better elucidate its pathophysiological mechanisms and to develop validated biomarkers as basis for early diagnosis and future adequate treatment modalities in order to prevent progression of this debilitating disorder.
Topics: Humans; Multiple System Atrophy; Magnetic Resonance Imaging; Cognitive Dysfunction; Neuroimaging; Gray Matter; Atrophy; Brain
PubMed: 37581647
DOI: 10.1007/s00702-023-02682-x -
Journal of Neurology Nov 2023Multiple system atrophy (MSA) is a sporadic, fatal, and rapidly progressive neurodegenerative disease of unknown etiology that is clinically characterized by autonomic... (Review)
Review
Multiple system atrophy (MSA) is a sporadic, fatal, and rapidly progressive neurodegenerative disease of unknown etiology that is clinically characterized by autonomic failure, parkinsonism, cerebellar ataxia, and pyramidal signs in any combination. Early onset and extensive autonomic dysfunction, including cardiovascular dysfunction characterized by orthostatic hypotension (OH) and supine hypertension, urinary dysfunction characterized by overactive bladder and incomplete bladder emptying, sexual dysfunction characterized by sexual desire deficiency and erectile dysfunction, and gastrointestinal dysfunction characterized by delayed gastric emptying and constipation, are the main features of MSA. Autonomic dysfunction greatly reduces quality of life and increases mortality. Therefore, early diagnosis and intervention are urgently needed to benefit MSA patients. In this review, we aim to discuss the systematic treatment of autonomic dysfunction in MSA, and focus on the current methods, starting from non-pharmacological methods, such as patient education, psychotherapy, diet change, surgery, and neuromodulation, to various drug treatments targeting autonomic nerve and its projection fibers. In addition, we also draw attention to the interactions among various treatments, and introduce novel methods proposed in recent years, such as gene therapy, stem cell therapy, and neural prosthesis implantation. Furthermore, we elaborate on the specific targets and mechanisms of action of various drugs. We would like to call for large-scale research to determine the efficacy of these methods in the future. Finally, we point out that studies on the pathogenesis of MSA and pathophysiological mechanisms of various autonomic dysfunction would also contribute to the development of new promising treatments and concepts.
Topics: Male; Humans; Multiple System Atrophy; Quality of Life; Autonomic Nervous System Diseases; Erectile Dysfunction; Parkinsonian Disorders
PubMed: 37477834
DOI: 10.1007/s00415-023-11876-y -
Hypertension (Dallas, Tex. : 1979) Oct 2023The prognostic role and the clinical significance of orthostatic hypertension (OHT) remained undefined for long because data were sparse and often inconsistent. In... (Review)
Review
The prognostic role and the clinical significance of orthostatic hypertension (OHT) remained undefined for long because data were sparse and often inconsistent. In recent years, evidence has been accumulating that OHT is associated with an increased risk of masked and sustained hypertension, hypertension-mediated organ damage, cardiovascular disease, and mortality. Most evidence came from studies in which OHT was defined using systolic blood pressure (BP) whereas the clinical relevance of diastolic OHT is still unclear. Recently, the American Autonomic Society and the Japanese Society of Hypertension defined OHT as an orthostatic systolic BP increase ≥20 mm Hg associated with a systolic BP of at least 140 mm Hg while standing. However, also smaller orthostatic BP increases have shown clinical relevance especially in people ≤45 years of age. A possible limitation of the BP response to standing is poor reproducibility. OHT concordance is better when the between-assessment interval is shorter, when OHT is evaluated using a larger number of BP readings, and if home BP measurement is used. The pathogenetic mechanisms leading to OHT are still controversial and may vary according to age. Excessive neurohumoral activation seems to be the main determinant in younger adults whereas vascular stiffness plays a more important role in older individuals. Conditions associated with higher activity of the sympathetic nervous system and/or baroreflex dysregulation, such as diabetes, essential hypertension, and aging have been found to be often associated with OHT. Measurement of orthostatic BP should be included in routine clinical practice especially in people with high-normal BP.
Topics: Adult; Humans; Aged; Reproducibility of Results; Hypertension; Blood Pressure; Blood Pressure Determination; Cardiovascular Diseases; Hypotension, Orthostatic
PubMed: 37417253
DOI: 10.1161/HYPERTENSIONAHA.123.21537 -
Neurological Sciences : Official... Jun 2024Maintaining cerebral perfusion in the early stages of recovery after stroke is paramount. Autoregulatory function may be impaired during this period leaving cerebral... (Review)
Review
Maintaining cerebral perfusion in the early stages of recovery after stroke is paramount. Autoregulatory function may be impaired during this period leaving cerebral perfusion directly reliant on intravascular volume and blood pressure (BP) with increased risk for expanding cerebral infarction during periods of low BP and hemorrhagic transformation during BP elevations. We suspected that dysautonomia is common during the acute period related to both pre-existing vascular risk factors and potentially independent of such conditions. Thus, we sought to understand the state of the science specific to dysautonomia and acute stroke. The scoping review search included multiple databases and key terms related to acute stroke and dysautonomia. The team employed a rigorous review process to identify, evaluate, and summarize relevant literature. We additionally summarized common clinical approaches used to detect dysautonomia at the bedside. The purpose of this scoping review is to understand the state of the science for the identification, treatment, and impact of dysautonomia on acute stroke patient outcomes. There is a high prevalence of dysautonomia among persons with stroke, though there is significant variability in the type of measures and definitions used to diagnose dysautonomia. While dysautonomia appears to be associated with poor functional outcome and post-stroke complications, there is a paucity of high-quality evidence, and generalizability is limited by heterogenous approaches to these studies. There is a need to establish common definitions, standard measurement tools, and a roadmap for incorporating these measures into clinical practice so that larger studies can be conducted.
Topics: Humans; Stroke; Primary Dysautonomias; Recovery of Function
PubMed: 38246939
DOI: 10.1007/s10072-023-07289-4 -
Nature Reviews. Neurology Feb 2024Following on from the COVID-19 pandemic is another worldwide public health challenge that is referred to variously as long COVID, post-COVID syndrome or post-acute... (Review)
Review
Following on from the COVID-19 pandemic is another worldwide public health challenge that is referred to variously as long COVID, post-COVID syndrome or post-acute sequelae of SARS-CoV-2 infection (PASC). PASC comes in many forms and affects all body organs. This heterogeneous presentation suggests involvement of the autonomic nervous system (ANS), which has numerous roles in the maintenance of homeostasis and coordination of responses to various stressors. Thus far, studies of ANS dysregulation in people with PASC have been largely observational and descriptive, based on symptom inventories or objective but indirect measures of cardiovascular function, and have paid little attention to the adrenomedullary, hormonal and enteric nervous components of the ANS. Such investigations do not consider the syndromic nature of autonomic dysfunction. This Review provides an update on the literature relating to ANS abnormalities in people with post-COVID syndrome and presents a theoretical perspective on how the ANS might participate in common features of PASC.
Topics: Humans; Post-Acute COVID-19 Syndrome; Pandemics; COVID-19; SARS-CoV-2; Autonomic Nervous System Diseases; Disease Progression
PubMed: 38212633
DOI: 10.1038/s41582-023-00917-9 -
Neurology India Mar 2024
Topics: Humans; Autonomic Nervous System Diseases; Flushing; Hypohidrosis
PubMed: 38817189
DOI: 10.4103/neurol-india.Neurol-India-D-24-00157 -
JAMA May 2024
Topics: Humans; Syncope; Tilt-Table Test; Female; Adult; COVID-19; Post-Acute COVID-19 Syndrome; Postural Orthostatic Tachycardia Syndrome
PubMed: 38602671
DOI: 10.1001/jama.2024.0004